Investigating Academic Outcomes of Children with Cleft Lip and Palate

DISCLOSURE STATEMENT MAKING THE GRADE: INVESTIGATING ACADEMIC • Neither I nor any member of my immediate family has a financial relationship OUTCOMES OF CHILDREN WITH or interest with any proprietary entity producing health care goods or services CLEFT LIP AND PALATE related to the content of this CME activity. • My content will not include discussion/reference of any commercial products or services. Emily Gallagher, MD, MPH • I do not intend to discuss an unapproved/investigative use of commercial products/devices. Seattle Children’s Craniofacial Center Assistant Professor, Department of Pediatrics

THANK YOU CINDY OBJECTIVES

• Illustrate trends in academic outcomes for children with orofacial clefts • Evaluate potential factors that contribute to observed academic deficits • Propose an intervention to improve outcomes

Functions of the palate

OROFACIAL CLEFTS: THE BASICS

• CLP: 1 in 700 births • CP: 1 in 2000 births

• Genetic and environmental factors • FEEDING • Isolated vs Syndromic • HEARING • SPEECH Will I feel comfortable taking my baby in public before lip repair? Will my baby have TIMELINE OF CARE other problems?

Palate repair Will other people bully my Lip repair PRENATAL DIAGNOSIS child because of the cleft? Newborn visit Lip/nose revision Bone graft Lip/nose revision, maxillary advancement Cleft repair (if needed) (if needed) (if needed) Is this because of something Ear tubes I did wrong? (if needed) Hearing screens (birth, 9mo, & annually) Dental care

Ear Tubes placed Does this mean my child will have learning problems?

IMPACTS OF OROFACIAL ACADEMIC OUTCOMES CLEFTS

Key Point: “There appears to be an innate human This does not mean that every child tendency to associate craniofacial with an orofacial cleft will have malformations with abnormal cognitive academic deficits. development.”

Cunningham, 2007

• Search strategy • Inclusion • Medline, Embase, PsychInfo, CINAHL • Patients <25 years with orofacial clefts • 1980-2017 • Measures of neurodevelopmental outcomes • English language • Search terms • Middle to high income economies SYSTEMATIC REVIEW SYSTEMATIC REVIEW • Cleft palate, cleft lip, orofacial cleft • Terms to include neurodevelopmental and • Exclusion academic outcomes • <10 cases • Qualitative studies 2270 references identified by search strategy SYSTEMATIC REVIEW: QUALITY SCORES 110 duplicate references excluded

2160 references screened for inclusion using Population based sample title and abstract, with full-text consulted when necessary Adequate demographic data reported

Participation rate reported

2080 references excluded that did Clear identification of syndromic patients not meet inclusion criteria Hearing data included 52 papers excluded that 80 references considered for inclusion by 3 additional Control group included did not meet inclusion 2 authors (EG, BC) references identified criteria on closer review Homogeneous group studied

Number of papers for Validated outcome measurement used 31 references included in final analysis each age group: Infant/toddler: 10 Unique cohort and outcomes Early school aged: 14 Adolescence: 7 0 5 10 15 20 25 30 35 Gallagher and Collett, Pediatrics, 2019 yes unclear no

INFANT TODDLER EARLY SCHOOL ADOLESCENT INFANT TODDLER EARLY SCHOOL ADOLESCENT

EXPRESSIVE LANGUAGE RECEPTIVE LANGUAGE (CLP) LANGUAGE IQ34 SCHOOL ATTENDANCE39 EXPRESSIVE LANGUAGE RECEPTIVE LANGUAGE (CLP) WORKING MEMORY SCHOOL ATTENDANCE39 WORKING MEMORY30 EARLY READING EXPRESSIVE LANGUAGE (CLP) EXPRESSIVE LANGUAGE (CLP) EARLY READING26 IQ (CP, uCLP)

2-WORD PHRASES 18 2-WORD PHRASES

AVERAGE WPPSI PIQ AVERAGE WPPSI VIQ (CP, uCLP)18

DUTCH BAYLEY EARLY READING27, 28 WISC-III42 DUTCH BAYLEY EARLY READING WISC-III42

SYSTEMATIC REVIEW:

9, 39, 40, 9, 39, 40, AVERAGE RANGE, RANGE, AVERAGE MOTOR SKILLS EXPRESSIVE LANGUAGE (CP) PHONOLOGIC MEMORYSUMMARY30 OF FINDINGSSTANDARDIZED TEST SCORES RANGE, AVERAGE MOTOR SKILLS EXPRESSIVE LANGUAGE (CP) IQ (bCLP) STANDARDIZED TEST SCORES BELOW CONTROLS BELOW 41 CONTROLS BELOW 41

RECEPTIVE LANGUAGE RECEPTIVE LANGUAGE (CP) NONWORD SPELLING28, 30 RECEPTIVE LANGUAGE RECEPTIVE LANGUAGE (CP) READING COMPREHENSION GRADES/SPECIAL EDUCATION40, 43, GRADES/SPECIAL EDUCATION40, 43, 44 44 BAYLEY BAYLEY SCHOOL ATTENDANCE37, 39 BAYLEY BAYLEY SCHOOL ATTENDANCE LEARNING DISABILITIES43 LEARNING DISABILITIES43 FINE MOTOR GRADES/SPECIAL EDUCATION15, 26, 36, 37, 38 FINE MOTOR GRADES GRADE RETENTION43 GRADE RETENTION43 STANDARDIZED TEST SCORES36 STANDARDIZED TEST SCORES GRADUATION CERTIFICATE44 GRADUATION CERTIFICATE44 READING/LEARNING DISABILITIES15, 31 SPECIAL EDUCATION

BELOW AVERAGE BELOW RAPID NAMING42 AVERAGE BELOW RAPID NAMING42 RATE OF READING28 LEARNING DISABILITIES SUSTAINED ATTENTION42 SUSTAINED ATTENTION42 READING COMPREHENSION28, 31

WPPSI VIQ (bCLP)18

INFANT TODDLER EARLY SCHOOL ADOLESCENT

EXPRESSIVE LANGUAGE RECEPTIVE LANGUAGE (CLP) WORKING MEMORY SCHOOL ATTENDANCE EARLY READING • Quality of studies was variable EXPRESSIVE LANGUAGE (CLP) IQ (CP, uCLP) 2-WORD PHRASES AVERAGE • Several high quality studies clearly show academic deficits DUTCH BAYLEY EARLY READING IQ SYSTEMATIC REVIEW • Deficits were present in a range of domains and ages

AVERAGE RANGE, RANGE, AVERAGE MOTOR SKILLS EXPRESSIVE LANGUAGE (CP) IQ (bCLP) SUSTAINED ATTENTION BELOW CONTROLS BELOW RECEPTIVE LANGUAGE RECEPTIVE LANGUAGE (CP) READING COMPREHENSION GRADES • Future studies should include more rigorous BAYLEY BAYLEY SCHOOL ATTENDANCE STANDARDIZED TEST SCORES review of participants

FINE MOTOR GRADES GRADUATION CERTIFICATE STANDARDIZED TEST SCORES SPECIAL EDUCATION • Children with orofacial clefts are at risk for SPECIAL EDUCATION LEARNING DISABILITIES neurodevelopmental deficits and should be BELOW AVERAGE BELOW monitored and supported LEARNING DISABILITIES GRADE RETENTION • Evaluating neurodevelopmental outcomes is complex INTRINSIC DIFFERENCES

• Functional and psychosocial impacts of orofacial clefts Association between IEP and cleft type ACADEMIC DEFICITS • Many potential factors • Intrinsic • Extrinsic

Gallagher et al. Associations between laterality of orofacial clefts and medical and academic outcomes. American Journal of Medical Genetics. 2017.

• Normally, adult gaze focuses on an infant’s • Does breastfeeding have a positive impact on eyes before 6 weeks, then includes more cognition and behavior for children? time on the mouth when infant starts to • Nutritional benefits support neural maturation vocalize. and may impact language development • Maternal eye contact predicts mother-infant • Some studies found better neurodevelopmental relationship 1 year later and has been linked outcomes after exclusive breast milk feeding PARENTAL BONDING to developmental outcomes. FEEDING/NUTRITION • More recent studies have been less clear • Maternal gaze was shifted when infant had a • Skin-to-skin contact may help with bonding and cleft lip subsequently behavior • Gaze towards infant’s body • Gaze towards facial areas other than eyes or mouth.

DePascalis et al. Maternal gaze to the infant face: Effects of infant age and facial AAP policy statement, 2012 configuration during mother-infant engagement in the first nine weeks. 2017, Girard et al. Breastfeeding, Cognitive and Noncognitive Development in Early Infant Behavior and Development. Childhood: A Population Study. Pediatrics, 2017.

VELO: VPI Effects on Life Outcomes

• Speech Limitations FEEDING/NUTRITION CL CLP CP SPEECH OUTCOMES • Swallowing problems • Situational difficulty • 5 year retrospective review of children with cleft palate • Emotional impact • Breast milk feeding (ever) was 29.5% • CDC report = 81% • Perception by others • Lower z-scores for weight and weight for length • Caregiver impact

Kaye et al. Initial Nutritional Assessment of Infants With Cleft Lip and/or Palate: Interventions and Return to Birth Weight; Cleft Palate-Craniofacial Journal, 2017. Gottschlich et al. A Retrospective Study Identifying Breast Milk Feeding Disparities in Infants with Cleft Palate; Journal of the Academy of Nutrition and Dietetics, 2018. • In non-cleft populations, few clear differences have been identified in developmental outcomes after anesthesia.

• Danish study: neurodevelopmental outcomes EXPOSURE TO ANESTHESIA of CL, CLP, CP SCHOOL ABSENCE • CL had higher scores, CP lowest scores • Cleft type, not number of surgeries, was associated with lower outcomes. Population-based cohort in Western Australia • Higher absence rates for CLP in grades 4-6 400 cases, 1800 controls • No difference in high school Quantifying school absence for children • Higher absences associated with lower Hu et al. Association between Exposure of Young Children to Procedures Requiring General with orofacial clefts Anesthesia and Learning and Behavioral Outcomes in a Population-based Birth Cohort. standardized test scores Anesthesiology. 2017. Impact of school absence on test scores O’Leary et al. Influence of Surgical Procedures and General Anesthesia on Child • Children with CP had lower scores Development Before Primary School Entry Among Matched Sibling Pairs. JAMA Pediatrics, 2018. regardless of absence rates Sun et al. Association Between a Single General Anesthesia Exposure Before Age 36 Months and Neurocognitive Outcomes in Later Childhood. 2017, JAMA. Clausen et al. Oral Clefts and Academic Performance in Adolescence: The Impact of Anesthesia-Related Neurotoxicity, Timing of Surgery, and Type of Oral Clefts. 2017, Cleft Palate-Craniofacial Journal.

• Narrative review of 148 quantitative and qualitative studies, 2004-2015

• 5 domains of adjustment: • Developmental trajectory PSYCHOSOCIAL OUTCOMES • Behavior HEARING LOSS • Emotional Well-being

• Social Experiences Conductive hearing loss • Satisfaction with Appearance and Treatment Chronic middle ear effusions Chronic otitis media • Contradictory results in all areas but overall impact of cleft seems low

What is the degree of hearing loss before palate repair for infants with cleft palate?

• Retrospective chart review HEARING LOSS • Cleft palate ± cleft lip • DOB 2008-2015 • Palate repaired at SCH before age 3 years • Audiograms in AudBase

Tubes placed with palatoplasty DEGREE OF HEARING LOSS BY AGE HEARING AFTER PALATE REPAIR

CLICK-EVOKED BAER, n=61 BEHAVIORAL AUDIOGRAMS, n=259

4.6%: mild or greater hearing loss

49.2%: mild hearing loss 32.0%: mild hearing loss 11.5%: moderate or greater hearing loss 30.5%: moderate or greater hearing loss

Potential targets for intervention: Oral Cleft

Home Language/Literacy Environment • Parental bonding • Shared Oral Reading INTERVENTION STUDY • Reciprocal Conversation • Breastmilk feeding • Parent Beliefs about Reading/Development • Hearing loss Can we change the home language environment? Pre-Reading Skills • Vocabulary/Grammar • Home language environment • Print Awareness • Phonological Awareness

EVIDENCE FOR REACH OUT AND READ

• Improves home literacy environment • Increases scores on testing • Frequency of shared reading • Receptive and expressive language • Availability of books in the home • Literacy scores at school entry INTERVENTION STUDY • Reading becomes a favorite shared • Low socioeconomic settings activity • English and non-English-speaking children Can Reach Out and Read be used to positively impact the Home Language Environment?

www.reachoutandread.org CRANIOFACIAL REACH OUT AND READ MEASURING THE HOME LANGUAGE ENVIRONMENT

• 2012: partnered with national ROR • 16 hours of recordings at home • Developed a list of books by age and • Sorts child vs others, TV, radio specific speech sounds • Software analyzes and provides • Follow ROR model but also • Adult Word Count demonstrate how to use books to • Child Vocalizations practice speech • Conversational Turns LENA device

• Feasibility study • Inclusion • Recruitment • CL, CLP, CP • Protocol implementation • SCH Craniofacial Center • English or Spanish-speaking

LENA ROR STUDY • Study population LENA ROR STUDY • Goal: 60 children with clefts • Exclusion • 9 months (±2 months) • Syndrome with known delays • Brain malformation, seizure • Profound hearing loss • Hypotonia • Hospitalized >6 weeks • State custody, adopted

BASELINE CHARACTERISTICS OF PARTICIPANTS STUDY DESIGN

• 78 approached, 27 enrolled • Consent rate: 35%

• Combined recordings with others from a different study to increase pre-intervention recordings IMPROVING ACADEMIC OUTCOMES

CHILD VOCALIZATIONS CONVERSATIONAL TURNS

MODIFIABLE TARGETS POTENTIAL INTERVENTIONS

BONDING COACHING

HEARING LOSS AMPLIFICATION

BREAST MILK PARENT SUPPORT

• Improvement in slope of the curve after intervention? • Home language environment is a modifiable target of an intervention HOME LANGUAGE READING • Feasibility study, need larger sample size • Future plans: multicenter randomized trial with reading intervention and coaching

QUESTIONS?

• Thank you! • Craniofacial Center • Seattle Children’s Hospital Academic Enrichment Fund • Research collaborators • Patients and families Agenda

• Introduction & Background ImprovingPracticeEfficiencytoDeliver • Implementation & Practice Workflow • Using Tools HighͲQualityPreventiveServices ‰ Maternal Depression ‰ Development/Autism Screening GregBlaschke,MD,MPH,FAAP ‰ Social Determinants of Health ShirleyR.Kuse ProfessorofPediatrics • Adolescent Well Visits DivisionHead,GeneralPediatrics OHSUDoernbecher Children’sHospital • Resources

FacultyDisclosure:GregBlaschke,MD,MPH,FAAP FacultyDisclosure:EdwardCurry,MD,FAAP

In the past 12 months, I have relevant financial relationships with the manufacturer(s) of any commercial product(s) and/or provider(s) of commercial services discussed in this CME activity. Small royalties from Up-2-Date reviews (donated to Cindy Ferrell Fund) In the past 12 months, I do not have any financial disclosures.

I do not intend to discuss an unapproved/investigative use of a commercial product/device in my presentation. I do not intend to discuss an unapproved/investigative use of a commercial product/device in my possession. I am one of the contributors/reviewers of the Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescent, 3rd and 4th Editions.

I acknowledge that today’s activity is certified for CME credit and thus cannot be promotional. I will give a balanced presentation about well-child care using the best available evidence to support my conclusions and recommendations.

ChangeinPractice ƌŝŐŚƚ&ƵƚƵƌĞƐ Participantswillbeableto: th …isasetofprinciples,strategiesandtoolsthataretheoryͲ ‰ReviewclinicalcontentinBrightFuturesGuidelines,4 Edition based,evidenceͲ driven,andsystemsͲ oriented,thatcanbe ‰IdentifyofficesystemsͲbasedstrategiestomaximizeflowand usedtoimprovethehealthandwellͲbeingofallchildren efficiencyforhealthpromotion throughculturallyappropriateinterventionsthataddressthe ‰UsepediatricianͲtestedstrategiesandBrightFuturestoolsto currentandemerginghealthpromotionneedsatthefamily, improvethequalityofpreventiveservicesdeliveredinthe clinicalpractice,community,healthsystemandpolicylevels. clinicalsetting ‰IdentifyopportunitiestotailorandapplyBrightFutures/AAP recommendationswithavailabletoolsandresources ƌŝŐŚƚ&ƵƚƵƌĞƐ ThePeriodicityScheduleandtheBrightFuturesGuidelines BrightFuturesisthehealthpromotion/disease preventionpartofthemedicalhome.

Attheheartofthemedicalhomeisthe relationshipbetweentheclinicianandthe familyoryouth The Periodicity Schedule tells you what to do in well- child visits, while the Bright Futures Guidelines tell you how to do it—and how to do it well.

ƌŝŐŚƚ&ƵƚƵƌĞƐ'ƵŝĚĞůŝŶĞƐ͕4th Edition ,ĞĂůƚŚWƌŽŵŽƚŝŽŶdŚĞŵĞƐ͗4th Edition Part 1: Health Promotion Themes • Promoting Lifelong Health for • Healthy Nutrition – 12 chapters highlighting key health promotion themes Families and Communities – New themes: Social determinants of health; Media use; Children and Youth with • Physical Activity Special Health Care Needs • Family Support • Oral Health • Promoting Health for Children and Part 2: Health Supervision Visits • Adolescent Development Youth with Special Health Care – Rationale and evidence for screening recommendations • Promoting the Healthy and Safe Needs – 32 age-specific visits (including prenatal visit) Use of Social Media – 5 health supervision priorities for each visit • Healthy Development • Safety and Injury Prevention • Designed to focus visit on most important issues for child that age • Mental Health • Includes: social determinants of health, health risks, developmental issues, positive reinforcement • Healthy Weight

ComponentsofaBrightFuturesVisit What’sNewaboutthe4th Edition?

¾ Social determinants of health are embedded in many visits • Tasks 9 Strengths and protective factors make a difference • Disease detection 9 Risk factors make a difference • Disease prevention ¾ Features updated milestones of development and developmental surveillance • Health promotion questions • Anticipatory guidance ¾ Provides new clinical content about the latest recommendations and provides guidance on implementation • Duration ¾ Includes updates to several adolescent screenings including cervical dysplasia; • Approx. 18 minutes depression; dyslipidemia; hearing; vision; tobacco, alcohol, or drug use ScreeningsUpdatedfromthe3rd Edition NewScreeningsSincethe3rd Edition

‰ Adolescent hearing screening: • Bilirubin screening: Universal at the Newborn Visit. ‰ 3rd Edition: Selective audiometry based on risk assessment at all Adolescent Visits • Maternal depression screening: Universal at the 1 Month through 6 Month Visits. ‰ 4th Edition: Universal audiometry (once during the Early, Middle, and Late Adolescence Visits) • Oral health: Universal fluoride varnish at the 6 Month (first tooth eruption) through 5 Year Visits, in addition to Selective fluoride supplementation at the 6 Month through 12 Month and 18 Month through ‰ Adolescent tobacco, alcohol, or drug use assessment: 16 Year Visits. ‰ 3rd Edition: Selective based on risk assessment for alcohol and drugs • Dyslipidemia screening: Universal once between the 9 and 11 Year Visits, in addition to the Universal ‰ 4th Edition: Tobacco, alcohol, or drugs – universal administration of an assessment tool at all Adolescent Visits dyslipidemia once between the 17 and 21 Year Visits carried over from the 3rd Edition. • Depression screening: Universal for adolescents, annually beginning at the 12 Year Visit. ‰ Cervical dysplasia: • Human immunodeficiency virus (HIV) screening: Universal once between the 15 and 18 Year Visits. ‰ 3rd Edition: Selective based on risk assessment at all Adolescent Visits ‰ 4th Edition: Universal beginning at the 21year visit in the 4th Edition

ƌŝŐŚƚ&ƵƚƵƌĞƐdŽŽůĂŶĚZĞƐŽƵƌĐĞ<ŝƚ,2nd Edition CoreTools:IntegratedFormat

Supporting Materials The toolkit consists of 2 main sections: ƒ Screening and Assessment Tools Core Forms ™Medical Screening Reference Tables These are the key documents to carry ™Commonly Used Screening Instruments and Tools out each Bright Futures visit: ƒ Additional forms that accompany the Visit ƒ Previsit Questionnaire Documentation Form ƒ Visit Documentation Form ™Initial History Questionnaire ƒ Bright Futures Parent-Patient ™Medication Record Handouts ™Problem List

™Problem Visit ‰ Previsit Questionnaire ‰ Documentation Form ‰ Parent/Patient Educational Handout ƒ Supplementary AAP Education Handouts – Surveillance tool allows healthcare – To document all pertinent information – Provides parental education for all Bright professional to gather pertinent information and fulfill quality measures Futures Priorities at each visit without using valuable time asking questions

Implementation&PracticeWorkflow HowDoesƌŝŐŚƚ&ƵƚƵƌĞƐ HelpYou?

Forhealthcareprofessionals: ForAAPChapters: WithBrightFutures,healthcare Providesresourcestoassistmembersin professionalscanaccomplish4tasksin18 followingtheGuidelinesandsharingbest minutes.Thetoolsandresourceshelp implementationpractices.BrightFutures clinicianstostructurevisitsandcreate servesasthebasisforquality practiceprocessestobetteraddress improvementprojects patientneeds.

Forpublichealthprofessionals: Forfamilies: Providesaroadmapforstructuringvisits Providesresourcesandeducational andsharinghealthinformationwiththe materialsspecifictoeachwellͲchildvisit. community;helpsidentifyprioritiesfor BrightFuturesrecognizesthestrengths fundingandprovidesrecommended thatfamiliesandparentsbringtothe standardizeddevelopmentalassessments. healthcarepartnership. ImplementingƌŝŐŚƚ&ƵƚƵƌĞƐŝŶƚŽĂŝůLJWƌĂĐƚŝĐĞ ImplementingƌŝŐŚƚ&ƵƚƵƌĞƐŝŶƚŽĂŝůLJWƌĂĐƚŝĐĞ

How it gets done in your practice setting in Can it be done? partnership with your patients and parents

You and your team are the experts YES!

OfficeͲBasedSystemsComponents Questionnaires ‰ Practice support and nursing staff in ‰ Utilize a preventive services prompting system ‰ Paper charge of how this happens: ‰ Utilize a recall/reminder system ƒ Have a staff session to reinforce ‰ Electronic importance and contribution ™ To address immunizations and well child visits ƒ Train how to distribute ƒ At the visit in the waiting or ƒ Develop a scoring system ‰ Utilize a system to track referral exam room ƒ Develop a system to alert the ™ Paper-based or electronic healthcare professional to know “when ƒ At home (via email or patient ready to proceed” ‰ Utilize a system to identify children with special health care needs ƒ Help parents/youth with literacy or portal) language differences ‰ Link families to appropriate community resources ‰ Make appointment time ƒ Have all tools and supplies ready ‰ Utilize a strength-based approach and shared decision-making strategy 15 minutes earlier ƒ Shift some responsibilities from the clinician to non-clinician staff where appropriate

WhatCanYouGetFromaƌŝŐŚƚ&ƵƚƵƌĞƐPrevisitQuestionnaire? Here are examples of what you can learn about how your patient and family are doing… ‰Parental/youth concerns and questions for this ‰ Developmental surveillance for young children visit ‰ Strengths/developmental surveillance for school ‰ Surveillance of patient/family strengths aged children & adolescents ‰ Surveillance of major changes in family ‰ Expanded anticipatory guidance questions such ‰ as: CaseStudies Medical risk assessment (unique for each age/visit) such as: ƒ Social Determinants of Health ƒ Caring for infant/child/adolescent ƒ TB, Lead, Anemia, STIs, Cholesterol ƒ Patient’s emotional well-being ƒ Vision and Hearing ƒ Safety ‰ Oral health risk assessment This surveillance tool also alerts the patient/family that they will be ƒ Dental home/fluoride H2O universally screened for topics based on their age/stage (eg, child development, autism, depression, etc.). UsingtheToolsThroughCaseStudies StrategiesforImplementingAdolescentWellVisits

¾ MaternalDepression(1MonthVisit) ¾ ChildDevelopment/Autism(18MonthVisit) ¾ SocialDeterminantsofHealth(3YearVisit)

AdolescentGeneralities AdolescentGeneralities

Adolescents are special! (like Start with strengths and practice Need to destigmatize, and do Practice is contextual - Best to be obvious and talk out loud Plain language newborns, 5 year old, preteen) building rapport universal screening modify to community, (no hidden agenda) • consent = giving permission epidemiology, setting “I ask all my patients these questions” • confidentiality = telling Adolescents are ‘hyper aware/ Have an office action plan for Visits are part of transition planning (rainbow flags help) others only if… in tune’ with environmental things we fear: • Becoming responsible for own • disclosures = can happen unintentionally (open record, clues and may ‘read things in’ • Pregnancy health over time For 10 years and over, Flow: together, separate, together billing, reminders) when not intended • Suicide • Letters for parents re: completing screening • Parent concerns and ability to • Addiction screeners together promotes promote understanding and • Violence • Letters for adolescents re: understanding discussion confidentiality, consent and disclosure

AdolescentGeneralities Suggestions 9 Convert Sports PE and explore further if complaint doesn’t = PE Statelawsvary Breakconfidentiality/disclosures • Talkwithpermission 9 Normalize asking questions • Generallywhenneeded(nocontraindications) 9 Do NOT ignore any concerning statement • Parentsinvolvementimprovesoutcome 9 Use motivational interviewing • Theydon’tneedtoknowall(orsometimesany) 9 Use tools! details 9 No such thing as Negative screen (thanks for answering, who could you talk about…XYZ) 9 Encourage longer appointments 9 Visit lasts over entire time in clinic (use team) 9 Continuity and longitudinal care (not everything in 1 visit) AdolescentWellVisits Confidentiality STRATEGIES PRACTICAL POINTS

• Schedule a longer visit • Explain why you’re asking the questions • Have an adolescent-friendly space 1. Parent and patient 3. Parent and patient together at beginning together to review • Clearly define confidentiality of visit assessment • Move from non-threatening questions to more sensitive topics • Remember surveillance is not 2. Parent and patient separated screening and vice versa during sensitive questions and physical exam

AdolescentWellVisits AdolescentWellVisits STRATEGIES STRATEGIES

• Avoid medical jargon – speak simply Treat all comments seriously • • Explore the adolescent’s issues • Be aware of nonverbal communication • Ask sensitive questions in the third Keep the tone non-judgmental • • Treat all comments seriously person (particularly for younger Avoid “Why?” • Don’t chart during the interview adolescents) • Use clarification, reflection, and interpretation as strategies • Use open-ended questions whenever possible

AdolescentWellVisits ConfidentialitySampleScript STRATEGIES “There are some things I talk about with everyone • Consider the adolescent’s your age. I keep this information private from your developmental stage, culture, parents if you don’t want to share it with them. If I ethnicity hear something that sounds dangerous to you or • Reassure when the adolescent someone else, I may need to tell your parents about seems uncomfortable that. I encourage everyone your age to talk to their • Encourage regular and open parents about important things, but if you don’t feel communication with parents ready, you can talk about those things here.” Caution AdolescentPrevisitQuestionnaires EXAMPLE

• Have an adolescent office action plan Universal screening • Suicide? imminent or past? recommendations Riskassessment

• Pregnancy Patient’s concerns Example:11Ͳ14YearVisits • Addiction (sensitivequestionsincluded) Patientstrengths • Disclosure of violence

Anticipatory • Use your full team and partners guidancequestions Development alsurveillance

Summary

• Interview the adolescent patient alone. • Explain to patients what you can and cannot do confidentially. Questions? • Explain limits of confidentiality. • Implement policies to protect confidentiality and inform staff. • Involvement of the family is optimal.

Workflow– 1MonthVisitExample EXAMPLE EstablishingaWorkflow:Review Workflow Needs to be Job-Specific, not Person-Specific ‰ Starts with initial entry point to medical office ƒ Receptionist provides age appropriate Previsit Questionnaire ƒ Pre-formatted age specific packet (1 Month Packet example) • 1 Month Previsit Questionnaire • Maternal Depression screening tool • Parental Educational Handout ƒ Parent would complete questionnaires/screening tools in waiting area ƒ Medical assistant on rooming child would make sure questionnaire is completed ƒ MA attaches questionnaire to chart or enter the results into the EHR ƒ Physician would review either paper copy or EHR ƒ Would document intervention in chart ƒ Completion of visit medical assistant would provide appropriate parent handout CommunityLinkageTipsfromthePractices TeamͲBasedApproach ‰Systems measure You don’t have to do all this alone! ƒ Do you have someone in your office or clinic who is in charge of liaisons with ¾ Multiple health supervision visits, thus multiple opportunities community organizations and updates to accessible list of community resources for parents? ¾ Sharing and delegation of tasks ‰Consider hiring a care coordinator, or use current staff with skills in this area ¾ Practice change management resources can be found on the following ‰Use community liaisons in the practice to handle referrals, communicate with specialists, websites: and coordinate services/resources for families o Bright Futures ‰Consider hosting “mixers” with potential referral sources in the community to establish o STAR Center relationships o National Resource Center for Patient/Family-Centered Medical Home ‰ If you have set it up, everything related to a difficult situation goes better o AAP Quality Improvement

Billing &Coding AccessingScreeningTools EXAMPLE ‰When standardized screening tools are administered, scored, and interpreted as part of preventive service visit, each screening can be individually coded for billing purposes.

‰Example:

https://toolkits.solutions.aap.org/ss/screening_tools.aspx

Source: https://www.aap.org/en-us/Documents/coding_preventive_care.pdf

PediatricPreventiveCodingResources MCH/TitleVConnection CHIPRA 2019 Core Measures

Title V MCH Services Block Grant WeightAssessmentandCounselingforNutritionand Coding at the AAP Website National Performance Measures PhysicalActivity ChlamydiaScreeninginWomenAges16Ͳ20 • One stop shop for all coding related resources from the AAP Breastfeeding SafeSleep ChildhoodImmunizationStatus • Includes ICD-10-CM information and all topic-specific coding fact DevelopmentalScreening ScreeningforDepressionandFollowͲUpPlan:Ages12Ͳ sheets 17(CDFͲCH) PhysicalActivity • Coding for Pediatric Preventive Care, 2019 Booklet WellͲChildVisitsintheFirst15MonthsofLife Bullying ImmunizationsforAdolescents • available at: https://www.aap.org/en- AdolescentWellͲVisit us/Documents/coding_preventive_care.pdf DevelopmentalScreeningintheFirstThreeYearsof MedicalHome Life • AAP Coding Hotline [email protected] for all your coding and Transition payer questions and issues!! AdolescentWellͲCareVisits PreventiveDentalVisit AccesstoPrimaryCarePractitioners Smoking medicaid.gov/medicaid/qualityͲofͲcare/performanceͲmeasurement/childͲ mchb.tvisdata.hrsa.gov/PrioritiesAndMeasures/NPMDistribution coreͲset/index.html EducationinQualityImprovementforPediatricPractice(EQIPP) WebsiteResources x Resources and tip sheets brightfutures.aap.org • EQIPP courses help you identify and close gaps in your practice x Resources for families, states and using practice tools. community health programs

• Bright Futures - Infancy and Early x Implementation strategies and Childhood Course stories from practices, states, and • Bright Futures - Middle Childhood communities that use Bright and Adolescence Course Futures

ƌŝŐŚƚ&ƵƚƵƌĞƐTools ChangesinPractice:Recap Below are some tools and resources available to assist with implementation of the 4th Edition: Participants can: th ƒ Bright Futures Guidelines, 4 Edition – Introductory Webinars ‰Review clinical content in Bright Futures Guidelines, 4th Edition o Available at: https://brightfutures.aap.org/materials-and-tools/Pages/Bright-Futures-Webinars.aspx ƒ Bright Futures Tool and Resource Kit, 2nd Edition – Overview (narrated PPT) ‰Identify office systems-based strategies to maximize flow and efficiency for health o Available at: https://brightfutures.aap.org/materials-and-tools/Pages/Presentations-and- promotion Handouts.aspx ƒ Screening and Priorities for each age/stage ‰Use pediatrician-tested strategies and Bright Futures tools to improve the quality of o Available at: https://brightfutures.aap.org/materials-and-tools/Pages/Presentations-and- preventive services delivered in the clinical setting Handouts.aspx ƒ Medical Screening Reference Tables ‰Identify opportunities to tailor and apply Bright Futures/AAP recommendations with o Available at: https://brightfutures.aap.org/materials-and-tools/tool-and-resource- available tools and resources kit/Pages/Medical-Screening-Reference-Tables.aspx

References

‰ Duncan P, Pirretti A, Earls MF, Stratbucker W, Healy JA, Shaw JS, Kairys S. Improving delivery of Bright Futures preventive services at the 9- and 24-month well child visit. Pediatrics. 2015;135(1)e178-e186. Available at: http://pediatrics.aappublications.org/content/135/1/e178

‰ Lannon CM, Flower K, Duncan P, Moore KS, Stuart J, Bassewitz J. The Bright Futures Training Intervention Project: implementing systems to support preventive and developmental services in practice. Pediatrics. 2008;122(1)e163-e171. Questions? Available at: http://pediatrics.aappublications.org/content/122/1/e163

‰ Hagan JF, Shaw JS, Duncan PM, eds. Bright Futures: Guidelines for Health Supervision of Infants, Children and Adolescents, 4th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017.

‰ Shaw JS, Hagan JF Jr, Shepard MT, Curry ES, Swanson JT, Janies KM, eds. Bright Futures Tool and Resource Kit. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2019 HowtoObtainƌŝŐŚƚ&ƵƚƵƌĞƐMaterials ContactInformation American Academy of Pediatrics Visit the Bright Futures Web site: brightfutures.aap.org Bright Futures National Center

Phone 630-626-6783 To order the Bright Futures Guidelines and Toolkit, go to shopAAP.org E-mail Sign up for the Bright Futures eNews and other alerts at [email protected] brightfutures.aap.org/Pages/contactus.aspx Website brightfutures.aap.org NeonatalHyperbilirubinemiaUpdates LearningObjectives

1. Reviewthebasicpathophysiologyofneonatal hyperbilirubinemia 2. UnderstandtheAAP’sclinicalpracticeguidelinesfor hyperbilirubinemiainnewbornsш35weeks 3. Reviewoutcomesofguidelinesimplementationand emergingdataaboutthepossiblerisksassociated withphototherapy E.HayesBakken,MD,IBCLC 4. DiscussNorthernCaliforniaNeonatalConsortium IlseLarson,MD,IBCLC ConsensusGuidelinesforScreening&Management

Withgratitudeto: ¾ EllenLaves,MD,CarriePhillipi,MD,PhD,andMinaTahai,MD(formanyoftheslides) ¾ TomNewman,MD,MPH(forallthelearnings)

NeonatalJaundice WhyNewborns?

60%ofhealthynewbornswillhaveclinicaljaundice • Increasedbilirubinproduction(јHgb&shortRBC lifespan) • LimitedbilirubinͲbindingcapacity(lowserum albumin) • DecreasedconjugaƟon(љglucoronysylͲ transferase activity) • Decreasedexcretionleadingtoreabsorptioninthe bowel(bowelflora,intestinalmotility,stool frequency,caloricintake,andfeedingfrequency)

What’sthesignificance? Reduced<30 TheKinder, Morephototherapy Reduced È BF GentlerEra kernicterus??? Acutebilirubinencephalopathy: 20! rates Ç BFrates • LethargyÆstupor • HypotoniaÆ hypertoniaÆ retrocolisͲopisthotonis 1980 •Poorfeeding,shrillcry 1950 1960 1970 1990 2004 2009 Kernicterus(chronicbilirubinencephalopathy): • Extrapyramidalsigns(athetosis),severedelays/MR Rhogam Vigintiphobia AAPPolicyShift USPSTF • Sensorineuralhearingloss Universal Statement Exchange Screening • Gazepalsies Transfusions • Dentaldysplasia

Phototherapy 2004AAPGuidelines AAPGuidelineGraphs 1. Promoteandsupportsuccessfulbreastfeeding. 2. Establishnurseryprotocolsfortheidentificationandevaluationof hyperbilirubinemia. 3. Measurethetotalserumbilirubin(TSB)ortranscutaneousbilirubin(TcB)levelon infantsjaundicedinthefirst24hours. 4. Recognizethatvisualestimationofthedegreeofjaundicecanleadtoerrors, particularlyindarklypigmentedinfants. 5. Interpretallbilirubinlevelsaccordingtotheinfant’sageinhours. 6. Recognizethatinfantsatlessthan38weeks’gestation,particularlythosewho arebreastfed,areathigherriskofdevelopinghyperbilirubinemiaandrequire closersurveillanceandmonitoring. 7. Performasystematicassessmentonallinfantsbeforedischargefortheriskof severehyperbilirubinemia. 8. Provideparentswithwrittenandverbalinformationaboutnewbornjaundice. 9. ProvideappropriatefollowͲupbasedonthetimeofdischargeandtherisk assessment. 10. Treatnewborns,whenindicated,withphototherapyorexchangetransfusion.

EffectofUniversalScreening EffectofUniversalScreening

Kuzniewiczetal.2009 38,182infants. • Only56%ofthosewhoreceivedphototherapyhadTsB above 10.6%werebornatfacilitieswithuniversalbilirubinscreening. threshold,comparedwith70%infacilitieswithoutuniversal screening. ComparedwithinfantsbornatfacilitiesthatwereNOT screening: • 62%lowerincidenceofTsB levelsovertheAAPthreshold (0.17%vs0.45%;P<.001), • Hadtwicetherateofinpatientphototherapy(9.1%vs4.2%;P <.001),and • Hadslightlylongerbirthhospitalizationlengthsofstay(50.9vs 48.7hours;P<.001).

PhototherapyNNT

Newman,etal2009 281,898AGAinfantsbornш35weeks’gestationat12Northern CaliforniaKaiserhospitalsfrom1995to2004. • 22,547withaTsB within3mg/dL oftheAAPphototherapy Isthistherightapproach? threshold • Usedmultiplelogisticregressiontoestimatetheefficacyof hospitalphototherapyinpreventingthebilirubinlevelfrom exceedingthe2004guideline’sexchangetransfusionthreshold within48hours. NNTs(95%CI) IntheSettingofUniversalScreening,doInfantsExceed ExchangeTransfusionLevels? Gestational AgeatQualifying AgeatQualifying AgeatQualifying AgeatQualifying Age,wk TSB:<24h TSB:24to<48h TSB:48to<72h TSB:ш72h Boys Flaherman etal.,2012~18,000newborns(2005Ͳ2007)intheKP 35 14(7–40) 26(14–57) 83(36–190) 171(70–426) NorthernCaliforniaHospitalsaftertheimplementationof 36 10(6–19) 19(12–39) 59(31–101) 122(68–236) universalscreening 37 16(10–28) 29(20–58) 95(52–168) 196(100–407) 38 35(14–100) 67(31–215) 222(107–502) 460(196–1352) • 22infants(14infants<38weeks)exceededexchange 39 74(31–244) 142(62–554) 476(197–1385) 989(373–3607) transfusionthreshold 40 106(44–256) 204(98–487) 682(367–1294) 1419(634–3755)

ш41 148(54–428) 284(127–780) 953(366–3017) 1983(676–8408) •Only1receivedanET Girls •Nodocumentedsequelae 35 21(12–49) 40(21–86) 126(50–267) 261(105–585)

36 15(11–26) 28(20–51) 90(43–146) 186(102–347)

37 23(16–39) 44(31–75) 145(73–243) 300(146–671)

38 53(23–134) 102(43–236) 339(154–730) 705(314–2016)

39 113(58–342) 217(103–713) 729(272–1730) 1516(614–4520)

40 162(75–400) 312(164–704) 1046(491–2136) 2176(922–6107)

ш41 226(92–702) 435(183–1140) 1461(510–4842) 3041(888–11096)

Table4:NewmanetalWĞĚŝĂƚƌŝĐƐ.2009May;123(5):1352–1359.doi:10.1542/peds.2008Ͳ1635

IntheSettingofUniversalScreening,doInfantsExceed JaundiceOutcomes ExchangeTransfusionLevels? Wickremasinghe,etal2015 •ScreeningTsB wasatleast“highͲintermediaterisk”forall22 infantsand“highͲrisk”forallш38weeks. • SNHL:Onlybilirubinlevelsш10mg/dlaboveexchange transfusionthresholds(orш35mg/dl)wereassociatedwitha •4outcomesmaybeattributabletoincompleteadherenceto significantlyincreasedrisk AAPguideline •13mighthavebeenpreventedbybetteradherencetoAAP Wu,etal2015 ĨŽůůŽǁͲƵƉ guideline • CerebralPalsyconsistentwithkernicterusoccurredonlyin BUT… infantswith2+riskfactorsforNTandTsB >5mg/dlabove •ReͲtestingwouldhaverequired2166 additionalbilirubintests exchangetransfusionthreshold toprevent(atmost)13outcomes Vandborg,etal2012 • Nosignificantdifferenceindevelopment atage1Ͳ5years (ASQ)ininfantswithapeakserumbilirubinover25mg/dl

WhoGetsKernicterus?

Kuzniewicz etal2014: KaiserNorthernCalifornia. 525,409infantsш35weeksgestationbetween1995Ͳ2011 • 47infantsidentifiedwithTsB ш30(8.6per100,000births) Arethererisksof •Medianfollowup7.9years phototherapy? DoesPhototherapyaffect DoesPhototherapyleadtoincreasedSeizure Breastfeeding? Risk? Waite,etal2016:smallreductioninbreastfeedingratesat12 Maimburg etal2016 monthsandinexclusivityat1,2,and4months • Increasedriskofepilepsyamongchildrentreatedwith phototherapy,theassociationwasseenonlyinboys(adjusted HR1.98,95%CI:1.40–2.78) Newman,etal2018 • Increasedriskofepilepsy,adjustedhazardratio(aHR)of1.22 (95%CI:1.05to1.42;P=0.009) • Boyswereathigherriskofseizuresoverall(aHR =1.18;95%CI: 1.10to1.27)andhadahigheraHR forphototherapy(1.33;95% CI:1.10to1.61)

IsPhototherapylinkedtoChildhoodCancer?

Newmanetal2016 Retrospectivecohortstudyof525,409childrenbornatш35 weeks’gestationbetween1995Ͳ2011at15KPNChospitals Exclusions:death,transfer,losttofollowͲupat<60days, cancerdxbefore60days •InitialcrudeIRRswereuniformlypositivewithlowpͲvalues. •Afteradjustingforconfoundingwerenolongersignificant Upperlimitofthehazardratiosismostconcerningforinfant’s withDownsyndromewiththeNNHbeing23attheupper limit

DevelopmentoftheNCNCGuidelines

Basedonconcernsthatthe2004AAPGuidelinewasbasedon limitedevidence,internallyinconsistentandrecommenda significantpracticeshiftat38weeksgestation,theUCSF NorthernCaliforniaNeonatalConsortiummemberscame togetherto: Isphototherapyworthevenasmallrisk? • Updatehyperbilirubinemiaclinicalpracticebasedonrecent research • DrawontheKPNorthernCaliforniaexperiencewithupdated clinicalpracticeguidelines

Fullexecutivesummaryandrecommendations: http://www.phototherapyguidelines.com/NeoHyperbilirubinemiaGuidelineFINAL_2018Ͳ0209.docx NCNCGraphs www.phototherapyguidelines.com

NCNCDefinitionofNeurotoxicityRisk AAPvs.NCNC(withriskfactors) Factors Neurotoxicityriskfactorsinclude: • Isoimmune hemolyticdisease,G6PDdeficiency,orotherhemolytic disease • Sepsisorsuspectedsepsis(sufficienttobecurrentlyonantibiotics) • Acidosis(BEчо8meq/LorpCO2>50mmHgwithinthelast24hr) • Albumin<3.0mg/dL • Anyclinicalinstability Examplesforwithneurotoxicityrisks,168HOLinfant 401/7NCNCthreshold=19(vs.18)Exchange=25.5(vs.22.5) 381/7NCNCthreshold=19(vs.18)Exchange=23.1(vs.22.5) 351/7NCNCthreshold=15.2(vs.15)Exchange=19.2(vs.19)

AAPvs.NCNC(noriskfactors) CaseComparisons

Ababyboyinclinicisnotedtohavejaundiceat48hoursoflife. MotherisAB+/AbͲ,sheisexpressingcolostrumandexclusively breastfeeding.Thebabyisfeedingwellwithappropriate output.Thisismom’sthirdbaby.Infant’sweightisdown7% frombirthweight.Thereisslightfacialbruising,butno cephalohematoma.TSBisobtainedandis15.5mcg/dL. ƒ Let’slookatAAPvs.NCNCrecommendationsfora41w1d, 37w6dand36w2dweekgestationalageinfant Examplesfornoneurotoxicityrisks,168HOLinfant 401/7NCNCthreshold=23(vs.21)Exchange=30.0(vs.25) 381/7NCNCthreshold=21.5(vs.21)Exchange28.3(vs.25) 351/7NCNCthreshold=19.1(vs.18)Exchange25.6(vs.22.5) 41w1d: 37w6d: AAPvs.NCNCRecommendations AAPvs.NCNCRecommendations

36w6d: AAPvs.NCNCRecommendations NextSteps??

Stepsthatreducephototherapy,butarestillinlinewiththeAAP Guidelines: Æ NophototherapyundertheAAPthresholds Æ Adjustaroundthemediumriskthresholdbygestationalage

Fromthe2004NomogramsText:

References

AdopttheNCNCGuidelines Flaherman V.J.,Kuzniewicz M.W.,EscobarG.J.,andNewmanT.B.TotalSerumBilirubinExceedingExchangeTransfusionThresholdsinthe SettingofUniversalScreening.JPeds 2012;160(5):796Ͳ801. Hoffman,etalConsensusGuidelinesforScreening&ManagementofHyperbilirubinemiainNeonates.UCSF(NC)2(NorthernCA NeonatologyConsortium).Originated1/2016.Lastrevised02/09/19.onlinehttp://www.phototherapyguidelines.com/ accessed:8/16/19. Kuzniewiicz M.W.Wickremasinghe A.C.,Wu,Y.W.,etal.Incidence,Etiology,andOutcomesofHazardousHyperbilirubinemiainNewborns. Pediatrics2014;134:504Ͳ509. Kuzniewicz M.W.,Escobar,G.J.andNewman,T.B..Impactofuniversalbilirubinscreeningonseverehyperbilirubinemiaandphototherapy • Evidencebaseisstrong use.Pediatrics2009;124(4):1031–1039. Maimburg,R.D.,Olsen,J.andSun,Y.2016.Neonatalhyperbilirubinemiaandtheriskoffebrileseizuresandchildhoodepilepsy.Epilepsy • InsiderintelligenceisthatforthcomingAAPguidelineswillnot Research2009;124:67–72. belowerthantheNCNCguidelines(2020?2021?) Maisels,M.J.andMcDonagh,A.F.Phototherapyforneonataljaundice.TheNewEnglandJournalofMedicine2008;358:920–928. NewmanT.B.,Kuzniewicz,M.W.,Liljestrand,P.,Wi,S.,McCulloch,C.andEscobar,G.J.Numbersneededtotreatwithphototherapy • OHSU’sED,Ward,MBU,andclinicsadoptedtheNCNC accordingtoAmericanAcademyofPediatricsguidelines.Pediatrics2009;123(5):1352–1359. NewmanT.B.,Wickremasinghe,A.C.,Walsh,E.M.,Grimes,B.A.,McCulloch,C.E.andKuzniewicz,M.W.Retrospectivecohortstudyof guidelinesSeptember9,2019 phototherapyandchildhoodcancerinNorthernCalifornia.Pediatrics2016;137(6). NewmanT.B.,Wu,Y.W.,Kuzniewicz,M.W.,Grimes,B.A.andMcCulloch,C.E.Childhoodseizuresafterphototherapy.Pediatrics2018; 142(4). Vandborg P.K.,Hansen,B.M.,Greisen,G.,Mathiasen,R.,Kasper,F.andEbbesen,F.2015.FollowͲupofextremeneonatal hyperbilirubinaemia in5Ͳ to10ͲyearͲoldchildren:aDanishpopulationͲbasedstudy.DevelopmentalMedicineandChildNeurology2018: 57(4):378–384. WaiteW.M.andTaylor,J.A.Phototherapyforthetreatmentofneonataljaundiceandbreastfeedingdurationandexclusivity.Breastfeeding medicineௗ:theofficialjournaloftheAcademyofBreastfeedingMedicine.2016;11:180–185. Wickremasinghe A.C.,Risley,R.J.,Kuzniewicz,M.W.,etal.Riskofsensorineuralhearinglossandbilirubinexchangetransfusionthresholds. Pediatrics2015;136(3):505–512. WuY.W.,Kuzniewicz,M.W.,Wickremasinghe,A.C.,etal..Riskforcerebralpalsyininfantswithtotalserumbilirubinlevelsatorabovethe exchangetransfusionthreshold:apopulationͲbasedstudy.JAMAPediatrics2015;169(3):239–246. Objectives Formulas and Vitamins- • Understandinfantandpediatricformulasandtheirappropriateuses Oh My! • Understandmainvitaminsandmineralsofconcern • Reviewcasestudy Briza York, RD, CSP, LD Clinical Pediatric Dietitian Specialist (Gastroenterology) Doernbecher Children’s Hospital Oregon Health and Science University

October 17th, 2019

InfantFormulas InfantFormulas Elecare Infant Neocate Infant Otherspecializedformulas: Puramino Infant(0Ͳ24mos) EnfamilPrematureandEnfaCare Alfamino Infant SimilacSpecialCareandNeosure • Breastmilkisbest!Butsometimesnotavailable Enfaport EnfamilPregestimil Similac PM60/40 • FDAregulated RossCarbohydrateFreeSoy EnfamilNutramigen Metabolicformulas(examples: • Standardconcentrationis20caloriesperounceformajorityof SimilacAlimentum Calcilo XD,PhenexͲ1,LMD) formulas GerberExtensiveHA • Specialrecipestomakeformulashigherincaloriesifneeded EnfamilGentlease • Prematuredischargeformulasare22caloriesperouncestandardmixing SimilacSensitive SimilacTotalComfort • Mainformulacompanies:EnfamilandSimilac GerberGentle

EnfamilProsobee EnfamilAR SimilacIsomil SimilacforSpitUp GerberSoy EnfamilInfant EnfamilEnspire SimilacAdvance SimilacOrganic

Thebottomhalfofthepyramidreally InfantFormulas Elecare Infant Neocate Infant Otherspecializedformulas: lookslikethis… *Notincluded:NonͲUS Puramino Infant(0Ͳ24mos) EnfamilPrematureandEnfaCare formulas(suchasHiPP, Alfamino Infant SimilacSpecialCareandNeosure Holle,etc) Enfaport EnfamilPregestimil Similac PM60/40 RossCarbohydrateFreeSoy EnfamilGentlease EnfamilNeuroPro Gentlease EnfamilNutramigen Metabolicformulas(examples: SimilacSensitive EnfamilEnspire Gentlease SimilacAlimentum Calcilo XD,PhenexͲ1,LMD) EnfamilReguline SimilacTotalComfort GerberExtensiveHA Earth’sBestOrganicGentle GerberGentle Similac ProSensitive EnfamilGentlease PlumOrganicsGentle Similac SensitiveNonͲGMO SimilacSensitive PlumOrganicsOrganicHappyPremiumBaby OrganicStage1 Similac forSpitUpNonͲGMO SimilacTotalComfort EnfamilProsobee EnfamilAR HappyBabyOrganicStage2 KirklandInfant GerberGentle SimilacIsomil SimilacforSpitUp HappyBabyOrganicSensitive GerberSoy EnfamilPremiumNewborn PureBlissbySimilac EnfamilProsobee EnfamilAR HonestCo.OrganicPremiumInfant EnfamilPremiumInfant PlumOrganicsOrganicPremium Earth’sBestOrganicDairy EnfamilInfant EnfamilEnspire Similac ProAdvance SimilacIsomil SimilacforSpitUp SimilacAdvance SimilacOrganic Similac AdvanceNonͲGMO GerberSoy Earth’sBestOrganicSensitivity EnfamilInfant EnfamilEnspire Similac forDiarrhea HonestCo.OrganicSensitive EnfamilNeuroPro Infant Similac forSupplementationNonͲGMO SimilacAdvance SimilacOrganic InfantFormulas InfantFormulas Forfussiness orgas,dairy protein somewhat brokendown, lowlactose

Soy Rice based starch thickened, EnfamilGentlease Dairy Niche, calories SimilacSensitive based, Dairy balanced SimilacTotalComfort standard based GerberGentle formulas EnfamilProsobee EnfamilAR EnfamilProsobee EnfamilAR SimilacIsomil SimilacforSpitUp SimilacIsomil SimilacforSpitUp GerberSoy GerberSoy EnfamilInfant EnfamilEnspire EnfamilInfant EnfamilEnspire SimilacAdvance SimilacOrganic SimilacAdvance SimilacOrganic

Infant Proteinneverbuilt, InfantFormulas Dairy Elecare Infant hypoallergenic,very proteinfullybroken Formulas Neocate Infant specialized down,fatcontent Puramino Infant(0Ͳ24mos) adjustedformoreMCT Alfamino Infant EnfamilPregestimil EnfamilPregestimil EnfamilNutramigen EnfamilNutramigen SimilacAlimentum Dairyproteinfullybroken SimilacAlimentum GerberExtensiveHA down,canbeusedfor GerberExtensiveHA milkproteinintolerance EnfamilGentlease EnfamilGentlease SimilacSensitive SimilacSensitive SimilacTotalComfort SimilacTotalComfort GerberGentle GerberGentle

EnfamilProsobee EnfamilAR EnfamilProsobee EnfamilAR SimilacIsomil SimilacforSpitUp SimilacIsomil SimilacforSpitUp GerberSoy GerberSoy EnfamilInfant EnfamilEnspire EnfamilInfant EnfamilEnspire SimilacAdvance SimilacOrganic SimilacAdvance SimilacOrganic

InfantFormulas Elecare Infant InappropriateInfantMilks Neocate Infant Otherspecializedformulas: Puramino Infant(0Ͳ24mos) EnfamilPrematureandEnfaCare Alfamino Infant SimilacSpecialCareandNeosure Enfaport • Friend’sbreastmilkorCraigslistbreastmilk EnfamilPregestimil Similac PM60/40 RossCarbohydrateFreeSoy • Goatmilk EnfamilNutramigen Metabolicformulas(examples: SimilacAlimentum Calcilo XD,PhenexͲ1,LMD) • Homemade“infantformulas” GerberExtensiveHA • Milkalternatives EnfamilGentlease SimilacSensitive SimilacTotalComfort GerberGentle

EnfamilProsobee EnfamilAR SimilacIsomil SimilacforSpitUp GerberSoy EnfamilInfant EnfamilEnspire SimilacAdvance SimilacOrganic OhDr.Google… Aboutgoatmilk…

• Goatmilkismostsimilarincompositiontocow’smilk • GoatmilkisNOT likebreastmilk • Goatmilkisnotsafeforanybaby,butespeciallynotforcow’smilk proteinintolerant/sensitivebabies • HomemadeformulasusinggoatmilkareNOTsafeornutritionally complete • Rawgoatmilkcancontaindangerousbacteria,includingE.Coli, Salmonella,Listeria,Campylobacter • Ifaninfantisongoatmilk,counselaboutthedangersandsend referraltoRegisteredDietitian

NutritionContentComparison NutritionContentComparison

• RecommendIntakeforAge:1.6Ͳ2.2g/kg/dayprotein,200Ͳ260mg/day Per100calories Breastmilk StandardInfant GoatMilk ofcalcium,65Ͳ80μg/dayoffolate,30Ͳ75mg/dayofmagnesium,400Ͳ Formula 700mg/dayofpotassium,and120Ͳ370mgofsodium Caloriesperounce 20 20 21 • Ifbabydrinks800caloriesperday: Protein 1.47g 2g 5.16g Breastmilk StandardInfant GoatMilk Calcium 46mg 78mg 194mg Formula Folate 7μg16μg1μg Protein 12g 16g 41g ~3xmore Magnesium 4mg 8mg 20mg Calcium 368mg 624mg 1,552mg ~4xmore Potassium 73mg 108mg 296mg Folate 56μg 128μg8μg Only12%ofneed Sodium 24mg 27mg 72mg Magnesium 32mg 64mg 160mg ~5xmore Potassium 584mg 864mg 2,368mg ~4xmore Sodium 192mg 216mg 576mg ~3xmore

InternationalFormulas PediatricFormulas

• HiPP,Holle,etc arepopular • Oralsupplementsortubefeeds • Unabletorecommendedat thistime • Completenutritionsource • Perarticle:“Thepotentialdangersare numerous.Childrencanfallillorbecome • Mostformulasare30calorieperounceor45calorieperounce malnourishedifparentsinadvertentlyusean incorrectformulaͲtoͲwaterratio;unofficial • Mainformulacompanies:AbbottandNestle formulavendorsmaynotstorethepowdered formulaproperly,raisingthepossibilityof • Blendedtubefeedingproductsaregaininginpopularity bacterialcontamination,productdeterioration orlossinnutrientdensity;thereisnosystemin placetonotifyconsumersintheUnitedStatesif anyoftheseformulasarerecalled;andwhile manyEuropeanformulascontainthenutrients Source:https://parenting.nytimes.com/feeding/europeanͲbabyͲformula requiredintheUnitedStates,somedonot.In addition,parentsintheUnitedStatesmaynot realizethatEuropeanformulaslabeled hypoallergenicaren’tmeantforchildrenwith cow’smilkallergies.” PediatricFormulas Thankfully Notincludedare“toddlerbeverages” Elecare Jr Elecare Jr ormetabolicformulas Neocate Jr Neocate Jr Neocate Splash onlyexpands Neocate Splash Puramino Jr alittlemore… Puramino Jr Alfamino Jr Alfamino Jr Otherspecializedformulas: Portagen KetoCal KetoVie Peptamen Jr(1.0and1.5) Peptamen Jr(1.0and1.5) RCF PediasurePeptide(1.0and1.5) PediasurePeptide(1.0and1.5) 1.0comesinunflavored, vanilla,strawberry 1.5comesinvanilla

Blendedtubefeedingproducts: Pediasurepowder Blendedtubefeedingproducts: SoyBrightBeginnings Compleat PediatricOrganicBlends PediasurewithFiber SoyBrightBeginnings Compleat PediatricOrganicBlends Nourish PediasureSidekicks Pediasure1.5withFiber Nourish KateFarms PediasureEnteral KateFarms NutrenJr BoostKidEssentials1.5 Compleat Pediatric PediasureEnteralwithFiber BoostKidEssentials1.5 Compleat Pediatric Pediasure Pediasure1.5 PediasureHarvest NutrenJr Pediasure1.5 PediasureHarvest RealFoodBlends* Pediasure RealFoodBlends*

Pediatric PediatricFormulElecareas Jr Elecare Jr Neocate Jr Formulas NeocateDairy Jr Neocate Splash Neocateprotein Splash Puramino Jr Puraminobroken Jr Alfamino Jr Alfaminodown Jr

Food based, Peptamen Jr(1.0and1.5) mostall Peptamen Jr(1.0and1.5) PediasurePeptideSoy (1.0and1.5) nutritionally PediasurePeptide(1.0and1.5) based complete Milk protein, Milk standard protein, higher formula Blendedtubefeedingproducts: Blendedtubefeedingproducts: calorie SoyBrightBeginnings Compleat PediatricOrganicBlends SoyBrightBeginnings Compleat PediatricOrganicBlends Nourish Nourish KateFarms KateFarms NutrenJr BoostKidEssentials1.5 Compleat Pediatric NutrenJr BoostKidEssentials1.5 Compleat Pediatric Pediasure Pediasure1.5 PediasureHarvest Pediasure Pediasure1.5 PediasureHarvest RealFoodBlends* RealFoodBlends*

Pediatric PediatricFormulas Elecare Jr Proteinneverbuilt, Elecare Jr Formulas Neocate Jr hypoallergenic,very Neocate Jr Neocate Splash specialized Neocate Splash Puramino Jr Puramino Jr Alfamino Jr Alfamino Jr

Peptamen Jr(1.0and1.5) Peptamen Jr(1.0and1.5) PediasurePeptide(1.0and1.5) PediasurePeptide(1.0and1.5)

Blendedtubefeedingproducts: Blendedtubefeedingproducts: SoyBrightBeginnings Compleat PediatricOrganicBlends SoyBrightBeginnings Compleat PediatricOrganicBlends Nourish Nourish KateFarms KateFarms NutrenJr BoostKidEssentials1.5 Compleat Pediatric NutrenJr BoostKidEssentials1.5 Compleat Pediatric Pediasure Pediasure1.5 PediasureHarvest Pediasure Pediasure1.5 PediasureHarvest RealFoodBlends* RealFoodBlends* Vitamins DietarySupplementRegulation

• Notalldietsarenutritionallycomplete • DietarySupplementHealthandEducationActof1994(DSHEA) • Malnutritioncancomeinmanyforms • Manufacturersanddistributorsprohibitedfrommarketing • Vitaminsupplementsaresometimesneeded adulteratedormisbrandedproducts • Limiteddietsduetopickyeating,medicalconditions,choice • Manufacturersanddistributorsareresponsibleforevaluatingthesafety andlabelingoftheirproducts • Conditionsthatcausemalabsorption • Geography • FDAwilltakeactionagainstadulteratedormisbrandeddietary • Increasednutrientneeds,metabolicconditions supplementsafteritreachesthemarket

Source:https://www.fda.gov/food/dietaryͲsupplements

Source: https://www.fennvilledl.michlibra ry.org/fennvilleͲfriends/sunͲ clipartͲfreeͲclipͲartͲimagesͲ 3.png/image_view_fullscreen VitaminSupplements VitaminD

• Ensurethatit’sageappropriate • Importantforcalciumabsorptionandbonemineralization • Notexcessive • Naturallyinveryfewfoods • Ironornoiron? • Breastfedinfantsrequire400internationalunitsdailyofvitaminD • Supplementspecificvitaminsbasedonlabvalues • FormulafedinfantsmayneedadditionalvitaminDdependingonvolume • VitaminD(25HDVitaminD) offormulaconsumed • Iron(CBC,Ironpanel,ferritin) • Olderchildren,vitaminDshouldbesupplementedbasedonlabvalues • Deficientvs.insufficient • Ageofpatient • Ergocalciferol (D2)orCholecalciferol(D3) • Rechecklabafter2Ͳ3mosofsupplementing

Iron EliminationDiets

• Importantforformationofhemoglobinandotherbloodandmuscle • Manypeopleareoneliminationdiets proteinsaswellasenzymes • Personalchoicevs.experiencewithfoodvs.medicaldiagnosis • Foodsources: • Thesearenotwithoutrisks • Heme:beef,poultry,shrimp,eggs • • NonͲheme:instantoatmeal,kidneybeans,tofu,spinach Dietiseasytochangeonown,butshouldbeguidedtoensure adequacy • IronabsorptionisincreasedwithvitaminC • Counselonsubstitutions • Calciumcandecreaseironabsorption • Ironbeconstipating,changestoolcolor • Supplementationbasedonlabvalues

Source:http://getdrawings.com/m anchesterͲunitedͲclipart Foods Mainnutrients Cow’smilk Protein,calcium,magnesium, phosphorus,vitaminsA,B6,B12, CaseStudy D,riboflavin,pantothenicacid(iodineinsomecountries) Soy Protein,calcium,phosphorus,magnesium,iron,zinc,thiamin, • 14yoboypresentswithfatigue riboflavin,vitaminB6,folate • Overallhealthyandwellnourishedpergrowthcharts Eggs Protein,iron,selenium,biotin, vitaminA,B12,pantothenicacid, • Pickyeater folate,riboflavin Wheat Carbohydrate,zinc,selenium,thiamin,niacin,riboflavin,folic • BloodtestsfoundmacrocyticanemiaandlowvitaminB12.No acid,iron,magnesium,dietaryfiber antibodiestointrinsicfactorortissuetransglutaminase Peanut/treenut Protein,selenium,zinc,manganese, magnesium,niacin, • GivenvitaminB12injectionsand“dietaryadvice” phosphorus,vitaminsE,B12,alphalinolenicacid,linoleicacid Fish/shellfish Protein,iodine, zinc,phosphorus,selenium,niacin Fattyfish:vitaminsA,D,omegaͲ3fattyacids

Groetch etal,2017 Harrisonetal,2019

CaseStudy Result ReferenceRange Hemoglobin, g/L 148 130Ͳ160 • Now15yodevelopedhearinglossfollowedbyvisionsymptoms Meancorpuscular volume,fL 100.4 83Ͳ100 • MRIandophthalmologyexamwerenormal Platelets, x10^9cells/L 250 150Ͳ450 • 2yrslater:progressivevisionlossfoundtohaveopticneuropathywith 20/200vision Creatinine,mg/dL 0.5 0.7Ͳ1.2 • NeurologicexamandanotherMRIwerenormal Totalbilirubin,mg/dL 1.3 <1.2 • Genetictests,GIscope/biospies,Fibroscan wereallnormal Alk Phos ʅkat/L 4.2 1Ͳ2.7 Totalprotein,g/L 74 60Ͳ80 Adjustedcalcium,mmol/L 2.23 2.2Ͳ2.6 CRP,nmol/L <9.5 <57.1

Harrisonetal,2019 Harrisonetal,2019

Result ReferenceRange VitaminA,ʅmol/L 0.8 0.8Ͳ2.2 CaseStudy VitaminE,ʅmol/L 14.3 10.2Ͳ39 25HDVitamin D,nmol/L 10 >50 • Persistentmacrocytosis withnormalferritin,folate,andB12 VitaminB12,pmol/L 135 132.8Ͳ664 Ferritin,pmol/L 90.8 74.2Ͳ898.9 • HomocysteineandMMAlevelselevatedindicatingfunctionalB12 Serumfolate,nmol/L 9.2 5.7Ͳ44.3 deficiency,whichledtonutritionalevaluation Zinc,ʅmol/L 26.8 11Ͳ23 • Noalcoholorsmoking Copper,ʅmol/L 9.8 12Ͳ23 • Growthwasgood Selenium,ʅmol/L 0.55 0.59Ͳ1.65 • Sinceelementaryschoolhasavoidedfoodswithcertaintextures Manganese,nmol/L 91.8 72.8Ͳ218.5 • WilleatFrenchfries,chips,whitebread,hamlunchmeat,andsausage Homocysteine,ʅmol/L 47.1 2Ͳ14.3 • Methylmalonic acid(urine), 7.2 0.7Ͳ3.2 Didn’tfinishpreviousvitaminB12injections ʅmol/mmol

Harrisonetal,2019 Harrisonetal,2019 CaseStudy References

• Providedsupplementsandreferredtomentalhealthforaneating • Groetch M,Verter C,Skypala I,VliegͲBoerstra B,Grimshaw K,DurbanR,etal.DietaryTherapyand NutritionManagementofEosinophilicEsophagitis:AWorkGroupReportoftheAmerican disorder AcademyofAllergy,Asthma,andImmunology.JAllergyClin Immunol Mar/Apr2017;5(2),312Ͳ • Visionstabilized,butdidnotimprove 324.e29 • HarrisonR,WarburtonV,LuxA,Atan D.Observation:CaseReport:BlindnessCausedbyJunkFood • Delayeddiagnosispossiblyd/ttreatedvitaminB12deficiency. Diet.AofInternalMedicineSept2019 Homocysteineandmethylmalonic acidaremoresensitiveindicators offunctionalvitaminB12deficiency • BMIisnottheonlyindicatorofmalnutrition

Harrisonetal,2019

Questions?

Thankyou! Disclosures • Ihavenothingtodisclose

PediatricChronicPain:TipsforPrimary CareProvidersforPreventionand Management

DATE: October 17, 2019 PRESENTED BY: Amy Holley PhD, Associate Professor of Pediatrics & Psychiatry

PresentationOverview Disclosures • Ihavenothingtodisclose • Describeprevalenceand impactofpediatricpain ExceptI’mgoingtospendthenexthour • Presentkeyfactorsthat talkingaboutpain… impactpainoutcomes • Describestrategies providerscanuseto bestsupportkidsand theirparents

Puttingafaceonthe NumericRatingScale Fromthemedicalrecord… PediatricChronicPainisCommon

Physicalfindingsdo Unremarkableexam.Iwonderif • 11Ͳ38%ofyouth notexplainher thereissomesomatization reportofpain • 5Ͳ10%havemoderateͲ severe disability • Prevalenceincreases withage; peak14Ͳ15yrs • Girls >thanboys

Assessforpossible psychogenic Painoutofproportion component withimaging Kingetal,Pain,2011

ImpactonChildren: PrevalenceofBackandNeckPainbyAge

Mood Missed Social Physical Sleep and School Function Function Problems Anxiety

AndParents:

Stressful Missed Financial Emotional Changesin interactions work Stress Distress familyroles withchild

Hakala etal.,BMJ,2002

MentalHealthComorbidity $19.5BILLION 44%ofyouthadmittedforchronic painhadmental healthdiagnosis: • mooddisorders(28%) • anxietydisorders(18%) • conversionandsomatizationdisorders(6%) 26%ofgeneralpediatricchronicpainsample have mentalhealthdiagnosis • Increasedriskfor: • anxietydisorders(OR2.42) • eatingdisorders(OR2.63) • depressivedisorders(OR2.32) • substanceusedisorders(OR2.11) 12 Coffelt etal,2013,Tegrethoff etal.,2015 ImpactExtendsintoAdulthood ParentandFamilyContext

Generalhealth Pain processing 1/6adultpainpatientsreportchronic Parentpain Pain history Memories paininchildhood Mood • Havingpediatricpainassociatedwithhigher Fearofpain Catastrophizing Acute disability Pain Responsesto School childpain Function

Anxiety Childhoodpainincreasesadultriskfor: Catastrophizing • Anxietydisorders(21.1vs.12.4%) Mood • Depressivedisorders(24.5vs.14.1%) Low physical • Lowerhouseholdincomeandhigherriskof activity Fearof Modelingofpain Pain unemployment andcoping • Opioidmisuse

Hassett etal.,2013;JPain,Noeletal.,2016;Pain,Groenwald,2019;JPain

DifferencesinPainResponses: ParentalChronicPainisCommon ParentswithandwithoutChronicPain =NUNGYGVGXKTZ]OZNINXUTOIVGOT% 25

• ?U[ZN]OZNINXUTOIVGOT#狨狥 20 • .KGRZN__U[ZN#狤狣 15 • ?U[ZNYKKQOTMIGXKLUXGI[ZK S[YI[RUYQKRKZGRVGOT#狨狢 10 • ?U[ZN]OZN0/'#狧狫 TUTGXZNXOZOY 5 VGOTOTVGXKTZY

0 ParentCatastrophizingaboutChildPain ParentProtectiveness ParentCP+ ParentCPͲ

6OOXG 6[RR[QGZ狤狢狢狨!)GSVUYKZGR狤狢狢狩! 9INGTHKXM KZGR狤狢狢狣!)RKSKTZOKZGR Wilson&Fales,Clin JPain,2015

QualitativeResults:ImpactofPainonParenting

PercentReporting

ReducedParentalInvolvement 86

Worrychildwilldeveloppain 75

Reducedchildphysicalactivity 63

Increasedimpatience 40

GuiltandselfͲdisappointment 37

Moreinconsistentdiscipline 28

0 102030405060708090100 PercentReporting

17 18 Wilson&Fales,Clin JPain,2015 TheIdeal:MultidisciplinaryModel Whatcanwe Physician do?

Patient Physical Nurse & Family Therapist

Psychologist

PsychologicalInterventionsare TheBarrierstoCare… Effective • Limitedavailabilityof pediatricpainspecialists

Psychologist • Numberofclinics/waitlists

• Transportation

• Insurance

• Providerunsurewhereto refer

ActivityEngagement ActivityEngagement Duration  Duration  Pain Pain

23 24 Whatcanyoudo? ExplainPainNeurobiology

Thebraincansense painevenifimaging doesnotshowtissue damage.

“ExplainPain”,Butler&Moseley

Andthat…. UseAnalogies

Chronicpainislikeacaralarm Levelof Levelof Pain Harm Persistentpainislikeadoorbellthatgoeshaywire

Chronicpainisaproblemwiththesoftware Thereisnothingwrongwiththehardwareinthebody (e.g.bones,muscles,organs),butthesoftwarethat sendsmessagesthroughoutyoursystemhasaglitch

Coakley&Schechter,PediatricPainLetter;2013

AssessParentRisks/Supports RiskClassificationassociatedwith ChildDisabilityandParentBehaviors

PRISM N(%) PainͲ Pain Parent Protective Parent Risk related Intensity Distress Behavior Catastroph. Group disability Low 76(33.2%) 16.8(9.9) 5.9(1.9) 8.0(5.4) 23.8(7.5) 22.4(8.9) (0Ͳ3)

Moderate 66(28.8%) 24.3(10.5) 6.2(1.6) 14.4(5.9) 28.4(8.2) 28.7(10.2) (4Ͳ6)

High 87(38.0%) 27.4(10.3) 6.1(1.7) 18.1(6.1) 31.8(8.4) 35.9(11.8) (7Ͳ10

Simonsetal.,2018;Pain AssessChildRisks/Supports AssessChildRisks/Supports

Simonsetal.,2015;Pain Simonsetal.,2015;Pain

RecognizePainAnxiety RecognizePainAnxiety

Icannotdoactivities Icannotdoactivities thatmakemypain Mypainisnevergoingto thatmakemypain Mypainisnevergoingto worse getbetter worse getbetter

Ineedtocancelplans Ican’tkeepstop Ineedtocancelplans Ican’tkeepstop whenIaminpain thinkingaboutit whenIaminpain thinkingaboutit

PainCatastrophizing PainCatastrophizing FearandAvoidance FearandAvoidance

Chowetal.,2016;JPain,Zaleetal.,JPain2013

FearAvoidanceModel FearAvoidanceModel

Vlaeyan,J.W.S.&Linton,S.J.;2000,Pain • Fearofpainmattersevenintheacutepainperiod • Sodoparentfactors!

T1Predictor B SE ɴ T1Predictor B ɴ Step2:PainIntensity .81 .76 .14 Step2:ChildAge .17 .15 SleepQuality Ͳ 6.58 2.99 Ͳ.34* ChildSex .77 .17 DepressiveSymptoms .09 .14 .09 FractureStatus(yes/no) Ͳ .36 Ͳ .08 TraitCatastrophizing Ͳ .17 .27 Ͳ.12 RelationtoChild Ͳ 1.43 Ͳ .19 State Catastrophizing Ͳ .27 .19 Ͳ.18 ParentChronic Pain(yes/no) 1.06 .24* Fear ofPain .35 .13 .51** ParentSomaticSymptoms Ͳ .10 Ͳ .15 PainProtectiveness .76 .23* CPMIndex Ͳ.15 .08 .046 *p<.05 p<.05,**p<.01TotalModelR2 =.35,p<.001 Includescovariates:sex,age,ethnicity,BMI–alln.s.)

Holley et al., Clin J Pain, 2017 Clementietal.,Clin JPain;2019

SetTreatmentExpectationsEarly SetTreatmentExpectationsEarly • Functionmayimprovebeforepain • Treatmentmayhavemultiplecomponents

PainͲrelated Disability

Improvement maybegradual (painhasbeen presentfora longtime)

4flattires:medicationinflatesonlyone TreatmentSession# LynchͲJordanetal.Pain,2014

ExplainhowParentingaChildwith GiveParentsSpecificRecommendations ChronicPainCanbeCounterintuitive

Ineedtoletherrestsoshe Limitpaincheckins canrecoverfromher symptoms

Expectyourchildto Ineedtoaskherabout graduallyreturnto herpainsosheknows attendingschool howmuchIcareabout her

Rewardyourchildfor Itscrueltoexpectmydaughter activityengagement toengageinactivitiesuntilher painisgone KnowWhentoReferto BehavioralHealth Whoneedsbehavioralhealthinterventions? Highfearavoidanceimpactingreturnto activity

Parentswhoneedtoadditionalsupport implementingoperantstrategies

SchoolreͲentry/504plandevelopment

CoͲoccurringsleepproblems

Mentalhealthassessment/treatment

Sendyourpatientstous!

Youcansubmitonlinereferralsthroughourwebsite! (Googlesearch:“OHSUComfortAbility”) Thankyou!

We recommend thisbook!

https://www.amazon.com/WhenͲYourͲChildͲHurtsͲStrategies/dp/0300204655

QUESTIONS? Objectives CraniofacialMedicine: • Evaluatinginfantheads ClinicalPearlsandCommon • Understandingwhentoreferornottorefer Cases • Syndromerecognition EmilyGallagher,MD,MPH Doernbecher AnnualReviewandUpdate October17,2019

Evaluatinginfantheads Fontanelsize

• Headsize:whentoworry? • Childrenwithrapidlygrowingbrainsandnormalbonehavebig • Noterelationshiptoothergrowthparameters fontanels • Measureparent/siblingheadsizes • Hydrocephalus,benignmacrocephaly • Developmentalassessment • Childrenwithnormalbrainsandpoorbonegrowthhavebigfontanels • Fewmanagementguidelinesexist! • Hypothyroidism,cleidocranial dysplasia • Childrenwithpoorlygrowingbrainsandnormalbonehavesmall fontanels • Primarymicrocephaly,hypoxicbraininjury • Childrenwithnormalbrainsandrapidlygrowingbonehavesmall fontanels • Craniosynostosis,hyperthyroidism

12montholdboy Another12montholdboy Previouslyhealthygirl 2yearoldgirl,milddelays

Headsize:whentoworry? Mechanicsofheadshapedifferences

• Macrocephaly • Microcephaly • Intrinsic:calvarial development • Extrinsic:plagiocephaly • Associatedwithdelays • Hypoxicbirthinjury • Craniosynostosis:premature • TheEpidemic • Dysmorphicfeatures • CNSmalformation fusionofinfantsuture • Treatment:whenisit“necessary”? • Departingnormalgrowth • Inuteroexposure curve • Alcohol,drugs • Hydrocephalus • Syndromes • Noteparentalheadsize • Metabolicdisorder • Common: • Maternalorinfant • Benignfamilialmacrocephaly • Congenitalinfection • IncreasedextraͲaxialfluid

Deformationalplagiocephaly Mostimportantviewswhenexaminingahead • Deformationofthecalvaria fromextrinsicforces • Onsetcanbeprenatalorpostnatal • Prenatal:inuteromoldingorconstraint • Postnatal:usuallyheadpositionpreference • Naturalhistory • Prenatalonset:spontaneousimprovement • Postnatalonset:noticedat1Ͳ2months,worsensuntil5Ͳ6months Calvarial suturesandnormalclosure

Metopic • Notadisease • Emphasisonprevention SUTURE CLOSUREBEGINS • Parent’sdecision • Referralby6months $3500 Coronal Metopic 3Ͳ9months

Sagittal Sagittal 22years Coronal 24years Lambdoid Lambdoid 26years

JohnnyJumpUp Ergo Moby Bumbo Exersaucer Tummytime $20Ͳ30 $100 $50 $35 $50 $0

Singlesuturecraniosynostosis A

metopic coronal

B

sagittal lambdoid

A B A B AB A B

HorizontalSkullBase

NAMETHEDIAGNOSIS? Syndromeevaluationinpatientswithclefts

Howoftendopatientswithcleftlipand/orpalatehavesyndromesor A BC associatedmalformations?

Metopic Positional Sagittal synostosis plagiocephaly synostosis • CLP:15Ͳ25% • CP:50%

A B

A:Holoprosencephaly ABC • Hypotelorism,depressednasalbridge • Midlinecleftlipandpalate B:Midlinecleftisnevernormal • Pyriformaperturestenosis Midlineencephalocele • Singlecentralincisor RobinSequence Micrognathia Glossoptosis Upperairwayobstruction +/Ͳ cleftpalate

Sticklersyndrome ~30%ofchildrenwithRS

TP63GeneMutations • 1gene,6syndromes • Ectodermaldysplasia • Clefting • Sparsehair • Riskofhyperthermia • Coneteethorhypodontia Disclosures

• None

“Top Neurology Cases” i.e. Headache, etc. Doernbecher Annual Review

DATE: October 3, 2019 BY: Kaitlin Greene, MD Director, Pediatric Headache OHSU Department of Pediatrics, Division of Pediatric Neurology

Case1:Headache Outline: • Goals: • Case 1: Headache • Review indications for imaging in patient presenting with headache • Case 2: Seizure • Review diagnostic criteria for migraine and migraine with aura in children and adolescents • Case 3: Stroke • Outline approach to acute and preventive treatment of headaches • Be comfortable prescribing a triptan!

• Discussion and Questions!

33

Case1:Headache Case1:Headache

• What are the headaches like? • 13 year old girl presenting with worsening headaches • Location: Mostly front, sometime back, • When did headaches start? sometimes more on one side or the other • Six months ago Age 8 • Quality: Pressure (throbbing when severe) • Short (~1 hour), infrequent (<1x/month), typically triggered by • Severity: Usually moderate, at least 2/month illness or dehydration, improved with ibuprofen severe • Over the past two years, frequency gradually increased to • What are the associated features 2x/month, then 4x/month, then to 2x/week by about 6 months ago • “Sensory sensitivity”: Light, sound, smell • Nausea when severe • Sees “flashes of light” for a few seconds with more severe headaches Itsoktobeweird.com Case1:Headache Case1:HeadacheͲ Diagnosis

• PMH: None • What is the diagnosis? Migraine! With Aura? • Family history: • Mom with “stress headaches” (Getssensitivetolight/noise,hastoliedown) • BUT first have to answer two • Younger sister gets headaches when sick questions: 1. Are there any “red flags” to necessitate • Medications: further work up? • Ibuprofen 200 mg as needed for headache 2. Does she meet diagnostic criteria for • Exam: Wt 50 kg. Normal including fundoscopic migraine or migraine with aura based on exam. the International Classification of headache disorders, 3rd edition (ICHD-3)?

Case1:HeadacheͲ Diagnosis Case1:HeadacheͲ Diagnosis

• Are there any “red flags”/indications for additional work up? • Does she meet criteria for migraine without aura (1.1) based on the • “SSNOOPP” ICHD-3 1? • Systemic symptoms (i.e. fever, rash, neck stiffness) A. Ȳ5 attacks fulfilling criteria B-D • Secondary risk factors (i.e. medical co-morbidities, history of cancer, B. Headache attacks lasting 2-72 hours (untreated or unsuccessfully treated) immunosuppression) C. Headache has at least two of the following four characteristics • 1. Unilateral location (Moreoftenbilateralinchildren2) Neurologic signs or symptoms: focal symptoms or focal findings on 2. Pulsating quality exam 3. Moderate or severe intensity • Onset: sudden, abrupt, maximum at onset (“thunderclap”) 4. Aggravation by or causing avoidance of routine physical activity D. During headache at least one of the following: • Older patient: age >50 (OR younger patient: age <6) 1. Nausea and/or vomiting • Progression and Prior headache history: major change in frequency, 2 Photophobia and phonophobia (Canbeinferredfrombehavior) severity or clinical features, new headache type or pattern (<6 months E. Not better accounted for by another diagnosis headache history) Comment: “Migraine headache is usually frontotemporal. Occipital headache in children is rare and calls for diagnostic caution.”

Dodick Adv StudMed 2003 1ICHDͲ3Cephalalgia 2018;2deGrauwetal.,Headache 1999

Case1:HeadacheͲ Diagnosis Case1:HeadacheͲ Diagnosis

• What about occipital headaches? Is it • Of children newly referred to Neurology and Headache rare? Does it call for diagnostic caution? Clinics, 6-16% have occipital headache1, 3 • Children with occipital headache are more likely to get • Study1:432childrenintheEDfor • Study2:150childrenintheED scanned BUT not more likely to find anything wrong!2, 3 HA1 forHA2 • In children with solely occipital headache, 91% were scanned (RR 4.9, 1.2-21) • 18/277 withdischargediagnosis(6%) • 2/150(1.3%)hadoccipital • No significant difference in abnormal findings on MRI had“lifeͲthreateningheadache” headacheandboth hadbrain • 3/18occipital,15/18 unableto tumors localize • 2/150(1.3%)hadbraintumorsbut • 17/18 hadheadachesfor<2months didNOThaveoccipitalheadache • 18/18(100%)hadobjective • 4/4(100%)withbraintumorshad neurologicalsigns abnormalneurologic examinations

1 2 3 1Conicellaet.al.,Headache 2008;2LewisandQureshi,Headache,2000 deGrauwet.al.,Headache 1999, BearJet.al.,AANAbstract2014, EidlitzͲMarcuset.al.,PediatricNeurology2014 Case1:Headache Case1:HeadacheͲ Diagnosis • What about aura? “Flashes of light for a few seconds” 1.2 Migraine with aura1: A. At least two attacks B. Ȳ 1 of the following fully reversible symptoms: • Visual, sensory, speech/language, motor, brainstem, retinal C. At least 3/6 characteristics: www.ohsu.edu • Aura symptom spreads gradually over Ȳ5 minutes • Occipital headache: Does it call for diagnostic caution? • Ȳ2 aura symptoms occur in succession • Depends on the context! • each individual aura symptom lasts 5-60 minutes • Ȳ 1 aura symptom is unilateral • In children presenting to the ED (or clinic) with NEW headache and • Ȳ 1aura symptom is “positive” ABNORMAL exam, caution is warranted regardless of location of headache • aura is accompanied, or followed within 60 minutes, by • BUT in a child with a normal neurologic exam and a headache phenotype headache consistent with migraine, occipital head pain location alone is not • Why does it matter? • Women with migraine with aura have a 2-fold increased necessarily associated with pathology risk of stroke more w/high-dose estrogen OCPs and smoking

1ICHDͲ3Cephalalgia 2018

Case1:Headache– Treatment Case1:Headache–AcuteTreatment

• Acute treatment: Decrease the • First-line: NSAIDs or Tylenol duration and severity of the attack • Acetaminophen and ibuprofen both studied • Inadequate acute treatment 1 optimization associated with a higher down to age 4 risk of developing chronic migraine • Both superior to placebo within one year in adults1 • Ibuprofen 2x more likely to abort migraine at 2h • Consider longer-acting NSAID • Naproxen less likely to cause medication overuse • Preventive treatment: Decrease the headache and may have some preventive frequency of attacks over time benefit2,3 • Consider when bothersome headache is occurring >1 day per week or >4 days per month iStock.com

LiptonNeurology2015 1Hamalainen Neurology1997;2LiptonNeurology2015;3CadyHeadache2014

Case1:Headache–AcuteTreatment Case1:Headache–AcuteTreatment

• Four triptans now FDA-approved for pediatric migraine • Second-line: Triptans (5-HT1B/1D agonists) • Triptan Forms Dose Approval Generally very safe and well-tolerated in children with healthy <40kg>40kg vessels! • Contraindications: Almotriptan PO 6.25mg 12.5mg 12Ͳ17yo (2009) • Underlying intracranial or cardiac vascular disease (including moyamoya, Rizatriptan MLT,tab 5mg 10mg 6Ͳ17yo (2011) prior stroke, ischemic heart disease) • Uncontrolled hypertension Sumatriptan/naproxen PO 10/60mg – 85/500mg 12Ͳ17 yo (2015) • WPW (sumatriptan (Sumatriptan alone:25mg(<40kg)–50 • Specific aura types (hemiplegic migraine and brainstem aura) alsoNSandSQ) mg(>40mg)

Zolmitriptan NS 2.5mg 5mg 12Ͳ17yo (2015)

FDA.gov;Lewiset.al.,Pediatrics2007;Hoet.al.,Cephalalgia 2012;Hewittet.al.,Headache2013;Linderet.al.,Headache2008 Case1:Headache–AcuteTreatment Case1:Headache–PreventiveTreatment

• • Triptan pearls: Topiramate is the only FDA-approved preventive treatment in • Better to take early when pain is MILD (53% pain free children based on two positive RCTs at 2h)1 • What about CHAMP? • BUT okay to take when mod/sev (38% pain free at 2h) • Take with naproxen! • Why?? • Higher 2h pain-free rate, lower 24h recurrence (adults)2 • Very high placebo-response • No need to re-dose rate, perhaps related to active co-interventions • Safe but no evidence for better efficacy • Frequent visits with providers • Limit to <10 days per month to decrease risk of 3 • Optimization of acute medication overuse treatment • Choose the formulation that makes the most sense! • Patients with very refractory • PO, MLT, NS, SQ migraine or continuous headache excluded FindingsfromCHAMP(PowersetalNEJM2017) 1Goadsby,Cephalalgia,2008;2Brandes et.al.,JAMA2007;3DeFelice AnnNeurol 2010

Case1:Headache Case1:HeadacheͲ Treatment

• Recommend discussing lifestyle modification and discussion of modifiable risk factors (Level B) • Recommend discussion of role or preventive treatments in those with frequent headaches, migraine-related disability and medication overuse (Level B) • Recommend informing families of placebo response rates in trials and that majority of preventives are not superior to placebo, with shared decision making about pros/cons of short-term treatment trials (Level B)

Case1:Headache–PreventiveTreatment Case1:Headache–PreventiveTreatment

• First-line: Lifestyle modifications! • Headachereliefguide.com • “Regularity” seems to be key – regular exercise, regular meals, regular fluid intake, regular sleep • Among teens, significantly higher odds of presenting to the ED with headache in Jan and Sept

Kedia et.al.,Cephalalgia 2013;CDC2018 Case1:Headache–PreventiveTreatment Case1:Headache–PreventiveTreatment

• Second-line: Over-the-counter • medications/supplements Third-line: Prescription • Riboflavin 200 mg BID (<40 kg: 100 mg BID) medications • Two negative RCT (very high placebo response rates)1,2 • Should discuss evidence • Recent positive placebo-controlled RCT3 for amitriptyline, • Coenzyme Q10 100 mg BID (1-3 mg/kg/d) topiramate, propranolol • One study in children with low CoQ10 showed decreased HA • Should have extended frequency with normalization of CoQ10 levels6 discussion of risks of 7 • One RCT in children with trend toward efficacy medication including • Melatonin 3 mg QHS (<40 kg: 1-2 mg QHS) concern for • One RCT in adolescents showed safety w/trend toward efficacy4 5 teratogenicity of valproic • Uncontrolled studies showing decreased frequency acid and topiramate

1BruijnetalCephalalgia 2010;2MacLennanJChildNeurol 2009;3TalebianNutrients2018;4MianoNeuralSci 2008;5FallahCurr DrugSaf 2015;6HersheyHeadache2007;7SlaterCephalalgia 2011 Oskoui etalNeurology2019

Case1:Headache–PreventiveTreatment Case1:Headache–PreventiveTreatment

• What about the new anti-CGRP monoclonal antibodies? • Anti-CGRP monoclonal antibodies: expert consensus for use in adolescents • Vasodilatory neuropeptide • Role in pathogenesis of migraine • Higher serum and saliva levels during migraine attacks • Levels decreased with triptan- induced pain relief • Infusion induces migraine in migraneurs only

RussoAnnu RevPharmacol Toxicol 2015 Szperka etal,Headache2018

Case1:HeadachePrevention Case1:Headache–PreventiveTreatment

• Cognitive Behavioral Therapy • Fourth-line: • • In children and adolescents age 10-17, Nerve blocks those who received CBT + amitriptyline • Botox? vs Headache Education (“placebo”) + • Insufficient evidence per amitriptyline had greater: practice parameter • Reduction in headache frequency (SMD 0.48 • Devices: TMS, Cefaly [95% CI 0.14-0.82] • Admission • Likelihood of Ȳ50% reduction in headache • DHE, thorazine, valproic frequency (RR 1.70 [95% CI 1.27-2.56] acid • Reduction in headache-related disability (SMD 0.43 [95% CI 0.09-0.77])

Powersetal,JAMA2013 Case1:Headache Case1:Headache–TakeͲAwayPoints

• Back to our case… • “SSNOOPP” pneumonic for imaging indications • Acute plan: • ICHD-3: Excellent source for diagnostic criteria • Find a quiet place to rest • Migraine with aura: symptoms evolve/spread over ~5 min • Mild/mod headache: Take naproxen 440 mg as needed up to 4 days/week and last 5-60 minutes • Mod/sev headache: Take sumatriptan 50 mg with naproxen 440 mg. Limit sumatriptan to 9 days per month. • Acute treatment: NSAID +/- triptan (safe and approved in • Preventive plan: kids! With choice of formulation!) • Regular sleep, regular hydration, regular exercise, regular meals! • Preventive treatment: First, do no harm! • Take riboflavin 200 mg twice a day. This will take at least 8 weeks to see • Emphasize on lifestyle and modification of risk factors benefit. • Think about CBT • New practice guideline from AAN/AHS in print • Amitriptyline, topiramate and propranolol may be considered

Case2:Seizure Case2:Seizure

• • CC: 4.5 yo boy with no significant PMH presents with three “spells” Goals: with alteration of consciousness over 10 days • Identify features of spells concerning for seizure • Description of spells: • Review differential for new onset seizures in childhood • Wakes from sleep and able to walk into mom’s room • • Behavioral arrest, unable to speak, appears “out of it”, doesn’t respond to Review general categorization of seizures mom’s voice • Outline steps of work up in a child with new concern for seizures • On one occasion, made “gurgling sounds” in throat • No unusual movements of face or body, no LOC, no incontinence, no tongue • Discuss treatment indications and natural history biting, no post-ictal state • Duration: 45 seconds • Any other symptoms? • Teacher has noticed some “staring spells” or “spacing out” episodes over the past 2-3 months • Has been more temperamental over the past 6 months (talking back, acting out)

Case2:Seizure Case2:SeizureͲ Diagnosis

• PMH: None • Differential diagnosis: • Family history: First cousin with childhood epilepsy • Seizure • TIA • Medications: None • Parasomnia • Exposures: None • Cardiogenic – arrhythmia, presyncope • No recent illness • Behavioral • No known ingestions or possible ingestions

• Exam: Normal between attacks Case2:SeizureͲ Diagnosis Case2:SeizureͲ Diagnosis

• What features are concerning for seizure? • Differential etiologies for new-onset seizures in children • Recurrent, stereotyped • Structural lesions • Brief duration • Trauma • Occurring out of sleep (or in sleep transition) • “Behavioral arrest” • Vascular event (Ischemic or hemorrhagic stroke) • Commonly asked “seizure features” • Infection (Meningitis, encephalitis) • Tongue biting (lateral) • Toxic (Ingestions, medication overdose) • 100% specificity, 30% sensitivity for seizure vs NES1 • Metabolic (Electrolyte disturbance, IEM) • Urinary incontinence • Remote neurologic injury or abnormal brain development • 2 57% specificity, 38% sensitivity in differentiating syncope vs NES vs seizure • Primary epilepsy • Ictal eye closure • 80% specificity, 58% sensitive for PNES3

1BrigoetalEpilepsyBehav 2012;2BrigoetalSeizure2012;Brigo etalSeizure2013

Case2:SeizureͲ Etiology Case2:SeizureͲ Evaluation

• Laboratory evaluation and toxicology for patients seen in ED • Types of seizures with first time seizure • Generalized seizures: Impaired awareness, bilateral motor • Head imaging symptoms • Emergent if concern for acute focal onset based on history, exam or EEG – • Focal (“partial”) seizures: with our without impairment of rule out hemorrhage or ischemia awareness • Outpatient MRI unless EEG confirms primary generalized epilepsy • • LP if febrile, concern for infection, not returning to baseline or <6 Motor: may have spread (“Jacksonian march”), versive movement (head or 1 eye deviation), vocalization or speech arrest (involvement of muscles of months of age phonation) • Sensory: Paresthesias, distortion, olfactory or gustatory, auditory, visual • Autonomic: “Rising” sensation, sweating, pupil changes

Arimgsas.com.au UofWashington Aboutkidshealth.ca AmericanAcademyofNeurology

Case2:SeizureͲ Evaluation Case2:SeizureͲ Prognosis

• EEG recommended for all patients • Recurrence risk presenting with new-onset seizures • All-comers: 42% recurrence • When to get EEG? • 88% of those in the first 2 years • Immediately post-ictal (<24 hours): can show • Awake with normal EEG: 19% generalized or focal slowing • Out of sleep with normal EEG: 37% • If otherwise well and back to baseline, can be done as an outpatient • Out of sleep with abnormal EEG: 63% • Consider more urgent EEG if not back to • >1 seizure in 24 hours: 41% baseline or concern for subclinical seizure • May provide insight into the etiology of seizure • Focal features OR characteristic findings of childhood epilepsy syndromes

Neupsykey.com Shinnar Pediatrics 1996 Case2:Seizure– Treatment Case2:Seizure– Treatment

• “Seizure safety” • AAN guideline: Treatment with AED after first seizure may • Caution around water, do not bathe or swim alone decrease risk of second seizure but dose not improve long- 1 • No rock climbing or sky diving term prognosis • Wear a helmet! • Recommend treatment after second afebrile seizure >24 • Consider rescue medication if seizure was hours apart prolonged or child was endangered • Focal seizures: Oxcarbazepine/carbamazepine, levetiracetam • First time seizure presenting in status has higher • Generalized seizures: Levetiracetam, topiramate, lamotrigine, likelihood of recurring with status valproic acid, zonisamide • Intranasal or buccal midazolam 0.2 mg/kg, max 10 mg • Rectal diazepam for younger children • Give instructions to call 9-1-1 with first administration

1Hirtz et al, American Academy of Neurology 2003, 2SpecchioandBeghi CNSDrugs2012

Case2:SeizureͲ Treatment Case2:Seizure

• Duration of treatment: goal 2 years seizure-free2 • Back to our patient… • 66-96% likelihood of seizure freedom at 1 year, 61-91% at 2 years • Focal seizure by description • Higher risk of relapse: adolescent onset, underlying neurologic • EEG showed significant left-sided epileptiform abnormalities and disorder, abnormal EEG suggestion of underlying structural lesion

Case2:Seizure Case2:Seizure

• Back to our patient… • Back to our patient…. • MRI brain showed left parieto- • Started on levetiracetam on admission but switched to occipital cortical dysplasia oxcarbazepine prior to discharge • Discussed future possibility of surgical intervention given focal cortical dysplasia

Pedsradiology.com Case2:Seizure–TakeͲAwayPoints Case3:Stroke

• Spell features concerning for seizure: • Goals: • Stereotyped, behavioral arrest, occurring at sleep transition • Tongue biting>eye closure, incontinence to differentiate from NES and syncope • Triage of acute onset of neurologic symptoms • Work-up of first-time seizure in Urgent Care/ED • Review basics of imaging techniques for stroke in children • Labs for all • Review of treatment protocol for acute stroke at OHSU • Head imaging if acute focal onset or abnormal exam (otherwise outpatient) • LP if concern for infection or <12 months • Recognize common presenting symptoms of stroke in children • EEG indications: all patients with new seizures • Review risk factors for stroke in children • Can be done outpatient unless not returning to baseline • Review secondary work-up and stroke prevention in children • Helps guide further work up • Helps predict recurrence risk • Rescue medication for those presenting in status • Initiation of AED after 2nd afebrile seizure

Case3:Stroke Case3Ͳ Stroke

• 8 year old previously healthy boy presents to the ED with • Differential acute onset of new headache, possible left facial droop and • Stroke – ischemic or hemorrhagic weakness 6 hours prior to arrival • Seizure • PMH: Unimmunized, limited primary care • Meningitis/encephalitis with focal infection • ROS: Fatigue, behavior changes and decreased PO for one • Migraine week • Tumor or other lesion with acute change (hemorrhage) • FH: No stroke, seizures, clotting or bleeding problems • What next? • Exam: T 101F, VSS, follows commands on the R, R gaze preference, L facial droop, L upper and lower extremity weakness

Case3:Stroke Case3:Stroke–WorkͲUp

• Labs: CBC, BMP, coags, type and screen, pregnancy test • “Supportive care” while awaiting imaging • Bed rest with HOB flat • IV fluids • Neurochecks • Normothermia – avoid fever!!! • Normotension • Fluids for hypotension • Labetelol for hypertension • Consider AED if concern for seizure Case3:Stroke–WorkͲUP Case3:Stroke–AcuteTreatment

CT/CTA/CTP MRI/MRA • Very sensitive for • Very sensitive blood for acute • Low sensitivity for ischemia acute ischemia within minutes, • May show up to 7-10 days hypodensity after 6-12 hours • Sensitive for • Will show vessel blood occlusion • Will show • Usually fastest to vessel get! Consultqd.Cleavelandclinic.org occlusion

Case3:Stroke–AcuteTreatment Case3:Stroke– DefinitionsandEpidemiology

• Why the concern about timing? • Stroke: “Acute onset neurological sign or symptom attributed • Goal is to reperfuse the to focal brain infarction or hemorrhage” “penumbra” or “tissue at risk” • 1-2 in 100000 children annually • For tissue that is already • Highest in children <5, boys>girls infarcted, reperfusion increases risk • “Neonatal” (>28 weeks gestation, <28 days postnatal) more common • Hemorrhagic transformation • Etiology • Reperfusion injury • Ischemic (~50%): Arterial ischemic stroke (AIS) or venous infarction • Complications related to due to cerebral sinovenous thrombosis (CSVT) catheterization • Hemorrhagic (~50%): intracerebral hemorrhage (ICH), intraventricular hemorraghe (IVH) or subarachnoid hemorrhage (SAH)

Ferriero etal,Stroke,2019

Case3:StrokeͲ Presentation Case3:StrokeͲ Etiology

• Cardiac (~30%) • Drugs • Congenital heart disease • Cocaine • Presenting symptoms: • Endocarditis • Chemotherapy (L-asp) • • Focal neurologic deficits Rheumatic heart disease • Metabolic/Genetic • Arrythmias • Homocystinuria • Vascular disease • Hemiparesis and hemi-facial weakness (67-90%) • Fabry’s disease • Intracranial arteriopathy (~45%) • Speech disturbance (20-50%) • Fibromuscular dysplasia • Focal Cerebral Arteriopathy (FCA) • Organic acidemias • Vision disturbance (10-15%) • Moyamoya • Majewski’s Osteopdysplastic Primordial • Ataxia (8-10%) • Extracranial arteriopathy (~7%) • Arterial dissection (esp posterior circulation) Dwarfism, type II • Altered mental status (17-38%) • Hematologic • Collagen vascular (e.g., Ehlers-Danlos) • Headaches (20-50%) – more common in children • Sickle cell disease • SLE • Leukemia • Neurocutaneous d/o’s • Acute seizure (15-25%) • Polycythemia • Neurofibromatosis • Hypercoaguable state • Tuberous sclerosis • Aquired: sepsis, nephrotic syndrome, liver failure, cancer, • PHACE syndrome OCPs • Inherited: protein c/s deficiency, AT III deficiency, Factor V Leiden, MTHFR, prothrombin 20210 Ferriero etal,Stroke,2019 Ferriero etal,Stroke,2019 Case3:Stroke–FocalCerebralArteriopathy Case3:StrokeͲ Etiology

• “FCA”: Unilateral stenosis and/or irregularity • Infection as a risk factor for of the large intracranial arteries of the 100 anterior circulation stroke Controls Cases • Often involves junction of distal ICA and MCA/ACA • Large case-control 80 international study of 355 • Three types 60

children 54%

children with AIS  • Inflammatory 46% of  • Dissection • 36% with definite arteriopathy, 40 39% • Undetermined 10% with possible arteriopathy 22% • Has been associated with viral infections • Infection ȱ1 week prior to Percent 20 18% including HSV, VZV stroke: 6.3-fold risk of AIS 3% • Course: Progression of symptoms over days- (p<0.0001; adjusted for age) 0 weeks, plateau over ~6 months, then • Unvaccinated: 7-fold risk of Priorweek Priormonth Priorsixmonths subsequent improvement stroke (p=0.0002) • BUT high 1-year recurrence rate (19-25%)

openi.nlm.nih.gov Ferriero etal,Stroke,2019 FullertonetalNeurology2015

Case3:Stroke– AdditionalEvaluation Case3:Stroke– SecondaryStrokePrevention

• Screen for common causes of stroke in children • High rate of recurrence • Cardiac structure and function • 10% for childhood ischemic stroke, 33% for arteriopathy • Intracranial vessel imaging (including “vessel wall imaging” to • No large studies to guide choice of antiplatelet vs look for inflammation of vessels if inflammatory FCA suspected) anticoagulant therapy • Neck vessel imaging • For cardioembolic or thrombophlic stroke, consensus statement • Thrombophilia screening recommends anti-coagulation with LMWH or warfarin for 3-6 • Inflammatory markers months • Screen for recent illness/infection • For all others, aspirin 3-5 mg/kg/d for ~2 years • Lumbar puncture in the case of FCA • HSV PCR, VZV PCR and IgG/IgM

Ferriero etal,Stroke,2019 Ferriero etal,Stroke,2019;IPSS

Case3:Stroke Case3:Stroke

• Back to our case… • Back to our case • MRI showed right MCA territory stroke with carotid occlusion • RVP positive for parainfluenza 1 and 3 • Not felt to be a candidate for acute intervention due to large territory of infarct and risk of reperfusion injury • Treated with aspirin and LMWH acutely, then long-term therapy with aspirin • Discharged home after inpatient rehab, ambulating independently Case3:Stroke–TakeͲAwayPoints

• Acute onset of focal neurologic symptoms is an emergency! • CT is often faster, but MRI is more sensitive for ischemia • Children > 8 years of age within 24 hours of onset of symptoms are candidates for acute intervention • tPA >12 years and <3 hours • Endovascular therapy >8 years and <24 hours • Focal neurologic deficits are most common presenting symptoms • Headache and seizure also common in children • Risk factors are more varied than in adults • Up to 45% of AIS in children are related to intracranial vasculopathy • Recent infection may be independent risk factor • Long-term therapy typically includes aspirin for 2 years Conflicts of Interest/Disclosure

I have no biomedical or financial conflicts of interest to disclose.

Craigan Usher, MD Division of Child & Adolescent Psychiatry Oregon Health & Science University 18 October 2019

Who am I? LEARNING OBJECTIVES Craigan Usher By the end of this session, participants should be able to: • Program Director, Child & Adolescent Psychiatry Training at OHSU 1) List three names that have been used to describe conversion • Kienle Scholar for Medical Humanities through Penn State College of Medicine phenomena • Assistant Editor—Book Forum, Journal of the American Academy of Child & 2) Name three stressors that are often “converted” in children/teens Adolescent Psychiatry 3) Explain the psychoanalytic roots of the term conversion and what • A few resource ideas: functional neuroimaging suggests are the underlying functional deficits that advance our understanding of conversion beyond the explanation offered by Freud 4) Discuss three ways to support youth with functional neurologic disorders

Why are we talking about? Why are we talking about conversion disorder?

DSM5 Criteria • Conversion disorder is a relatively common diagnosis in children and adults A) One or more symptoms of altered voluntary motor or sensory • CD is rare before age 7 and common between ages 12-16 function • Conversion disorders can lead to very significant distress, with B) Clinical findings provide evidence of incompatibility between the patients, parents, school teachers, providers, and others often symptom and recognized neurological or medical conditions feeling confused, that there efforts are futile C) This symptom or deficit is not better explained by another • Up to 30% of new neurology outpatient visits may involve medical or mental disorder functional symptoms with 8% meeting criteria for conversion D) The symptom or deficit causes clinically significant distress or disorder impairment in social, occupational, or other important areas of • 10-20% of patients with intractable epilepsy have non-electrical functioning or warrants medical evaluation. seizures (NES) • The prognosis for adults is generally poor (50% improving), but many remain symptomatic • The course of pediatric conversion is not well studied, but generally thought to improve more quickly

Hubschmid M, Aybek S, Maccaferri GE, Chocron O, Gholamrezaee MM, Rossetti AO, Vingerhoets F, Berney A. Efficacy of brief American Psychiatric Association. Diagnostic and statistical manual of mental disorders (DSM-5). American interdisciplinary psychotherapeutic intervention for motor conversion disorder and nonepileptic attacks. General hospital psychiatry. Psychiatric Pub; 2013 May 22. 2015 Sep 1;37(5):448-55. Data on Prevalence & Characteristics Vocabulary • Around 50% of children/teens have a “co-morbid” psychiatric disorder, “Hysteria” was first described by Egyptian the most common including anxiety and depression and Greek philosophers and physicians and • Often associated with/precipitated by stressors, including: referred to a “wandering womb” etiological • Family conflict theory. • Bullying • Separation from a family member In the 19th Century, Jean-Marie Charcot • Academic problems noted that both men and women could • In 42 children at CHoP, in a 3yr period (02/2015 – 07/2018) they suffer from “hysteria,” but that male hysteria found: was due to trauma while female hysteria • Children with CD made up 10.7% of the CAP inpatient consults could be both traumatic and constitutional. • Antecedent stressors (usually family structure, conflict) found in 95% of patients • A history of trauma found in only 14% Charcot noted that many of his “hysteric” patient were more susceptible to hypnosis • 25% demonstrated la belle indifference while 45% had moderate and that this may offer a cure. to severe distress • c/w other researchers, they found an even distribution of young A Clinical Lesson at the Salpêtrière by André Brouillet men:young women at 13, but more females effected in later teens

Samuels A, Tuvia T, Patterson D, Briklin O, Shaffer S, Walker A. Characteristics of Conversion Disorder in an Urban Academic Children’s Medical Center. Clinical pediatrics. 2019 Oct;58(11-12):1250-4.

Sigmund Freud’s Original Freud’s Structural Model Conceptualization of “Konversion” Case 4 & 5 Freud considered his models theoretical placeholders— until more sophisticated means of neural inquiry were Originally wrote about conversion in “The Neuro-Psychoses of Defence” available. (1894) and in Studies on Hysteria (1895) Superego One can thus easily imagine Freud replacing his model Conversion consists in a transposition of psychical conflict into, and it’s with contemporary language, seeing: attempted resolution through, somatic symptoms which may be either of 1. The Id (Das Es) as the insistence of the Limbic System a motor nature (e.g. paralyses) or of a sensory one (e.g. localised (amygdala, nucleus accumbens) pushing for pleasure anasthesias or pains). or vengeance 2. The Ego (Ich) various regions of the posterior cortex Essentially, Freud argued that “through bodily symptoms, repressed responsible for how we represent the outside world ideas ‘join in the conversation’.” 3. The Super Ego (Das Über-Ich) as the Prefrontal Cortex Id responsible for having a conversation restraint / top- down regulation Ego

Pontalis JB, Laplanche J. The Language of Psycho- Analysis. New York: WW Norton; 1973. p90

Freud’s Topological Model The Archaeological Model of Therapeutic Action

• Repressionactsasadam,activelykeepingthe • If one could simply “dig” deeper, revealing to the individualkeepingfromconsciousawareness patient what was being converted and hence kept painfulthoughts,feelings,memories,and from their awareness, then the symptoms could be impulses. relieved. “Iftheperceptionofofrealityentailsunpleasure, Superego thatperception—thatis,thetruth—mustbe sacrificed.”

ǦFreud,SEXXIII,p237

Id

Ego Classic Example: How this theory and “cure” are supposed to work NEUROIMAGING FINDINGS CONVERSION DISORDER: • A 12-year-old young man, Sam, discovers that his parents are not faithful to one another and are planning to separate. Sam cries and announces that he “can’t stand this.” When Sam wakes the next morning, his legs feel wobbly, his gait is unsteady, he has difficulty swallowing and he complains of nausea. Disrupted Abnormal Top Down • That day, Sam sees his pediatrician for an urgent visit. She witnesses Sam’s extremely abnormal gait that Emotional Sense of Regulation seems to change character. She finds that the patient’s physical and neurological examinations are completely normal. Having read Freud and taken a clear history of the past 24 hours, Sam’s doctor Processing Agency Problems encourages him to recognize the link between his emotional pain, the traumatizing sudden rupture of expectation that he’s gone through, his neurologic symptoms and things he’s said to his parents (“you two Traumatic Agency is internal One study found make me sick” “I’m totally grossed out by you” “I can’t stomach this” “I wont’ stand for this” etc). experience may sense: “I made that reduced activation predispose happen.” of hand movement • With improved insight, the patient’s symptoms resolve and he learns to cope with what he sees as his individuals to having Neurologically, it when observing parents’ betrayal. less activity in the appears to have an hand movements supplementary afterwardness. (decreased motor area and mirroring) BUT NOT greater activity in Reduced activion in increased inhibitory the R amygdala, the temporo-parietal control from frontal PLEASE RAISE YOUR HAND IF YOU HAVE EVER HAD A REAL-LIFE insula, and bilateral junction and lobe. Aybek S, Nicholson TR, O’Daly O, Zelaya F, Kanaan RA, David AS. posterior cingulate reduced connectivity CLINICAL EXPERIENCE THAT WORKED LIKE THIS. Emotion-motion interactions in conversion disorder: an FMRI study. cortices. in sensorimotor PLoS One. 2015 Apr 10;10(4):e0123273. cortex and cerebellum in pts w/ Roelofs JJ, Teodoro T, Edwards MJ. Neuroimaging in functional movement disorders. Current neurology and neuroscience reports. functional tremors. 2019 Mar 1;19(3):12.

TIPS FOR TALKING ABOUT CONVERSION DISORDER TIPS FOR TALKING ABOUT CONVERSION DISORDER

Destigmatize & Legitimize Educate & Explore Inquire about the details of a patient’s (OT, PT, Bring people up by their strengths, not their CBT, SLP) treatment weaknesses • “Functional neurological disorders are common. They can be brought on by something painful in your life.” • Who are you meeting with? • Inquire about friends, hobbies, activities, things about • This is a brain disorder. Period. which the patient is proud that do not relate to the • “The amazing thing is, it come from your brain and • How often? • What questions do you have about the nature of functional deficit your brain can be part of the solution.” this problem? • What shared goals do you have? • Demonstrate for parents how best to ignore/avoid • “But the part of your brain that CAN solve the • What therapeutic activities are you doing? reinforcing panic/concern about functional symptoms problem, just doesn’t know it yet. It needs training.” • Emphasize the importance of this work—call or • Highlight and reinforce engagement/patient’s • Explore predisposing vulnerabilities, acute email in front of your patient to collaborate. strengths and note that you would like to hear about precipitants and perpetuating factors an event or hobby, a favorite pet, book, movie, videogame, and perhaps for the patient to bring in a picture/sample at the next visit • Encourage follow-up visits for progress NOT “if things are going well.”

Adams C, Anderson J, Madva EN, LaFrance Jr WC, Perez DL. You’ve made the diagnosis of functional neurological disorder: now what? Pract Neurol 2018;18:323-330.

TIPS FOR TREATING CONVERSION DISORDER CONVERSION DISORDER: CASE EXAMPLES

Connect with School Personnel Document – Ideally in sharable EMR

• Create an assessment and safety plan • Outline previous work-up and rationale behind A 12-year-old young man with A 16-year-old with NES, A 13-year-old with headaches, diagnosis • Again, destigmatize and de-escalate sense of urinary incontinence and LE multiple ED and ICU treatments weakness, speech articulation alarm that is often associated with PNES and • Delineate safety steps to take weakness for acute episodes problems other FNDs • Note patient’s strengths (the reader may not know • Clarify to whom they can reach out for support, that the Freudian archeological dig and reveal when, and how therapeutic approach is ineffective) • Emphasize on-going outpatient treatment plan • Clarify recommended treatment course that cautions against use of potentially habit-forming pharmacologic interventions QUESTIONS & YOUR CASES?

“Life can only be understood backwards; but it must be lived forwards.” - Soren Kierkegaard

LEARNING OBJECTIVES: REVISITED LEARNING OBJECTIVES: REVISITED So, today you learned that: 3) Sigmund Freud coined the term conversion disorder and he characterized this as a way that affects, ideas, and experiences that were actively being repressed by an dynamic unconscious force 1) Conversion disorder and conversion phenomena have also been called: could “join the conversation” by being expressed neurologically. Functional neuroimaging has • hysteria advanced this by discovering deficits in 1-emotional processing; 2-one’s sense of agency; and 3-top • psychogenic disorders down regulation/mirroring. • non-organic syndromes • pseudoseizures 4) Some important ways of supporting children/teens and their families/friends/teachers: • psychogenic non-epileptic seizures (PNES) • functional neurologic symptom disorder • Combat stigma: these are real, treatable disorders • functional neurologic disorder (FND) • Offer education and the neurological understanding of what maintains symptoms—it’s skill opposed to will 2) Stressors that are often “converted” include: • Refer and inquire about therapies including CBT, OT, PT; collaborate • family conflict • Bring people up by their strengths; reinforce these! • bullying • Connect with schools • separation from a family member • academic problems • Develop a treatment plan and place an alert/put this atop every note

CRAIGAN USHER, MD [email protected] Slide 1

RP1 Enlarge font! Be proud! Randall Phelps, 10/13/2019

Tic Disorders and Tourette Syndrome

Evaluation, Diagnosis, and Treatments

October 18th, 2019 RP1

PRESENTED BY: Amelia B. Roth, MD

RP2

Vignette #1: RP3 Disclosures: X Smart, social 6 year old boy dx with ADHD at age 5 by PCP

X Continues to be disruptive, strong willed, anxious, and 1. No financial disclosures inflexible at home and school.

2. Clinical vignettes are used but patient information is X Methylphenidate and Strattera have been tried, with protected mixed results…

3. Off-label medication use is described, as is common in X In the exam room he is fun and interactive, and frequently pediatrics honks at me…

X On further questioning, he also has a history of repetitive throat clearing, grunting, crotch grabbing, and saying words over and over since toddlerhood

RP4

Slide 3

RP2 I prefer "vignette" to "Case". Case sounds cold and clinical. I think vignette sounds warmer. Randall Phelps, 10/13/2019 RP3 Yeah, not so sure about this "case title". How about just giving him a name. Again, less objectifying. What is a tic? Randall Phelps, 10/13/2019 RP4 I would leave this off for now. The implication here is that guanfacine is the treatment for TS, which, I don't think is the impression you want to leave. Yes, guanfacine can be a helpful and well-tolerated tool, but Rx shouldn't be emphasized as the primary cure for TS! Randall Phelps, 10/13/2019 X A fragment of normal behavior that occurs quickly and in isolation, but more repetitive and less variable

X Not voluntary and they are not involuntary, they are “unvoluntary” RP5 X Can be easily described/reproduced by observers

X Wax and wane, and can be suppressed at least temporarily

X Feels like an itch that has to be scratched or a RP6 sneeze that is hard to suppress

X The tic itself is often not as much of a problem as the comorbidities… Slide 4

RP5 No--I didn't coin this term. I heard it from Dr Sam Zinner of UW first, but I don't think he coined it either. I don't think you need to attribute the term. Randall Phelps, 10/13/2019 Tics versus Stereotypic Movements RP6 subjectively, FEELS like an itch... Randall Phelps, 10/13/2019

X Tics: generally ego dystonic, most have a premonitory sensation and while they can be suppressed, tension exists when the tic is not released

X Stereotypies: ego syntonic, (though kids can become embarrassed by them), and suppression of the stereotypy does not cause as much tension

X Hand flapping, shuddering, complex hand movements, head nodding and banging, body rocking, sometimes accompanied by open mouth and staring, and sometimes vocalizations

Slide 6

Common Childhood Motor Tics RP9 contrast again with stereotypies. Stereotypies in contect of developmental disabilities, such as ASD or profound ID, can include significant self-injurious behavior Randall Phelps, 10/13/2019 X Hard/frequent eye blinks, winks

X Eyes darting

X Facial grimaces, jaw movements

X Opening mouth

X Shoulder shrugging, neck stretching

X Torso shifting, jerking

X Hand to face/GU area/head/etc…

X Scrunching nose

X Copropraxia (rude gestures) and echopraxia (imitating gestures)

X Hopping, twirling, jumping

X Repetitive tensing of abdominal/limb muscles RP9 X Truly dangerous tics are rare, but muscle soreness can occur, as opposed to stereotypes, which can include significant self-injurious behavior

Common Childhood Phonic Tics Premonitory Sensation X Repetitive throat clearing

X Grunting, honking X Burning in the eye prior to a blink X Meowing, hissing, barking

X Induced belching X Tension in neck relieved with a stretch or jerk

X Making sounds with mouth X Feeling of tightness relieved with extension X Snorting, sniffing

X Gasping, sharp inhalations X Kids get referred to PT’s for “neck problems”,

X Short, sharp vocalizations: “oop” “eep” and what is really occurring is a motor tic

X Rarely, coprolalia and echolalia, and palilalia (repeating own words)

X Hooting, shouting

X Words or phrases that are not part of a conversation (can be barked or grunted) Slide 9

RP7 I recommend consistency in font. RP7 Randall Phelps, 10/13/2019 RP8 RP8 Great quote. “People believe that if Randall Phelps, 10/13/2019 you can shut off your Tourette’s for a period of time, then you can always shut it off. I try to explain to people that if I spent my whole life trying to control my tics, that’s all I would have time for.” – Dash Mihok (actor)

Types of Tic Disorders Types of Tics

X Transient: motor, phonic, or both for > 2 X Simple Tics: Sudden, brief, a limited number weeks and < 1 year of muscle groups

X Chronic Motor or Vocal Tic: Motor tic OR X Complex Tics: coordinated between more Vocal tic > 1 year than 1 muscle group (rolling eyes back while

X Tourette Syndrome: At least 2 motor and at sniffing and shrugging shoulders)

least one vocal tic > 1 year, (generally X Complex Tic or OCD Ritual? Is a tic really a waxing and waning but mostly present) manifestation of OCD? On obsession followed by a compulsion?

Vignette #2: RP10

Who gets tics? X 8 year old boy diagnosed with ADHD, ODD, and Social Anxiety at age 6 at the CDRC here for f/u

X 1 out of 100 kids between 5 and 17 years X Parents and Psychiatrist still think it’s autism of age has a tic disorder X He has a 1:1 aid at school

X 1 out of 160 kids between 5 and 17 have X He is a perfectionist and easily escalates saying “I want to Tourette Syndrome die”, and now curses and hits walls

X He can be sweet, is eager to please, makes great eye X 3-4 boys diagnosed for every girl contact, and is socially engaged. He hates that he curses X Tics tend to emerge around age 5/6, and gets violent with objects…

worsen around age 10/11, and improve by X The only medicine tried so for was Risperidone

18, then sometimes recur in middle age X I notice that older brother in room has a phonic tic…

X On further questioning, he makes a lot of random noises and movements,RP11 and taps his forehead in a repetitive way... Slide 13

RP10 again--I recommend "vignette" rather than "Case" and I would ditch the sub-titles. Randall Phelps, 10/13/2019 RP11 where are you going with this vignette? Is there an epilogue? How do you address the diagnostic confusion (e.g. were they wanting ABA? DDS? ) And what were the side effects of Risperidone? Developmental Disability Services Randall Phelps, 10/13/2019

X People seeking an autism diagnosis are sometimes seeking services…

X DDS offers respite care, personal support workers paid through the state, behavioral evaluations, and some money for the purchase of non-billable items (crash pads, sensory tools)

X Tourette Syndrome is now an eligibility for DDS, provided there is proof of global functional impairment, as are the diagnoses of an Autism Spectrum Disorder, Intellectual Disability, Global Developmental Delay, and FASD

Tourette Syndrome Vignette #3 RP12

X Most have normal IQ X 14 year-old boy comes in with mom X “Does he have autism or is he just a (a jerk)?”, mom asks in front X School performance often affected by OCD, anxiety, and of son ADHD X Difficulty making friends; annoyed with others easily X Onset between ages of 2 and 15 years, the mean is around age X Many annoying habits, including throat-clearing, coughing, making body function noises, bouncing, tapping, head-rolling, 6 or 7 years and fidgeting

X Tics tends to be most severe in late childhood/early teen years X Teased about these behaviors and he would like to stop X He has been diagnosed in the past with ADHD and treated with X Half of kids are tic free by age 18, though they can come back stimulant, which caused exacerbation of sounds/movements, weight-loss, and diminished energy. He has begun to hoard in middle adulthood things and was dx with OCD.

X Remember, mild cases are more common than severe cases! X Aggression towards sister and cat had escalated and the family was beginning to consider residential treatment… X Only 15-20% have coprolalia or copropraxia X A psychiatrist dx high functioning autism and prescribed an anti-psychotic medication, with some improvement in behavior, but also significant weight-gain and sedation

Slide 16

RP12 again: change to vignette, and use pseudonym instead of sub-title. Randall Phelps, 10/13/2019

VignetteRP13 #3

X On Exam he is pleasant, cooperative, with typical social referencing and reciprocity, typical prosody of speech

X A few subtle tics seen in office, some fidgetiness

X ADOS—non-clinical

X Normal cognitive and language skills

X Now he is obese, secondary to atypical antipsychotic med

X He gained 30 lbs. in one year, and kept increasing doses

X He is now teased more for his weight than for his tics…

RP28 Slide 17

RP13 vignette, cont'd--data Randall Phelps, 10/13/2019 RP28 Note that the obesity was the direct result of Risperidone, with 30 lbs weight gain in 1 year on it. Note that the Risperidone helped with tics initially, but that the benefits waned, necessitating increases in doses over the year. Note that child now says that he is teased RP14 more for being fat than he was ever teased for tics and that he would rather tic than have the extra 30#, and that he tics now anyway Conclusions on Risperidone Randall Phelps, 10/13/2019

X Tourette syndrome, with secondary social impairments. X The key is that the teen was very bothered by these habits. X With new diagnosis, mom softened and was more receptive to him X He was referred to counseling, and school accommodations where recommended, as well as sports/exercise X On follow-up he was doing well, both academically and socially, and off of all medication

Slide 18

RP14 conclusion: Randall Phelps, 10/13/2019 Comorbidities RP15

X ADHD

X Anxiety and OCD (20-40% have OCD, almost all have some elements of OCD)

X If you have OCD, you have a 20% risk of developing tics and 7% risk of TS

X Mood challenges

X “fiery temperaments”

X Social Development challenges

X Sleep challenges and parasomnias

X Comorbidities are often a bigger challenge than the tics!

X Target treatment to whatever causes the most interference with functioning

Slide 19

RP15 emphasize that the co-morbidies are OFTEN a bigger problem for folks than the tics themselves! Target treatment to whatever causes the most interference with function/participation! Randall Phelps, 10/13/2019 Heritability

X Tourette Syndrome tends to be a highly penetrant dominant trait, males tend to have ADHD and tics, RP16 females tend to have OCD (externalization versus internalization)

X Stimulants provoke tics in predisposed kids, as can steroids, stress, illness, and lack of sleep Slide 20

RP16 note that, in general, males externalize and females internalize, so this makes sense Randall Phelps, 10/13/2019 Worsening Factors

X Sleep deprivation/Exhaustion

X Anxiety

X Excitement

X Anger RP17 X Illnesses – virus, strep… RP20

X Pain, injury RP18 X Being alone (feeling more comfortable)

X Lack of exercise

X Feeling too hot or too cold

X Sensory irritants like tags, turtle necks, tight or itchy clothes

RP19 Slide 21

RP17 do you want to mention PANDAS here? That it seems that the issue is that infections generally increase tics and OCD sx, as well as other behavioral symptoms? Not necessarily immune-mediated--but that there is that hypothesis? Randall Phelps, 10/13/2019 RP20 Ah--never mind. I see next slide. Good. Randall Phelps, 10/13/2019 Do you Believe in Pandas? RP18 this could relate to feeling comfortable ticcing when alone Randall Phelps, 10/13/2019

X Tics and OCD tend to worsen with illness, and particularly with strep

X There is a theory that it’s an immune mediated process, similar to Sydenham’s Chorea

Slide 22

RP19 Randall Phelps, 10/13/2019 Alleviating Factors

X Sleep

X Calm

X Focusing on a task

X playing a musical instrument, (drums!)

X Vigorous exercise

X Regulating body temperature

X Staying healthy RP21 RP22 Slide 24 RP24 RP21 Great slide! Randall Phelps, 10/13/2019 Lifestyle and Behavioral Management RP22 Add that working with schools is very important here--recommending 504 or IEP with scheduled sensory/tic breaks is important! Randall Phelps, 10/13/2019 RP24 Ah, I see you got to this later, too--good! X First: optimize sleep! Decrease screen time! Randall Phelps, 10/13/2019

X Second: optimize physical activity and outdoor time

X Third: get child into a physical or musical activity they enjoy like martial arts, running, swimming, ball sports, drumming, other musical instruments

X Fourth: Cognitive Behavioral Therapy (CBT) for anxiety/OCD and Comprehensive Behavioral Intervention for Tics (CBIT)

X Parents and teachers can redirect or distract when child is having tics, but should not keep asking child to stop, or make the child feel ashamed

X Celebrate neurodiversity in the home, school, and community

Comprehensive Behavioral Intervention for Tics Medical Management: Optimize Sleep

X 1. Training the patient to be more self-aware of tics (but not X First, optimize sleep! more self-conscious) X Start with 0.25 mg Melatonin at bedtime if sleep onset is X 2. Training the patient to do competing behaviors when they challenging, slowly increase as needed feel the urge to tic (slow breathing instead of throat RP23 X Next step would be Clonidine, start with 0.05 to 0.1 mg at clearing) Æ so, not suppressing the tic (which is exhausting), bedtime but practicing behaviors that are incompatible with ticcing until the urge goes away X Consider adding in long-acting Clonidine if waking up in night and ticcing X 3. making changes in daily routines that can be helpful in reducing tics (manage anxiety and stress) X If sleeping very well, AND still having problematic day time tics, consider day time medications as well, such as X 4. Many people living with tics already use similar strategies guanfacine they have discovered on their own

Slide 26

RP23 As you know, I agree. Clonidine helps with sleep and reduces tics, so it's a great choice. But, just to note: sleep specialists in attendance may object. One response to such an objection would be that not treating sleep ticcing can result in need for stronger Rx, with more side effects, so it is often in child's best interest to treat with Clonidine or Guanfacine.... Randall Phelps, 10/13/2019 Medical Management: Day Time

X Consider starting guanfacine, usually short acting

X For young kids, start with 0.25 mg BID, then can slowly increase as needed

X If starting long acting guanfacine, start at night if not already on clonidine, then move to AM once adjusted to soporific effects

X Once sleep is optimized, and day time tics are improved, consider addressing ADHD if needed with stimulants

X Consider managing anxiety/OCD with an SSRI if needed Medical Management for ADHD in Tips and Tricks in the Classroom kids with tics X Consider a 504 plan to allow for tic accommodations, or an IEP if X Stimulants usually worsen tics, but occasionally can help significant ADHD also present interfering with learning

X Kids with Tourette Syndrome/Tics tend to do better with X Tic Breaks, or timing tics with other loud noises in the class (such stimulants when used synergistically with alpha agonists as clapping or laughing)

X Kids tends to do better with Dexmethylphenidate (Focalin) X Sports water bottle at desk can help than Methylphenidate (Ritalin) X Chewing gum

X Strattera can be helpful for some, though many report X Fidgets in the hands or pockets like putty, pieces of felt

feeling unwell on this X Movement breaks

X Subtle hand signals between teacher and student to communicate needs

X Treat the underlying Anxiety, OCD, ADHD, Sleep Disorders

Slide 30

RP26 Yes. Highlight this earlier? in reference to a vignette? Perhaps one of the vignettes where the family insists on ASD--they want ASD so they can get DDS? Resources for Families Randall Phelps, 10/13/2019

,The Tourette Association of America ڼ www.tourette.org, established in 1972

Check out the video: “I have Tourette Syndrome but ڼ Tourette Syndrome Doesn’t Have Me”

If there are global adaptive impairments, kids can be RP26 ڼ eligible for Developmental Disability Services, and possibly SSI depending on family income Objectives

Adolescent suicide prevention: ɵ Recognize adolescent suicide risk Risk screening, assessment, and safety planning ɵ Identify strategies for screening of suicide risk ɵ Describe assessment and management of those at Melissa Weddle, MD, MPH increased risk Pediatric Review and Update October 18, 2018

Youth Suicide in Oregon

PART 1

The Evidence FOR SUICIDE RISK SCREENING

3

Youth Suicide in Oregon Youth Suicide in Oregon Contemplated Suicide in the last 12 Months

Source: 2013, 2015, 2017 Oregon Healthy Teens Survey PUBLIC HEALTH DIVISION Note: “Transgender or gender..” includes those who identified as PUBLIC HEALTH DIVISION Adolescent and School Health transgender, gender fluid, genderqueer, gender nonconforming, Source: 2017 Oregon Healthy Teens Survey Adolescent and School Health intersex/intergender, multiple responses, and “not sure of gender” Youth Suicide in Oregon Youth Suicide in Oregon Attempted Suicide in the Last 12 Months Contemplated Suicide in the last 12 Months

Note: “Transgender or gender..” includes those who identified as transgender, gender fluid, genderqueer, gender nonconforming, intersex/intergender, multiple responses, and “not sure of Source: 2017 Oregon Healthy Teens Survey PUBLIC HEALTH DIVISION gender” PUBLIC HEALTH DIVISION Adolescent and School Health Source: 2017 Oregon Healthy Teens Survey Adolescent and School Health

Youth Suicide in Oregon Youth Suicide in Oregon Attempted Suicide in the Last 12 Months

PUBLIC HEALTH DIVISION Source: 2017 Oregon Healthy Teens Survey Adolescent and School Health Source: 2017 Oregon Healthy Teens Survey PUBLIC HEALTH DIVISION Adolescent and School Health

Youth Suicide in Oregon Youth Suicide in Oregon Suicide deaths by age, Oregon 2017 Suicide deaths by gender, Oregon 2017

Source:OregonViolentDeathReportingSystem

11 12 OHSU Child & Adolescent Psychiatry Consultation-Liaison service OHSU Child & Adolescent Psychiatry Consultation-Liaison service

National Recommendations

American Academy of Pediatrics recommends that pediatricians ask questions about mood disorders, sexual PART 2 orientation, suicidal thoughts, and other risk factors associated with suicide during routine health care visits Recommended American Academy of Child and Adolescent Psychiatry SCREENING & ASSESSMENT TOOLS recommends that physicians be aware of patients at high risk for suicide American Medical Association Guidelines for Adolescent Preventive Services recommends that all adolescents be asked annually about behaviors or emotions that indicate risk for suicide

15 16

Why should Primary Care Practitioners Screen? Barriers to PCP Screening & Assessment

ɵ Suicide is the #2 cause of death of 10 – 24 year olds Time 32.8% ɵ 70% of adolescents seen by PCP annually ɵ Adolescents more comfortable with PCP Adequate training 25.5% ɵ Patients who died by suicide visited PCPs over 2 times as often Adequate knowledge 32.9% as mental health clinicians Comfort discussing suicide 64.2%

17 18 Why screen in the hospital or ED? Minor Consent and Confidentiality

ORS 109.675 - a minor who is 14 years or older may access outpatient mental health, drug, or alcohol treatment without parental consent ɵ 30% of adolescents have not been seen by a

PCP in the past year ORS 109.860 - for mental health and chemical dependency services, the provider may disclose health information to a minor’s parent or guardian if: ɵ PCP may not have screened or had ɵ It is clinically appropriate and in the minor’s best interests adequate training ɵ The minor must be admitted to a detoxification program ɵ The minor is at risk of committing suicide and requires hospital admission.

Confidentiality Exceptions: ɵ Risk of harm to self or others ɵ Abuse 19 20

Risk Factors for Suicide Risk Factors for Suicide

ɵ Family history of suicide or child ɵ Feelings of hopelessness maltreatment ɵ Isolation ɵ Previous suicide attempt(s) ɵ Barriers to accessing mental health ɵ History of trauma and/or personality or treatment mood disorders ɵ Loss (relational, social, work, or financial) ɵ History of alcohol and substance abuse

21 22

Warning Signs Warning Signs ɵ Talking about wanting to die ɵ Sleeping too little or too much ɵ Talking about being a burden to others ɵ Withdrawing from family or friends or feeling ɵ Increasing use of alcohol or drugs isolated ɵ ɵ Acting anxious or agitated, behaving Displaying extreme mood swings recklessly ɵ Saying good-bye to loved ones, giving belongings away

23 24 Protective Factors Components of Evaluation ɵ Family and community support (connectedness) ɵ Screening

ɵ Self-esteem and a sense of purpose and ɵ Assessment meaning ɵ Problem solving, conflict resolution, coping, and ɵ Safety Plan nonviolent communications skills ɵ Lethal Means Counseling ɵ Cultural or religious beliefs ɵ Disposition ɵ Effective clinical care

25

Suicide Risk Screening and Assessment Tools Depression and Suicide Risk Screening PHQ-9 Modified for Adolescents Screening Tools PHQ-9 plus suicide questions ɵ PHQ-A (Patient Health Questionnaire for Adolescents) 11-17 years old ɵ asQ (Ask Suicide-Screening Questions) The PHQ-A can be considered ɵ C-SSRS (Columbia-Suicide Screening Rating Scale) a suicide risk screening tool ONLY if suicide questions are Assessment Tools included and everyone ɵ asQ BSSA (Brief Suicide Screening Assessment) answers them (e.g. not only ɵ C-SSRS when PHQ-2 is positive)

28

Suicide Risk Screening - asQ Suicide Risk Screening - asQ asQ Suicide Risk Screening Tool asQ Information Sheet Available in multiple languages

Developed for patients 10-24, for Takes 1-2 minutes to screen use in pediatric EDs, inpatient, and primary care settings 100% Sensitivity in Primary Care For use by non-psychiatric clinicians 88% Specificity in Primary Care Negative Screen: “No” on first 4 questions; end of screen

12.1% of US adolescents experience Positive screen: “Yes” to any of first 4 suicide ideation, 4% develop a questions requires answer to question 5, suicide plan, and 4.1% attempt patients cannot leave until evaluated for suicide safety Acute positive screen: “Yes” on question 5, patient requires STAT safety/full mental health Solely relying on depression evaluation screening through PHQ-9 missed up Non-acute positive screen: “No” on question to 28% of participants at risk for 5, use asQ Brief Suicide Safety Assessment suicide (BSSA) (~10-15 minutes) 30 Brief Suicide Safety Assessment

asQ BSSA (Outpatient Version) Developed for primary care For use by non-psychiatric clinicians Contains protocol and scripts for talking to pediatric patients and parents

32

Brief Suicide Safety Assessment BSSA Step 1: Praise Patient

asQ BSSA (Outpatient Version)

Cues each step of process: 1. Praise patient

2. Assess the patient

3. Interview patient & parent/guardian together

4. Make a safety plan with the patient

5. Determine disposition

6. Provide Resources to all patients

33 34

BSSA Step 2: Assess the Patient BSSA Step 2a: Frequency of Suicidal Thoughts

asQ BSSA (Outpatient Version) Step 2: Assess the patient Frequency of suicide thoughts Suicide plan Past behaviors Symptoms Social supports and stressors

35 36 BSSA Step 2b: Suicide Plan BSSA Step 2c: Past Behavior

37 38

BSSA Step 2d: Symptoms BSSA Step 2e: Social Support & Stressors

39 40

BSSA Step 3: Interview Parent/Guardian Together BSSA Step 4: Make a Safety Plan with the Patient

41 42 BSSA Step 5: Determine Disposition BSSA Step 6: Provide Resources to all Patients

Outcomes based on assessment: 1. Immediate referral to mental health provider

2. Safety planning with urgent referral to mental health provider within 72 hours

3. Safety planning with non-urgent referral to mental health provider

4. No further intervention needed at this time

43 44

Oregon Resources: OPAL-K Lines For Life - National Suicide Prevention Lifeline above • Phone consultation to Psychiatry for re-directs here assistancePART 3 in treatment and support of YouthLine – a teen to teen crisis and help line; teens available to help daily from 4-10PM, off-hours call re- patients with mental health difficulties direct to Lines for Life Management, Referral, and Call: 877-968-8491 Washington’sStructured PAL Clinician’s Follow-up guide is available Text: teen2teen to 839863 www.palforkids.org/resources.html Chat: http://www.oregonyouthline.org

46

Safety Planning Template Safety Planning Intervention Example Steps: Safety Plan Template (Brown and Stanley) Step 1: Recognize warning signs Free to use after registering on website Step 2: Identify and employ internal coping strategies ~20-30 minutes to complete Step 3: Use healthy social with patient, collaborative contacts as a means of process distraction. Step 4: Contact family and Identifies friends for help Internal coping strategies Step 5: Contact MH Enhancing social support professional or Professional Supports emergency services if needed Emergency contacts Step 6: Reduce access to lethal means 47 48 Lethal Means Statistics Lethal Means: Special Issues Related to Suicidal Youth What is it about guns? Involve parents and guardians whenever possible. Ask questions about means restriction with parents privately. ɵ 85% lethality Gently assume there may be guns in the home. ɵ > 33% of households have guns Example scripts: “Let’s talk about securing your guns so we can ɵ Irreversible damage keep your child safe”

ɵ 85% come from the victim’s home “Now might be a good time to give your guns to a friend or family member for safe-keeping”

49

Lethal Means: Special Issues Related to Suicidal Youth Means Safety Resources

It is important to remove and limit access to other lethal means: ɵ material that could be used for hanging ɵ medication lockbox

Lockmed.com

51 52

Referrals OPAL-K • Phone consultation to Psychiatry for Local Mental Health Resources assistancePART 4 in treatment and support of Identify community mental health partners OPAL-K patients with mental health difficulties Can assist with diagnostic questions Implementation Lines For Life Washington’s PAL Clinician’s guide is available Can assist with identifying local community mental health providers and www.palforkids.org/resources.html resources

53 54 Implementation Implementation

1. Education of staff about importance of “It’s not how are we going to do this, but screening how are we going to handle it if we lose 2. Identify a champion(s) one of our patients?” 3. Provide information about confidentiality ~Ted Abernathy, MD (Pilot Pediatrican for asQ Implementation)

55 56

Office Implementation Office Implementation 4. Establish flow of screening forms 5. Can forms be embedded in EMR? When and where do patients receive screen?

Confidential space for patient to complete 6. Establish tracking system to follow-up screen? with patients

Who will review/score screen?

How is provider notified of results?

How are results documented in the chart?

57 58

OPAL-K OPAL-K (Oregon Psychiatric Access Line about Kids) • Phone consultation to Psychiatry for Psychiatric phone consultation for medical assistancePART 5 in treatment and support of practitioners who treat children and patients with mental health difficulties adolescents with mental health difficulties Resources 9 am to 5 pm, Monday through Friday Washington’s PAL Clinician’s guide is available 855-966-7255 (toll-free) or 503-346-1000 www.palforkids.org/resources.html (Portland metro) Register online: www.ohsu.edu/opalk Fax: 503-346-1389 Email: [email protected]

59 60 Other Resources/Toolkits

Resources for providers OCCAP (Oregon Council of Child and Adolescent Psychiatry) Zero Suicide Suicide Prevention Resource Center (SPRC) Suicide Prevention in Primary Care Settings Toolkit (Deschutes County) Resources for youth Lines For Life YouthLine Teens Finding Hope Trevor Project Youth ERA Resources for parents Child Mind NAMI (National Alliance on Mental Illness) Toolkit OFSN (Oregon Families Support Network) Teens Finding Hope

61 62

Thanks to Oregon Pediatric Society and the Adolescent Suicide Prevention Task Force members who generously provided their time and expertise

Barbara Long, MD, MPH Kyle Johnson, MD Greg Blaschke, MD, MPH Rita Lahlou, MD Kristin Case, FNP Stewart Newman, MD Colbie Caughlan, MPH Kristi Nix, MD Keith Cheng, MD Teri Petterson, MD Kristan Collins, MD Liz Stevenson, JD, MPH Michael Harris, PhD Liz Thorne, MPH Ajit Jetmalani, MD Melissa Weddle, MD, MPH WhatEveryPediatricianNeeds toKnowAboutDrowning

BenjaminHoffmanMDCPSTͲIFAAP ProfessorofPediatrics,OregonHealthandScienceuniversity Chair,AAPCouncilonInjuryViolenceandPoisonPrevention

12childrenperweek Drowning1Ͳ18years

Objectives

• Bytheendofthispresentation,youshouldbeableto: • Discusstheepidemiologyofdrowningforchildrenandteens • DiscussthekeypointsfromtherecentlyrevisedAAPpolicy statementondrowningprevention • List5keytipstohelpyoudecreasedrowningrisksforyour patientsandtheirfamilies • Describelayersofprotectionindrowningprevention

Deaths1Ͳ18year2007Ͳ2017 UnintentionalDrowningDeathRateofUSInfantsandChildren Ages0Ͳ19byGender, 1981Ͳ2017

6 MVMVTrafficTraffic 66866686 1195 23318 DrowningDrowning 5 Male In2017: 28010 23318 OtherOtherUnintentionalUnintentionalInj.Inj. Female 913total 1488 1195 28010 4 Total unintentional 19061488 SuicideSuicide 1906 drowningdeaths 1952 HomocideHomocide 3 14809 1952 14809 MalignancyMalignancy 7599 44594459 13561 14276 Congen.Congen.AnomaliesAnomalies 2 13950 14276 13561 HeartHeartDz Dz

7599 Rate per 100,000 population 13950 1 Flu/PneumoniaFlu/Pneumonia

ChronicChronicRespRespDz Dz 0 CV CV SepsisSepsis Source:AAPAnalysisofCDCWISQARSfatalinjuryreports.February 2019 AllOthersAllOthers (https://webappa.cdc.gov/sasweb/ncipc/mortrate.html) Deaths1Ͳ4year2007Ͳ2017 UnintentionalDrowningDeathRateofUSInfantsandChildren, byAgeGroup, 1981Ͳ2017 6 0Ͳ4yrs Drowning 5Ͳ9yrs 5 3309 OtherUnintentionalInj. 10Ͳ14yrs CongenAnomalies 4 15Ͳ19yrs 9990 Homicide 7710 Malignancy 3 HeartDz 394 2769 3668 2848 Flu/Pneumonia 2 463

Sepsis Rate per 100,000 population 802 1 ChronicResp.Dz. Other 0

Source:AAPAnalysisofCDCWISQARSfatalinjuryreports.February 2019 (https://webappa.cdc.gov/sasweb/ncipc/mortrate.html)

Unintentional Drowning Deaths (Rate per 100,000) among US Children AfricanAmericanKidsDrownatMuch (ages 1-19) by Race/Ethnicity by Age Group, 2008-2017 Average 4 AmericanIndian/AlaskanNative 3 HigherRates Black White 3 2.5 Hispanic Asian/PacificIslander 2 100,000

 2 per 100,000   1.5 Per  Rate 1 Rate

 1

0 0.5

1Ͳ4yrs 5Ͳ9yrs 10Ͳ14yrs 15Ͳ19yrs Mortality

Source:AAPanalysisofNationalCenterforInjuryPreventionandControl/CDCWISQARS™ 0 (WebͲbasedInjuryStatisticsQueryandReportingSystem),April2019. 1Ͳ4yr 5Ͳ9yr 10Ͳ14yr 15Ͳ19yr Note:AmericanIndian/AlaskanNative,Black,White,andAsian/PacificIslanderrefertothosewhoidentifyasnonͲHispanic. White Black AI/AN Asian/Pac.Isl CDCWISQARS 2007Ͳ2017

Fatal Unintentional Injuries (rates per 100,000) among US Children (ages 0-19) by Race/Ethnicity, 1990-2017 UnintentionalDrowningDeathRate(per100,000Population)ofUSInfants andChildrenAges0Ͳ19,byState, 2008Ͳ2014AnnualizedAverage AmericanIndian/AlaskanNative 60 Black White 50 Hispanic Asian/PacificIslander 40

30

20

10 Rate per 100,000

0 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015 2016 2017

Source:AAPanalysisofNationalCenterforInjuryPreventionandControl/CDCWISQARS™ (WebͲbasedInjuryStatisticsQueryandReportingSystem),March2019. Source:NCHSVitalStatisticsSystemfornumbersofdeaths;USCensusBureauforpopulationestimates. Note:AmericanIndian/AlaskanNative,Black,White,andAsian/PacificIslanderrefertothosewhoidentifyasnonͲHispanic. RetrievedfromWISQARSFataInjuryMapping.CDC AAPPolicy

“theAAPlaysoutstrategiesto protectchildrenateachstageof theirlife.Newparentsareadvised tobevigilantatbathtimeandto emptyallbucketsandwading poolsimmediately.Allchildren shouldlearntoswim,andchildren andteensshouldwearlifejackets whilenearopenbodiesofwater. TeenscanlearnCPRandother watersafetyskills.”

Barriers Constant,CloseandCapableSupervision

SteenJamesMDFAAP LifeJackets ThereisNO EVIDENCEthat InfantSurvival SwimClasses Work

CPR

Aap.org/drowning

PSAwithNicoleHughes

https://www.youtube.com/watch?v=DQsro78hQC8 LayersofProtection • Questions? Barriers • Constant,Close, Capable Supervision • WaterCompetence • LifeJackets • CPR Outline Pediatric Oral & Dental y Tooth Basics Care for the y Caries Primary Care Provider y Prevention y Oral Pathology Sarah Kate Lee, DDS y Dental Trauma y Dental “Emergencies”

Basics Basics

y Anatomy of a tooth • Tooth Surfaces y Crown (portion seen in mouth) made of 3 layers – Incisal (anterior) & Occlusal (posterior) – biting/chewing surfaces y Enamel: outermost layer, white, strongest – Facial (anterior) & Buccal (posterior) – substance in the body, where cavities begin surface touching the lips & cheek y Dentin: middle layer, yellowish, cavities progress – Lingual – surface touching the tongue much more quickly – Palatal- surface towards the palate in y Pulp: blood and nerve supply. upper arch y When cavities reach this far then endodontics – Proximal – surfaces that are next to (root canal therapy) or extraction indicated each other y Teeth either hurt a lot or not at all depending on • Mesial – surface facing towards the health of pulp midline y Root (portion in bone, surrounded by periodontal • Distal – surface facing away from the ligament) midline y Root canal is extension of the pulp down the root • Cavities are most common – y Pulp is within the root and provides – Grooves on occlusal surfaces of communication to the body via blood supply posterior teeth within – On proximal surfaces where teeth touch (can only be cleaned w/ floss)

Primary Dentition – “Baby Teeth” “Do baby teeth really matter?” y Eating • Esthetics, social implications • Eruption of primary teeth y Preservation of facial form begins around 6 months of • Healthy teeth aid in development age and continues until 30 y Preservation of arch length for months permanent dentition • Order of eruption is also important

• First permanent tooth to erupt is the first molar – Erupts behind primary teeth around age of 6

• Primary teeth are replaced by permanent teeth Permanent Dentition – “Adult teeth” Caries Process

y Multifactorial disease that leads to the localized destruction of • Full permanent dentition hard dental tissues y Destruction of hard tissues by around age 12 the weak acids produced by • Third molars (“wisdom bacterial carbohydrate fermentation teeth”) tend to cause y Typically a slow process – symptoms in late teen remember the enamel is very strong! years to early adulthood y Left untreated, caries can lead • Order and timing are to tooth pain, infection, and/or abscess both important y Most common chronic • Mandible erupts prior to disease of children aged 6 to 11 years and adolescents aged maxilla 12 to 19 years • “Shark teeth” common y Elementary school students miss an average of 2.3 days/yr for dental issues

Caries Process Caries

Dr. Emanouela Carlson

Early Childhood Caries-ECC Dentoalveolar Abscess & Infection y Cause from caries, trauma, periodontal disease

y Systemic involvement (i.e. fever, facial swelling, asymmetry) warrant • Early childhood caries: emergency attention presence of more than one y Concern for risk for endocarditis, brain abscess, Ludwig’s angina decayed, missing (due to y Tx. indicated is extraction or root canal decay), or filled tooth therapy (only permanent teeth) – urgent surface in a child under 6 dental referral years old y Prescribe antibiotics • Severe ECC: any sign of y Amoxicillin and Clindamycin commonly smooth surface caries in a child under 3 years of age

Caretaker education and establishing a dental home is vital! Caries Risk Factors Prevention

y Caries is a 100% preventable disease! y Diet y Avoid sticky, starchy, sweet y Duration and frequency matter y Juice, sports drinks, and soda are dangerous! y Breast feeding vs bottle feeding y Establishing a dental home y First dental visit between eruption of first tooth & age 1

Prevention Prevention y Fluoride Recommendations y Toothpaste y FLUORIDE y Brushing should be supervised y Converts hydroxyapetite to fluoroapetite until child has manual dexterity y Fluoroapetite is 100 times less soluble! to tie their shoes or write their y Helps to slow demineralization name in cursive y 2x/day for 2 minutes each time y Promotes remineralization of tooth structure y “Smear” or “Grain of rice” – y Inhibits dental plaque bacteria metabolism younger than 3 years old y This reduces amount of acid produced y “Pea-sized” – 3 to 6 years old y Public water fluoridation y Optimal level 0.7ppm F (mg/L) y Supplementation y Safety y Toxic dose 5 mg/kg (10 kg child= 1.8 oz of 1000ppm toothpaste (2 travel size tubes)) y Lethal dose 32- 64 mg/kg

Prevention y Fluorosis y Occurs during tooth formation y White, opaque discoloration of enamel y Typically scattered around middle to incisal 1/3 of tooth Oral Pathology y 84.5% of people unaffected in optimally fluoridated areas y Vast majority of cases are mild Neonatal White Spot Lesions Aphthous Ulcer (canker sore) y Ulcerative appearance primarily and when matured y Bohn’s nodules y Epstein pearls y Dental Lamina y Painful, self-limiting, no systemic manifestations y Mucus gland tissue y Trapped Cysts y Common locations: buccal mucosa, floor of mouth, oropharynx, vestibule, tongue present on maxillary epithelial y Trapped y One to few lesions present at a time typically alveolar ridge remnants on epithelial y Idiopathic, but can be associated with systemic disease: Bechet, Celiac, Crohn, midpalatal raphe remnants on Neutropenia, Immunodeficiency syndrome, GERD y Present in 80% alveolar ridge y Treatment: Palliative, avoid trauma to area, topical steroid of newborns

No treatment indicated. Resolve spontaneously.

Herpetic Lesion Primary herpetic gingivostomatitis y Vesicle appearance primarily and ulcerative (shallow, punctate) when mature y Caused by HSV-1 y Common locations: attached gingiva, hard palate, vermillion border y Most common under age 5 y Few to several lesions present at a time typically y Presents with fever, lymphadenopathy, headache, malaise, y Caused by HSV-1 intense gingival erythema, painful oral vesicles throughout y Treatment: palliative, systemic antiviral (valacyclovir) agents if within 72 hours mouth y Treatment: systemic acyclovir, valacyclovir may be warranted, palliative care

Geographic Tongue Natal Teeth

y Benign migratory glossitis y Mineralized tooth-like structures present at birth or shortly thereafter y Usually asymptomatic, but may have tingling or y 90% are the primary incisors burning sensation y Tx: Remove teeth if they are interfering with feeding or y May disappear and highly mobile and an aspiration risk reoccur

y Tx: no treatment y If painful, can consider Candida infection Eruption Cyst/Hematoma y Red, purple gingival enlargement on the alveolar ridge y Can occur in primary or permanent dentition y Tx: None; resolve as tooth erupts y If symptomatic or causing delayed eruption, can make an Dental Trauma incision

Avulsions Intrusions

y PERMANENT TEETH: y Intrusions y Greatest chance of keeping tooth y Tooth pushed into the socket, which viable is replanting ASAP typically fractures as a result y Dry time of >60 minutes = no viable y No immediate tx. needed PDL cells y urgent referral to dentist for y Only grab tooth by the crown (white evaluation part) y Pain management y If dirty, rinse root with isotonic solution (Hank’s Balanced Salt Solution), milk, y Depending on extent on intrusion cold running water treatments include: y Reposition tooth in socket w/ firm y Waiting for spontaneous eruption or finger pressure extraction (primary teeth) y If unable to – store tooth in milk, saline, y Waiting for spontaneous eruption, or special storage media- NOT WATER! orthodontic repositioning, or surgical y Seek emergency dental treatment repositioning (permanent teeth) immediately y Tetanus status? NEVER replant a primary tooth

Dental Trauma y Fractures y Tx. based on extent of fracture y Only enamel exposed: smooth sharp edges y Dentin exposed: seal w/ glass ionomer y Pulp exposed: pulp capping and restore or extraction (primary tooth), pulp capping or root canal therapy and restore or extraction (permanent tooth) y Root fracture: extraction likely y Pain Management (no antibiotics indicated) y In ED: place dy-cal over pulp area, refer to see dentist ASAP Dental “Emergencies”

Eruption Teething Symptoms

y Pain during eruption y There is tooth growing out y Cavities are a possible the side of another tooth?! explanation, but pain in the back y Encourage child to wiggle especially if it’s in multiple areas of the mouth may be related to out the tooth eruption of first permanent molars y If refuse and causing pain, y Teething dentist can extract y Occurs w/ eruption of primary dentition (btwn. 6-30 mos.) and y Concern for decreased oral permanent molars (6 & 12 yrs.) hygiene in the area due to y Symptoms can include: drooling, pain rash (from drooling), pain y Teething does NOT cause fever! y Recommend cold washcloth, cool teething rings, ibuprofen or Tylenol

“Wisdom Teeth” Special Thank You! y Jaw pain posteriorly y Third molars or “Wisdom y Robert Steelman MD, DDS Teeth” y Can start erupting anywhere y Ian Bell DDS btwn. 15-21 years old y Most people don’t have space in their mouth for them (often impacted as a result) y Pericoronitis –gum inflammation around partially erupted tooth common y Proximity to Inferior Alveolar Nerve y Extraction recommended y Important consideration prior to chemo/radiation treatment (especially if IV bisphosphonates planned to avoid osteonecrosis of the jaw) Top Endocrine Cases Objectives Cheryl Hanna MD

z Puberty early and late z Growth throughout childhood z Thyroid function : elevated of Free T4

Pediatric Endocrinology visit Case 1 z EN is a 7y 4m girl referred for evaluation of early puberty z PMHx z Mother’s observations – Born small; 5lb 3 oz, 18 ¾ inches at 38 weeks – 6y 9m vaginal discharge, ? breast development – Genetics evaluation at 2y 10m for mild developmental delay and – 7y papules on face, Ĺhair in genital area ĺ dermatology dx: acne short stature z Pediatrician evaluation – Pediatrician w/u at 4y 3m for short stature – 17 OHP 244 ng/dl, Total Testosterone 34 ng/dl, normal thyroid function • Bone age 3 proximally, 4y 2m distally – Referral pediatric endocrinology • Genetics report – not Turners – complex chromosomal rearrangement of unknown significance • IGF 1 89 ng/ml (32-179), IGF BP3 2.9 mg/L (1.7-4.9)

Pediatric Endocrinology visit

z PMHx – Born small; 5lb 3 oz, 18 ¾ inches at 38 weeks – Genetics evaluation at 2y 10m for mild developmental delay and short stature – Pediatrician w/u at 4y 3m for short stature • Exam- no puberty • Bone age 3 proximally, 4y 2m distally • Genetics report not Turners • IGF 1 89 ng/ml (32-179), IGF BP3 2.9 mg/L (1.7-4.9) z FHx: Mother 63 in, Father 69.5 in Target height 63 3/4in Brother 50% z Shx: Mother from Albania, shy but doing well in school

OK135S058 Pediatric Endocrinology visit Puberty Features Breasts Pubic hair z Physical Exam at 7y 4 m Accelerated growth – Ht 122 cm, weight 26kg – General not dysmorphic – Pubertal exam • Tanner III-IV breast • Tanner III pubic hair –Skin • Mild acne, increased hair on legs

OK135S058

Differential Diagnosis z 7 y 4m old girl with early puberty Pediatrics (2009) 123 e932 – Normal early puberty – Central precocious puberty – Mild congenital adrenal hyperplasia advancing 1991-1993 bone age to the biologic time for puberty 2095 girls 5.6-20years – Adrenal or ovarian tumor producing androgens 2006-2008 and estrogens 1100 girls

Examined by palpation

Early puberty : when to evaluate The interval from B2 to menarche increased from 2.5 years to 3.3 years z EARLY – Boys younger than age 9 – Girls with breast or pubic hair development before • age 7 (white) • age 6 (African American) – Older girls (6/7 to 8 years) • rapid progression of puberty • rapid bone age advancement • new CNS findings • emotional state adversely affected Premature Adrenarche Premature Adrenarche Clinical signs of male androgen production (pubic hair, body odor, acne) without Clinical signs of male androgen production (pubic hair, body odor, acne) without signs of true puberty (no enlargement of the penis, testis or breast development) signs of true puberty (no enlargement of the penis, testis or breast development)

Idiopathic Mild CAH First sign of real Clinical features: puberty tall for family mildly advanced BA Associated with: Exposure to topical Metabolic syndrome testosterone Obesity Mild CAH 21 hydroxylase deficiency Insulin resistance AM 17 OHP<100ng/dl rules it out FHx Type 2 diabetes 17 OHP >1000ng confirms dx SGA Adrenal tumor

Evaluation

z Bone age: 10 proximally, 10 ½ distally Puberty Features Breasts z Labs Pubic hair – LH 7.9 mIU/ml ( prepubertal <0.3) Accelerated growth – FSH 7.6 mIU/ml (prepubertal <4.2) Advanced BA z Conclusion: she is in central puberty z Potential explanations: Could premature adrenarche have advanced BA and started normal puberty??

OK135S058

Evaluation Treatment z Bone age: 10 proximally, 10 ½ distally z Treated central puberty z Labs – LHRH agonist – LH 7.9 mIU/ml ( prepubertal <0.3) • Estrogen is advancer of BA – FSH 7.6 mIU/ml (prepubertal <4.2) • More effective in younger girls CAH work up z Did not treat mild CAH – AM 17 OHP 244 ng/dl – Treatment Ĺ risk of adrenal insufficiency

– 17 OH post ACTH 1233 ng/dl – Over treatment may stunt growth – Cortisol post ACTH 26 mcg/dl – Parents fearful of steroids Significant past history Case 2

z FL age 14y 8 m referred to pediatric endocrinology z 5 y crampy abd pain during/post eating for short stature and delayed puberty • w/u age 10 negative H pylori, fecal calprotectin z PMHx • w/u age 13 normal endoscopy – Birth history • Miralax helps with constipation • 7lb 8 oz product of a term pregnancy z • 2 days in NICU for meconium aspiration ADHD treatment started second grade • No hypoglycemia or jaundice • Gained 5 pounds in last several weeks off medication

Family History Exam: TH Not dysmorphic z Mom 5’5” BP 107/65 GU tanner II – menarche at 16-17 Testes 3-4 ml – Irritable bowel syndrome – 2 maternal relatives with inflammatory bowel disease z Dad 5’7” – Normal puberty z Sister ADHD

Constitutional Growth Delay Etiology of Delayed Puberty

Constitutional Growth Delay Chronic illness Endocrine disease which delays bone age Failure of the hypothalamic pituitary gonadal axis

OK135S057 Constitutional Growth Delay

Positive Family History

Delayed BA

Delayed puberty

Chronic illness and hormone deficiency ruled out

OK135S057

J Child Psychology Psychiatry:58;663 (2017) Growth in Children on ADHD medications J Child Psychology Psychiatry:58;663 (2017)

z Observational long term follow up – 515 ADHD (age 7-10) – Treatment monitored to age 18

z 289 classmates without ADHD (LNGC) Observe height z Height at age 25 z Conclusion: extended use of medication associated with suppression of adult height Height Z score Failure of the Hypothalamic Pituitary Gonadal Axis Laboratory evaluation z Hypothalamic Pituitary Dysfunction z Age 14 – LH/FSH deficiency- isolated as in Kallmann’s – freeT4 1.36, TSH 1.66 syndrome or as part of hypopituitarism – Serum IgA 118, TTg 0 – Hyperprolactinemia- prolactinoma or medication induced z Age 14 ½ – Functional deficiency due to calorie – BA 12 ½ to 13 ½ insufficiency or excessive exercise z Age 14y 8m z Gonadal Failure – Females: Turner syndrome, oophoritis, – IGF 1 316 ng/dl (156-554) galactosemia, chemotherapy, XX or XY – LH 2.2 (prepubertal <0.3), FSH 3.8 gonadal dysgenesis – Testosterone 67 ng/dl (Tanner II 18-150) – Males: vanishing testis syndrome, chemo or radiation

Bone age

Exam: TH Not dysmorphic BP 107/65 GU tanner II Testes 3-4 ml

Case 3 Conclusion AV is a 15 year old girl referred for evaluation of abnormal thyroid function tests discovered in a work up z Most likely constitutional growth delay – Bone age non specific test for fatigue – At a bone age >12 ½ should be in puberty; exam and labs suggest he is – No idea about tempo, could be partial gonadotropin deficiency Date Free T4 (.58-1.64) TSH (0.5-4.3) z No lab evidence for growth hormone or thyroid hormone deficiency z Are ADHD meds responsible? Does he have a hidden GI illness? 4/11/2019 4.4 0.58 z Plan: observe progress in growth and puberty over next 6 months 4/20/2019 5.71 0.94 z Seen 4/26/2019 z Exam: height 154.6 cm, weight 63.5 kg, BMI – Fatigue since September 2018 26.6 BP 119/49 • Often naps after school • Always exhausted – General- well appearing, no tremor, no sweaty – Sleeps well at night time hands – Heavy periods since age 10 ½ started on ocp 4/11/2019 – HEENT: no exophthalmos, no thyromegaly – Often hot – Tanner V – Lightheaded when stands – Neuro: DTRs 2+ – No increased appetite, no racing heart beat – Biotin supplement for 1 year recommended by hair dresser z Family HX negative for thyroid disease

Interpreting Thyroid Function Tests Elevated Free T4 (free T4 5.7, TSH 0.94)

z Elevated free T4 and low TSH think hyperthyroidism z TSH should be actually low in hyperthyroidism z Low free T4 and elevated TSH think z Could this be thyroid hormone resistance? primary hypothyroidism z Could this be an abnormality of thyroid binding z Low free T4 and normal TSH think hypopituitarism or non thyroidal showing up in the particular “direct” free T4 assay? illness – Birth control pills with estrogen raise TBG z Could something be interfering with these assays?

Signs and Symptoms of Hyperthyroidism Graves’ Hyperthyroidism: Epidemiology

z Goiter z Prominent eyes z n HR z Children 1:5,000 z Nervousness z Adults 1:500 z Sweating z Peak age 11-15 years z n appetite z Weight loss z Ƃ:ƃ = 5:1 z Deterioration in school z Labs: Ĺ Free T4, Ļ TSH z Emotional disturbance z Heat intolerance z Fatigue/breathlessness z Diarrhea z Thyroid hormone action defect – Thyroid hormone receptor beta gene defects – Increased T4 – Normal TSH

AV is a 15 year old girl referred for evaluation of abnormal thyroid function tests discovered in a work up for fatigue

z Interfere with endogenous thyroid function Date Free T4 (.58-1.64) TSH (0.5-4.3) z Interfere with thyroid hormone therapy 10/28/2016 1.37 0.92 4/11/2019 4.4 0.58 z Interfere with thyroid labs 4/26/2019 5.71 0.94 AV is a 15 year old girl referred for evaluation of abnormal thyroid function tests discovered in a work up for fatigue

Date Free T4 (.58-1.64) TSH (0.5-4.3) Free T4 Equilibrium dialysis (0.8-1.7) 10/28/2016 1.37 0.92 4/11/2019 4.4 0.58 4/20/2019 5.71 0.94 5/7/2019 >6 0.76 off biotin

AV is a 15 year old girl referred for evaluation of Free T4 by equilibrium dialysis abnormal thyroid function tests discovered in a work up for fatigue z Gold standard z Helpful in patients on medications known to interfere with thyroid labs Date Free T4 (.58-1.64) TSH (0.5-4.3) Free T4 Equilibrium dialysis z Helpful when things do not make sense (0.8-1.7) 10/28/2016 1.37 0.92 4/11/2019 4.4 0.58 4/20/2019 5.71 0.94 5/7/2019 >6 0.76 1.2 ƒ•‡͓ͳ

• CC:Abnormalanus

Peds ED Greatest Hits! • HPI:25doboywithpoorrectaltone,decreasedPOintake.Adoptivemomnoted rectalprotrusionafewdaysPTP,feelsitisgettingworse.Constanttrickleofstool, ...well, actually, misses… mustardyyellow,hasnotseenblooduntiltodaywhenshe'snoticedsomesmall well, actually, my misses… bloodfromareaofmucosalbreakdownonrightperianalarea • Novomiting.Feedingreducedsignificantlyinlast24h,usuallyeats3ozatatime, now1oz,stilleatingq3h.Nofevers.Nocoughing.Nosignificantnasalsecretions.

• NormalMRItoevaluatesacraldimple1dayPTP. Beech Burns, MD,MCR October 18th, 2019

ƒ•‡͓ͳ ƒ•‡͓ͳ

• PMH: • BP83/61 T36.7 HR130 RR48 SpO2100% • Csection.Nocomplications.Immunizationsutd.Fullterm • Gen:Nodistress.Interactive,suckingonpacifier • FH: • Head:NCAT,AFSF,scatteredpetechiaearoundeyesbilaterally • Maternalasthma • GU:normal • SH: • Skin:Smallulcerwithdenudedheadapproximately0.5cmdiameteratrightside • Liveswithadoptiveparentsandadoptedsiblings immediatelyadjacenttoanus.Abnormalappearinganus,noanalwink.LargelowͲ • ROS: lyingsacraldimple • Otherwisenegative

ƒ•‡͓ͳ Šƒ–‹•–Š‹•ǫ

• Rectalprolapse?

• Milkproteinallergy • Neurogenicdysfunctionrelatedtotetheredcord • Infection

• Whataboutthefacialpetechiae? Šƒ–•Š‘—Ž†™‡Šƒ˜‡†‘‡ǫ Šƒ–™‡†‹†

• CBC,BMP,Coags • Normal • Neurosurgeryconsultforabnormalrectaltone • Imagingreassuring.FollowͲupinclinic • PediatricSurgeryconsult • Closeoutpatientf/u,changeformulatohydrolyzed,barrierointment • Evaluatedbyhospitalistatbedside • Discharged

Šƒ–Šƒ’’‡‡†ǫ Šƒ–Šƒ’’‡‡†ǫ

• RTED23dayslater… • BP123/72 T35.9 HR128 RR48 SpO2100% • “7woboybroughtinforALTE.PatientandDadwerehomealone,patientwascrying • Welldeveloped,active,strongcry andinconsolable,Dadwenttochangehisdiaperwhenhefeltthatpatientwentlimp • Head:AFSF,somescalptendernessonleftside andunresponsiveforatleast30minutes.Daddeniesthathewaschokingorhad • Skin:Nobruisingnoted repetitiveshakingmovements.OnceMomgotbackpatientstillwasnotacting normally.Hiseyeswerepulledopenandtheywererolledupwards,Momtriedto openhismouthtocheckonhistongue(andmakesurehewasn'tchokingonit)and feltthathisjawwasclampedshut.911wascalled.”

‡ƒ† Šƒ–Šƒ’’‡‡†‡š–

• Neurosurgery,traumaconsult • AdmittedtoPICU • SCANteamrecommendedCBC,CMP,urinalysis,UDS • Socialworkconsult • DHSreportmade • ChildabuseinvestigationteamfromPortlandPoliceDepartmentcametoPeds ED Šƒ–Šƒ’’‡‡†‡š– Šƒ–‹•ƒDz•‡–‹‡Ž‹Œ—”›dzǫ

Intracranialinjury: Leftparietalskullfracture,bilateralsubduralhygromas,falcine subduralhematomas • Asentinelinjuryisaminorinjuryinayoungchildthatispoorlyexplainedand Scatteredsubarachnoidblood thereforeconcerningforphysicalabuse Concernforshearinjurynearcorpuscallosum

Skeletalinjuries: • Abusetendstogetmoresevereovertime Bilateralwristfractures Subacutemultisegmentalbilateralribfractures Rightproximalfemurfracture • Failuretorecognizeandtakeactionwhenrelativelyminor,suspiciousinjuriesoccur

Ocularfindings: mayhavedevastatingconsequencesfortheinfantandfamily. BilateraldiffusescatteredintraͲretinalandpreͲretinalhemorrhages Diffuseroth spotsscatteredthroughoutperiphery,botheyes

Abdominalinjury: Smallliverlacerationsx2

‡–‹‡Ž Œ—”‹‡• ‘••‹„Ž‡‡–‹‡Ž Œ—”‹‡•

• About30%ofchildrenwithAHTand20%ofabusivefracturesareinitiallymissed • Bruisesinunusuallocations • In2006study,30%ofchildrenwhodiedofchildabusehaddocumentedhealth • Bruisesinunusualpatterns carevisitsforreasonsotherthanroutinewellͲchildcareintheyearbeforetheir • Burns death • Bitemarks • 19%ofthesechildrenhadvisits1monthbeforetheirdeath • Intraoralinjuries • In2013studyof400casecontrols,27.5%ofdefinitelyͲabusedpatientshada • Fractures previoussentinelinjurycomparedwith0%ofnonͲabusedchildren • IndefinitelyͲabusedgroup,42%ofsentinelinjurieswereknownbymedicalprovider • In1999studyinJAMA,diagnosisofAHTmorelikelytobemissedinintact,nonͲ minorityfamilies

‘••‹„Ž‡‡–‹‡Ž Œ—”‹‡• Šƒ–ƒ”‡™‡Ž‹‡Ž›–‘•‡‡ǫ

• HighestRisk? • Fracture(rib)

• Mostcommon? • 80%bruise • 11%intraoralinjury • 7%fracture —– ‘‡ ‡›ƒ‡ƒ™ƒ›•ȋˆ‘”‡Ȍ

• 9monthwellchildcheck • Takeathoroughhistory,scrutinizeit

• Newadoptivefamilywith4 • Examinethepatientclosely biologicalkids,1fosterchild, 1exchangestudent,and2 • Detectionofsentinelinjuriesmaysaveachild’slife adoptedchildren

• Normalgrowthand development

ƒ•‡͓ʹ ƒ•‡͓ʹ

• CC:Rash • HR:106BP:122/82RR:22Temp:37.1O2Sat:99%RA • Gen:Wellappearing,welldeveloped,nonͲdistressed • HPI:3yr oldhealthyboywithrash.Started2daysagoaschappedlips.1dayago • developedaneckrash+crustinessonleftscalp.Parentsalsomentionmildswelling HEENT:Smallwhitepatchesontonsilswithmilderythema;nolesionsonbuccal onforeskinofpenis(improvingoverpastfewweeks).Sorethroat1dayago.Nonew mucosa;crustinginEACs.Earsarebotherythematousbutnottender.Perioral exposures.Nosignificantsunexposure erythema.3cmdiametercrustedlesiononLparietalscalpw/oerythema • GU:Penisnormal,noswellingorerythema • PMH:None • MSK:Patientholdsarmsflexedagainstbodyandresistsattemptstomovethem upwards;erythemainACfossaeandtheaxillaebilaterally;noaxillaryLAD • RelevantPSHx,Meds,Allergies,SocialHx: • Skin:Blanchingerythematousrashcircumferentiallyaroundtheneck,ontheears, • Nosurgeries,dailymedications,allergies,livesathomewithparents,nosibs andtheperioralarea

ƒ•‡͓ʹ Šƒ–‹•–Š‹•ǫ

• Cellulitis • Contactdermatitis • Scarletfever • Idreactiontotineainfection • Roseola • Seborrhea • Pharyngitis • StevensͲJohnsonSyndrome Šƒ–•Š‘—Ž†™‡Šƒ˜‡†‘‡ǫ Šƒ–†‹†™‡†‘ǫ

• Rapidstrepnegative,culturenegative(previousday)

• Received400mgPOTylenol+9mg(0.6mg/kg)PODecadron

• Observedwithnochangeinappearancebutimprovedpain.Dischargedhomewith 14daycourseofGriseofulvin.Returnforhighfever,n/v,decreasedmentalstatus

Šƒ–Šƒ’’‡‡†ǫ

• RTED2dayslater(day4ofrash):

• Rashworsearoundlipsandmouth(ulcerated).NowinvolvingperiͲorbitalregion. Extremediscomfort–refusingtoopeneyesormouthwithnooralintakein1day. Eyesnotredbutwithyellowdrainage.Sloughingrashinarmpits

• PhysicalExam: • VitalSigns:HR:107BP:107/62RR:22Temp:36.7O2Sat:99%RA • Skin:Skiniswarm,CR<2sec.Plaqueswithscaleandcrustingonneck,around mouth,behindears.Crustinginexternalauditorycanalswithsomedraining.Lipsare ulceratedandedematous.Desquamationpresent.Conj withoutinjectionbutlids matted,yellowdrainagebilaterally. • GU:Inguinalfoldswitherythematousmaculesandpapules Returnvisit

Šƒ–Šƒ’’‡‡†‡š–ǫ ‹ƒ‰‘•‹•ǫ

• CMP,CBC,lactateallreassuring • CRPandESRnormal • RapidStrep:Negative • Blood+PerioralSkinCulturesdrawn Staphylococcal Scalded Skin Syndrome!!

• GivenIVfluidsandMorphine

• ConsultedDermatology • Admitted ‹•ƒŽŽ‰”‘™—’ǥ™‡ŽŽǡƒ–Ž‡ƒ•–’‘––› —–‹•ǯ––Šƒ–ˆ‘”„ƒ„‹‡•ǫ –”ƒ‹‹‰ CasesofSSSSbyAge

700 658 • 2018study: 600 • 1259patientsbetween2011Ͳ 2016 500 • 84%ч4yearsold 400 Cases 

# 300 231 200 168 180

100 22 0 0Ͳ59days 2Ͳ11months 1Ͳ4years 5Ͳ10years 11Ͳ18years AgeGroup

—–™Šƒ–†‘‡•‹–Ž‘‘Ž‹‡‹‘Ž†‡”‹†•ǫ Šƒ–†‘‡•‹–Ž‘‘Ž‹‡‹‘Ž†‡”‹†•ǫ

• “Firstsignsaremacular “Thickcrustingand erythemaandskinpain,initially radialfissuringoften accentuatedintheskinfolds, developsaroundthe suchastheneck,axillae, mouth” inguinalfolds,andglutealcleft” “Thecrusting, • ‘Patientholdsarmsflexedagainstbody andresistsattemptstomovethem fissuring,and upwards;erythemainACfossaeand erythemacanbe theaxillaebilaterally’ strikingandis classicallyreferredto asSSSS‘sadface’”

Patel,G.K.&Finlay,A.Y.AmJClin Dermatol (2003)4:165. doi:10.2165/00128071Ͳ200304030Ͳ00003

—–™Š›†‘‡•‹–Ž‘‘†‹ˆˆ‡”‡–ǫ Šƒ–ƒ„‘—––Šƒ–ˆ—‰ƒŽ‹ˆ‡ –‹‘ǫǫ

• “ProtectiveantitoxinAbsin • ComorbiditiesforSSSS somechildrenandmostadults limitsthelesionstoafew localizedblistersinmilder forms,whereaslackofAbsin generalizedSSSSallows hematogenousdisseminationof ETtoproduceexfoliationthat maycovertheentirebody surface.”– “Difficultiesin diagnosisandmanagementof theSSSS”inPIDJ2000 —– ‘‡ ‡›ƒ‡ƒ™ƒ›•ȋˆ‘”‡Ȍ

• Derm recs: • IVAncef(100mg/kg/daydivq8)+IVClindamycin(30mg/kg/daydivq8) • Staphscaldedskinsyndromeisnotstrictlyaneonataldisease • Vaselinetoaffectedskinareas (childrenunder4mostcommon)

• Ophtho recs: • Nocorneal/conjunctivalinvolvement • Polytrim tolidmarginsTID • Thepresentationmaybemoresubtle…skinpainisakeyinitial • WarmorColdCompresstobreakupeyelidcrust feature • 2daysafteradmission: • Rashimproving–SkinCx:MSSA • AbxnarrowedtoIVClindamycin • Akidwhowon’tshowyouhisarmpitshasSSSSuntilproven • 4daysafteradmission: otherwise…orhe’sticklish… • DischargedonPOKeflexx10days • Polysporin BID(Eyelid)+Mupirocin/VaselineTID • Ketoconazoleshampoodailyx2weeksthentwiceweeklyasneeded

ƒ•‡͓͵ ƒ•‡͓͵

• CC: Abdominalpain,hypoxia • PMH: • Healthy,nohospitalizations,chronicmedproblems • HPI: 5yo boytransferredfromOSHwithconcernforabdominalpainandhypoxia. N/V/Ddeveloped5daysPTP.Tactilefeverdaily.Vomitinganddiarrhearesolved, • RelevantPSHx,Meds,Allergies,SocialHx: thencongestion,coughdeveloped.Today,familynotedincreasedrespiratoryrate, • Liveswithparents workinghardertobreathe.TakentoOSHED.There,febrileandhypoxic.Startedon • Imm: 3LNC.CMPwithNa128,K3.2,Cl88,AP86,AST58,ALT48.CBCwithWBC26.8K, • UTDperreport bands49%

Šƒ–‹•–Š‹•ǫŠ›‹•–Š‹• Š‹Ž†Š›’‘š‹ ǫ ƒ•‡͓͵ Š‹•†‘‡•ǯ–•‘—†Ž‹‡ƒ’’‡†‹ ‹–‹•ǥ VitalSigns:HR:124BP:96/60RR:40Temp:38.4O2Sat:88% Constitutional:Listlessyoungboyinmoderaterespiratorydistress • Bacterialpneumonia CV:Tachycardia,nom/r/g • ComplicatedPNAwitheffusion Resp:Inrespiratorydistress.DecreasedAM,verydecreasedonright.+retractions.No rales,rhonchi,wheeze • Atelectasisduetosplintingfromabdominalprocess Abd:Soft,BSnormal.Nodistension.VeryTTPinRLQ,periumbilicalarea Neuro:Normal Skin:Norash,CR<3sec Šƒ–•Š‘—Ž†™‡Šƒ˜‡†‘‡ǫ Šƒ–†‹†™‡†‘ǫ

• Increasedoxygento4L

• GaveNSbolus

• ObtainedachestXͲray

ŠƒǨ‘—Ž†–Š‹•„‡’‡—‘‹ƒ ƒ•“—‡”ƒ†‹‰ƒ•ƒ’’‡†‹ ‹–‹•ǫǫǫ

Let’slookattheliterature!

• N=250kidsexaminedforacuteabdomenbetween1972Ͳ1975 • 12casesofPNA(4.8%) • Allwithpainsevereenoughtosuggestacuteappendicitis • N=1168 3hadappendectomies(!) 1986Ͳ1992,admittedwithabdominalpain

• “(Ourfindingsare)indeedastrongargumentforobtainingchestroentgenogramsonall childrenwhohavesymptomsofanacuteabdomen.” Backtoourcase…

• Cough?

• Retrospectivestudy. • Fever? • N=1613ptsunder12whogotKUBandCXR • 30casesofpneumonia(1.89%) • Allbut2hadfever,cough,orURIsymptoms • URIsymptoms?

‹ ‡–”›ǡ‹†Ǩ‘—Šƒ˜‡–‘™ƒ‡—’’”‡––›‡ƒ”Ž›‹–Š‡‘”‹‰–‘ˆ‘‘Ž‡ǥ

 ‹‡Ž‹‡‘ˆƒ”‡

• 5yo boywhopresentswithnausea,vomiting,anddiarrhea,followedby • 0023 T38.4HR124BP96/60RR40SpO288%3L developmentoffeverandcoughfoundonexamtohavedecreasedbreathsoundson therightandhypoxiaconcerningforbacterialpneumonia.Differentialdiagnosisalso • 0047 CXRordered includesviralpneumonia,pleuraleffusion,atelectasiswithabdominalpathology includingappendicitis.Patienthasmarkedleukocytosiswithbandemia concerning forbacterialinfection. • 0107 Ampicillin,NSbolusordered

• CXRobtained,whichrevealedrightlowerlobeandrightmiddlelobepneumonia. • 0130 HR115BP89/58RR42SpO298%on4L

• Oxygensaturationswere88%on3Lonarrival.Increased4Lwithincreaseinoxygen • 0238 T37.1HR112,patientreportsbellyfeelsbetter. saturationsto98%.Withoxygenrequirement,tachypnea,signsofdehydration, DecreasedWOB.Awaitingadmission. patientwarrantsadmissionforfurthercare.WegaveampicillinIV×1dose.Wealso gaveanormalsalinebolus.Givenmildhyponatremia,hypokalemia,and • 0306 Admittedtoward hypochloremia,electrolytesshouldbefollowedonadmission

‘–Š‡”Œ‘„™‡ŽŽ†‘‡ǥ ‘ŽŽ‘™‹‰ƒ†‹••‹‘

• 0837 Radiologycallsinpatientteamafterreadingthe film…

• Meatthenurse’sstation… Impression:Extensiverightlowerlobeconsolidationisnotedwithadjacent pleuraleffusion.Leftlowerlobeatelectasisalsopresent.Bilateralairway thickeningandlowlungvolumes.Ovoidloculated gasprojectsovertheliver notdefinitivelywithinbowel.Ifthereisconcernforbowelperforationor abscess,consideraleftlateraldecubitusview. Šƒ–Šƒ’’‡‡†‡š–ǫ • • • Surgery Started RLQ  US LLDecub Film ACloserLook…   Zosyn consult ƒ†‹‘Ž‘‰›‡ƒ†ǣ • • ǣ lower Findings Pneumoperitoneum, IMPRESSION • • • tubular appendicitis noncompressible, Impression: Tubular 4.9  x 

quadrant OutsideFilm  3.6  are   structure structure  x   concerning 2.3   Consistent with   pain. thick   adjacent  11mm 10mm   gaseous walled  for  with   in  fecalith perforated    mass, abscess diameter,   bowel perforated  centered  distention   appendicitis   around  and   given multiple   history  air Ͳ fluid  of  fevers  levels.  and  right  Šƒ–ƒ„‘—––Š‡’‡—‘‹ƒǫŠƒ–ƒ  Šƒ–Šƒ’’‡‡†‡š–ǫ •—’’‘•‡†–‘–‡ŽŽ–Š‹•‰—›ǫ • Laparascopic appendectomy

• Omentum adherenttoright abdominalwall.Large abscessinrightpericolic gutter.Secondlargerabscess foundinthesuprahepatic space.JPdrainleftinplace.

• Empyemaandlungabscessascomplicationofaperforatedappendicitisinapregnant woman.Int JSurg CaseRep.,2012;3(12)

• Rightpostoperativepleuraleffusionfollowinglaparascopic appendectomies:acase Šǥ–Š‡‘Ž†”‡ƒ –‹˜‡Ǧ’Ž‡—”ƒŽǦ series.AnnRColl Surg Engl.,2010;92(5) ‡ˆˆ—•‹‘Ǧ•‡ ‘†ƒ”›Ǧ–‘Ǧ • 3consecutivecases,allwithrupturedappendix • Empyema.Ararepresentationofperforatedappendicitis.JAMA,1978;240(23) ƒ„†‘‹ƒŽǦƒ„• ‡••–”‹ ǥ • 2cases–50yo woman,5yo boy

Q:Howcommonisthis? ǣ‘–˜‡”›ǥ

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• POD#0:PICUformonitoring 1.Reviewallavailablediagnostics • POD#7:Recurrentfevers.CT abdomenshowed2recurrent 2.Phoneyour(radiology)friends abscesses.IRdrainedand percutaneousdrainleftin 3. Thinkhorses…thenzebras… place. thenthinkaboutsomeanimalyou’ve • POD#15:DischargedwithIR neverheardof… draininplacewithplanfor clinicf/u.Dischargedon 4.Practicehumilityinallthings Augmentin. ‡ˆ‡”‡ ‡•

• SheetsLK,etal.SentinelInjuriesinInfantsEvaluatedforChildPhysicalAbuse. Pediatrics2013;131:701Ͳ7 • KingW,etal.ChildAbuseFatalities:AreweMissingOpportunitiesforIntervention? PediatricEmergencyCare2006;22(4):211Ͳ4 • Staiman A,etal.EpidemiologyofStaphylococcalScaldedSkinSyndromeinU.S. Children.BritishJournalofDermatology2017;178(3):704Ͳ8 • NeubauerHC,etal.VariationinDiagnosticTestUseandAssociatedOutcomesin SSSSatChildren’sHospitals.HospitalPediatrics2018;8(9):530Ͳ7 • Jona JZ,etal.BasilarPneumoniaSimulatingAcuteAppendicitisinChildren.Archives ofSurgery1976;111 Questions? • HomierV,etal.PrevalenceofPneumoniainChildrenunder12yearsofagewho    Ǩ UndergoAbdominalRadiographyintheEmergencyDepartment.CanadianJournalof EmergencyMedicine2007;9(5) Multi-disciplinary clinics within pediatric otolaryngology

X 1. Craniofacial clinic (Cleft lip and palate, craniofacial abnormalities )

X 2. Aero-digestive clinic (Pediatric ENT, Pulmonary, Speech and Feeding, GI)

X 3. Vascular anomalies (Pediatric ENT, Dermatology, Interventional Radiology)

Challenging Pediatric X 4. Voice clinic (Pediatric ENT, Voice therapy) Otolaryngology Cases X 5. Hearing loss clinic (Pediatric ENT, Speech, and Audiology)

X 6. Thyroid clinic (Pediatric ENT, Endocrinology, Radiology) Monica Deshpande, APNP, Department of Pediatric Otolaryngology, [email protected]

Case 1. – Vascular anomalies How do you treat her? X 3 year old girl with a known lymphatic malformation on her neck comes in to see you in clinic with increased pain and swelling A. No treatment, it will get better on alone on neck. It has doubled in size and she B. Order an MRI or CT on patient also has a URI symptoms (fever, cold). C. Refer to ENT immediately D. Tx with antibiotics X No difficulty breathing. Otherwise stable. E. Treat with antibiotics and steroids

Lymphatic malformations Lymphatic malformations-

X Lymphatic malformations are collections of dilated lymphatic channels which can vary widely in terms of their size and age of presentation X Can get infected very easily, especially with onset of sickness X Short term treatment is to treat infections with both antibiotics and steroids (usually 2 weeks abx, 5 days steroids at 2 mg/kg/day) X Later treatments can include sclerotherapy, surgery, and new treatments such as sirolimus

Figure 1: http://www.sickkids.ca/PlasticSurgery/What-we-do/Vascular-Anomalies-Clinic/Vascular- Malformations/Lympathic%20Malformations/index.html Case 2 – Hoarseness What is the most likely cause of her hoarseness?

X2 year old ex 28w preemie with a 1. Vocal cord nodules history of cardiac surgery(PDA ligation) 2. Vocal cord paralysis comes in with a chronic history of 3. GERD hoarseness and voice straining during a well child check up. Mom complains 4. Laryngeal cleft that she still tends to cough and choke with liquids.

Vocal cord paralysis Vocal cord paralysis

X 1. Most commonly associated with cardiac surgery, prolonged intubation, thyroidectomy, and TEF repair. X 2. Weak cry in infants X 3. Difficulty feeding X 4. Breathy soft voice X 5. Tx include, voice therapy, surgery

Vocal cord nodules Vocal cord nodules

X 1. Most common cause of chronic hoarseness in school age children X 2. Boys> Girls X 3. Located at the junction of the anterior 1/3 and posterior 2/3 of vocal cords X 4. Develop from repeated trauma to vocal cords X 5. Voice therapy most indicated treatment- rarely surgery Case 3 1. Repeat swallow study (MBBS)

-15 month old comes in with choking with liquids 2. FEES (flexible fiberoptic laryngoscopy and and recurrent pneumonia. function endoscopic evaluation) – better than -Also with chronic cough and not gaining weight. MBBS in visualizing laryngeal function -Mom said symptoms are worse when lying down. - Has tried a trial of omeprazole with no help. - No stridor. No history of intubation. 3. GERD Testing –pH probe -prior MBBS at 6 months of age showed aspiration 4. Chest- xray (normal) What is the best type of test to order at this time?

MBBS (Modified Barium swallow study) What is the child’s diagnosis

X Inconsistent micro aspiration with the thin and X 1. Laryngomalacia nectar thick liquids. Resolved with nectar X 2. GERD- Plus thick liquids and pureed to dry soluble solid X 3. Laryngeal cleft foods. X 4. Vocal cord paralysis

Type I Laryngeal cleft Laryngeal cleft

Type I –extends to level of vocal cords Type II – extends below vocal cords into cricoid cartilage Type III –extends to trachea/esophagus X Feeding issues Type IV – extends to level of trachea/esophagus X Failure to thrive X Recurrent pulmonary issues (aspiration) X Hoarseness X 75% will have aspiration on MBBS

Figure 2: http://www.laryngeal-cleft.com/What-is-laryngeal-cleft Treatment of laryngeal cleft Case 4

X 3 month female former preemie 32 week old presents with loud stridor and follow up from ED. X 1. Direct laryngoscopy to view airway X Was diagnosed with croup and RSV, but unresponsive to X 2. If cleft is present (type I, II), endoscopic treatment in ED. Mom said noisy breathing is worse when feeding. Seems to be getting louder. No history of repair considered intubation. X 3. Surgical repair outcomes favorable for X Gaining good weight cessation of aspiration X No wheezing X 4. Repeat swallow study in 3 months X Chest x-ray is normal X Swallow study normal

What is the cause of her stridor? Case 4

1. Laryngomalacia X ENT consulted to do beside scope – No evidence of laryngomalacia 2. Foreign body X Taken back for airway evaluation (MDL, Bronch) 3. Airway hemangioma 4. Vascular ring

Airway hemangioma Presentation of airway hemangioma

X More common in preemie, Caucasian, F > M X Stridor occurring around 6-8 weeks of age- Figure 3 https://www.sciencedirect.com/science worse with feeding /article/pii/S0030666508000844 X Other hemangiomas in “beard” distribution Airway hemangioma Case 5 – Voice clinic

X Treated with propranolol 2mg/kg/day divided tid X 14 year old presents with shortness of breath with activity till 6 months of age, then can go to bid dosing- that began 2 years ago. must give with feeding X Worse with activity. She is a competitive soccer player and began experiencing symptoms when starting more X Stay on this dose till one year of age –PCP can competitive play adjust X She is a straight A student, highly motivated X Symptoms resolve (stridor) in one to two weeks- X Keeps her from performing sport, has tried albuterol no need for f/u airway exam. inhaler given by PCP for exercise induced asthma, but not helping

Exercise induced laryngeal obstruction What we do in Voice Clinic…

X Usually in Adolescent females involved with competitive X Seen by Voice/Speech Therapist first who does flexible sports laryngoscope ( may re-create symptoms by running up and X Anxiety and high stress commonly noted down stairs) X X Majority treated with albuterol inhaler although PFT were ENT evaluates scope with Speech (looks for an airway abnormalities that may be causing stridor- webbing, normal. nodules, stenosis, etc.. X Come up with a treatment plan for the patient and family

Vocal cord dysfunction Case 6

X Misdiagnosed as Asthma X 2 year old girl, enlarging neck mass for 3 weeks X Triggered by exercise, stress X Non-tender X Co occurs with asthma – 50% X No associated symptoms X Responds very well to voice therapy (preventative and interruption technique) X Healthy child X NO cats, one dog X Sensation of throat tightness, sudden onset, trouble breathing in, and stridor on inhalation X Non-responsive to inhalers X PE: Afebrile, X Left submandibular mass -2.5 X 2 cm, starting to turn purple and has had some drainage Atypical mycobacterial lymphadenitis

X 2-5 years of age; rare > 12 years X Otherwise healthy X Fish, turtles, birds X Painless mass – overtime skin changes X Submandibular X Usually unilateral X > 3cm in 80% What is this neck mass? X Onset over weeks Labs: Bartonella, PPD X 35-40% suppurate

Treatment of NTM (non-tuberculosis Case 7 mycobacteria)

X 8 year old boy comes in for a well child - Usually no need for imaging check with a known left sided - Surgical excision better than FNA SNHL(sensorineural hearing loss). He has - Surgical excision has a 96% cure

- About 67 % respond to antibiotics (clarithromycin + rifampin(lots of recently moved and tells you he feels like choices) his hearing has changed. - (takes 12 weeks to respond to abx and need monitoring)

- Could go away without any treatment in 12 months (observation) After reviewing his records, You find the reason for his hearing loss is an enlarged vestibular aqueduct found on MRI.

Enlarged vestibular aqueduct (EVA) Enlarged Vestibular Aqueduct

X 40 % of kids with EVA with experience progression of hearing loss over time, on one side or both X Head trauma may cause symptoms to worsen- controversial X Accounts for about 23% of unilateral hearing loss X Can have issues with balance and dizziness Unilateral hearing loss Immunizations for cochlear implants – CDC recommendations X 59% of children with unilateral hearing loss can have academic or behavioral problems- speech, etc X Infants below 2- Prevnar 13 routine X Children (between 2nd and 6th birthday) – two doses of Prevnar 13 if they have X Preferential seating not gotten PCV 7 or 13 previously. If they finished PCV 7- one dose of PCV 13

X FM system X Between ages 6 -18 – single dose of Prevnar 13 regardless of whether they received PCV7 or PPSV23. X Keeping other ear healthy- monitoring for ear infections X HA use (binaural hearing) X In addition all children age 2 years and older who have completed the Prevnar X Regular audiograms series should receive one dose Pnenumovax 23 (PPSV 23). Wait at least 2 months after last dose of Prevnar to receive Pneuomovax23. X Some may be candidates for cochlear implants

Case 8 What to do?

X 2 year old with a history of recurrent ear X 1. Put her on an oral antibiotics infections. She had tubes placed 6 months ago by X 2. Get an ear culture and start antibiotic ear drops ENT. She presents to your office with a draining X 3. Refer back to ENT ears. You start on her on ear drops and drainage X 4. Do not treat goes away. She is back in your office again a month later with draining ears.

Clinical practice guidelines- American academy of otolaryngology

X Topical antibiotic eardrops only, without oral antibiotics, for children with uncomplicated acute tympanostomy tube otorrhea

Figure 6: https://pediatrics.aappublications.org/content/139/6/e20170667 References

X Campisi, E.S., Schniderman, J.E., Owen, B., Moraes, T.J., Campisi, P., (2019). Exercise-induced laryngeal obstruction: Quality initiative to improve assessment and management. International Journal of Pediatric Otorhinolaryngology, Advance online publication. doi.org/10.11016/j.ijporl.2019.109677.

X Elluru, R.G., Balakrishnan, K., Paudua, H.M., (2014)., Lymphatic malformations: Diagnosis and Management, Seminars in Pediatric Surgery. August: 23(4): 178-185.

X Evans AK, Cunningham MJ. Atypical mycobacterial cervical lymphadenitis in children: a disease as old as mankind, yet a persistent challenge. Am J Otolaryngol 2005; 26:337

X Kelchner, L.N., Brehm, S.B.& Weinrich, B.D (2014). Pediatric Voice, A Modern, Collaborative Approach to Care. San Diego, CA: Plural Publishing.

X Johnson, D.R, Watters, K, Ferari, L.R, Reza, R., (2014). Laryngeal cleft: Evaluation and Management, International Journal of Pediatric Otorhinolarngology, 78 (6), 905-911.

X Mathers-Schmidt, B. A., (2001). Paradoxical Vocal Fold Motion: At Tutorial on a Complex Disorder and the Speech- Language Pathologists Role, American Journal of Speech-Language Pathology, 10, 111-125.

X Shah, R.K, Harvey-Woodnorhth, G., Glynn, A, Nuss, R.C., (2006). Perceptual voice characterisitics in pediatric unilateral vocal fold paralysis, Otolaryngology Head and Neck Surgery, April, 134(4): 618-21/ Thank You X Steele, D. W., Adam, G.P, Di, Mengyang, et. Al, (2017), Prevention and Treatment of Tympanostomy Tube Otorrhea: A Meta-analysis. Pediatrics 139 (6

X Vila, P., Lieu, J.E., (2015). Asymmetric and Unilateral Hearing loss in Children. Cell tissue Research. July: 36 (1), 271- 278 Disclosures

IhavenofinancialrelationshipsorConflictsofInterest(COIs)todisclose

Top Cases in Pediatric Infectious Diseases (and lessons learned)

LouiseElaineVazMDMPH AssociateProfessorofPediatrics DivisionofPediatricInfectiousDiseases OregonHealth&ScienceUniversity

October 2019 Doernbecher Pediatric Review 2

Objectives It is really hard to be a PCP At the conclusion of this session, participants will be able to: Disclaimer:theseareweirdcases! 1. Describe an approach to the differential according to presenting signs and symptoms for common pediatric Imagineyouhadthesepatients–wouldyoudoanythingdifferently? complaints Whattools/resources/testsareavailable? 2. Discuss the diagnostics of challenging cases in infectious diseases 3. Describe emerging infectious diseases affecting patients in the Pacific Northwest RESPECT

Presenting Symptom: Headache HPI:

ම Several weeks of headaches; Cold around Christmas. ම PCP visit– social issues highlighted (mental health, drug use). ම “Headache is located across his forehead and is described as throbbing. He 14 year old male with several weeks of rates the pain at 9/10 currently, but 10/10 at night. The intensity of the pain waxes and wanes. He reports associated photophobia and hyperacusis. He also reports worsening headache. mild dizziness when walking. He denies any fevers. No nausea or vomiting. ම “He is very argumentative during our visit saying that he is just going to sleep and not go to school and he is going to eat whatever the hell he wants.” ම ED: Non contrast Head CT normal; labs normal ම Initial diagnoses: Acute non-intractable headache vs. Tension type Pertinent History 1 day prior to arrival • ED: “Headache is throbbing, anterior, and equal bilaterally. Pain has been මPMH: Type 1 DM (poorly controlled A1c 10.6); temporarily alleviated with use of ibuprofen and tylenol at home. Today he reports his depression; obesity; immunized; pain is uncontrolled despite use of ibuprofen around 1:30. The patient reports associated nausea with one episode of vomiting” මFMH: Crohn’s disease, stroke (father), Substance • Concern about exposure to antidepressants at the patient's mother's house. DHS is abuse (mother); Autoimmune (diabetes, thyroid) – involved. maternal side • No recreational or other substance exposure has been confirmed. මROS: No focal neuro deficits noted; මNo fevers/vomiting/rashes/cough Dx: Acute non-intractable headache, unspecified headache type

Challenging case Day of admission (January) Worseningheadache ම Parents report: child was asleep most of day and then woke up for dinner Vomiting/Nausea ම Began having tingling in hands and fingers Photophobiaorothersigns ම Rapid neurologic change: incomprehensible speech and not following commands appropriately. Didn’t know where he was ම To ED – concern for ingestion Couldthisbeamigraine? CTisnegative ම Exam: dilated pupils, combative, afebrile Neurovisitismonthsaway ම CTA: No official report - verbal was that there were no concerns for stroke. Socialoverlay ම Intubated for LP: CSF - R 2750 W: 680 L: 71 M:9 N:20; Glc: 108 Protein IncreasingnumberofEDvisitsÆ thingsnotgettingbetter 145; Meningoencephalitis panel negative. ම Transfer to DCH PICU for altered mental status

Differential? Meningitis Encephalitis Sphenoid sinusitis Brain abscess Other brain lesion

Tunkel AR et al. Practice guidelines for the management of bacterial meningitis. Clin Infect Dis. 2004 Nov 1;39(9):1267-84. Tunkel AR. The management of encephalitis: clinical practice guidelines by the Infectious Diseases Society of America. Clin Infect Dis. 2008 Aug 1;47(3):303-27. Exposures and Social History Exam following transfer • Lives: Recently in Southern Oregon: lived near woods with known ticks. Moved back up with father T 39.7 C P 65 R 20 BP 110/58 SpO2 100% and stepmother in Eugene (town). Intubated, heavily sedated • Recreation: Camping in southern Oregon. Neuro/Psych: ම Becomes agitated and combative with exam, not oriented • Food: No raw or uncooked meats; no hunting; likes to garden, particularly, tomatoes. ම When disturbed very agitated, opens eyes, no redirectable, beats hand on bed, not cooperative ම Normal reflexes • Animals: 2 cats, multiple dogs. Exam otherwise normal • MRSA/TB/HSV: Stepmother + HSV cold sores, none in past 2 months. No foreign travel. Outside Non contrast CTA: No extra-axial fluid collections. No parenchymal or subarachnoid hemorrhage. No midline shift or mass effect. There is an area of nonspecific hypoattenuation within the inferior left frontal • SDRR: Not sexually active, smoked marijuana, none in past month; Mom and her boyfriend smoke a lobe. This measures 4.5 x 2.1 cm in size. lot. Additional small focus of hypoattenuation is seen within the anterior left frontal lobe on axial image 20. A small focus of hypoattenuation is seen in the right parietal cortical region on axial image 26. • Mental Health: Recently removed from mother’s home due to maternal substance abuse (alcoholism No morphological abnormalities of the ventricles. The sellar and pineal regions are unremarkable. No and MJ); sister attempted suicide with Benadryl overdose abnormalities of the basal cisterns.

Brain MRI Multifocal rim-enhancing circular lesions. Largest at the left inferior frontal lobe. Mostly at grey-white matter junction Left lateral ventricular intraventricular cyst Mild diffuse leptomeningeal enhancement

What the non contrast CT missed

Chest imaging: LLL PNA Differential Infectious ම Neurocystercercosis ම Toxoplasmosis ම Fungal/mold ම TB ම Echinococcosis ම Bacterial abscesses

Neoplastic ම Lymphoma ම Metastatic lesions from unknown primary site

Autoimmune What antimicrobials would you start? Additional studies Vancomycin, ceftriaxone, and liposomal ම LP: Opening pressure: 52 cm WBC 594 (47% PMN), RBC 4, P79, amphotericin B G: 21 ම Meningoencephalitis panel: negative Other considerations: ම EVD was placed for high ICP Acyclovir; doxycycline ම Right frontal endoscopic approach for biopsy of right frontal ventricular lesion RIPE or empiric treatment for toxo or ම Findings: Small pink exophytic mass; Not a cyst/larva/worm; neurcystercercosis? Touch prep revealed abnormal cells steroids?

Microbiology Hospital course Amphotericin B (5 mg/k/day) and 5-FC ම Broad Range PCR: Amplified something with fungal primers (ultimately Full neurologic recovery by day 7 of amphotericin unable to further identify) ම EVD removed at this time ම Day 4: CSF CrAg 1:10 Serum CrAg 1:2560 Complicated by acute kidney injury: Serum creatinine: 0.56 Æ1.63 ම Path: Yeast forms identified in specimen B, most compatible with Cryptococcus neoformans Remained inpatient for entire 6-week course of AmphoB ම Fungal CSF culture: Cryptococcus gatti Molecular subtype: VGIIa Discharged on fluconazole; has chronic kidney disease (Stage 3) ම – took several weeks to get this;

Outpatient Course Imaging considerations Has been on fluconazole for over 1.5 • CT:Inpatientswithneurologicsymptomssuchasmoderateorsevereimpairmentof years. consciousnessorneurologicdeficits(notincludingcranialnerveabnormalities),performingCT beforelumbarpunctureisrecommended. MR this summer: There is continued regression of multiple contrast enhancing foci involving the Æ considercontrastCTtoruleoutabscess supratentorial brain. Plan: continue until completely • MR:essentialindetectingcomplicationofmeningitissuchasvenousthrombosis,smallvessel resolved infarct/ischemia,cerebritis,ventriculitis,subdural/epiduralempyema,andvasculitis,andto discerntheetiologyandrouteofspreadofinfectiousmeningitis. • AdvancedMRItechniquessuchasmagnetictransfersequence,diffusionͲweightedimaging (DWI),diffusiontensorimaging(DTI),MRspectroscopy,andperfusionimaginghavesignificantly contributedtotheevaluationofmeningitiscomplications

TopicsinMagneticResonanceImaging.23(5):315–325,OCTOBER2014 Despite fancy diagnostics, there are still challenges Meningoencephalitis Panel

1. Make sure you know which organism are on your Date WBC RBC Glucose Protein M/E Fungal CSF Serum institutional panel culture CrAg CrAg 2. Know what is NOT on the panel (GAS, Staph aureus, other Virus Bacteria Other 1/6 680 2750 108 145 Negative gram negatives) 3. Know when the ME panel is most helpful CMV E.Coli Cryptococcus 1/8 594 4 21 79 Negative C gattii - Pretreated Bacterial Meningitis VZV H.Influenzae 1/9 20 1333 1:10 - Entero/parechovirus Enterovirus Listeria m. 1/13 1:2056 - When it is not helpful: HHV6 HSV 1/2 Group BStrep 4. Know there are concerns regarding false negative and HHV6 S. pneumoniae 1/29 36 2 89 104 Negative false positive results, particularly with HSV. Parechovirus 2/20 20 1340 109 58 1:40 - Obtain alternative testing

Rememberallthevariousdiagnostictoolsavailabletous:serologies,culture,broadrangePCR,pathology!

BardJDandAlbyK.PointͲCounterpoint:Meningitis/EncephalitisSyndromicTestingintheClinicalLaboratory.JClin Microbiol.2018Apr;56(4):e00018Ͳ18. Radmard Setal.ClinicalUtilizationoftheFilmArray Meningitis/Encephalitis(ME)MultiplexPolymeraseChainReaction(PCR)Assay.FrontNeurol.2019;10:281. Leber etal.MulticenterEvaluationofBioFire FilmArray Meningitis/EncephalitisPanelforDetectionofBacteria,Viruses,andYeastinCerebrospinalFluidSpecimens.JClin Microbiol. 2016Sep;54(9):2251Ͳ61.

New emerging infection: C. gattii • First identified in Oregon in 2004 • Travel history to: British Columbia, Washington and Oregon. • Disease in healthy, immunocompetent persons and immunocompromised. • 65 cases occurred among Oregon residents in 2017. Officially reportable in Oregon since 2011. • Association: decaying wood, especially Douglas Fir in the Pacific Northwest (deBess 2014). Similar climatic areas are found in Oregon’s Willamette Valley. • Concern for expansion: increase of logging in the coastal temperate rain forest zone

DeBess E et al. Isolation of Cryptococcus gattii from Oregon soil and tree bark, 2010-2011. BMC Microbiol. 2014 Dec 21;14:323. https://www.oregon.gov/oha/PH/DISEASESCONDITIONS/COMMUNICABLEDISEASE/DISEASESURVEILLANCEDATA/ANNUALREPORTS/Documents/2017/2017-Cryptococcus.pdf Smith RM et al. Treatment and outcomes among patients with Cryptococcus gattii infections in the United States Pacific Northwest. PLoS One. 2014 Feb 19;9(2):e88875. https://www.oregon.gov/oha/PH/DISEASESCONDITIONS/COMMUNICABLEDISEASE/DISEASESURVEILLANCEDATA/ANNUALREPORTS/Documents/2017/2017-Cryptococcus.pdf

•Acquisition: inhalation of Isn’t this a weird presentation? spores from the environment. No zoonotic transmission. Whydidthisboygetsosick? •Incubation: 2 to 13 months, with a median of 6–7 months • Clinical presentation: cryptococcomas in the lung and brain (often large, multifocal lesions)

https://www.cdc.gov/fungal/diseases/cryptococcosis-gattii/index.html Sentinel Immunodeficiency Pearls Newly recognized immune deficits in otherwise healthy persons Non contrast CT may miss early lesions. ම Auto-antibodies against GM-CSF detected in serum Æ If infection concern, need contrast (or diffusion weighted) to exclude infection Meningo-encephalitis panel may not always be helpful (concerns about false ම Also causes acquired Pulmonary alveolar proteinosis (PAP), positive/false negatives) leading to a build-up of surfactant in the alveoli and inhibiting gas exchange ÆIf index of suspicion is high, confirm with other tests Severe cryptococcal disease (namely C gattii) can occur in previously healthy hosts here ම Ig Deficiencies (CVID) in the Pacific Northwest ම HIV or Idiopathic lymphopenia Æ It’s here! Be on the look out If a weird infection presents and is severe, think about possible immunodeficiency. Æ We now have a pediatric immunologist!

RosenLBetal.AntiͲGMͲCSFautoantibodiesinpatientswithcryptococcal meningitis. JImmunol. 2013Apr15;190(8):3959Ͳ66. MarrKA.Cryptococcusgattii infectioninhealthyhosts:asentinelforsubclinicalimmunodeficiency?Clin InfectDis. 2012Jan1;54(1):153Ͳ4.

Presenting Symptom: Swollen glands HPI: •Previously healthy; had a cold prior to this. • ER: Temp 37.5, red pharynx, diagnosed Strep without a rapid strep and gave amoxicillin. 5 year old with a lump on R neck in May accompanied • Saw PCP for follow-up: Didn’t think this was strep. Large lymph node at R neck. Not by 1 day fever (102.5 F) fluctuant or tender. Ordered labs – including cat scratch and presumptively started him on azithromycin and clindamycin. • Over 1 month: Node continued to enlarge. Referred to local ENT (adult). CT scan. • Admitted for IV antibiotics and surgery with partial IandD. Discharged on Augmentin. No growth. • Referred to ID for persistent draining wound.

Pertinent History Exposure History ROS: 1 day of fever; +night sweats; decreased • Attends preschool and otherwise home activity/fatigue • TB/MRSA/HSV: No homeless shelter/ jail exposures. No one with cold sores. - Pertinent negative: no blue, pink or purple toned • Food/Water: Well water for bathing and teeth brushing skin; no weight loss, no belly pain, no other lymph nodes • Recreation: No hot tub/warm springs; lots of gardening; no hanging PMH: Hydrocele and hernia repair x2; Lymphangioma plants; no composting on site; Organic soil delivered 2 years ago. Tonka removal right scalp trucks in dirt; has eaten dirt per mom. • Animals: 2 dogs, 1 fish, 15 chickens, 5 ducks; Scratched by cat 6 months FMH: Mom- Celiac disease; MGGF-TB in the 1950s ago. Common causes of lymphadenopathy Bacterial Adenitis Fever Edema Erythema Tenderness WBC

Remember the pharyngeal spaces

https://www.aafp.org/afp/2014/0301/p353.html

Mycobacteria Cat Scratch: Bartonella henselae Scrofula: TB vs. NTM SX: A small papule may develop at the Sx: Develops over weeks to months; Tender and site of inoculation; can take 2+ weeks rubbery, Discolored skin over the node. to develop adenopathy Cervical>>Axillary>Groin Dx: Serology, Blood or PCR Dx: clinical; biopsy shows necrotizing granulomas; culture or PCR + Tx: Azithromycin, Bactrim Tx: Excisional biopsy preferred; If involves facial nerve, Cx: Retinitis, osteomyelitis, may require abx (azithromycin, rifampin +/- hepatosplenic lesions, endocarditis. ethambutol). Cx: If Iand D is doneÆmay lead to sinus tract and Consider Tularemia with an eschar cutaneous drainage for up to 12 months HELPFULCLUES:AGE,LOCATION,APPEARANCE

https://cmr.asm.org/content/24/4/701 https://www.dermatologyadvisor.com/dermatology/cat-scratch-disease-bartonella-infection/article/691691/

Toxoplasmosis Congenital Conditions Mimicking Adenopathy Sx: malaise, fever, sore throat, and • Thyroglossal duct cyst (see myalgia. picture) • Dermoid cysts or tumors Dx: Serologic testing; Tissue PCR • Branchial cleft (see picture) Tx: Self limited; • Lympho-vascular malformations pyrimethamine/sulfadiazine + leucovorin rescue • Hemangioma • Ectopic thymus Cx: Retinitis, myocarditis and pneumonitis. • Epidermoid cyst • Cystic Hygroma

https://www.pathologyoutlines.com/topic/lymphnodestoxoplasma.html http://fortworthent.net/ear-nose-throat/thyroglossal-duct-cyst-removal/ https://www.nejm.org/doi/full/10.1056/NEJMicm1503044 Imaging Considerations BACK TO OUR CASE Chest x-ray Æ prolonged fevers, constitutional symptoms; concern for TB, cancer, etc. Ultrasonography Æ defining the presence and extent of an abscess; Liver/spleen/Masses CT Æ congenital / structural concerns; pre-op MR Æ not used often – helpful if want to avoid radiation but may need sedation

AmericanJournalofRoentgenology.2012;199:1105Ͳ1113.10.2214/AJR.12.8629.ImagingofCervicalLymphadenopathyinChildrenand YoungAdults.https://www.ajronline.org/doi/10.2214/AJR.12.8629

Microbiology Differential •Broad Range Bacterial PCR: Legionella longbeachae •6/1: WBC 18.8 HCT 32.6 Plt 390 77N ESR 83 Mycobacterium species •Bartonella IgG = <1:64 (neg); IgM= <1:16 (neg) Unusual fungus •TST: negative / Quantiferon Gold: negative Nocardia •Wound culture from OR on 6/13: negative for bacteria. Other (who knows?)

•No AFB or fungal cultures were done. BroadRangePCR:nonontuberculousmycobacteriadetected; •Path report: Necrotizing granulomatous lymphadenitis. GMS and AFB noTBdetected stains for mycobacteria and fungus were negative. • ID Eval: Large infectious serological panel was negative (toxoplasmosis, fungal, tularemia).

Course Emerging Infection: Legionellosis •Underwent a more extensive excision and •At least 60 different species of Legionella debridement with Peds ENT. • Most disease is caused by Legionella pneumophila, particularly serogroup 1 •Confirmed the Broad range PCR result with a • Longbeachae: Underdiagnosed cause of Legionnaires’ disease second sample • First isolated from a patient in Long Beach, California in 1980. • Highly recognized in Australia and New Zealand •Completed 21 days of azithromycin with clinical • Bacteria is found in soil and compost-derived products resolution. • Diagnosis: culture (slow grower); urine antigen may not be helpful, serology (paired); PCR

Whiley H,BenthamR. Legionellalongbeachae andlegionellosis.Emerg InfectDis2011Apr KenagyE.RiskFactorsfor Legionellalongbeachae Legionnaires’Disease,NewZealand.EIDJul;23(7):1148Ͳ1154. CIDVolume38,Issue10,15May2004,Pagese102–e106 https://www.cdc.gov/legionella/clinicians/diagnosticͲtesting.html Legionellosis Red Flags •Transmission: No Person-to-person; Inhalation and ingestion are •Non healing wound possible modes •Night sweats or weight loss •Clinical: early symptoms include fever, chills, headache, shortness •Lack of infectious symptoms in the ear, nose, and throat regions of breath, sometimes dry cough, and muscle aches and pain. •Unexplained fevers > 1 week Pontiac fever (without pneumonia) •Lymph nodes > 2 cm in size; does NOT Wax / Wane Other: Osteomyelitis; Cutaneous (non healing wound); Adenopathy •Supraclavicular or axillary lymph nodes •Risk factors: Exposure to compost or potting mix. Gardening •Hard, rubbery consistency; fixed/matted behaviors, including having unwashed hands near the face after •Abnormal CXR exposure to or tipping and troweling compost or potting mix. •Hepatosplenomegaly • Abnormal labs (CRP, ESR, WBC, etc.)

McClellandM.PneumoniaandOsteomyelitisDueto Legionellalongbeachae inaWomanwithSystemicLupusErythematosus

Pearls Presenting Symptom: Difficulty Seeing Avoid I and D unless bacterial abscess •ÆPoor wound healing may signify continued infection •Æ Excisional biopsy is best. You may not be able to identify the underlying etiology in every patient. 6 year old girl with new onset vision loss •ÆNewer molecular diagnostic studies (Broad Range PCR) may be helpful, particularly with more unusual presentations. Know the red flags •Æ If it waxes/wanes – you are generally ok, but close follow-up can identify early lesions

HPI Pertinent History • Used father’s reading glasses when looking at books. ROS negative: NO fever, headache, neurologic or constitutional symptoms; +vision loss • Over the next few months noted she was holding objects close to face; unable to read PMH: normal pregnancy, labor, delivery, infancy large projection screen at church; ම Hx of urticaria lasting 12 months between ages 3 and 4 years; symptomatic treatment; resolved spontaneously • Denies eye pain or headaches or preceding illnesses ම No medications • Visited the optometrist where glasses were recommended. ම Unimmunized • Incidental screening exam abnormal Social History and Exposures: youngest of 11 children; lives on the northern Oregon ම Strabismus coast; No travel; 3 cats, dogs and chinchilla; no sick contacts ම Fundus exam: disc edema FMH: brother with myopia and astigmatism Referral to Casey Eye A quick review of the eye exam Eye Anatomy 1.Visualacuity 2.Pupils(withafferentcheck–swinginglight) 3.Extraocularmotilityandalignment(bothandmonocular) 4.Intraocularpressure 5.Visualfields 6.Externalexam 7.Slitlamp:Lids/lashes/lacrimalsystem:;Conjunctiva/sclera;Cornea;Anteriorchamber;Iris; Lens,Anteriorvitreous 8.Funduscopic:Opticnerve,macula,vessels,periphery

https://kidshealth.org/en/parents/eyes.html

Eye Concerns Initial Evaluation at Casey Eye Institute

Keratitis Scleritis Uveitis Retinitis/optic Acuity Refraction neuropathies ම Right 20/60 ම Sphere Mechanism inflammation and Inflammation of Inflammation of Optic nerve ම Left 20/200 ම Right +1.25 ulceration of the sclera iris/ciliary body, lesions ම Left +0.75 Slit Lamp- normal cornea choroid Macular lesions Pressure: normal Etiology HSV, RA, Crohns Syphilis, TB, Infection, bacteria/fungi toxoplasmosis, autoimmune, Motor histo; ischemic, ම Intermittent exotropia autoimmune neoplasms ම Deprivation amblyopia Symptoms Pain, decreased Dull pain, intense Pain, Pain is variable acuity, irritation, redness, loss of photophobia, Vision loss tearing, vision blurred vision, photophobia, mild redness, pupillary conjunctivitis constriction

MacularStar Fundus Exam

Right Left Disc Discfullnesswithexudativematerial Discedemaespecially temporaltodisc inferoͲnasally

Macula Faintmacularscarseennasally Prominentmacularstar Vessels Normal Normal Periphery Normal Normal Right Left Swollenopticdisc Macular Star and Strabismus Differential Diagnosis: neuroretinitis •Macular star formation is caused by the deposition of lipid exudates along the outer Inflammatory: MS, sarcoid, Behcet, Sjogren, Lupus, Guillain- plexiform layer of the macula. • Vision loss due to maculopathy NOT optic nerve issue Barre, Wegner’s, IBD •Strabismus: - Post infectious: measles, mumps, varicella, influenza, EBV ම Failure of eyes to look in the same direction at the same time ම Weakness of muscles of one eye: (superior oblique, interior oblique, lateral) Infections: ම Childhood: associated with amblyopia (decreased vision in one eye) - Complication of meningitis or encephalitis either as a direct effect ම Types 1. Esotropia: convergent - cross eye of one eye of the infectious organism or from a secondary vasculitis 2. Exotropia: divergent - one eye turns outward - Acute viral infections (CMV), Toxoplasmosis, Syphilis, Tuberculosis, Cat Scratch Disease, West Nile, Cryptococcus, Ebola, Zika, Lyme, RMSF

Infectious Neuroretinitis Lab Evaluation CBC and CMP were normal Toxoplasma retinitis Syphilis retinitis Sarcoid: ACE normal Syphilis: NR Tuberculosis: Quantiferon - NR B. henselae: IgG 1:1024, IgM 1:16

CMV retinitis Lyme retinitis

Balansudaram et al. Outbreak of Acquired Ocular Toxoplasmosis Arq. Bras. Oftalmol. vol.77 no.5 São Paulo Sept./Oct. 2014 Involving 248 Patients. Arch Ophthalmol. 2010 Jan;128(1):28-32. Mora D. Int J Med Sci 2009; 6(3):124-125.

Emerging Infection: Bartonellosis •Bartonella henselae: Facultative, intracellular gram negative rod; fastidious Rash •NOT Bartonella Quintana (trench fever) or Bartonella bacilliformis (Carrion’s disease) Hepatosplenic •Incidence highest among in the southern United States (6.4 cases/100,000 population) dissemination; and among children 5–9 years of age (9.4 cases/100,000 population). Osteomyelitis •12,000 outpatients are given a CSD diagnosis and 500 inpatients are hospitalized for Encephalopathy CSD. Endocarditis •Normal flora in kittens; maintained through contact/fleas; transmission from cat bite, Eye Disease scratch, lick

Nelson Cat-Scratch Disease in the United States, 2005–2013. EID Volume 22, Number 10—October 2016 https://cvi.asm.org/content/9/1/8/figuresͲonly Cat Scratch Ocular Disease Treatment Unclear benefit in healthy hosts, but lesions may resolve faster Many agents potentially active: Macrolides, tetracyclines, • Ocular involvement of cat-scratch disease occurs aminoglycosides, TMP-SMX in 5–10% of cases, and is the most common non- lymphatic organ involvement. Retinitis – visual prognosis is usually excellent • Parinaud's oculoglandular syndrome: occurs in ම Doxycycline plus Rifampin or Fluoroquinolone based on case series 5% of cases. ම 2-4-6 weeks • Neuroretinitis is seen in 1-2% ම +/- steroids

Ghazi A case of cat-scratch disease with unusual ophthalmic manifestations. Middle East Afr J Ophthalmol. 2012 Apr-Jun;19(2):243-6. Arango-Ferreira. Parinaud’s Oculoglandular Syndrome in Cat Scratch Disease N Engl J Med 2018; 37

Eye Exams Initial +10 +30 +42 (stop +360 therapy) Pearls Acuity (R) 20/60 20/40 R: 20/25 20/30 20/20 - Acute vision changes should prompt referral to an eye specialist Acuity (L) 20/200 20/70 L: 20/70 20/80 20/50 Æ Dilated exam and slit lamp key Macula (R) faint macular scar trace macular trace macular trace macular Normal seen nasally star star star - Common infections can have unusual presentations Macula (L) prominent Trace exudate, mild Small Æ the most common cause of neuroretinitis is cat scratch disease. macular star macular star exudate/macular hypopigmented star scarring on - While CSD is usually self-limited, use of doxycycline or fluroquinolones may be needed inferior fovea for disseminated disease Disc (R) Disc fullness with Mild edema mild disc edema mild disc edema Normal Æ could consider other regimens: azithromycin or trim-sulfamethoxazole exudative material temporal to disc

Disc (L) disc edema Disc edema Disc edema Inferior temporal especially especially especially gliosis inferonasally inferolaterally inferonasally

Summary 1. Described a differential according to presenting signs and symptoms for headache, lymphadenopathy, and vision loss 2. There are many diagnostic tools available in infectious diseases, including newer molecular tests 3. Cryptococcal disease, Legionellosis, and Cat Scratch disease are rare but emerging infections affecting patients in the Pacific Northwest Thank You

Questions?

72 Conflict of Interest Statement

• I have no financial disclosure Can a Screening EKG Save A • This presentation does not contain trade names Pediatric Athlete’s Life • This presentation does not contain advertising.

Brendan Kelly, MD Pediatric Cardiology Oregon Health & Science University NW Permanente Physician

BUT I AM A CARDIOLOGIST! Hayward Demison

• Portland Central Catholic High School Star athlete • Cardiac arrest during a football game in 2010 after scoring a touchdown • Successful resuscitation by members of the audience. • Echo showed: Anomalous left coronary artery • Probable cause of event: VF due to acute ischemia. • Recovered after surgery for coronary artery re-implantation

Hank Gathers ‘90 “Pistol” Pete Maravich ‘88 Marc Vivien Foe ‘03

Reggie Lewis ‘93 Sudden Death in Children How about Portland Oregon?

• 1.3 per 100,000 (1-22yrs) • Chugh et al, Oregon Sudden Unexpected – Minnesota, Driscoll et al 1985 Death Study. Heart Rhythm 2009. • 3.3 per 100,000 (1-20yrs) • 7.5 per 100,000 (0-17yrs) – Northern England, Wren et al 2000 • 1.9 per 100,000 (1-17yrs) • 2.7 per 100,000 (1-18yrs) • 3.0 per 100,000 (1-4yrs) – Taiwan, Wu et al 2009 • 2.4 per 100,000 (5-9yrs) • 1.7 per 100,000 (10-14yrs)

Harmon et al 2015

• 514 deaths in NCAA athletes 2003-2013 • Accidents responsible for 6.1/100,000 pty • Sudden cardiac death in 79 athletes – 1.9 deaths/100,000 pty – Male vs female NCAA athletes 2.6 vs 0.8/100,000 pty – Black vs white NCAA athletes 4.7 vs 1.5 /100,000 pty – Male NCAA div I basketball player 19.2/100,000 pty • 25% were autopsy negative SCD • Different risk for different populations

Deaths During Sports are RARE

• But….. – Highly visible deaths, prime of life What are the causes of the – Kids being active like doctors prescribe • “No child should die that way.” problem? • “Can you tell me my child won’t die?” • “I have good insurance, I want all the tests.” • Community screening programs – EKG only – EKG and echo • What about the non-athletes? Maron et al 2007 Disease ECG abnormal? Echo/MRI Inherited? abnormal? HCM Y* Y Y

Abnormal Coronary N Y* N

Long QT Y N Y

ARVC Y* ? Y

CPVT ? N Y

Brugada syndrome Y N Y

WPWYNN

CHD Y* Y N*

Myocarditis NA NA N

Commotio cordis NA NA N

Prevention of SCD

• Primary Prevention • History Currently a part of Screening In The US: • Family History pre-participation • Physical examination screening • ECG Pre-Participation Evaluation (PPE) • Echocardiography for the 12-25 year old athletes • Stress test • Secondary Prevention • CPR + AED Programs

AHA Statement Recommendations. Maron 1996, Revised 2007 Pre-participation Evaluation (PPE)

• Current AHA recommendation • 14 point History & Physical components • Most states including Oregon have adopted this document Before You Refer

• Think about asthma • Rule out anemia • Vasovagal episodes are common • We all have symptoms with exertion • In my practice: – Close family member means 1st degree relative • Innocent murmurs are common

Italian Experience

• Screening program for athletes introduced 1982. • 12-35 year old athletes screened per Italian law – History, physical exam, 12 lead EKG What about other developed • Corrado et al in 2006 – 1979-2004 55 SCD in 50 males and 5 females countries? – SCD age mean 23.3yrs, median 23yrs – 90% white population – Decrease in SCD per 100,000 person years with screening • 4.19 (1.94-7.59) to 0.87 (0.46-1.28) – Greatest decline in death from cardiomyopathies (especially arrhythmogenic right ventricular cardiomyopathy) • 36% to 17% • 7-9% false positive rate of screening

Corrado et al JAMA 2006. Veneto region of Italy But…. • Risk of SCD goes up with age – 0.13/100,000 in 12-19yo – 1.45/100,000 in 20-24yo • Risk of SCD is higher in males vs females – 0.75/100,000 vs 0.13/100,000 • SCD risk in US 12-25yo <1/100,000 • SCD risk in Italy 12-35yo 3/100,000 – 82% were males • In US 1/3 SCD due to hypertrophic cardiomyopathy • In Italy 1/4 SCD due to ARVC Italian approach Overview

• Specialized sports medicine physicians • Used insurance claims to assign cause of death • Has been adopted with modifications by: • 2.28M person-y versus 2.93 person-y – ESC, IOC, FIFA, many US professional sports teams • Universities – Harvard, UW, Stanford, UVA, U Wisconsin, Georgetown • US military for aviators

Steinvil et al 2011

• Review of 24 newspaper reported sudden death events during sports in competitive athletes from 1985-2009 in Israel • 1997 mandated screening instituted – H&P, resting ECG, exercise test screening by certified physicians – 12-44 years old • No decrease in event rate with ECG – 2.54 events per 100,000 athlete-years prior to 1997 – 2.66 events per 100,000 athlete-years after 1997

Not All ECGs Are Typical!

• HCM: 10% normal; sub-clinical/pre-clinical • WPW: can be intermittent, or subtle • 53/212 pediatric cardiologists who returned a survey • 8 normal EKGs • LQT: can be tough & subtle. • 10 abnormals (LQT, WPW, HCM, PHTN, myocarditis) – Can even be normal. • Brugada: often normal. – May need provocative testing (fever, IV Procainamide). • ARVC: subtle repolarization abnormalities • CPVT: usually normal – PVCs or VT with exercise; usually suspected when story suggests LQT but ECG is “normal”. Author Population Positive ECG

Fuller 1997 5615 HS athletes 2.6%

Pelliccia 2006 32652 athletes Italy 9%

Pelliccia 2007 4450 athletes Italy 12% Let’s Talk About the False Magalski 2008 964 college athletes 10% Wilson 2008 2720 HS athletes UK 4%

Positive ECGs Bessem 2009 428 athletes NL 6%

Baggish 2010 510 college athletes 16%

Weiner 2011 510 college athletes 10%

Vetter 2011 400 children 5-19 8%

Chandra 2014 7764 non athletes UK 22%

Chandra 2014 4081 athletes UK 33%

So Change EKG Reading Criteria for Athletes?

European Society of Cardiology 2010 Seattle Criteria 2013 Refined Criteria 2014 International 2017

Chandra et al. JACC 2014 Chandra et al. JACC 2014 Sharma et al: International recommendations for electrocardiographic interpretation in athletes. European Heart Journal 2018. Asymptomatic Athletes 12-35 year old

Sharma et al: International recommendations for electrocardiographic interpretation in athletes. European Heart Journal 2018.

Malhotra et al BMJ 2019 Malhotra et al BMJ 2019

• International criteria was the best – Specificity of 98% • 11,168 soccer players between 1996-2016 – Sensitivity 86% • Health questionnaire, EKG, echocardiogram. – PPV 17% • 95% male, 91% white • History • Compared ESC 2010, Seattle 2013, Refined 2014, – Specificity 96%, Sensitivity 7%, PPV 2.8% and International 2017 • Physical • All four criteria identified 36 of 42 athletes with – Specificity 98%, Sensitivity 5%, PPV 1.9% serious cardiac conditions (86%)

But there is always echo and MRI right? HCM

Anomalous Left coronary Screening echo • Can detect obvious cardiomyopathy • Imaging coronary arteries takes skills – Often need cardiac CT in suspicious cases Talk to me about the $$MONEY$$

A life – how much is it worth?

Ann Intern Med. 2010;152(5):276-286. • Priceless….in theory. • Screening societal threshold • Addition of EKG to H&P – $50,000-$100,000/life year – $42,900/life year • Is this money better spent in other areas of • Assumes <9% abnormal EKGs health care? • Assumes EKG cost of $5 • Assumes secondary testing of $330 • Assumes risk reduction of 84% – 50% reduction leads to $63,600/life year

Leslie et al. Circulation 2012 Maron 2007 $3.4 million per life saved Fuller 2000 $44,000 per life year saved

• Simulation models incorporating prevalence, Wheeler 2010 $46,000 per life year saved sensitivity, specificity Malhotra 2011 $69,000 per diagnosis – HCM, LQTS, WPW – 2 EKG screening populations ADHD and athletics Leslie 2012 $91,000 per life year saved • Treatment algorithms generated and analyzed Halkin 2012 $10-14 million per life saved

• Screening at age 8: $91,000/life year Dhutia 2016 $36,000 per diagnosis • Screening at age 14: $204,000/life year USA Cost • ~10 million competitive athletes. • 60 million people aged 12-25. • “A billion here and a billion there and – Can screening ethically be restricted to pretty soon you are talking real money!” “athletes”? Senator Everett Dirksen • Current cost estimate: – $2.5-3.5 billion per year. • Not enough cardiology providers to read all the EKGs • Insurance company payment is an issue

Evidence pro ECG screening Evidence con ECG screening • ECG abnormal in almost all HCM patients with • Italian study has not been replicated even in Italy. hypertrophy. • USA study (Maron) and Israel Study did not • Can detect LQT, WPW, Brugada, CPVT etc support ECG screening. • A high cut off QTc value > 460 in boys and 480 in • False positives: Almost 30% in athletes? girls can pick up clear LQT. – Mild LVH, mild RVH, borderline QTc. • ECG is “cheap” and easy to do. • False negatives: will occur regardless of • Current PPE is less cost effective screening than technique ECG. – Coronary artery abnormalities are hard to • False positives can be reduced with a clear & detect. modified EKG reading protocol

So, where are we? ACC AHA guidelines • Debate at every meeting. Both sides have good points but seem to selectively choose data. • AHA/ACC panel does not support mandatory national ECG screening. • Data being collected. Child Safety Research • They cite: Consortium; mainly pediatric EP and cardiology – Low prevalence (PACES), lay advocates and FDA. Working to set – Low risk in those with conditions associated with SD common standards on data collection and – Large population size reporting. – Imperfections of ECG • Do support local efforts in small cohorts with close physician involvement. The logic of Cascade screening What can we do? Disease ECG abnormal? Echo/MRI Inherited? abnormal? • Screen patients with symptoms HCM Y* Y Y

– Syncope or near-syncope: ECG. WPWYNN – Syncope or chest pain during exercise: ECG + Long QT Y N Y cardiology referral (will likely need echo) ARVC Y* ? Y • Screen patients with positive family history. CPVT ? N Y – Cascade screening. Brugada syndrome Y N Y Abnormal Coronary N Y* N • Widespread availability of AED. CHD Y* Y N*

– ~$53,000/QALY Myocarditis NA NA N • Widespread CPR & AED training. Commotio cordis NA NA N

Abnormal EKG Examples

Long QT: QT = 600 ms; with sinus bradycardia WPW Brugada ECG

Final Thoughts and Questions? •NO disclosures

Prevent an Eating Disorder- Save an Athlete Dr. Melissa Novak D.O. Primary Care Sports Medicine Oregon Health Sciences University

20 Year Old Collegiate Track Athlete 2014 Female Athlete Triad Coalition Consensus Statement

7

What we are going to talk about

• Define Female Athlete Triad Syndrome

• Explain How YOU can Prevent and Screen in the during routine well child checks

•Explore Diagnosis and Return to Play Guidelines Age 22, Multi-organ Failure, 60lbs Christy Henrich

Born: July 18, 1972 Died: July 26, 1994

Meet Sarah.

• “I realized that as I worked harder and lost some weight, my times were TO THIN TO TRAIN?? improving,”

• “So I figured that if a TO THIN TO TRAIN? little weight loss was good, a lot would be even better.” Simple Logic: Improved cardiovascular fitness Increased strength and power Decreased morbidity and mortality • Sarah's downward spiral into the depths of Decreased high-risk behavior anorexia is perhaps most disturbing for its simple logic: Decreased risk of breast cancer Improved cognitive function • If a few pounds were good for performance, a lot of pounds would be amazing… Improved bone strength Improved self-esteem Healthy aging

“Smarten up”

• “Even though your score is suppose to be based on your routine, you must know that you are giving the judge lots of signals…approach the apparatus with your head high, clothes tidy, hair in place. You will be “saying” to the judge you Unrealistic have trained well…Judges will see you in a standards of positive light. They may even be tempted to run out on the floor and pinch your cheek because appearance and If a little weight loss is good, you are killing them with “cute”. Judges love performance More is Better “cute” so work it babe!”

Female Athlete Triad- Defined in 1992 The Female Athlete Prism-The Spectrum of the Female Athlete Triad Screening Recommendations Female Triad Coalition Questions??

• Have you ever had a menstrual period? • Female Athlete Triad Coalition • How old were you when you had your first menstrual period? recommends screening once a year with • *When was your most recent menstrual period? • How many periods have you had in the last self reported questionnaire 12 months? • *Are you presently taking any female hormones (estrogen, • If there is any one symptom of the triad progesterone, birth control pills)? • Do you worry about your weight? further investigation should be initiated • Are you trying to or has any one recommended that you gain or lose weight? • Are you on a special diet or do you avoid certain types of foods or food groups? • Have you ever had an eating disorder? • Have you ever had a stress fracture? • Have you ever been told you have low bone density (osteopenia or osteoporosis?)

Low Energy Availability How Can You Assess Low Energy Availability

Energy Expenditure • Energy availability calculator on Female Athlete Coalition Website Energy – http://www.femaleathletetriad.org/calculators/ Intake • Nutrition assessment with sports dietician • Energy expenditure apps

Energy Balance

Consequences of Low Energy Availability How Athlete’s Reduce Energy-disordered eating

• Abnormal eating behaviors – Fasting – Binge-eating – Purging – Diet pills – Laxatives – Diuretics –Enemas • Eating disorders/mental health disorder – Anorexia/Bulimia Menstrual Dysfunction Osteopenia/Osteoporosis

Bone loss is often irreversible • Amenorrhea: primary or secondary – Primary: delay of menarche – Secondary: cessation after regular menstrual cycles have been established May be present without • Underlying factor is inadequate energy menstrual dysfunction availability • Amenorrheic women are infertile due to absence of ovulation, BUT they may ovulate before Stress fractures occur more menses is restored = unintended pregnancy! often with menstrual irregularities

Health Consequences Sarah: “I felt alone…”

• Psychological Health • For most health issues, off – Low self esteem, depression, anxiety to the PCP… – 5.4% athletes with eating disorders reported suicide • “When I went to see my attempts PCP, it was not helpful” • Medical Complications – “I was told I should gain weight to reach 120 pounds” – Cardiovascular, endocrine, reproductive, skeletal GI, renal and central nervous systems – “That’s more than I ever weighed before I even began running”

Well Meaning Useless Advice… Prevention/Early Detection “I FELT ALONE”

• Disconnect between a PCPs advice and • Education!! the goals of an athlete – Athletes, parents, coaches, athletic trainers, – No constructive path for an athlete to follow judges, administrators – Yes, she needed to add some pounds back • Pre-participation Physical on, but she wasn’t willing to give up her • Presentation with any associated clinic athletic dreams to do so syndrome • Rule changes “I felt alone” – Discourage unhealthy weight loss practices Identify Athletes at Greatest Risk Identify Athletes Most at Risk for Stress Fracture

• Restrict dietary energy intake • Low BMD • Exercise for prolonged periods • Menstrual disturbance • Vegetarian • Late menarche • Limit the foods they will eat • Dietary insufficiency • Early start of sport-specific training and • Genetic predisposition dieting, injury and sudden increase in • Biomechanical abnormalities training volume • Training errors • Bone geometry

Nonpharmacologic Treatment Recovery

• Main goal of treating the triad is increasing energy availability • Recovery of Bone Mineral Density • Goals: Improved bone health and – Process: YEARS menstrual function • Recovery of Menstrual Cycle • Multidisciplinary team is key – Process: MONTHS • Time course is different for each athlete • Recovery of Energy Status – Process: DAYS TO WEEKS

Treatment Treatment

• Recommend increasing dietary energy intake and decrease exercise energy • Weight gain to achieve a BMI of >18.5 expenditure or both • Individual treatment plans: diet quality, • Return of body weight associated with timing, incorporation of energy dense foods, normal menses adjustments for training • Reversal of recent weight loss • Increase energy intake gradually 20-30% over baseline needs • Weight gain of approx 0.5 kg every 7-10d • Regular monitoring with sports dietitian Calcium and Vitamin D Pharmacological Therapy

• 9-18 years • Lack of evidence based studies to recommend pharmacological therapy – Vitamin D: RDA 600 units • Would only be considered in athlete if lacking – Calcium: RDA 1300mg response to non-pharmacologic management with low BMD + clinical significant fracture • 19-50 years history – Vitamin D: RDA 600 units • In general we do NOT treat with oral – Calcium: RDA 1000mg contraceptives as they mask the menstrual problems and do not increase bone density

Triad Clearance Evidence Based risk factors associated with Poor outcomes

• Conundrum: many athletes cleared • Low energy availability with or without without proper management and disordered eating/eating disorder assessment • Low BMI • Return to Play: • Delayed menarche – Athletes often return after triad associated • Oligo/amenorrhea injures or illness without adequate management or follow up • Low BMD • Stress reaction/fracture history • Leanness sport

Athlete Participation in Sport

• Athlete must agree: – To comply with all treatment strategies – To be closely monitored by health-care professionals – Place a precedence on treatment over training and competition – Modify type, duration, and intensity of training and competition • Often useful to have a written contract with the agreements Return to Play- Complex Equation Clearance…

• Need to respect the athletes privacy, very • Willingness of athlete to comply with goals sensitive issue • Sport-specific training demands • However communication with coaching staff • Is the sport an increased risk of medical extremely important and/or psychological risk to the athlete – Coaches may be a part of the solution – Yes: consider limiting or withholding • If disqualified specific steps need to be outlined training/competition for the athlete – Withholding training/competition can be motivating – Who should they meet with – What are the consequences – Timeframe for return to training and competition

Questions before I summarize? Female Athlete Triad- Summary

• Spectrum of health and disease based on energy availability – Disordered Eating – Menstrual Dysfunction – Bone Mineral Density • Identification of those at risk • Treatment team is multi-disciplinary

Sarah’s parting words-

• “Your body can’t run on nothing. Eventually, you will crash and burn. If a friend or coach says something, be open to considering what they’re telling you. The sooner Thank you! you get help, the easier it Melissa Novak, DO will be to get your life Primary Care Sports Medicine back.” Oregon Health & Science University [email protected]

Disclosures

• Authorofchapterinoneoftextwill recommend • Otherwise– none.

CasesofHorsesandZebrasof PediatricSportsInjuries RyanPeteringMD,CAQSM OHSUSports&FamilyMedicine OHSUSportsMedicineFellowshipDirector

Agenda RESOURCES • Orthobullets • Casebasedapproachtocommonand • AFP(AmericanFamilyPhysician) uncommonPediatricSportsinjuries • FractureManagementPrimaryCare

SalterHarrisClassification SalterHarrisClassification

UpToDate:Generalprinciplesoffracturemanagement:Fracturepatternsanddescriptioninchildren UpToDate:Generalprinciplesoffracturemanagement:Fracturepatternsanddescriptioninchildren 10yo malesoccerplayerwith2Ͳ3 weeksofheelpain.Noinjury. SeversDisease

• ApophysitisoftheAchillestendonatcalcaneus insertion • 8Ͳ11yearsold/boys>girls • Primarilyrelativeresttreatment • Heelraiseinsert • DoesNOTrequirestrictrestorsportsavoidance. • DoesNOTmandatexray (preferredtoavoid) • QuestionableifPThelpfulwhencomparedtowaitand see,heelraise(n=101) – JPediatrOrthop. 2016Mar;36(2):152Ͳ7.TreatmentofCalcaneal Apophysitis: WaitandSeeVersusOrthoticDeviceVersus PhysicalTherapy:APragmatic TherapeuticRandomizedClinicalTrial.

Sindig LarsenJohannson

13yomalebasketballplayerwith recurrent/chronickneepain–anterior – withnotriggerͲ worsewithactivity.

OsgoodSchlater/Sindig Johanson OsgoodSchlatter LarsenDisease • Apophysitis ofthepatellartendonattibia(OS) orthepatella(SJL) • Primarilyrelativeresttreatment • Chopat strap • DoesNOTrequirestrictrestorsports avoidance. • DoesNOTmandatexray (preferredtoavoid) Pediatrics.2011Nov;128(5):e1121Ͳ8.Epub 2011Oct3. HyperosmolardextroseinjectionforrecalcitrantOsgoodͲ Schlatter disease. • N=65 • Comparedwithusualcareat3months,unalteredsportwasmorecommoninbothdextroseͲ treated(21of21vs 13of22;P=.001)andlidocaineͲtreated(20of22vs 13of22;P=.034) knees,andasymptomaticsportwasmorefrequentindextroseͲtreatedkneesthaneither lidocaineͲtreated(14of21vs 5of22;P=.006)orusualͲcareͲtreated(14of21vs 3of22;P< .001)knees. • At1year,asymptomaticsportwasmore commonindextroseͲtreatedkneesthanknees treatedwithonlylidocaine (32of38vs 6of 13;P=.024)oronlyusualcare(32of38vs 2 of14;P<.0001).

RectusFemoris Avulsion

14yosprinter,duringcompetition, suddenonsetofright thigh/anteriorhippain.

RectusFemoris Avulsion

• Suddenonsetanteriorhippain • Sprinter/soccer/explosivesportsplayer 15yoactivemalewithrecurrent, • Ofteninitiallyunabletowalk • Xrays AREneeded–toassessifbonyavulsion chronicleftkneepainandeffusion– • UltrasoundorMRIneededoftentoquantifyiftearin nofocalevent/injury–andanexam tendonorjustavulsion • Nonoperative management–weightbearingas normalexceptforeffusion tolerated • Largeboneavulsion,tendonretraction– indication toreferOrtho. • Typically6Ͳ12weekfullreturntosport Osteochondral Lesion Osteochondral Dissecans (OCD)

Mostcommonlocation: Diagnosis: • Femoralcondyles • Xray:Tunnelviewoftheknee • Capitellum humerus @elbow (4thview) • Talar dome – Considergettingbilateralfilms Epidemiology • MRI:Confirm/Staging • Mostcommonagegroup:– Adolescence Management • Activityrestriction/reduction Presentation: • Nonweightbearingifseverepain • Jointpain • Bracing • SWELLING=EFFUSION • LimitedROM • Surgicaloptions • Mechanicalsymptoms

15yobaseballpitcherwithmedial elbowpainx2weeks.

ThrowersElbow LittleLeaguersElbow

• Riskfactors – Greaterthan80pitchespergame – Morethan8monthsofcompetitivepitchingperyear – Fastballspeed>85mph – Continuedpitchingdespitearmfatigue/pain – Participatinginshowcases/tournament http://m.mlb.com/pitchs mart/pitchingͲguidelines LittleLeaguersElbow

• Treatment 11yoobesemalefootballplayer– – Nonoperative mostcommon new/acuteonsetlefthippainwith – Surgicalconsiderationifbonyfragment(debateas tosizeoffragmentneededforsurgery) gradualworseningover2Ͳ3days–no – Pitchcountadherence trigger/focalevent – Gradualreturntobaseball– withdelayedreturn topitching

SCFE(SlippedCapFemoralEpiphysis)

PediatricRheumatology7(1):10ͼJune2009

SCFE(SlippedCapFemoralEpiphysis)

• Greatestriskfactor? • Mostcommonageonset? • Presentingsign? • Management? SCFE(SlippedCapFemoralEpiphysis)

• Greatestriskfactor? – Obese – Male – AfricanAmerican/Islanders • Mostcommonageonset? – 13yoboys/12yofemale • Presentingsign? – Groin/hippainmostcommon – Kneepainnotrare • Management? – Surgery – Crutches/emergentreferral

5yomalesoccerplaywithlimp,thigh painandkneepainx4monthswithno trigger/causativeevent.Normalknee andthighexam.

LeggCalvePerthes LeggCalvePerthes

• Idiopathicavascularnecrosisofproximal femoralepiphysis • M>F(5:1) • 4Ͳ8yo(5yomostcommon) • Bilateral~10Ͳ15% • Riskfactors – positivefamilyhistory – lowbirthweight – abnormalbirthpresentation – secondhandsmoke – Asian,Inuit,andCentralEuropeandecent LeggCalvePerthes

• Nonoperative 15yofemalesoccerplayerwithsudden – Activitymodification onsetkneepainwhilecutting/pivoting – Maintainmotion – Noroleforbracing/casting/splinting • Operative – Typically>8yo

ACLtear

• F>M(~5:1) • Commonnoncontact/pivoting • Effusioncanoccurin<1hour • Painvs instability • Surgeryornosurgery? • Timeframeforsurgery? • PEPprogram http://smsmf.org/files/PEP_Program_04122011.pdf

AnteriorDrawer Lachman 15yofemaleballerinawithacute worseningofchronickneepain– mediallyknee

AneurysmalBoneCyst(ABC)

• Benign&nonneoplasticbonelesion • 75%<20yo@diagnosis • Spine(25%),longbones(25%) • Painandswelling • Maypresentaspathologicfracture • Missedoftenonplainfilms • Treatment– usuallysurgical – Curettage+/Ͳ bonegrafting – Cements,otheradjuvants

StressFracturevs ShinSplit

15yobaseballplayerwithshinpain– anterior–x4weeks,nowpreventing running. RiskCategoryStressFracture ShinSplintvs StressFracture

Shinsplint (MedialTibial Stress StressFracture Syndrome) Diffuse painlocationanteriortibia; arch Focalpainlocation;archcollapse/medial collapse/medialkneedeviation/hipdrop kneedeviation/hipdrop Mayimprovewithrunning(initially) Worse withrunning Associated withactivity–doesnothave Typicallyassociatedwithdramatic tobeintenseactivity–orwithincrease; (relative)increaseinvolumeofexercise morecommoninnoviceexercisers Xray negative Xray negative(unless3Ͳ4weeksof symptoms–mayhavecallus);MRIor bonescantypicallyneeded Activityreductioniskey pain Typicallynonweightbearingandconsider management boot/castinitially Shoechanges, inserts,archsupportand Gradualrampup ofactivity– medialtibial lowerlegstrengtheningexercises stressreaction– typically6Ͳ8weeksout ofrunning(bestcase)

Spondylolysis vs Spondylolisthesis

13yofemalegymnastwith3Ͳ4months oflowbackpain Spondylolysis vs Spondylolisthesis

• Stressreaction/fractureparsinterarticularis =spondylolysis • Anteriormotionoflumbarvertabrae – spondylolisthesis • Gradingbasedonhowmuchanteriormotion(lawof25%) • Adolescentswithrecurrenthyperextensionofback • Typicallymanymonthsdurationwhendiagnosed– misdiagnosedaslowbackstrain. • Xray – needobliqueviewsbilaterallyvsMRI/CT • Nonoperativeiflessthan50%anteriormotion • Backbracingcontroversial/unclearbenefitiflessthan25Ͳ50% • Typically90daysofnoncompetitionifspondy

R.Grazina etal./PhysicalTherapyinSport37(2019)34e43

Spondylolysis vs Spondylolisthesis

• Returntoplayatanylevelwasapprox.90%returntothepreͲ injurysportsactivitylevel 16yofootballkicker– collisiononfield • Themeantimetoreturntosportswas4+months. – likelyLOCforapprox.10sec– • Approx 90%returnwithnonsurgical/conservative management assessedonsideline–andSCAT5 • Surgicallymanagedpatientshad6+monthstoreturnto assessmentperformed sports • Approx 80%returnwithsurgicalmanagement.

R.Grazina etal./PhysicalTherapyinSport37(2019)34e43

Concussion Summary • Recognize,Remove,Recover,Returnto Learn/Play • Respectthephysis! • Physicalactivityrecommendations • Rememberthesecommondiagnosis– • SubͲsymptomthreshold approachpatientwithgoaltomakesureyour patientdoesnothaveoneofthese! • Returntoplay • Whenthereistrauma– thinkbrokenbone • AvoidED,avoidimaging,avoidpredetermined • timeoff Usereferences • Focusoncommonprimarycaretopics– poor sleep,anxietyandotherpsychosocialissues Thankyou

RyanPeteringMD OHSUFamily&SportsMedicine petering@ohsu.edu What is neuropsychology?

Clinical Psychology

Neuropsychological Factors that Neurology Influence Concussion Management and Neuroscience Recovery Neuropsychology Pediatric Review 2019

PRESENTED BY: Tyler Duffield, Ph.D.

Neuropsychology Application Epidemiology of Concussion • Distinguish injury from non-injury factors: • CDC: approximately 1.7 million Americans sustain annual • Neurologic vs. traumatic brain injury (TBI) • Psychiatric vs. – approximately 70% (i.e., 1.2 million) considered mild (mTBI) • Neurodevelopmental vs. • Several groups of authors have noted that the actual number of • Psychosocial/Family factors TBIs annually is likely much higher, as many go undiagnosed, unreported, and thus uncounted.

• Or more often the case, a combination of • Estimated total expenditures exceeding $21.5 billion per annum for mTBI alone these factors

Characterizing TBI • 5 subtypes: – Cognitive – Ocular-motor – Headache/migraine – Vestibular – Anxiety/mood

• Also considered sleep disturbance and cervical strain as associated conditions

Oregon Legislation Oregon Legislation • Max’s Law (2010) applies only to Oregon School Districts. • Senate Bill 1547 (2018) takes effect in 7/2020 • Jenna’s Law (2014) extends the intent of Max’s Law to Oregon youth sports – Allow a larger range of medical professions to make medical and referee organizations. clearance decisions if they undergo an education module • Both Max’s and Jenna’s Laws require school and non-school youth athletic programs to: – Previously allowed: – Create policies and procedures. • Physicians, nurse practitioners, physician assistants and – Provide training. (neuro)psychologists

– Track training. – Now also allowed: – Ensure that staff practice good concussion management. • Chiropractors, naturopaths, physical therapists and – Restrict play when a concussion is suspected. occupational therapists – Provide educational materials/programs. • However, not athletic trainers!

Why a Neuropsychologist in Primary Care?

– 82% (n = 6624) first visit within primary care

– 5% (n = 418) within specialty care (e.g., neurology)

– 12% (n = 947) within the ED • Age: Significantly higher rate of <4 y/o • Race/Ethnicity: 42% AA patients compared to 5% white patients • Payor: – 37% children insured by Medicaid – 24% self-pay – 7% private insurance • TBI rates averaged 1,237 per 100,000 population – 1,457 for males and 1,006 for females

• Majority of TBI cases (92%) were treated in an ED and released

• Most TBIs are unintentional (93.7%), but small subset due to assault (6.1%)

• Nearly three-fourths (72.2%) associated with consumer product

• Product-related TBIs were more frequent among: – <1 year (90.6%) – 1–4 years (81.4%) – 5–9 years (71.9%) – 10–14 years (75.1%) – 15–19 years (54.8%)

Big Take Aways Big Take Aways • 5-9 y/o, bicycle crashes often contribute to TBI • <10 y/o, beds leading cause of TBI – Consistent with prior findings • 10–19 y/o sustain TBIs from contact sports, primarily football

• Placing infants on beds/furniture and fall/roll off • TBIs associated with floors and stairs are common in children and adolescents of all ages (account for approximately 11%) • Bunk beds are especially risky – danger of top bunk – Structural designs, such as uneven flooring and prefabricated stairs

• <1 y/o, car seats problematic, particularly when used as carrier inappropriately – Hard or non-resilient surfaces, such as asphalt and concrete, are associated with skull and upper extremity fractures – e.g., placing on countertop and falling/knocked off Prevention Strategies in and Around the Home • Removing tripping hazards such as area rugs • Improving lighting • Avoiding hard surface playgrounds • Increasing use of home safety devices – Stair gates and guard rails that are easily grasped and no sharp edges • Avoid use of prefabricated stairs – Create tripping hazard when the builder raises/lowers the top-step riser to adjust the stairway height to match the actual height rise between floors • Caregiver education and home safety visits • Enforcement of game and playground safety rules, consistent and proper use of safety gear, notably helmets, adult supervision, and education of youth athletes, parents, and coaches

• 16% history of concussion at index concussion • Clinical course and symptom burden risk of repeat injury: – 22% of those repeat within 2 years vs 15% w/o history of concussion – 2 times greater for one month vs. one week of symptoms • 8.4% (n = 45) repeat concussion within 1 year – 2.5 times greater for Ȳ11 symptoms vs. 0–2 symptoms – Highly correlated, constructed multivariate models • 16.2% (n = 87) repeat concussion within 2 years – including 3.4% (n = 18) with 2 additional concussions • Predicted risk increased • Presence of co-occurring condition non-significant • Median (IQR) time to repeat concussion was 11.8 (5.8–17.8) months

• • Risk among 12-to 15-y/o was 1.85 times that of 9-to 11-y/o The 2-year risk of a repeat concussion did not vary by: – Sex • Risk was 1.5 times higher Ȳ1 pre-existing co-occurring condition – Insurance payor – Migraine/headache (28.6%) – Mechanism of injury – Anxiety (25.0%)

Concussion Severity/Grading & Return to Play (RTP) Defining Concussion… • 14 guidelines identified by Collins et al. (1999)

• 3 emerged as the most widely used: • The Cantu Grading Scales • The Colorado Medical Society Guidelines (CMS) • The American Academy of Neurology guidelines (AAN)

• All use mild, moderate, severe ratings

• Generally based upon symptom duration, post-traumatic amnesia (PTA), and loss of consciousness (LOC)

• “An examination of the grading systems reveals little agreement in grading concussion severity.” • Echemendia, Giza, and Kutcher (2015) Defining Concussion… • 1601 articles screened, 36 studies included • 14 reported on criteria for SRC definitions • 22 on biomechanical aspects of concussion • 6 different operational definitions

• Summary/Conclusions: SRC is a TBI that is defined as a complex pathophysiological process affecting the brain, induced by biomechanical forces with several common features that help define its nature.

• Direct or Impulsive forces • Linear and rotational forces • 70 – 100 g of force

• Hitting your head does not equate concussion • Linear/sequential recovery process • Physiologic recovery continues after resolution of clinical symptoms

Non-specific Symptoms

• Symptoms of concussion have large overlap with: • Sickness (e.g., cold) • Poor sleep • Stress • Anxiety • Depression

https://www.cdc.gov/traumaticbraininjury/symptoms.html General Symptom Resolution Trajectory

• Resolution of clinical symptoms from self-report and objective testing typically 1-2 weeks with age moderation

• Physiologic recovery as demonstrated by MRS, fMRI, qEEG, etc. is variable and outlasts clinical recovery, but latter recovery is 45 days to 3 months typically. • Kamins et al., 2017

• As a provider: • Linear/sequential recovery process, symptoms do not wax and wane • Considerationofpremorbid/concomitantfactorsforprolongedrecovery • Exceptionissymptomexacerbationwithphysicalexertioninacuterecoveryperiod • Symptom report in acute recovery period is most reliable

• Single mTBI vs. multiple mTBI very small differences (d = .06) – Limited to trivial cumulative impact

• Executive functions most susceptible to multiple mTBI – White matter maturation occurs last in frontal lobes

• Yet to identify threshold (e.g., 5th concussion) that predicts longstanding neuropsychological impairment

• The long term cumulative effects of concussion regarding cognition is a contentious research topic: Concussions vs. Repetitive Sub-Concussive Impacts

– Some reviews find negligible impairments or inconclusive findings • High contact athletes (football) perform worse • Karr, Areshenkoff, & Garcia-Barrera, 2014; than low contact athletes (basketball, baseball, soccer, wrestling, volleyball, paddling, and • Solomon, Ott, & Lovell, 2011; cheerleading) on ImPACT testing. • Yumul & McKinlay, 2016 – Tsushima et al. (2016)

– While others show long-term cognitive effects from repeated • High contact (lineman) youth football players concussion primarily related to elite athlete status perform worse than low contact (receivers and • Manley et al., 2017 defensive backs) players on ImPACT testing. • Vos, Nieuwenhuijsen, & Sluiter, 2018 – Tsushima et al. (2017) 1-time NP Consultation as PCS Intervention Concussions vs. Repetitive Sub-Concussive Impacts

• Exposure to contact football before or after age 12

– >2 times increased odds for problems with behavioral regulation (e.g., easily angered), apathy, and executive function (e.g., organizing/planning)

– >3 times increased odds for depression • Alosco et al., 2017

Kirkwood et al., 2016

• Minimal impact on school grades, national exam scores, and graduation rates at a group level.

• PCS symptoms and self-reported executive dysfunction more predictive of poor school performance than cognitive testing.

• Concussion team at school still very important for reintegration into school following rest.

• How much does missed school matter?

Pain

Sleep Concussion Psychiatric

Iatrogenic Best Predictors of Outcome in Concussion

• Age: mixed findings • Headache (post-injury):worse outcomes • Sex: mixed findings • Total symptom report: strong • Prior Concussions: mixed findings evidence of worse outcomes

• • Migraine: mixed findings Mental health history: strong evidence of worse outcomes

• ADHD, LD, etc.: minimal support

• LOC: minimal support

• PTA: minimal support

• Co-morbid problems like depression, anxiety, and sleeplessness are inherent in chronic pain.

• The brain responds to ‘painful’ or nociceptive events in a host of brain regions/ circuits in a flexibly accessible manner: – Sensory – Discriminatory – Emotional/affective – Cognitive/decision making – Brainstem modulatory – Motor

• People have higher ratings of pain when anxious • People have higher ratings of pain when sad – hippocampus/entorhinal complex with interactions to – Higher activations in emotional regulatory the anterior insula and mid anterior cingulate circuitry (e.g., orbitofrontal cortex) Î Î – higher pain processing activation (e.g. amygdala, insula, inferior frontal gyrus, anterior cingulate). – higher pain processing activation (e.g. amygdala, insula, inferior frontal gyrus, anterior cingulate). • Descending Pain Modulatory System (DPMS) Depression, anxiety, and – Inhibitory and facilitatory modulatory action threat act as a physiological largely based upon expectation amplifier for pain. – Healthy controls given intravenous painkiller during brain-imaging study while given painful stimuli throughout. • Hidden injection • Positive expectation • Negative expectation

Iverson et al., 2015

Iverson et al., 2015 Iverson et al., 2015 Iverson et al., 2015 Iverson et al., 2015

Iverson et al., 2015 Iverson et al., 2015

Article • The most methodologically rigorous studies to date have not demonstrated benefit of an initial Prolonged Activity Restriction After period of 5 to 6 days of complete rest over an Concussion: Are We Worsening Outcomes? earlier return to activity.

, Noah D. Silverberg, PhD , Michael W. Kirkwood, PhD , Raquel Bernier, Marc DiFazio, MD1 2,3 4,5 MD1, and Grant L. Iverson, PhD6,7,8,9 • Authors could not find studies suggesting that Clinical Pediatrics 2016, Vol. 55(5) 443–451 © The Author(s) 2015 Reprints and permissions: thinking, reading, or studying cause sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922815589914 cpj.sagepub.com neurometabolic demands, or changes in the brain that could be harmful. Harmful Effects? Additional Psychological Factors Related to Recovery • Nocebo effect • Coping Style/Illness Perception – Remember the DPMS – Anderson & Fitzgerald, 2018 – Priming effects • Good Old Days Bias • Activity Restriction Model of Depression – The tendency to underestimate pre-injury problems and overestimate pre- injury health. • Physical Deconditioning • Brooks et al., 2014

• Conclusion: Gradual/graded return to normal life activities • Cogniphobia following 2-3 days in most cases. – Avoidance of mental exertion out of a fear of developing or exacerbating a headache. • Silverberg, Iverson, & Panenka, 2017 • Similarly, a more recent systematic review concluded 24-48 hours of cognitive and physical rest is a appropriate for most patients. • Diagnosis Threat – Form of stereotype threat - reduced cognitive/academic performance due – Schneider et al., 2017 to beliefs or reminders following a neurologic injury. • Fresson, Dardenne, & Meulemans 2018

Sleep and Mental Health – Blake et al., 2017 Sleep and General Health

• 30-40% of US youth experience inadequate sleep • Sleep deprivation increases risk of: • 30% have a sleep disorder – Illness susceptibility (4x increase of cold less than 6 hours) – Insomnia – – Delayed Sleep Phase Disorder Orthopedic injuries • Pervasive in psychiatric disorders – <8 hours 2x increase in concussion rates in youth – Share highest % of connected symptoms within all symptoms of DSM-IV – Lifestyle disease (e.g., diabetes, obesity, heart disease) – Dementias • May precipitate and maintain psychiatric conditions • 60% of Alzheimer’s patients have sleep disorder that preceded – Ð Sleep ÎÏAnxiety & Depression diagnosis by several years – Mortality MORE THAN • Decades decrease in life expectancy with chronic sleep deprivation – Ï Anxiety & Depression ÎÐSleep Sleep and TBI risk Sleep Disturbance Following Concussion

• Sleep deprivation hinders: • 30-70% report sleep difficulties 1-3 weeks post-injury – Reaction time – Hypersomnia is common – Judgment • Following acute phase of recovery – Balance – 30% report insomnia – Coordination – Approximately 40% can have circadian rhythm shift (delayed) – Proprioception – 40-70% report fatigue – General cognition (learning, memory, problem solving, etc.) – 30% report sleep apnea • The pattern and time frame of sleep disturbance may vary substantially among patients who have sustained a concussion. – Mosti, Spiers, & Kloss, 2016

Sleep and Concussion Sleep and Concussion • Subjective sleep complaints are 3x more likely to develop concomitant • headaches in the first 6 weeks following an MTBI. Switching between sleep and wake is complex: – Ventrolateral preoptic nucleus – Also more likely to have depressive symptoms and irritability. • ƀ-aminobutyric acid and Galanin producing neurons that, when • Chaput et al., 2009 stimulated, are responsible for normal sleep – Posterior lateral hypothalamus • Sleep disturbance in the acute TBI period was associated with • Produces orexin increased symptoms of depression, anxiety and apathy (mild TBI group only) 12 months post-injury. – Tuberomammillary nucleus • • Rao et al., 2014 Releases histamine – Dorsal Raphe Nucleus • Produces Serotonin • In fact, sleep disturbance, even in the acute post-TBI period, predicted the development of anxiety and depression in the chronic period for all – Locus Coeruleus severities of TBI. • Produces Noradrenaline • Morse & Garner, 2018 • Similar “switches” regulate the transitions between NREM and REM sleep

Sleep&Concussion Consultation & Management • Exact mechanisms by which concussion affects sleep are not yet Model fully understood.

• Disturbances in orexin, serotonin, histamine, and noradrenaline have all been proposed as potential mechanisms for concussion- induced sleep dysregulation.

• In addition, neuro-inflammation and disturbances in the newly described glymphatic pathway could also play a role in the concussion-sleep disturbance relationship. Generally

• Set positive and realistic expectation! – Expectancy effect – Importance of early education – well validated intervention – Null effects for cognitive rehabilitation per 2 systematic reviews and empirical support for vision therapy is tenuous

• Resume normal activities as soon as reasonably possible, including light exercise!

• Reinforce progress! – Prolonged symptom pacing recommendations = iatrogenic

• Prospective, multicenter cohort study (9 EDs) – 5-18 cohort (average was 12) – 2413 participants (40% female)

• Physical activity participation and PCS severity were rated using standardized questionnaires in the ED and at days 7 and 28 post-injury.

• Physical activity within 7 days of acute injury compared with no physical activity was associated with reduced risk of PCS at one month.

• N = 103 – (aerobic exercise: n = 52; 24 female [46%]; stretching, n = 51; 24 female Neuropsychology Service in [47%]) Family Med/Sports Med • Exercise group seen a mean (SD) of 4.9 (2.2) days after SRC • Stretching group seen a mean (SD) of 4.8 (2.4) days after SRC • Evaluation:

• No differences in age, sex, previous concussions, time from injury, initial symptom severity score, or initial exercise treadmill test and – Half day and full day evaluations physical examination results. • Concussion/mTBI • Exercise recovered in a median of 13 (IQR = 10-18.5) days • Stretching recovered in a median of 17 (IQR = 13-23) days – (P = .009 by Mann-Whitney test) • Neurodevelopmental disabilities

• Nonsignificant lower incidence of delayed recovery in the aerobic exercise group (2 participants [4%] in the aerobic group vs 7 [14%] in • General neurological conditions the placebo group; P = .08). OHSU Concussion Program

• Concussion Treatment Clinic:

– The concussion follow-up clinic: 3-6 sessions

– Partnership of ATC, NP, Sports MD

– ATC: treadmill test, sensory/motor intervention

– NP: sleep protocol, behavioral activation, exposure

Practical take homes • Pre-injury mental health and sleep quality will predict outcomes

• High acute symptom burden (particularly headache), onset of sleep dysregulation and/or activity withdrawal will prolong recovery – Dr. Herring’s perspective on disability

• Early exercise and sleep intervention will likely improve clinical outcome

• Returning to normal daily activities (physical, recreational, social) as soon as possible (2-3 days), often gradually/incrementally, will likely improve clinical outcome

Practical take homes • Linear/sequential recovery process, symptoms do not wax and wane – Consideration of premorbid/concomitant factors for prolonged recovery – Exception is symptom exacerbation with physical exertion in acute recovery period

• Symptom report in acute recovery period is most reliable

• Consider the person who sustained the concussion, not just persistent symptoms through the medical lens. – The more distal from injury, consider referring to a mental health therapist rather than a rehabilitation therapist.