Pediatric FEES Workshop

Presented by: J. Paul Willging, MD Claire K. Miller, PhD

March 2 & 3, 2018 Lady Cilento Children’s Hospital South Brisbane, QLD Cincinnati Children's Hospital Medical Center - CCHMC

CCHMC 678 beds Level IV NICU Dr. Sabin -Oral Polio Vaccine Dr. Whitsett – Surfactant Brief Biography Jay Paul Willging, MD Jay Paul Willging,MD • Born and raised in Cincinnati, Ohio

• Did not know my first name was JAY until I was in first grade – computers DO NOT understand Education • University of Cincinnati • BS in Biology 1974 - 1978 • Master’s Studies in Vertebrate Ecology 1978 - 1981 • MD 1981 - 1985 • General Surgery training 1985 – 1987 • Otolaryngology – Head and Neck Surgery Residency 1987 – 1991 • Cincinnati Children's Hospital Medical Center • Pediatric Otolaryngology Fellowship 1991 - 1992 Professional Activities • University of Cincinnati • Department of Otolaryngology – Head and Neck Surgery 1992 – present • Tenured Professor • Cincinnati Children’s Hospital Medical Center • Division of Pediatric Otolaryngology 1992 – present • Director of the Pediatric Otolaryngology Fellowship training program 1998 – present • Director of Clinical Operations 2013 to present • Multidisciplinary Clinics • FEES, VPI, Craniofacial Personal Interests

• Married with four adult children • 4 grandchildren • Interests outside of medicine • Farm activities • Hunting • Water sports • Flying Pediatric Otolaryngology • 35,000 clinic visits per year • 12,000 surgical patients per year • Airway Reconstructions • 140 per year • Cochlear Implantation • 60 per year • Fourteen Faculty as of July 2018 Claire Kane Miller, PhD Education Miami University of Ohio Bachelor of Science in Education,1980 Master of Science in Speech Pathology,1982 University of Cincinnati PhD – Communication Sciences and Disorders, 2006 MHA – Master of Health Administration, 2011 Professional Activities • Cincinnati Children’s Hospital Medical Center • Program Director, Aerodigestive and Esophageal Center/Interdisciplinary Feeding Team • Speech-Language Pathologist, clinical and research activities • University of Cincinnati • Adjunct Assistant Professor, Department of Communication Sciences & Disorders • Field Service Associate Professor – Affiliated, University of Cincinnati, Department of Otolaryngology Interests • Married with 2 (adult) children • Interests • Reading • Travel • Lakes, oceans, and sunsets • Baking – with a chocolate focus Division of Speech-Language Pathology

Base program with 8 satellites = 140+ speech pathologists • Inpatient, outpatient, team, and clinic-based • High Risk Infant Clinic • Interdisciplinary Feeding Team Clinic & Multidisciplinary Feeding Treatment Program • Voice Clinic • Velopharyngeal Insufficiency Clinic (VPI Clinic) • Craniofacial Team • FEES Clinic (Fiberoptic Endoscopic Evaluation of ) • Video Swallow Clinic • Neurodevelopment Clinic for Heart Institute patients FEES Course Objectives • Describe the protocol used for pediatric fiberoptic endoscopic evaluation of swallowing (FEES) across conditions and locations (NICU, ICU, cardiac unit, and the outpatient clinic) • Identify the clinical indications for FEES, compare and contrast to the videofluoroscopic swallowing assessment • Describe the equipment components needed for FEES and the procedures for cleaning and safety • Increase recognition of anatomical landmarks and key structures in infants, children, and adolescent patients • Describe available methodology for the assessment of aspiration • Explain how to implement the use of FEES in the clinical setting Pediatric Fiberoptic Endoscopic Evaluation of Swallowing (FEES) Workshop

J. Paul Willging, MD Claire Kane Miller, PhD Lady Cilento Children’s Hospital March 2 & 3, 2018 Learner Outcomes

• To describe the components of a comprehensive aerodigestive evaluation Overview of the CCHMC Aerodigestive & Esophageal Center Complexity of Feeding and Swallowing Issues • Feeding issues span the scope of multiple disciplines • Multiple cases to be discussed in the course are “Aerodigestive/Interdisciplinary Feeding Team” patients • What is the Aerodigestive and Esophageal Center? CCHMC Aerodigestive & Esophageal Center

• The Aerodigestive & Esophageal Center (ADEC) - formed in 2001 Evaluation/management of congenital or acquired anatomic, structural, or functional anomalies of the larynx and/or trachea that interfere with normal function Patients seeking decannulation or answers regarding complicated airway, digestive, and feeding issues Aerodigestive & Esophageal Center Cincinnati Children’s Hospital Medical Center

• Mission: • Provision of interdisciplinary care for patients with complex disorders of the airway and digestive tract • Goal • Improve clinical outcomes for patients by coordinating diagnostic and operative procedures and implementing an integrated treatment plan Aerodigestive Assessment

• Otolaryngology • *Airway Protection/Swallowing • Pulmonary Medicine • – reflux issues, laryngopharyngeal reflux, extra-esophageal reflux • Pediatric Surgery • Consultative services – Radiology, Speech Pathology, Nutrition Therapy, Social Work, Occupational Therapy, Interdisciplinary Feeding Team Esophageal Center

• Established in 2011 for treatment of children with complex esophageal disorders – under the Aerodigestive “umbrella” – (EA), including long gap esophageal atresia – Tracheoesophageal fistulas (TEF) – Esophageal duplications – Bronchogenic cysts – Esophageal strictures (narrowing of the ) – Caustic ingestions Center for Pediatric Voice Disorders

• The Center for Pediatric Voice Disorders Established in 2004 – Examining acoustic, aerodynamic, perceptual and endoscopic data – Focus on voice and swallowing outcomes in children with complex airway disorders – Offer treatment and voice care guidelines as part of perioperative care – Includes 3 voice scientists/clinicians, voice clinician, and 2 ENTs “LEAD” SERVICES in the ADEC

• ENT • Major airway issues, seeking airway reconstruction – not currently on ventilator • Pulmonary Medicine • Respiratory issues, ventilator dependent, patients who have had surgical issues put on hold secondary to pulmonary issues • Gastroenterology – • Structural and functional conditions – Eosinophilic Esophagitis, colonic interposition, esophageal dysmotility, EA, esophageal web/stricture, GER, intestinal disorders, malabsorption disease, motility disorders • General Pediatric Surgery/Esophageal Center Significant reflux, TEF/EA with esophageal or reflux issues without major airway issues, patients with anatomic intestinal issues, omphalocele • Interdisciplinary Feeding Team • Feeding issues are primary concern, known or suspected problems with airway protection/swallowing, problems tolerating tube feeds, slow/inadequate weight gain, limited oral intake • Patient must require 2 or > services on the team: MD, Nurse Practitioner, RD, RN, SLP, OT, SW Interdisciplinary Feeding Team

Administrative Registered Social Worker Support Dietician

Physicians Nurse Speech Medical Director – ENT Practitioner Pathologist Gastroenterologist Human Genetics Physician

Occupational Family and Nurse Therapist Patient Interdisciplinary Feeding Team Visit

• Nutritional Assessment • Oral motor/feeding evaluation • Social Work consultation • Nurse Practitioner/Physician assessment • Team discussion of impressions and consensus • Recommendations – Treatment Plan Communication: The "O" Drive – shared drive for internal use only

Patient: • Reason for referral/Parental Goal: Home Phone/Cell Phone • PAST MEDICAL HISTORY PER PARENTAL MR: REPORT (as of initial intake by ): DOB: • Meds: Pt’s home City, State: • Allergies: Email: • SBE: Referring Physician: • Isolation: Primary Care Physician: • Specials: Other Local Physicians: • Wt/Date: Aerodigestive primary MD: • Previous Tests & Procedures: Aerodigestive lead RN: • Aerodigestive team: • ADSC Evaluation: ______• PLAN: ______• REVISED: Intake Process

• Lead service completes initial intake with parent • Synopsis of history, evaluates primary problems and concerns, additional factors • Tentative plan for assessment Assessment Components

• Anesthesia Consult • MLB, Flexible Bronchoscopy, EGD • High resolution CT +/- • Impedance • FEES exam • Videofluoroscopic Swallowing Study • Interdisciplinary Feeding Team Evaluation Diagnostic Assessment Overview

J. Paul Willging, M.D. Professor of Otolaryngology - Head & Neck Surgery Cincinnati Children’s Hospital Medical Center University of Cincinnati Director, CCHMC Pediatric FEES Clinic Maturational Development of Swallow Function

• Matures in concert with • Anatomic changes • Neuromuscular development • Critical periods for learning Children Will Compensate for Change

• Swallowing process matures in concert with physical development • Require continued oral stimulation and feeding opportunities during growth and development • The problem associated with NPO status Anything that interferes with a child’s ability to feed orally creates a condition where a feeding disorder may develop Epidemiology of Pediatric Feeding & Swallowing Problems

• Minor “feeding problems” occur in up to 25%-35% of normally developing children • Arvedson, 2008; Burklow et al., 1998 • Incidence and prevalence increases in children with medical problems - structural, neurological, metabolic, or mixed etiology (40%-70%) • Rudolph’s Pediatrics, 2003 • Rudolph & Link, 2002 • Manikam & Perman, 2000 Minor Feeding Problems

Maladaptive Feeding Behaviors

• Refusal to get into the highchair • Batting at spoon • Crying through meal • Spitting food out • Throwing food • Gagging and/or vomiting Biobehavioral Nature of Feeding

• >85 % of patients have a behavioral component contributing to their current feeding problem • <15% have problems which are solely behavioral in nature • Interaction of biology and behavior How to Approach a Child With a Suspected Feeding Problem

• Classify the significance of the feeding problem • Determine immediate disposition of the child • Develop an initial plan of evaluation Comprehensive Diagnostic Assessment • The mixed nature of etiologies spans the scope of multiple disciplines • Structural • Neurologic • Metabolic • Sensory • Cardiorespiratory • Not sure • Overlap Overlap

• Etiologies are not mutually exclusive • Multiple interacting characteristics • i.e. Neurological issues and cardiorespiratory compromise may be present with structural diagnosis • Classifying complex pediatric feeding disorders. Burklow, et al., 1998 • Rommel et al., 2004 (Complexity of feeding problems) Evaluation

• Significance of the • Nutritional assessment problem • Physical examination • Document the safety of • Oral motor evaluation swallowing • Examination of structure • Investigate the etiology of the problem • Objective studies • Medical / Surgical • VSS History • FEES • Endoscopy • MRI / CT History

• Age of onset of • Timing • Acute, progressive, or chronic • Related events • Surgical intervention • Seizure activity • Caustic ingestion history • Constant or intermittent • During meals, between meals, or both • Solids, liquids, or both • Foreign body possible History

• Accompanying symptoms • Sialorrhea / drooling • Hoarseness, voice changes, or other laryngeal symptoms • Weight loss / failure to thrive • Odynophagia • Correlation between feeding • Coughing, choking, gagging, nausea, or vomiting • Stridor, cyanosis, or apnea • Symptoms of GERD / LPRD History • Pregnancy • Maternal infections • Drug use • Thyroid dysfunction • Polyhydramnios • Birth history • Traumatic delivery • Respiratory distress • APGAR scores History • Neonatal Hx • NICU • Intubation • Surgical interventions • Growth charts • Respiratory status • Infection history Family / Social History • Primary caregivers • Other persons in the home • Feeding problems in the family • Environmental factors Nutritional Assessment

• Anthropometric Measurements • Laboratory Data / Medical History • Diet History • Vitamin Supplements / Herbal Therapies Nutritional Intervention • Establish caloric, protein, vitamin/mineral and fluid goals • Formula Selection / Supplements • Suggestions to Increase the Caloric Density of Diet • Feeding Schedule Clinical Evaluation

• Starting point: baseline assessment of feeding status, oral stage problems, related medical problems, identify clinical signs & symptoms of dysphagia • Limitations: cannot assess beyond the oral phase • Outcome: provides basis for clinical pathway to determine further assessments, instrumental & other Pediatric Clinical Oral Motor/Feeding Assessments

• Clinical assessment provides basis for clinical pathway -next steps • Available tools – population specific, age specific, assessment domains – behavioral, sensory, oral motor • Strengths and limitations of available instruments • Accuracy (+/-) of prediction/detection of airway protection issues • Heckathorn, 2016; Rommel & Hamdy, 2016; deMatteo et al., 2005 Oral Motor Evaluation

• Oral sensory • Hypersensitivity • Sensitive to touch, gagging, texture refusal, resistance to utensils • Hyposensitivity • Pocketing, inefficient bolus movement, limited OM movements • Impaired oral tactile discrimination • Difficulty differentiating between various textures simultaneously Oral-Pharyngeal Skills

• Oral anatomy • Oral motor patterns • Symmetry • Lip retraction, lip pursing hyposensitivity • protrusion, thrust, retraction or hypotonicity • Wide jaw excursions, phasic biting or limited range of motion • Developmentally appropriate skills • Bottle feeding • Cup Drinking • Utensil Use • Transitioning to solids • Advancing textures Cervical Auscultation

• Screening Tool • Auditory assessment of respiration/swallow, changes during feeding • Make inferences about the pharyngeal phase • Reliability/Validity questionable: • (refs) • Therapist dependent • Reynolds, et al., 2009 Physical Examination

• Head and Neck exam • Thorough oral cavity exam • lips, tongue, teeth, palate, tonsils, suck reflex • Cranial nerve exam • Assessment for nasal patency • Assessment of laryngeal function • Flexible fiberoptic laryngoscopy can be performed in office setting without sedation Physical Examination • Overall level of alertness • Communication skills • Basic posture and position • Control of secretions • Oral structures (lips, tongue, palate) • Oral function (range of movements, strength) • Assessment of nutritive skills Respiratory Compromise

• Inability to coordinate respiration and swallowing • Cardiac – congenital anomalies • Dysphagia after heart procedures (Kohr, et al., 2003; Haarbauer- Krupa, 2006) • Injury to the recurrent laryngeal nerve • Lung – cystic fibrosis, bronchiectasis, prematurity • Congenital diaphragmatic hernia • Airway anomalies – subglottic stenosis, hemangioma, VCP, laryngomalacia, laryngeal stenosis, tracheal stenosis • Pulmonary rings/slings resulting in compression of trachea and/or esophagus Laryngomalacia • Interference with respiration and coordination of swallowing Clinical Signs and Symptoms of Possible Swallowing Dysfunction • Coughing, choking, gagging during feeds • Noisy, wet respiration associated with feeding • Physiologic signs: apneic spells, color changes, bradycardia, increased respiratory rate, oxygen desaturations • Evidence of food or liquid in tracheostomy tube during/after feed • Congestion, pneumonia/unexplained respiratory illnesses

• Loughlin & Lefton-Greif, 1994; Arvedson & Lefton-Greif, 1998; Newman, 2001; Arvedson & Brodsky, 2002; Lefton-Greif, 2008; Weir, 2009; Prasse & Kikano, 2009; Wallis et al., 2010 Decision-making • Medical work-up – additional referrals • Instrumental Examinations • Videofluoroscopic Swallowing Study • Fiberoptic Endoscopic Evaluation of Swallowing Pediatric Video Swallow Studies • Radiographic view of oral, pharyngeal, and cervical esophageal phases of swallowing • Screening view of esophageal phase • Collaborative examination - Radiologist & Speech Pathologist VFSS: Why & When? • Patient is at risk for swallowing dysfunction and/or aspiration by history or observation - need to define oral, pharyngeal, & upper esophageal components of the swallow

• Patient presents with hx of prior aspiration pneumonia

• *When - do in baseline health status, not during acute illness with rare exceptions VFSS Aims • Define movement patterns of structures in oral cavity, , larynx, & through upper (cervical) esophagus • Measure efficiency of swallow • Determine reason for swallowing dysfunction – bolus propulsion issue or airway protection failure – both? • If aspiration occurs, determine when, why, how much, & response or lack of response VFSS Aims • Examine intervention possibilities in the context of the study • Postural/position changes • Compensatory strategies – eg flow rate • Diet modifications (viscosity, texture changes) • Maneuvers in children (not infants) Interpretation

CCHMC 2018

Drop-down boxes for items in flowsheet format in EMR Video Swallow Standardization with Infants & Young Children - Difficult • Not always possible to start thin liquid in a measured bolus & increase systematically • Breast feeders: How accurate can findings be with EBM via nipple? • Nipple feeder: How many suck/swallow sequences should be observed? VSS Advantages

Advantages: • View of all phases of the swallow – oral, pharyngeal, cervical esophageal • No discomfort • Opportunity to try compensatory strategies • Provides ongoing view of airway protection during rapid chain swallowing sequences i.e. bottle-feeds VSS: Disadvantages • Radiation exposure – particularly with repeat exams • Child may resist barium – may not get sufficient sample for meaningful interpretation • Not feasible if child has negligible oral intake • May be dependent on personnel involved Summary

• Described as the “gold standard” – but is it? • Need more evidence as to accuracy in pediatrics and relationship to long term outcomes • Issues with inter-rator reliability – implications for accuracy of interpretation and appropriateness of subsequent recommendations re feeds • Stoeckli, et al., 2003; Kuhlemeier et al., 1998 Pediatric FEES

• Fiberoptic Endoscopic Evaluation of Swallowing • Adaptation of Susan Langmore’s FEES™ procedure, use of flexible endoscope transnasally to assess swallow function • Pediatric application developed at CCHMC in 1993 • Protocol for infants, children, and adolescents • NICU • Ideal for Breastfeeding • No adverse events in our experience • *Collaborative exam – Speech Pathology & Otolaryngology Focus of FEES • Assessment of Anatomy, Function, and Sensation • Clear definition of anatomy • Degree of sensitivity • Airway protection capability • Overall safety of swallowing

• Willging, Miller, Thompson, & Rudolph, 2000 Potential FEES Candidates • Abnormal videofluoroscopy results – more info needed re swallowing function, anatomy, sensory awareness • Pre-operative assessment of swallowing ability, risk for aspiration post-operatively • Unable to be positioned for videofluoroscopic swallowing study • Need for interval exams – avoid radiation • Questionable secretion management ability • Ideal for infants and children who accept little or no oral intake, or who breastfeed only Swallowing Parameters Assessed During FEES • Secretion Management • Timing of Swallow Onset • Penetration – location and depth • Aspiration – before/after swallow – not during • Residue – following swallow • Sensory Assessment View of Anatomy & Function Pediatric FEES Protocol Feeding Portion

• Guidelines – order and protocol may differ depending on patient needs • If patient NPO, has never fed – may start with drop of food coloring flavored with small amount of carbonation, med flavor syrup, or juice • Sensory enhancement • Pelletier & Lawless, 2003 • Depending on toleration & airway protection ability, advance to small amounts pureed: applesauce, pudding • Advance to soft solid, thin, thickened liquids as appropriate Utility of FEES

• Specific focus on airway protection –identification of anatomic abnormalities • Assessment of sensory threshold • Presence of spontaneous clearing swallows • No radiation, time limitations, or need for barium • Can be used as an initial, interval, or follow-up exam Advantages of FEES • Clear view of structures - clear view of functional ability to protect airway • Allows assessment of a child’s ability to mange secretions • No time limits • Portability • Can assess sensory function • Can help to determine readiness for oral feeds, surgical candidacy • Does not require alteration of food/liquid with barium Disadvantages of FEES

• Discomfort with scope passage is possible – usually subsides once stimulation of nasal mucosa ends • Presence of scope may trigger gagging &/or vomiting • View disappears briefly during moment of swallow “white out” – therefore unable to detect events during the swallow • Consistent loss of view during sequential swallowing i.e. bottle intake – implications for interpretation • Focus limited to pharyngeal phase of swallowing Contraindications for FEES

• Patients with choanal atresia • Patients who are orally intubated • Rapid sequential swallows need to be assessed – FEES does not allow a clear view during rapid sequential swallows • Physiologic instability – high respiratory rate, low oxygen saturation UGI: Upper Gastrointestinal Series

• View of esophagus, stomach and small bowel under fluoroscopy • Define structural integrity, rule out anatomical factors such as innominate artery compression, malrotation • Completion prior to video swallow study ideal Achalasia Cricopharyngeal Achalasia

• Inability of the upper esophageal sphincter to relax • Cricopharyngeal bar on VSS • Treatment • Dilation of UES • Botox injection into the UES • Cricopharyngeal myotomy Pharyngoesophageal Manometry

• Transnasal passage of transducer catheter • Pharyngeal and esophageal peristalsis • UES and LES sphincter function and pressure • Technically challenging in children • Indications evolving • Patients at risk for motility issues • Caustic ingestion • Tracheoesophageal fistula • Esophageal resection • Esophageal stricture Accessory Imaging Studies

• MRI head • suspected brainstem and skull base problems • Chiari Dye Testing

• Dye added to liquids, foods – in trach patients, can monitor presence of dye via suctioning • Issues with reliability, validity • Evidence questionable as to from above or below in some cases

• Ref: O’Neil-Pirozzi, et al., (2003); Dysphagia Scintigraphy

• Spit Scan • Radionuclide isotope in buccal cavity • Technectium 99 • Indicates aspiration in lungs • Scanning done at intervals over a 2 hour period • Questionable: material aspirated during swallowing of saliva or during a reflux event • Not a “true” evaluation of swallowing function Microlaryngoscopy and Bronchoscopy

• General anesthesia in the operating room • Diagnostic assessment of the larynx and trachea Structural Problems

• Mechanical interference with swallowing • Laryngomalacia • Tracheoesophageal fistula • Laryngeal cleft • Hypopharyngeal stenosis • Cricopharyngeal achalasia Rigid Endoscopy

• Rigid endoscopy – Rigid endoscopy under general anesthesia with spontaneous breathing • Appropriate size bronchoscope always available • Careful evaluation of the stenosis - thickness, length, extension to adjacent sites, distance from the vocal cords, second airway lesions • Vocal cord mobility, active and passive • Posterior glottic stenosis, stomal problems, tracheomalacia, etc RIGID BRONCHOSCOPY OR AND FLEXIBLE BRONCHOSCOPY Advantages of Rigid Scopes

• Optics • Ability to manipulate • Exposure/visualization tissue • Instrumentation • Geometry Advantages of Flexible Scopes

• Greater peripheral range • Access to upper lobes • Patients with “impossible anatomy” • Mandibular ankylosis, hypoplasia • Cervical ankylosis • Oropharyngeal tumor masses • Patients with artificial airways • Scopes follow natural airway with minimal anatomic distortion • BAL/suctioning Bronchoalveloar Lavage

• Lipid laden macrophages • Quantitative cultures High Resolution Chest CT Interventions • Is it safe to feed • How to feed • Interventions to improve safety of feeding • Medical interventions • Surgical interventions • Monitoring Who Needs Special Evaluations • Many evaluation options • Not everyone needs everything • Who needs Multidisciplinary Care? • Two or more medical disciplines Multidisciplinary Team • Specialists • Collaborative working relationship • Good communication • Trust • Multidisciplinary treatment approach • Longitudinal follow-up References • Arvedson, J. C., & Brodsky, L. (2002). Pediatric swallowing and feeding: Assessment and management: Cengage Learning. • Arvedson, J. C., & Lefton-Greif, M. A. (1998). Pediatric videofluoroscopic swallow studies: A professional manual with caregiver guidelines: Communication Skill Builders/Psychological Corporation. • Burklow, K. A., McGrath, A. M., Valerius, K. S., & Rudolph, C. (2002). Relationship between feeding difficulties, medical complexity, and gestational age. Nutrition in Clinical Practice, 17(6), 373-378. • Burklow, K. A., Phelps, A. N., Schultz, J. R., McConnell, K., & Rudolph, C. (1998). Classifying complex pediatric feeding disorders. Journal of Pediatric Gastroenterology and Nutrition, 27(2), 143-147. • Heckathorn D, Speyer R, Taylor J, et al. (2016). Systematic review: Non-instrumental swallowing and feeding assessments in pediatrics. Dysphagia. 31(1):1-23.

84 References • Kuhlemeier, KV, Yates P, & Palmer, JP.(1998). Intra-and interrater variation in the evaluation of videofluorographic swallowing studies.13(3): 142-147. • Loughlin, G. M., & Lefton-Greif, M. (1994). Dysfunctional swallowing and respiratory disease in children. Advances in pediatrics, 41, 135. • Manikam R. & Perman JA. (2000). Pediatric feeding disorders. J Clin Gastroenterolog, 30: 34-46. • Newman, L. A., Keckley, C., Petersen, M. C., & Hamner, A. (2001). Swallowing function and medical diagnoses in infants suspected of dysphagia. Pediatrics, 108(6), e106-e106. • O’Neil-Pirozzi, T.M., Lisiecki, D.J., Momose, D.K., et al.(2003). Simultaneous modified barium swallow and blue dye tests: A determination of the accuracy of blue dye test aspiration findings. Dysphagia, 18(7):32-38.

85 References • Pelletier, C.A. & Lawless, H.(2003). Effect of citric acid and citric acide-sucrose mixtures on swallowing in neurogenic oropharyngeal dysphagia. Dysphagia, 18(4):231-241. • Prasse, J & Kikano, G. (2009). An overview of pediatric dysphagia. Clinical Pediatrics, 48(3): 247-251. • Rommel, N 7 Hamdy, S. (2016). Oropharyngeal dysphagia: manifestations and diagnosis. Nature Reviews, 13:49-59. • Reynolds, EW, Vice FL, & Gewolb, IH. Variability of swallow- associated sounds in adults and infants.(2009). Dysphagia, 24(1):13-19. • Rudolph, C. D. (1994). Feeding disorders in infants and children. J Pediatr, 125(6 Pt 2), S116-124. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/7983566 • Stoeckli SJ, Huisman T, Burkhardt A, et al. (2003). Interrater reliability of videofluoroscopic evaluation.Dysphagia, 18(1): 53-57.

86 References

• Wallis, C., & Ryan, M. (2012). Assessing the role of aspiration in pediatric lung disease. Pediatric Allergy, Immunology, and Pulmonology, 25(3), 132-142. • Weir, K., McMahon, S., Barry, L., Masters, I., & Chang, A. (2009). Clinical signs and symptoms of oropharyngeal aspiration and dysphagia in children. European Respiratory Journal, 33(3), 604- 611. • Willging, JP. (2000). Benefit of feeding assessment prior to airway reconstruction.Laryngoscope, 110(5): 825-834.

87 Pediatric FEES History Roles on the Pediatric FEES Team

J. Paul Willging, M.D. Claire Miller, Ph.D. CCC/SLP

Lady Cilento Children’s Hospital South Brisbane QLD March 2 & 3, 2018 Learner Outcomes • Explain roles in the pediatric FEES clinic and the logistics of how to implement FEES in the clinical setting Development of the FEES Procedure • Susan Langmore, Ph.D. (1988, 1991) • Robert Bastian, M.D. (1991, 1993) • Martyn Mendelsohn (1993) • Pediatric Applications (March 1993) • Willging (1994; 1996) • Willging, Miller, Link, & Rudolph (2000); Leder & Karas, 2000; Link, et al., 2000; Hartnick et al., 2000 • Manrique et al., 2003 • Leder & Murray, 2008 • Thottam, et al, 2015 • Suterwala, et al., FEES in the NICU, 2017 • Vetter-Laracy et al., 2018 Opportunity • Gastroenterologist – Colin Rudolph • Pediatric Otolaryngologist – Robin Cotton • New Faculty – me • Speech Language Pathologist with swallowing expertise – Claire Miller • Question: think you can put a telescope into the hypopharynx and watch a child swallow without making them gag? • Enrolled in Langmore’s course in Dallas, Texas Pediatric FEES Pilot Study

• Permission obtained from IRB to evaluate swallowing in patients already undergoing nasopharyngoscopy (nodules, VPI) • Goal to determine feasibility of doing FEES with pediatric patients • Are we creating abnormal swallowing parameters • Normative data collected Expansion of Study

• Inclusion of pediatric patients with known or suspected airway protection problems

• First Pediatric FEES at CCHMC March 1993 Verification of the Technique - I

• Does the presence of an endoscope create abnormal swallowing parameters patients • Comparison of VSS & FEES Results • Split screen studies: simultaneous fluoroscopy and FEES exams Verification of the Technique - II • VSS • Immediately repeat VSS with simultaneous FEES • Compare • Initial VSS results with Subsequent VSS • VSS findings compared with FEES findings • Recommendations from VSS to FEES • No Differences Found Eventual Development of the Pediatric FEES Clinic • Collaborative team effort • Pediatric Otolaryngologist, Director • Pediatric Speech Pathologist • Otolaryngology Nurse • Value of collaboration - multidisciplinary • Appreciation of specific and overlapping knowledge areas Role of the Nurse in the Pediatric FEES Clinic Interview Prior to FEES

• In collaboration with • Confirm allergies SLP □Food Coloring • Pertinent medical and □Pontocaine/Afrin developmental history □Food • Surgical history – airway surgeries • Current medications • Diet – oral, enteral Preparation • Describe Procedure • Parent/caretaker expectation • Hold child on lap and gently cradle arms • Take care not to let arms free during exam • Nurse will support and steady head Numbing Nose

• Spray nose with Pontocaine and Afrin spray according to age Contraindications to Spray

 Infants < 1 year old  Neurological Deficit  Inability to manage secretions  Known allergy to Pontocaine or Afrin  Gut feeling Video Room Set-up

 Appropriate size scope Alcohol Pad Scan serial number of Towels scope into medical record Gown O2 Set-up Gloves Suction Emesis Basin Topical Anesthetic CaviWipe XL During the FEES Examination • Nurse assists with achieving and maintaining appropriate positioning of child on parent’s lap • Arranges supplies for ready access during exam • Assists with special needs of patient • Tracheal suctioning, NP suctioning, vomiting FEES Procedure FEES Procedure Special Considerations

• Isolation • MRSA • Food Allergies • Parental Needs • Pregnancy • Fear of holding child After Procedure • Patient leaves procedure room, returns to original clinic room • SLP and ENT interpret findings and then return to family to discuss • RN assist family in discharge – referrals, patient instructions Role of the Speech Pathologist in the Pediatric FEES Clinic Prior to FEES

• Review pertinent medical and feeding history, oral motor/feeding assessment in separate exam room • Assemble and prepare feeding equipment and food items prior to exam in the FEES room Observation & Assessment • Overall level of alertness • Communication skills • Basic posture and position • Control of secretions • Oral structures (lips, tongue, palate) • Oral function (range of movements, strength) • Assessment of nutritive skills During the Procedure • Assist with patient positioning during exam • Communicate with ENT - anatomic findings and airway protection ability During the FEES Assessment… • SLP presents the solids & liquids to the patient • Judges swallowing dynamics • swallow response time, penetration(degree) aspiration (response), residue (degree/clearance) • Introduction of compensatory strategies: pacing, changes in flow, viscosity, alternating fluids, solids Interpreting Findings • Assist patient & family following exam – patient returns to first exam room • Review of FEES exam: analyze exam with ENT • Agree upon recommendations, discuss with the family Management Decisions • In discussion with the family: • Address the safety of feeding • What types of liquids, foods are appropriate? • What compensatory strategies improve swallowing function? • Focus upon collaborative decision-making Scope of Practice • Use of endoscopy for swallowing assessment is within the scope of practice of Speech Pathologist • www.asha.org • Position statement • Training Guidelines, Knowledge & Skills documents • Know individual state laws/regulations Role of the Physician in the Pediatric FEES Clinic Role of the Physician

• Medical evaluation of the patient as to the appropriateness of testing • Passage of the endoscope • Needs to be within the scope of practice of the individual physically passing the endoscope • Not an issue of what discipline passes the endoscope Role of the Physician • Determination of anatomy and function • Nasal cavity • Nasopharynx • Velum • Oropharynx • Hypopharynx • Larynx • Esophageal inlet Anatomic & Functional Assessment • Contractile function of the hypopharyngeal musculature • Symmetry of contraction • Secretion management • Protection of the airway • Sensory awareness of materials in hypopharynx • Evaluation of swallowing parameters in conjunction with SLP FEES: Adverse Effects • No significant adverse events in > 6,000 pediatric FEES examinations • and tens of thousands of pediatric flexible laryngoscopy examinations in the office and emergency room setting • Epistaxis • Vasovagal response (fainting) • Patient • Caregiver FEES: Adverse Effects • Laryngospasm • Generally seen in anesthetized patients • Stimulation of the larynx by secretions • Reflexive closure of the glottis • Inability to move air • Life threatening emergency • Low risk of occurrence in awake state • Treatment requires positive pressure mask ventilation, possible paralytic agents Beware Oxidant Dyes Methylene Blue Blue Dye #1 (present in green dyes) • Enteral administration associated with possible problems in premature infants and patients with mitochondrial disorders: • Jaundice • Rash • Anemia • Methemoglobinemia • Safe alternatives: • Betacarotene • Chlorophyll • Vitamin suspension - AquADEKS Possible Contraindications to FEES • All are relative – there are few absolute contraindications to FEES and those pertain to the ability to mechanically perform the test, not the medical condition of the patient • Anatomic conditions that would limit visualization • Nasal obstruction • Choanal atresia • Retrognathia • Pharyngeal stenosis • Medically fragile patients Special Considerations to FEES • Subacute Bacterial Endocarditis (SBE) Prophylactic antibiotics • Risk of developing bacterial vegetations on the heart valves in patients with cardiac abnormalities • Generally NOT indicated in flexible endoscopy unless biopsy is taken • Pulse oximetry, supplemental oxygen, suction equipment, resuscitation equipment Logistics of How to Develop a Pediatric FEES Clinic Infrastructure Support • Personnel • Equipment • Space

• Specifics will be unique to every institution Support for Personnel

• Division Director (s) • Needs to understand the proposal - multidisciplinary • Needs to support time allocation to the clinic • Needs to understand the time-line to grow the program • Return on Investment - cost accounting • Direct collections for the procedure • Indirect benefits to the facility • Resource utilization – other testing performed • Surgical procedures resulting from management of this patient population • Other consults / therapy services generated The Argument for Space and Equipment

• Division Director (s) need to ‘sell’ the program • Priorities of the institution • Multidisciplinary programs carry weight • Vulnerable patient populations • Identification of medical problems EARLY such that medical morbidity can be avoided and medical costs saved • Improved outcomes The Argument for Space and Equipment

• Equipment • Generally present within the facility • Can usually start clinics with existing equipment • Need plan to cover expansion of program with time • Nursing support • Often tied to Divisional activity • Nursing support usually available for new programs • Space • Often the largest obstacle to overcome • Need a champion who can align program with set goals of the institution • Need the ability to handle growth from the outset Success of the Program

• Track numbers • Track revenue • Follow all activities generated by patients seen • Satisfaction surveys • Patient / Family satisfaction • Satisfaction of the referral base • Value to referring physicians • Support to existing programs • Less referrals out of the institution References

• Willging JP. Endoscopic evaluation of swallowing in children. Int J Pediatr Otorhinolaryngol. 1995;32 Suppl:S107-108. • Miller CK WJ, Strife JL, Rudolph CD. Fiberoptic endoscopic examination of swallowing in infants and children with feeding disorders. Dysphagia. 1994;9(4):266. • Leder SB, Karas DE. Fiberoptic endoscopic evaluation of swallowing in the pediatric population. Laryngoscope. 2000;110(7):1132-1136. • Link DT, Willging JP, Miller CK, Cotton RT, Rudolph CD. Pediatric laryngopharyngeal sensory testing during flexible endoscopic evaluation of swallowing: feasible and correlative. Ann Otol Rhinol Laryngol. 2000;109(10 Pt 1):899-905. References

• Willging JP MC, Hogan MJ, Rudolph CD. . Fiberoptic endoscopic evaluation of swallowing in children: A preliminary report of 100 procedures. Dysphagia. 1996;11:2. • Hartnick CJ, Hartley BE, Miller C, Willging JP. Pediatric fiberoptic endoscopic evaluation of swallowing. Ann Otol Rhinol Laryngol. 2000;109(11):996-999. • Manrique D, Melo EC, Buhler RB. Fiberoptic endoscopic swallowing disorders in chronic encephalopathy. J Pediatr (Rio J). 2002;78(1):67-70. • Thottam PJ, Silva RC, McLevy JD, Simons JP, Mehta DK. Use of fiberoptic endoscopic evaluation of swallowing (FEES) in the management of psychogenic dysphagia in children. International journal of pediatric otorhinolaryngology. 2015;79(2):108-110. References

• Willging JP. Benefit of feeding assessment before pediatric airway reconstruction. Laryngoscope. 2000;110(5 Pt 1):825-834. • Willging JP, Thompson DM. Pediatric FEESST: fiberoptic endoscopic evaluation of swallowing with sensory testing. Curr Gastroenterol Rep. 2005;7(3):240-243. • Suterwala MS, Reynolds J, Carroll, et al. Using fiberoptic endoscopic evaluation of swallowing to detect laryngeal penetration and aspiration in infants in the neonatal care intensive unit. J of Perinatology.2017; 37: 404-408. • Vetter-Laracy S, Osona B, Roca A, et al. Neonatal swallowing assessment using fiberoptic endoscopic evaluation of swallowing (FEES). Pediatric Pulmonology. 2018; 1-6. https://doi.org/10.1002/ppul.23946. Anatomy, Normal & Abnormal Swallowing Parameters

J. Paul Willging, M.D. Learner Outcomes • Recognize and identify anatomical landmarks and key structures in the upper aerodigestive tract Focus of FEES in the Pediatric Population • Assessment of : • Anatomy as it affects swallowing function • Movement and sensation of critical structures in the hypopharynx and laryngopharynx • Secretion management • Airway protection during swallowing – food/liquid • Response to therapeutic interventions to improve safety of swallow Utility of FEES • Specific focus on airway protection • Clear view of anatomy • Assessment of sensitivity • Ability to manage secretions; presence of spontaneous clearing swallows • No radiation, time limitation, or barium • Initial, interval, and follow-up exam Limitations • White-out during moment of swallow • Possible discomfort with passage of scope • Focus is limited to the pharyngeal phase • Requires specific training Anatomy

• Nasopharynx, Oropharynx, Hypopharynx Orientation/Identification of Anatomy

• During transnasal passage of the scope, identify: • Nasal septum • Turbinates • Middle meatus • Adenoids • Soft palate • Tongue base Endoscope Insertion – Inferior Meatus Nasal Anatomy - Left Nasal Anatomy- Left

Middle turbinate

Septum

Inferior turbinate Nasal floor Inferior meatus Nasal Anatomy- Left Nasal Anatomy- Left

Middle turbinate

Septum

Inferior turbinate

Inferior meatus Nasal Anatomy- Left Nasal Anatomy- Left

Middle Turbinate

Middle Meatus

Septum

Inferior turbinate Nasal Anatomy- Left Nasal Anatomy- Left

Middle Turbinate Lateral Nasal Wall Adenoid

Middle Meatus Septum

Inferior turbinate Inferior meatus

Nasal floor Nasal Anatomy- Left Nasal Anatomy- Left

Adenoid Posterior pharyngeal wall

Soft Palate

Posterior edge of septum Nasal Anatomy- Left Nasal Anatomy

Adenoid

Soft Palate Nasal Anatomy Nasal Anatomy

Adenoid

Posterior Soft Palate pharyngeal wall Nasal Anatomy- Left Nasal Anatomy - Right Nasopharynx

Torus Tubarius Septum Skull Base Fossa of Rosenmueller

Pharyngeal Wall

Eustachian Tube Orifice Identify in the Nose and Nasopharynx: • Septum • Posterior pharyngeal wall • Turbinate • Soft Palate (velum) • Inferior • Middle • Fossa of Rosenmueller • Meatus • Eustachian Tube Orifice • Inferior • Torus Tubarius • Middle • Skull Base • Nasal Floor • Adenoid • Nasopharynx Structures in lateral oropharynx Oropharynx Soft Palate Posterior Tonsillar Pillar Uvula Anterior Tonsillar Pillar

Tonsil Pharyngeal Wall

Tongue

Molars Identify in the Oropharynx:

• Tongue • Tonsil • Anterior Tonsillar Pillar • Posterior Tonsillar Pillar • Soft Palate • Uvula

Larynx

Posterior commissure Esophageal inlet Interarytenoid notch Pyriform Sinus

Arytenoid

False Vocal Cord

Pharyngoepiglottic fold

True Vocal Cord Aryepiglottic fold

Vallecula Anterior commissure Epiglottis Hypopharynx Hypopharynx

Pharyngeal wall Pyriform Sinus Tonsil

Epiglottis

Glossoepiglottic ligament

Pharyngoepiglottic fold Vallecula Hypopharynx Hypopharynx

Pyriform Sinus Pharyngeal wall Postcricoid area

Aryepiglottic fold Arytenoid

Epiglottis Hypopharynx Hypopharynx

Vocal cord Pharyngeal wall Arytenoid

Pyriform Sinus

Pharyngoepiglottic fold Epiglottis Hypopharynx Hypopharynx

Pharyngeal wall Arytenoid Pyriform Sinus

Pharyngoepiglottic fold

Epiglottis Vallecula Identify in the Hypopharynx:

• Lateral and posterior pharyngeal walls • Pharygoepiglottic band • Epiglottis • Glossoepiglottic fold • Valleculae (right & left) • Pyriform sinuses • Aryepiglottic folds • Arytenoids, interarytenoid space • False vocal folds • True vocal folds • Anterior & posterior commissure • Post-cricoid area Appearance of Structures • Configuration of hypopharynx • Lateral channels/pyriforms: normal, shallow, small • Epiglottis: normal, omega shape, position • Arytenoids: normal, erythematous, edematous, prolapse • True vocal folds • Posterior commissure • Reflux irritation ? Anatomic Observations, continued

• Tongue base obstruction • Supraglottic collapse • Vocal fold immobility • Anatomic variations Only One Way to Learn Function of Key Structures Viewed Endoscopically

• Velopharyngeal closure • Secretion management & swallow frequency • Airway protection • Phonation • Pharyngeal musculature Observe Function – prior to feeding • Velopharyngeal closure • Secretion management, spontaneous swallows to clear • Presence of secretions: highly predictive of subsequent aspiration: Murray et al., 1996; Link et al., 2000, Donzelli et al., 2003 • Laryngeal function during respiration & phonation • Overall swallowing frequency Swallowing Function with Bolus Presentation • Presentation guided by oral motor ability, patient tolerance, & clinical judgement • Evaluation: swallowing physiology, coordination, and associated events Reviewing Swallowing Physiology

• Infants – suck/swallow synchrony • Toddlers/Children – transitional feeding, change in swallowing dynamics • Older Children/Adolescents/Adults – temporal aspects of the swallow Infants • Infant swallowing dynamics • Bu’Lock et al., 1990; Mathew, 1985; Koenig et al., 1990; Kramer & Eicher, 1993; Newman, 1992; 2001 • Sucking bursts – transition to intermittent sucking phase • Limitations of FEES during rapid sequential swallows Toddlers/Children • Transition to temporal aspects of mature swallow • Overlapping functions of breathing/swallowing • Coordination of respiratory & swallowing function – ongoing research re highly integrated functions, not mutally exclusive Adolescents/Adults • Temporal coordination of laryngeal dynamics and respiration during swallowing • Martin-Harris, Brodsky, Price, Michel, & Walters, 2003 • Martin-Harris, Michel, & Castell, 2005 – physiologic model of oropharyngeal swallowing revisited • GI Motility, online, 2006 Assessment of Function During Swallowing • General guidelines for presentation of foods and liquids • Order of consistencies • Therapeutic strategies Airway Protection • Arytenoid approximation & forward movement • True & false vocal fold approximation • Posterior movement of epiglottis, retroflexion • Lateral pharyngeal wall movement Normal Swallow - FEES Identification of Abnormal Swallowing Parameters • Poor secretion management ability, pooling secretions • Premature spillage • Laryngeal penetration • Aspiration • Residue • Sensory response: calibrated puff of air, should induce LAR, quantify sensory level Pooling Secretions • Secretions which accumulate in the hypopharynx without initiation of a spontaneous, clearing swallow response • Immediately apparent • Secondary to underlying sensory deficit • Weak pharyngeal contraction • Inadequate relaxation of the cricopharyngeal sphincter Pooled secretions without clearing swallow Premature Spillage • Escape of material over the tongue base in the absence of purposeful oral motor movements/tongue base retraction • Material may enter valleculae or overflow into the pyriform sinuses • Degree of spillage should be interpreted in the context of ability to initiate a timely swallow • Implications of continued flow of bolus without swallowing initiation Premature Spillage Premature Spillage Laryngeal Penetration • Passage of food or liquid within the confines of the endolarynx • Contact may be made onto the laryngeal surface of the epiglottis, aryepiglottic folds, or arytenoids • Location described as “shallow” or “deep” (proximity to airway opening) • FEES can identify point of entry Laryngeal Penetration Aspiration • Passage of secretions or food/liquid below the true vocal folds • During FEES, aspiration that may occur during the swallow cannot be observed because of “white-out” • Can identify before swallow • Can detect evidence after swallow • Inferences Aspiration Residue • Persistence of food or liquid in the hypopharynx following swallowing • May be related to the strength of pharyngeal contraction • May be secondary to cricopharyngeal dysfunction • ↓ sensory awareness Residue Regurgitation

Laryngeal Cleft Structural Changes with Age Maturational Changes in Swallowing Dynamics

Normal Swallowing Parameters VFSS & FEES

J. Paul Willging, MD Claire K. Miller, Ph.D. Learner Outcomes

• Identify the changes in anatomy and swallowing function that occur with maturation • Identify normal swallowing parameters viewed during videofluoroscopy and endoscopy Developmental Considerations in Laryngeal Sensitivity and Airway Protection

• How are infants different from adults? • Anatomic contrasts – to review • Consider - maturation of laryngeal sensory receptors • Cough – in neonatal population more likely response may be repeated swallows, airway closure, apnea

Thach, 2001; 2007 Overview of Anatomic Contrasts – Infants/Children Swallowing Physiology Contrasts in Anatomy

• Infants – relatively small oral cavity, forward resting tongue position, buccal sucking pads • Tongue maintains approximation with the lips, gums, hard/soft palate • Tongue base, soft palate, and pharyngeal walls in close approximation • Uvula may rest against epiglottis, frequent contact between the epiglottis and soft palate throughout first 6 months Anatomic Contrasts – Fluoroscopic View Anatomic Contrasts – Endoscopic View

• In infants, epiglottis is infant proportionally narrower – vertical, tubular shape (Greek letter omega) • The tip of the epiglottis is parallel to the body of the second cervical vertebrae and reaches superiorly to the soft palate, usually until 4-6 child months of age Anatomic Contrasts

• Vocal folds are 6-8 mm long, nearly ½ cartilaginous, compared to 1/3 or less in adults • Arytenoids are "bulky" • Mucosa of infant larynx is more reactive than adults – sensitive to refluxate • Trachea is smaller, shorter, and narrower in infants • Trachea: 5.7 cm long at 3 months, increases to 8.1 cm between 8-12 months Anatomic & Functional Changes • Downward growth of the mandible creates larger intraoral space, room for mastication • Cricoid cartilage loses excess bulk; arytenoids decrease in relative size • Downward movement of hyoid and larynx @ 4-6 months • Larynx continues to grow, gradual descent (complete by age 3 to C5) • Downward and forward growth of mandible, elongation of pharynx • The larynx and hyoid develop mobility necessary for effective airway protection during swallowing • Modification of the swallowing process occurs in response to structural changes Changes in Anatomy

• With maturation, structures continue to elongate and separate • Continued separation of the epiglottis and palate occurs, facilitating oral breathing • By six years, near adult configuration • Cricoid at lower border of C5 Concurrent Development of the Brain • Myelination: the process by which glia produce the fat sheath that covers the axons of neurons • Myelin speeds up the transmission of neural impulses Cortical Control of Swallowing • Central controls for voluntary swallowing are diffuse - Ertekin & Aydogdu, 2003 • Bilateral but asymmetrical • Dynamic: changes depending on continuous and various inputs (bolus characteristics) • Shift depending on phase • Oral phase largely under cortical control - *myelination key • tongue movements • Start/stop chewing Brainstem Controls • Pharyngeal phase is reflexive and primarily mediated by the central pattern generator (CPG) within the medullary brainstem • Sharing of respiratory and swallowing motorneurons • Networks of interneurons for chewing, sucking, laryngeal and respiratory activity • Central control to coordinate sensory input and motor fx • Nucleus Tractus Solitarius; Nucleus Ambiguus • Dorsal and ventral swallowing groups • (Ertekin and Aydogdu, 2003; Paydarfar, 2011) Changes in Infants at 4-6 months of age • Myelination enhances propagation of electrical impulse along the nerve fiber – • Slow gliding occurs on un-mylelinated fibers • Increases in myelination by ~ 5 months may contribute to increased changes in oral motor control/swallowing function at 6 months • Oral cavity enlarges relative to the tongue, masticatory space is created, eruption of deciduous dentition Airway Protection During Swallowing • Key component of airway protection across the lifespan • Occurs both during nutritive and non-nutritive swallowing • Breathing is suspended and airway closes • Apnea or respiratory pause; deglutition apnea • Respiratory flow or respiratory phase Airway Closure during Swallowing • As the swallow begins, the arytenoids move medially and anteriorly • During tongue base propulsion and retraction velopharyngeal closure occurs • Hyoid elevates - epiglottis retroverts Respiration & Swallowing

• Respiratory patterns and swallowing are precisely coordinated • Sucking and breathing may occur simultaneously, but at the moment of swallowing, the airway is closed Coordination of Breathing and Swallowing • Neural control mechanisms • Sensory: mechanical, chemesthetic, thermoreception, gustatory, olfaction • Motor: interneuronal network of premotor neurons; motor neuron pools; cranial nerves • Integration by way of Central Pattern Generator (CPG) • Cortical and sub-cortical input Infant Swallowing Dynamics

• Small size of infant oral cavity relative to tongue facilitates rhythmic alternation of compression, suction, expression, swallow-breathe (SW-BR) • Nasal airflow during breathing maintained during sucking and feeding – at moment of swallow, distinct apneic interval • Respiration inhibited during pharyngeal swallow – oral feeding always interferes with ventilation to some extent • *Key is infant's ability to adapt/integrate • Nixon, et al., 2008; Kelly et al., 2007; Gewolb et al., 2006 Swallowing Apnea Duration in Infants

• Respiratory pause during swallow appears to be mature at term birth – non-nutritive, nutritive • SAD strongly influenced by feeding • Irrespective of age, SAD significantly shorter during feeding

~Kelly, et al., 2006 Breathing and Swallowing Coordination in Infants • Developmental time course of respiratory phase and swallowing coordination suggests respiratory rhythm more stable with maturity • Kelly, 2007; Lau, Smith, & Schanler, 2003; Lau, 2015; Mathew, 1991 • Feeding places additional demands on coordinative relationship between breathing and swallowing in early infancy • Impact of feeding in infants with neurologic or cardiorespiratory compromise – implications for airway protection - *NICU Normal Swallowing Parameters Videofluoroscopy Fiberoptic Endoscopic Evaluation of Swallowing Physiology of Infant Swallow • Tongue forms central groove in response to tactile input of nipple • Compression of the nipple via sucking creates positive pressure • As the posterior portion of the tongue and jaw are depressed during sucking, increased negative pressure draws the bolus into the pharynx • For effective sucking, the oral cavity must be fully sealed or the tongue and jaw motions will be ineffective in creating suction Suck-Swallow Process - VFSS

• Close proximity of the structures provides the positional stability needed • Epiglottis – less active during infant swallowing secondary to location and proximity of the structures • Collection of liquid in the vallecular space prior to swallow initiation can be a normal variation in infants Older Child – Video Swallow Study Infant FEES Older Infant - FEES Older Child - FEES References

• Ertekin C & Aydogdu I.Neurophysiology of swallowing.Clinical Neurophysiology. 2003; 114(12):2226-2244. • Gewolb IH & Vice FL. Maturational changes in the rhythms, patterning, and coordination of respiration and swallow during feeding in preterm and term infants. Dev Med Child Neurol. 2006; 48(7):589-594. • Kelly BN, Huckabee ML, Jones, RD et al. The early impact of feeding on infant breathing-swallowing coordination. Resp Physiology & Neurobiology. 2007;156(2):147-153. • Kelly BN, Huckabee M, Jones RD et al. Nutritive and non-nutritive swallowing apnea duration in term infants: Implications for neural control mechanisms. Resp Physiology & Neurobiology.2006; 372- 378. References • Lau, C. Development of suck and swallow mechanisms in infants. Ann Nutr Metab. 2015; 66:7-14. • Lau C, Smith EO, Schanler RJ. Coordination of suck-swallow and swallow respiration in preterm infants. 2003; 92:721-727. • Matthew OP. Breathing patterns of preterm infant during bottle feeding: Role of milk flow. J of Pediatrics.1991; 119(6):960-965. • Mizuno K. Neonatal feeding performance as a predictor of neurodevelopmental outcome at 18 months. Dev Med & Child Neurology. 2007; 47(5): 299-304. • Nixon G, Charbonneau I, Kermack AS, et al. Respiratory- swallowing interactions during sleep in premature infants at term. Resp Physiology & Neurobiology. 2008; 160:76-82. References

• Paydafar, D. Protecting the airway during swallowing: what is the role for afferent surveillance? Head & Neck. 2011; DOI 10.1002/hed. • Thach BT. Maturation and transformation of reflexes that protect the laryngeal airway from liquid aspiration from fetal to adult life. Am J of Medicine. 2001;111(8A): 69S-77S. • Thach BT. Maturation of the cough and other reflexes that protect the fetal and neonatal airway. Pulm Pharmacol Ther. 2007;20(4):365-370. • Wilson SL, Thach BT, Brouillette RT, Abu-Osba YK. Coordination of breathing and swallowing in human infants. J Appl Physiol Respir Environ Exerc Physiol. 1981;50(4):851-858. Pediatric FEES Protocol Overview

J. Paul Willging, M.D. Claire Kane Miller, Ph.D. Learner Outcomes • Describe the general protocol for conducting a FEES examination in the outpatient clinic or at the bedside Setting • Clinic or bedside General Protocol for Pediatric FEES Examination Pre-FEES • Review pertinent developmental and medical history • Establish the reason for the FEES • Baseline oral motor assessment FEES Examination Protocol • Select appropriate endoscope • Determine if spray will be appropriate FEES Data Sheet – in Binder • CCHMC FEES Examination Clinic Data Points – EMR Flowsheet • Patient Name: • Date of Birth: • Date of Exam: • Dx: • Initial Exam: □ • Follow-up Exam: □ Nutritional Intake

• Current oral intake : □ NPO □ tastes of no appreciable volume • Oral : □ liquids □ purees □ solids □ age appropriate diet • Supplemental : □ GT □ NGT □GJ □ J Oral Motor Assessment

• Drop-down boxes in Epic template correspond • Orofacial symmetry and tone at rest • Range and strength of oral motor movements – non- nutritive, nutritive • Current oral intake and method • Note vocal quality – communication method Oral Motor Assessment • Oral Motor Assessment • Orofacial symmetry and tone at rest □normal □abnormal □other • Range of oral motor movements : □no restrictions in range of cheek, jaw, lip, and tongue motion □ limited active movements □significant limitations in active movements □*comment • Strength of oral motor movements : □ within normal limits □ decreased □*comment Pediatric FEES Protocol info: scope in - pre-feeding

• Document appearance of structures at rest • Normal/abnormal appearance • Symmetry Appearance of Hypopharynx/Larynx

Assessment • Erythema • Edema • Supraglottic collapse • Post-cricoid swelling • Post-cricoid venous plexus • Pachydermia Assessment of Vocal Fold Mobility

• Respiration – observing structures at rest, during breathing, cue to “sniff” (as possible) to assess abduction • Airway protection: cue to “cough”, hold breath • Phonation: cue or model: “eeeee” high/low • Normal mobility? • Immobile? • Arytenoid prolapse • Visualization - adequacy Vocal Fold Mobility

• normal mobility bilaterally □yes □no • immobility □yes □no • limited abduction □yes □no • vocal folds fixed in midline □yes □no • Bilateral arytenoid prolapse □yes □no • unable to visualize vocal folds □yes □no • Left vocal fold: normal □yes □no; immobile □yes □no; in paramedian position □yes □no; in lateral position □yes □no; arytenoid prolapse □yes □no • Right vocal fold: normal □yes □no; immobile □yes □no; in paramedian position □yes □no; in lateral position □yes □no; arytenoid prolapse □yes □no FEES Data Sheet • Appearance of Hypopharynx and Larynx at rest • Appearance: □normal □abnormal • Symmetry: □symmetrical □asymmetrical • Erythema □yes □no • Edema □yes □no • Supraglottic collapse □yes □no • Post-cricoid swelling □yes □no • Post-cricoid venous plexus □yes □no • Pachydermia □yes □no Pachydermia Post-cricoid venous plexus Secretion Management & Swallowing Frequency • Judge appearance of secretions • Standing secretions/ response • Pooling within the laryngeal vestibule • Evidence of leakage into the subglottis • Patient response to secretions: normal spontaneous swallows to clear, no attempt to clear, ineffective attempts to clear • Vocal quality Secretion Management • Frequency of spontaneous swallowing □ within normal limits □reduced □*other • Amount of secretions in hypopharynx: □none □present at beginning of examination and increased as examination progressed □standing secretions: location: VS□ PS□ VS + PS □ • Attempts to clear □ successful □ unsuccessful no attempt to clear □ • Response to aspiration of secretions: □no response □attempts to clear □inconsistent clearing response □effective clearing • Vocal quality: □normal □intermittent wet vocal quality □consistent wet vocal quality Sensory Assessment • Laryngopharyngeal sensation • Assess prior to feeding • Laryngeal adductor reflex (LAR) • Tapping region of the aryepiglottic fold • More precise – calibrated duration and intensity- controlled air pulse -*scope is no longer available • Response – cough with or without swallow; brief non-rhythmic break in respiration Sensory Assessment • Laryngeal sensation is essential for airway protection • Prior Data - LPST > 4.5 mm Hg correlated with: • Laryngeal penetration and aspiration • Pooled hypopharyngeal secretions • Pneumonia • Neurologic disorder • Gastroesophageal reflux • Link et al., 2000 (median age of subjects = 2.7) Pediatric FEES Protocol Feeding Portion • Guidelines – order and protocol may differ depending on patient needs • If patient is NPO, has never orally fed – may begin with drop of food coloring flavored with small amount of carbonation, med flavor syrup, or juice – NICU - AQUADEKS • Sensory enhancement: Logemann, 1995; Pelletier & Lawless, 2003, Lundine, Bates, & Yin, 2015 • Depending on toleration & airway protection ability, may advance to small amounts pureed: applesauce, pudding, or solid • Presentations are guided by oral motor ability, patient tolerance, & clinical judgment Rating Swallows

• Oral control of bolus, swallow response time, laryngeal penetration (location, depth), aspiration, residue • Swallow Onset Time • Pharyngeal clearance • Airway protection threat • Protective reactions/responsiveness to abnormalities in swallow function • Cough or other reaction to clear • Generation of additional swallows to clear residue • Sensory awareness PenAsp Scale

• 1. Material does not enter the airway • 2. Material enters the airway, remains above the vocal folds, and is ejected from the airway • 3. Material enters the airway, remains above the vocal folds, and is not ejected from the airway • 4. Material enters the airway, contacts the vocal folds, and is ejected from the airway • 5. Material enters the airway, contacts the vocal folds, and is not ejected from the airway • 6. Material enters the airway, passes below the vocal folds and is ejected out of the airway • 7. Material enters the airway, passes below the vocal folds, and is not ejected from the trachea despite effort • 8. Material enters the airway, passes below the vocal folds, and no effort is made to eject *Rosenbek, et al., 1996 Ratings – Pharyngeal Contraction/Clearance

• Pharyngeal Contraction: complete □ incomplete □ • Pharyngoesophageal Segment Opening: □complete, no obstruction to flow □partial obstruction to flow □marked obstruction to flow □total obstruction of flow □other: *** • Pharyngeal Residue • Residue of liquid: □none □ minimal □estimate volume______• □required multiple swallows to clear □responsive to verbal cue to use additional swallows to clear □ used spontaneous swallows to clear • Residue of pureed: □none □minimal □estimate volume ______• □required multiple swallows to clear □responsive to verbal cues to clear □used spontaneous swallows to clear • Residue of solids: □none □ minimal □estimate volume______• □required multiple swallows to clear □responsive to verbal cues to use additional swallows to clear □used spontaneous swallows to clear FEES Data Sheet Summary • Document - type of bolus, swallow onset time, penetration (depth, frequency, response), aspiration (frequency, response), residue (degree, response) • Note - response to compensatory strategies Introduction of Compensatory Strategies • Clinical judgment • Differs in infants & children With Infants: • Positioning – upright v sidelying • Pacing – adjusting rate of intake • Flow Rate – adjusting flow dynamics • Viscosity – altering liquid, or textures of food Management Decisions – Following Exam • In discussion with the family: • Address the overall safety of feeding as judged during the exam • What types of liquids, foods are appropriate? • What compensatory strategies improve swallowing function? Goals for future dysphagia treatment? • Focus upon collaborative decision-making with the family Identifying Abnormal Swallowing Parameters

Assessment & Effects of Chronic Aspiration

J. Paul Willging, MD Claire Kane Miller, PhD Learner Outcomes • Identify abnormalities in the swallowing process that create airway protection compromise • Discuss the effects of chronic aspiration on overall pulmonary status • Describe available methodology for assessment of aspiration Swallowing Abnormalities • Problems with bolus propulsion: generation of positive pressures insufficient • Poor tongue base retraction, weak pharyngeal contraction, cricopharyngeal dysfunction • Issues with airway protection • Inadequate vocal fold adduction, poor closure/movement of supraglottic structures, inadequate retroversion of the epiglottis • Problems with maintaining coordination of bolus flow/airway protection • Depressed sensory response Abnormal Swallowing Parameters

• Poor secretion management • Premature spillage - delayed swallow onset • Laryngeal penetration • Aspiration • Residue • Abnormal sensory response Secretion Management & Swallowing Frequency

• Judge appearance of secretions • Pooling within the laryngeal vestibule • Evidence of leakage into the subglottis • Patient response to secretions: normal spontaneous swallows to clear, no attempt to clear, ineffective attempts to clear • Vocal quality Poor Secretion Management Rating Secretion Management • Frequency of spontaneous swallowing □ within normal limits □reduced □*other • Amount of secretions in hypopharynx: □none □present at beginning of examination and increased as examination progressed □standing secretions: location: VS□ PS□ VS + PS □ • Attempts to clear □ successful □ unsuccessful no attempt to clear □ • Response to aspiration of secretions: □no response □attempts to clear □inconsistent clearing response □effective clearing • Vocal quality: □normal □intermittent wet vocal quality □consistent wet vocal quality Sensory Assessment • Laryngopharyngeal sensation • Assess prior to feeding • Laryngeal adductor reflex (LAR) • Tapping region of the aryepiglottic fold • More precise – calibrated duration and intensity- controlled air pulse -*scope is no longer available • Response – cough with or without swallow; brief non-rhythmic break in respiration Sensory Assessment • Laryngeal sensation is essential for airway protection • Prior Data - LPST > 4.5 mm Hg correlated with: • Laryngeal penetration and aspiration • Pooled hypopharyngeal secretions • Pneumonia • Neurologic disorder • Gastroesophageal reflux • Link et al., 2000 (median age of subjects = 2.7) Rating Swallows

• Oral control of bolus, swallow response time, laryngeal penetration (location, depth), aspiration, residue • Swallow Onset Time • Pharyngeal clearance • Airway protection threat • Protective reactions/responsiveness to abnormalities in swallow function • Cough or other reaction to clear • Generation of additional swallows to clear residue • Sensory awareness Premature Spillage • Escape of material over the tongue base in the absence of purposeful oral motor movements for transfer/tongue base retraction • Material may enter valleculae or overflow into the pyriform sinuses • Degree of spillage should be interpreted in the context of ability to initiate a timely swallow • Implications of continued flow of bolus without swallowing initiation Premature Spillage - Stills

• Oral control/delayed swallow onset Premature Spillage Laryngeal Penetration • Passage of food or liquid within the confines of the endolarynx • Contact onto the laryngeal surface of the epiglottis, aryepiglottic folds, or the arytenoids • Location described as “shallow” or “deep” (proximity to airway opening)

Laryngeal Penetration Penetration-Aspiration -PenAsp Scale Penetration/Aspiration Scale Key • 1. Material does not enter the airway • 2. Material enters the airway, remains above the vocal folds, and is ejected from the airway • 3. Material enters the airway, remains above the vocal folds, and is not ejected from the airway • 4. Material enters the airway, contacts the vocal folds, and is ejected from the airway • 5. Material enters the airway, contacts the vocal folds, and is not ejected from the airway • 6. Material enters the airway, passes below the vocal folds and is ejected out of the airway • 7. Material enters the airway, passes below the vocal folds, and is not ejected from the trachea despite effort • 8. Material enters the airway, passes below the vocal folds, and no effort is made to eject *Rosenbek, et al., 1996 Aspiration • Passage of secretions or food/liquid below the true vocal folds • During FEES, aspiration that may occur during the swallow cannot be observed because of “white-out” • Can identify before and after the swallow • Can detect evidence after swallow • Inferences Aspiration Residue • Persistence of food or liquid in the hypopharynx following swallowing • May be related to the strength of pharyngeal contraction • May be secondary to cricopharyngeal dysfunction • ↓ sensory awareness Residue • Amount • Location • Effectiveness of strategies to clear Ratings – Pharyngeal Contraction/Clearance

• Pharyngeal Contraction: complete □ incomplete □ • Pharyngoesophageal Segment Opening: □complete, no • obstruction to flow □partial obstruction to flow □marked obstruction to flow □total obstruction of flow □other: *** • Pharyngeal Residue • Residue of liquid: □none □ minimal □estimate volume______• □ required multiple swallows to clear □responsive to verbal cue to use additional swallows to clear □ used spontaneous swallows to clear • Residue of pureed: □none □minimal □estimate volume ______• □ required multiple swallows to clear □responsive to verbal cues to clear □used spontaneous swallows to clear • Residue of solids: □none □ minimal □estimate volume______• □required multiple swallows to clear □responsive to verbal cues to use additional swallows to clear □used spontaneous swallows to clear FEES Data Sheet Summary • Document - type of bolus, swallow onset time, penetration (depth, frequency, response), aspiration (frequency, response), residue (degree, response) • Note - response to compensatory strategies Introduction of Compensatory Strategies • Clinical judgment • Differs in infants & children With Infants: • Positioning – upright v sidelying • Pacing – adjusting rate of intake • Flow Rate – adjusting flow dynamics • Viscosity – altering liquid, or textures of food Compensatory Strategies in Children/Adolescents • Verbal cueing – i.e. learn to use supraglottic swallow sequence to assist with airway closure • Alternation of sips of liquid with bites of solids to improve pharyngeal clearance • Use of maneuvers +/- • Chin tuck – head tilt – head turn to modify bolus path – dependent on ability to follow directions • Thickened liquid +/- • The best evidence for the efficacy of strategy = the patient during the exam Management Decisions – Following Exam • In discussion with the family: • Address the overall safety of feeding as judged during the exam • What types of liquids, foods are appropriate? • What compensatory strategies improve swallowing function? Goals for future dysphagia treatment? • Focus upon collaborative decision-making with the family Chronic • Repeated passage of material from the oropharynx into the lower airways • May occur with: • Oral intake • Gastric refluxate • Saliva • Any of us in this room may do it… • Normal adults may aspirate secretions during sleep

• Gleeson, K. et al. Quantitative aspiration during sleep in normal subjects. Chest. 1997; 111: 1266-1272. Chronic Pulmonary Aspiration • Central neurologic disorders • Neuromuscular weakness • Chiari malformation • Vocal fold paralysis • Upper airway abnormalities • Laryngoesophageal cleft • H type tracheoesophageal fistula Chronic Pulmonary Aspiration - GER • Gastroesophageal reflux • May present with respiratory symptoms with or without aspiration

• Laryngeal sensation may be impaired by exposure to acid reflux

• Phua, et al. Patients with gastroesophageal reflux disease and cough have impaired laryngopharyngeal mechanosensitivity. Thorax 2005; 60:488-491 Salivary Aspiration • Aspiration of secretions with or without sialorrhea • Genetic syndromes • CHARGE • Moebius • Opitz BBB • Congenital viral infections • Varicella • Coxsackie Clinical Presentation of Aspiration Variable • Chronic cough • Recurrent wheeze • Recurrent pneumonia or bronchitis • Dysphagia • Failure to Thrive • Abnormal radiographic studies Differential Dx: Chronic Cough • Reflux • Chronic pulmonary aspiration • Allergic Rhinitis • Asthma • Sinusitis • Cystic Fibrosis • Post-infectious • Primary Ciliary Dyskinesia • Pertussis • Tracheomalacia • Tuberculosis • Lower Airway Compression • Endobronchial mass • Foreign Body • Interstitial Lung Disease • Protracted Bacterial Bronchitis • Pulmonary Edema • Bronchiectasis • Psychogenic cough • Chronic Lung Disease of Prematurity Differential Dx: Wheezing

• Chronic pulmonary aspiration • Cystic Fibrosis • Asthma • Primary Ciliary Dyskinesia • Tracheomalacia • Bronchiectasis • Bronchomalcia • Endobronchial mass • Tracheal/bronchial stenosis • Airway hemangioma • Lower airway compression • Mediastinal mass • Bronchiolitis • Chronic lung disease of • Post-infectious prematurity • Vocal cord paralysis • Foreign body • Laryngomalacia Differential Dx: Recurrent Pneumonia

• Chronic pulmonary aspiration • Bronchial stenosis • Cystic Fibrosis • Endobronchial mass • Primary Ciliary Dyskinesia • Lymphadenopathy • Immunosuppression • Foreign body • HIV/AIDS • Congenital lobar emphysema • Chronic pulmonary aspiration • Pulmonary sequestration • Bronchiectasis • Pulmonary AV malformation • Tuberculosis • Pulmonary hemosiderosis • Neuromuscular weakness Long Term Complication of Chronic Aspiration: Bronchiectasis • Signs and symptoms • Loose and productive cough • Chest pain • Dyspnea • Rales or rhonchi on examination • May have chest wall deformity or digital clubbing • Failure to thrive Bronchiectasis

• Chronically dilated bronchi and bronchioles • Traditionally defined as irreversible damage • Early disease has been noted to resolve in some cases* • Result of chronic inflammation • Mucosal thickening • Damage to muscular and elastic layers of airways • Destruction of bronchial cartilage

• *Gaillard EA, et al. Reversible bronchial dilatation in children: comparison of serial high-resolution computer tomography scans of the lungs. Eur J Radiol. 2003 Sept; 47(3): 215-220. Bronchiectasis • In advanced stages: • Bronchial wall collapse • Development of chronic obstructive lung disease • Bronchial vascular proliferation causing hemoptysis • Hypoxemia • Pulmonary hypertension • Significant burden of disease and impact on quality of life*

• *Kapur N, et al. The burden of disease on pediatric non-cystic fibrosis bronchiectasis. Chest 2012 Apr; 141(4): 1018-24. Bronchiectasis in Aspiration • 66% of children with aspiration were found to have bronchiectasis* • Most commonly in lower lobes or right upper lobe • Risk factors include neurologic impairment and reported history of reflux • Aspiration may be cause of non-CF bronchiectasis in only 2-18% of cases • Other diagnoses must be considered in children with chronic cough and bronchiectasis • *B. Piccione JC et al. Bronchiectasis in chronic pulmonary aspiration: risk factors and clinical implications. Pediatric Pulmonol. 2012 May: 47(5): 447-52. Differential Dx: Bronchiectasis

• Cystic Fibrosis • Congenital airway • Primary Ciliary Dyskinesia malformations • Bronchiolitis obliterans • Immunodeficiency syndromes syndrome • HIV infection • Connective tissue disorders • Allergic bronchopulmonary aspergillosis (ABPA) • Autoimmune disease • Severe or recurrent lower • Inflammatory bowel disease respiratory tract infections • Post-infectious • Post-obstructive pneumonia • Bordetella pertussis • Foreign body • Mycobacterium tuberculosis • Congenital or developed • Atypical Mycobacterium obstructive airway lesions species • Influenza • Chronic pulmonary aspiration • Adenovirus Bronchiectasis - Evaluation • Complete history is critical • Sweat chloride • Ciliary biopsy • Genetic testing • Immune evaluation • HIV and/or TB testing • Swallowing evaluation – VSS and FEES • Triple scope • Impedance probe • Spirometry Bronchiectasis – Medical Management • Preventatively and symptomatically • Underlying disease should be identified and treated • Targeted antibiotics during symptom exacerbation • Bronchodilators • Inhaled steroids • Routine immunizations including annual influenza vaccine Aspiration - Imaging • Chest radiograph • May be normal • Poor sensitivity in detecting early lung disease • Evidence of dilated airways • “Tram track” sign seen in severe cases Aspiration - Imaging

• High Resolution CT is gold standard • Air trapping • Bronchial wall thickening • Tree-in-bud opacities Aspiration - Imaging • Bronchiectasis • Dilated peripheral bronchi • Bronchial wall thickening • Presence of bronchi within 1 cm of pleura • Bronchi with an internal diameter of greater than that of the adjacent pulmonary artery, or “signet ring” sign CT Scanning

• Excellent evaluation of long term consequences of aspiration • But the damage is already done – tells you about the past, not necessarily the present • Requires GA and radiation Flexible Bronchoscopy • Assist in identifying anatomic abnormalities • Limitations when compared to rigid bronchoscopy • Obtain bronchoalveolar lavage (BAL) • Cytological analysis of lower respiratory secretions • Culture Flexible Bronchoscopy • Advantages • Ability to take sample for culture, and lipid evaluation • Lipid laden macrophages • Can access distal tracheobronchial tree, and go round corners • Disadvantages • GA • Does not visualize posterior glottis well Rigid Bronchoscopy

• Advantages •Disadvantages • Excellent for looking for •A rigid telescope anatomical anomalies •Skilled bronchoscopist • Superior optics helpful Anatomical Pathology Lipid-Laden Macrophages

• Bronchoalveolar lavage • In theory, the presence of lipid in macrophages suggests aspiration of particulate • Significant limitations • Interobserver reliability is poor • Lipid may be present in macrophages for variable amount of time • Non-specific Lipid-Laden Macrophages

• May not have desired sensitivity • Among 100 children with documented aspiration, 72% had fewer than 5% macrophages stained for lipid* • Provides supporting evidence of aspiration • Context is critical

• *B. Piccione JC et al. Bronchiectasis in chronic pulmonary aspiration: risk factors and clinical implications. Pediatric Pulmonol. 2012 May: 47(5): 447-52. Dye Testing • This is an investigation useful in children WITH A TRACHEOTOMY • Concept • Green food dye is mixed with food / drink / G tube feeds / saliva, etc • If green staining is suctioned from the tracheotomy tube, child is aspirating Advantages • Not a window in time • May be repeatedly performed • Several times a day over several weeks • Many substances may be dyed • Cheap • Convenient • Good “customer buy-in” Reflux

• If gastrostomy tube fed, may dye the feed • Differing times of day relevant, especially at night • Continuous feeds vs bolus feeds Limitations / Dangers

• Only useful in patients with a tracheotomy • Not useful if no connection between airway and pharynx • Grade 4 subglottic stenosis • Laryngotracheal separation • Concerns of Blue dye # 1 and methylene blue poisoning • Methemoglobinaemia Children at Risk

• Dye testing has confirmed some groups of children are especially at risk of aspiration • CHARGE syndrome • MRCP (a progressive problem in some children) • Laryngeal cleft • Pierre Robin sequence • Vocal cord paralysis • Extreme prematurity Treatment Options for Salivary Aspiration • Therapy • Oral motor skill development • Positioning • Medical • Glycopyrolate (Robinul) • Botox injection • Surgical • Ligation of parotid ducts • Excision of submandibular glands The “Drool” Procedure

• Bilateral submandibular gland excision • Bilateral parotid duct ligation Complications • Parotid duct recanalization or mucocele formation • Radionucleotide parotid scan diagnostic • Re-ligate • With tympanic neurectomy? • With Botox? “Drool” Procedure Failures • Even if surgery a success, patient may be a failure • May continue to aspirate • Especially if poor swallow, or immotile esophagus Laryngotracheal Separation

• NO aspiration • No voicing either Interpretation of the FEES Examination Case Studies

J. Paul Willging, M.D. Claire Kane Miller, Ph.D. Learner Outcomes • Describe the evaluation protocol, scoring, and interpretation system used in pediatric FEES • Participate in group rating and comparison using an audience response system FEES Data Sheet • Demographics • Nutritional intake • Oral sensorimotor status • Transnasal passage of endoscope – appearance of structures at rest • Symmetry • Erythema • Edema • Supraglottic collapse • Post-cricoid swelling • Post-cricoid venous plexus • Pachydermia FEES Data Sheet • Sensory assessment • Swallowing parameters • Rating swallows Case JJ • Infant born with hypoplastic left heart syndrome • Candidate for Norwood procedure, performed at DOL 4 • Demonstrating coordinated suck-swallow sequence during non-nutritive sucking • Accepting 5 mL during oral feeding trials • FEES study requested at 2 weeks of age to assess vocal fold function, airway protection skills, and safety of swallow Case JJ FEES Study Appearance of the Hypopharynx and Larynx at rest A. Symmetrical B. Asymmetrical Amount of Secretions in the Hypopharynx A. None B. Present at the beginning of the examination and increased C. Standing secretions in the pyriform sinus Vocal fold mobility A. Normal vocal fold mobility B. Left vocal fold immobility C. Right vocal fold immobility Swallowing Response Time A. Timely swallowing onset B. Bolus head in pyriform sinus prior to swallowing initiation C. Bolus head in vallecular space prior to swallowing initiation Case Lady H • 4 month old female infant • Infantile spasms • Aicardi Syndrome • Referred for VSS and FEES FEES Clip – LH Anatomy FEES Clip – LH Swallowing Function Rate Depth of Laryngeal Penetration A. No laryngeal penetration occurred B. Liquid makes contact with the laryngeal surface of the epiglottis C. Liquid makes contact with the aryepiglottic fold and interarytenoid notch D. B + C Case DR – Recurrent Pneumonia • Born at 35 weeks gestation • Recurrent bronchiolitis, pneumonia, wheezing, persistent reflux • “aspiration-type” symptoms FEES Clip – Case DR In Case DR A. Swallowing onset is delayed B. Swallowing onset is delayed and penetration occurs C. Swallowing onset is delayed with penetration and subsequent aspiration Case CS • 9 month old • Trached since 2 weeks of age • Hx of PDA, chronic kidney disease • Referred for second opinion from OSH – description – glottic web, subglottic stenosis, normal esophagus FEES Clip - CS In this case, the primary issue appears to be: A. Inability to achieve glottis closure and airway protection B. Delayed onset of the swallowing response C. Incomplete hypopharyngeal clearance D. Both B & C Case AF - Neuroblastoma • 8 year old female • Enteral feeds – continuous at night, bolus during day • Tastes – no volume, spitting out secretions • FEES requested to assess swallowing function Management of Secretions Case AF: Rate Secretion Management A. Minimal secretions in the hypopharynx B. Secretions are primarily in the valleculae C. Secretions are in the valleculae and pyrifrom sinus D. Secretions are diffuse throughout the hypopharynx without spontaneous efforts to clear Case BT – Brain Tumor • 12 year old with medulloblastoma, s/p resection, in remission but now with tumor recurrence • 100% per oral intake • GT and trach have been removed • Recent issues with coughing during oral intake, wet vocal quality Rate: Swallow Response Time Case BT: Rate Swallow Onset Time A. Timely swallowing onset B. Swallow initiated as bolus enters the vallecular space C. Swallow initiated as bolus entered the pyriform sinus BT Puree Rate Hypopharyngeal Clearance Following Swallows A. No residue post swallow B. Residue in valleculae, not consistently cleared C. Residue in valleculae and pyrifrom sinus region, not consistently cleared D. Residue primarily in right pyriform sinus region Case 3 – Down Syndrome • 8 year old – full po intake • Sleep apnea • Persistent pulmonary issues Define the Event The Event in the Prior Clip is: A. Aspiration B. Evidence of weak pharyngeal contraction C. Retrograde flow from the UES after the swallow Case JC – 3 year old • Diagnosed with BVCP after respiratory illness at 6 months of age • Full po up until time of illness – no feeding issues • Trached – significant feeding problems • Relies on oral suction – 100% What is the Underlying Issue for this Patient? • What is Known: • Bilateral vocal fold paralysis • Complete reliance on oral suction • Fed orally prior to respiratory illness by report Clip Interesting Finding • Patient was scheduled to undergo a MLB, EGD, and Flexible Bronchoscopy • Findings in the OR • Current Status Introduction to Equipment

Logistics of Scope Passage J. Paul Willging, M.D. Objectives • Describe and identify the equipment components needed for FEES • Discuss proper procedures for positioning and manipulation of the endoscope Types of Nasopharyngoscopes • Fiberoptic bundle • 2.2, 3.5, 3.7, 4.0 mm diameters • Video (Chip-in-the-tip) scope to capture image • Manufacturers • Olympus • Pentax • Machida • Storz • Costs range from $3,000 – $15,000 Equipment and Supplies • Flexible endoscope • Assorted sizes beneficial • Light source • Camera head and processor • Video recorder • Monitor

Reimbursement requires that the examination is recorded Equipment

• Nasopharyngoscope • Light source • Recording equipment • Camera • Recording device • Video • Digital Equipment and Supplies • Packages: • Kay – Pentax • Olympus • Stryker • Others • Large units vs freely portable Recording of the Exam • Multiple purpose • Allow review of the examination immediately after the procedure to allow determination of findings and the development of recommendations • Archiving of material for comparison with future exams • Teaching purposes Archiving Options • Need storage • VHS analogue tapes • Digital tapes • DVD-ram • DVD • Removable hard drives • Remote server • Storage is costly • Access to examinations is essential Patient Selection • Any patient can tolerate flexible endoscopy • Easy of performing the procedure • Age dependent • Congenital abnormalities • Developmental age • Monitoring of patients is generally not necessary Anesthetic • Topical anesthesia • Increase cooperation of the child with the examination • 1:1 mixture of oxymetazoline and 2% pontocaine • 15 minutes activation time • Not necessary, but improves cooperation in children • Avoid topical spray • Infants less than 1 year • Neurologic deficit • Inability to handle secretions • Known allergy to Pontocaine or Afrin • Gut feeling they won’t do well Prior to Performing the Exam

• Pertinent history • Baseline oral motor/feeding skills status • Discuss the test with the family Patient Preparation

• Interact with the child • Let them see the endoscope • Distract them – put them at ease • Power of suggestion • Avoid words with connotation of pain / discomfort • Know when delay is counter productive • Anticipation is often worse than the actual procedure • Select the appropriate endoscope Selection of Appropriate Endoscope

• What scopes are available • 3.5 mm endoscopes are most versatile • Robust, cheapest, easy to manuever • Ideal • Largest scope that can be easily inserted with minimal discomfort • Need endoscope to remain above inferior turbinate to view velopharyngeal sphincter area from above • Patient factors • Narrow nose, choanal atresia / stenosis history • Large nose Patient Positioning

• Age dependent • Sitting alone upright in a chair • Sitting in the lap of a caregiver • Parent puts their arms around the child, holding the arms and hands of the child under theirs • Assistant steadies the child’s head against the chest of the parent • Equipment best positioned to the right of the patient Passsing the Endoscope

• Pass scope along floor of the nose or above the inferior turbinate into the middle meatus • Check each side to assess which nasal passage appears least obstructed • Passage of scope through largest nasal passage • If feeding or impedance catheter in nose, attempt to pass on the same side • Anchor hand against the nasal tip and the forehead • Assistants are controlling the head and the arms Manipulation of the Endoscope

• Secure the endoscope by bracing insertion hand against the nasal bridge and forehead of the child • Fingertips grasp nasal tip when necessary • Insertion hand advances scope forward; controlling hand manipulates endoscope tip • Expect movement • Prepare family for child discomfort / reaction • Move insertion hand with the child Complications

• Epistaxis • Very rare event • Generally no intervention necessary • Afrin and pressure may assist in control • Vasovagal reflex – fainting • Patient or Parent • Remove endoscope • Protect from injury • Reclining position – lower head, raise legs Equipment Options

Swallow Signals Lab • Real time display & review/examination of physiologic signals related to swallowing • Manometer – measures pressure at sensor locations (pharyngeal and upper esophageal sphincter) • Electromyography (EMG) – surface (sEMG) • Nasal Cannula • Tongue Array sEMG • Records muscle activity in specific area • 2 active electrodes, 1 ground electrode • 2 EMG channels allows recording from two separate areas simulataneously • Non-invasive, easy to administer, well-tolerated • Provides visual display for patient sEMG in Swallowing • Buccinator/masseter muscles • Obicularis oris • Submandibular musculature – anterior belly digastric, stylohyoid, mylohyoid • Facilitate learning of “effortful” swallow, other appropriate therapeutic maneuvers Tongue Pressure Data • Tongue pressure data • Silaca strip • Three small air-filled bulbs - calibrated • 13 mm diameter • Strip placed along midline of palate • Anterior bulb – alveolar ridge • Medial bulb – midline of hard palate • Posterior bulb – posterior edge of hard palate – near juncture of the hard and soft palate Tongue Array • Each bulb on tongue-bulb array measures pressure applied by the tongue • Array placed intraorally, clinician (or patient) holds stem externally, can bend as necessary • Substrate should be against hard palate, bulbs oriented downward so that tongue makes direct contact (during swallowing or pushing) • Arrays are reusable by same patient Clinical Applications -Tongue Array

• Tongue strength and symmetry • Visual cueing • Visual targets in therapy • *note that asymmetry in tongue strength can occur in the normal population • Careful judgment re significance of aysmmetry Nasal Cannula • Displays respiratory phase (inspiration, expiration, apnea) in relation to swallowing • Visual display show respiratory phases in real time • Can color code phases on display • Can use in conjunction with EMG, or stethoscopic microphone • Minimally invasive • Inexpensive • Reliable signal for monitoring respiratory phase Digital Video Recording • Video and audio recording • Archiving • Event marking • Image digitization and measurement • FEES & VSS • Ultrasound Respiratory Phases • Swallowing apnea (SA) can occur within various respiratory phases: • Mid-inspiration (II) • Between inspiration and expiration (IE) • In mid-expiration (EE)* • Between expiration and inspiration (EI) • During apneic pauses (P)

• Martin, Logemann, Shaker, Dodds, 1994 Respiratory Phase Categories in Healthy Infants* • EE most dominant pattern at 48 hours • IE is second most dominant pattern • IE pattern dominant at 12 months in Kelly study • Some variation during the intervening months (0- 12) • Marked reduction of repeated swallows during apneic pause from birth to 12 months of age

**Kelly BN, Huckabee M, Jones R, & Frampton C. (2007).The first year of human life: coordinating respiration and nutritive swallowing. Dysphagia 22:37-43. Breathing and Swallowing Coordination in Infants • Feeding places additional demands on coordinative relationship between breathing and swallowing in early infancy • Apnea duration shorter for nutritive versus non-nutritive sucking • Developmental time course of respiratory phase and swallowing coordination suggests respiratory rhythm more stable with maturity • Kelly, 2007; Lau, Smith, & Schanler, 2003; Mizuno & Ueda, 2003 • Impact of feeding in infants with neurologic or cardiorespiratory compromise – implications for airway protection Respiratory Swallowing Integration in Infants and Children • SAD may be useful marker of neurologic immaturity or neural damage (Kelly, 2006) • Atypical breathing-swallowing coordination has been identified in children with cerebral palsy, bronchiolitis, and chronic lung disease • McPherson et al., 1992; Pinnington, 2000; Mizuno, 2007 • Additional research needed - potential links between respiratory and swallowing integration to help establish clinical pathways for care • Cass et al., 2005: Assessing pulmonary consequences of dysphagia in children with neurologic disabilities: when to intervene? Clinical Implications

• Understanding respiratory-swallow coordinative patterns will allow interventions to be focused toward production of optimal patterning during feeding • Need normative data beyond the neonatal age for identification of aberrant patterns • Determine what therapeutic strategies to use in intervention • Pacing during feeding – imposed pause intervals • Therapeutic sensory stimulation – volume, viscosity, taste • Positioning Pilot Data • The specific respiratory phase patterns & duration of apneic pause in relation to abnormal and normal swallowing parameters in pediatric patients is presently unknown • Purpose of our pilot study: • To analyze duration of apneic pause and respiratory phase patterns in a cohort of pediatric patients undergoing video swallow studies Simultaneous Display - Respiratory Phase and Interruption of Nasal Airflow Associated with Swallowing

Puree Bolus – Expiration- Swallow – Expiration (EE) pattern

Expiration – Swallow - Inspiration Pattern Note Depth of Bolus

Inspiration – Inspiration Pattern with trace aspiration

Repeated Swallows during Apneic Pause

References

• Cass H, Wallis C, Ryan M. et al. (2007). Assessing pulmonary consequences of dysphagia in children with neurological disabilities: when to intervene? Dev Med & Child Neruology. 47: 347-352. • Kelly BN, Huckabee M, Jones R, & Frampton C. (2007).The first year of human life: coordinating respiration and nutritive swallowing. Dysphagia. 22:37-43. • Kelly B, Huckabee M, Jones R, Frampton C. (2006).Nutritive and non- nutritive swallowing apnea duration in term infants: Implications for neural control mechanisms. Respiratory Physiology & Neurobiology 154:372-378 • Lau C, Smith EO, & Schanler RJ. (2003). Coordination of suck-swallow and swallow respiration in preterm infants. Acta Paediatr. 92(6): 721-727. • Martin BJ, Logemann JA, Shaker R, & Dodds WJ. (1994). Coordination between respiration and swallowing: respiratory phase relationships and temporal integration. J Appl Physiol. 76(2):714-723. • McPherson KA, Kenny DJ, Koheil R, Bablich K, Sochaniwskyj A, Milner M. (1992). Ventilation and swallowing interactions of normal children and children with cerebral palsy. Dev Med child Neurol 34(7): 577-588, 1992. References

• Mizuno K, Nishida Y, Taki M, Hibino S, et al. Infants with bronchopulmonary dysplasia suckle with weak pressures to maintain breathing during feeding. Pediatrics. 120(4): 1035-1042. • Pinnington LL, Smith CM, Ellis RE, Morton RE. (2000). Feeding efficiency and respiratory integration in infants with acute viral bronchiolitis. J Pediatr 137 (4):523-526. Hands On Lab Session Groups Special Considerations for the Use of FEES in Infants

J. Paul Willging, MD Claire Kane Miller, PhD Objectives • Describe indications and protocols for use of FEES in special populations • Protocol for using FEES with infants who are exclusively breast feeding Pediatric Feeding & Swallowing Problems

• Medical and technological progress in last several decades - ↑ survival of infants and children with complex medical conditions • Incidence and prevalence of dysphagia ↑ in children with medical problems with structural, neurological, metabolic, or mixed etiologies Medically Fragile Infants Units/Populations at CCHMC • CCHMC Units • NICU* • Medical/Surgical • Fetal Care Center • GI • Level IV, 54 beds • Transplant • Common diagnoses • Extreme prematurity • Rehabilitation • Congenital diaphragmatic • Psychiatric hernia • Gastroschisis • Transitional Care* • Genetic Syndromes • Pulmonary* • Craniofacial conditions • Neurology* • Neurologic conditions • Tracheoesophageal fistula • Complex Airway* /Esophageal atresia • Pediatric ICU • Cardiac ICU and Step Down Range of Patient Diagnoses • Structural issues • Laryngeal clefts, LTE clefts • Glottic webs, pharyngeal stenosis, buccopharyngeal membrane • Congenital & acquired vocal fold paralysis, subglottic stenosis • Cystic hygroma, cervical lymphatic malformation • Syndromes • CHARGE, VATER, Down Syndrome, DiGeorge, Pierre Robin Sequence, Cri du Chat • Chromosomal abnormalities • Neurologic Conditions • Prematurity, Chiari malformation, traumatic brain injury, neurodegenerative disease, anoxic brain injury, shaken baby syndrome • *Many patients with multiple diagnoses Feeding Issues • Assessment begins with the bedside clinical assessment (as appropriate medically + developmentally) • Consider effect of diagnosis and current medical status • Appropriate function/intervention for their point in recovery • Autonomic stability • Feeding Status (TPN, Enteral, Windows, Consolidating) • Behavioral competence (state, state maintenance) • Reaction to sensory input (light, sound, touch, movement) • Global motor function • Positioning • Structures • Oral Reflexes • Secretion management Therapist’s Role as Related to Feeding • Create/support unit environment maximizing feeding/swallowing potential (especially in NICU) • Educate staff and family about supportive practices for long term goal of oral feeding • Oral care, taping, kangaroo care • Monitor medical recovery for appropriate timing of assessment and interventions considering effect of underlying systems • Respiratory (O2 support, Respiratory Rate, WOB) • Cardiac status • Digestive (TPN, enteral feeds, consolidation, weight gain) • Neurological status • Age (maturity, developmental and behavioral readiness) Clinical Signs and Symptoms of Possible Swallowing Dysfunction

• Coughing, choking, gagging during feeds • Noisy, wet respiration associated with feeding • Physiologic signs: apneic spells, color changes, bradycardia, increased respiratory rate, oxygen desaturations • Evidence of food or liquid in tracheotomy tube during/after feed • Congestion, pneumonia/unexplained respiratory illnesses Considerations for Instrumental Assessment • Clinical signs and symptoms of potential airway protection compromise with feeding/swallowing • Secretion management concerns • Respiratory/lung changes • Increased respiratory rate or work of breathing with oral feeding attempts • Color change • Lack of or decreasing coordination with feeding task (monitor cues) • Increasing avoidance, poor progress • Integration of considerations will assure best assessment • Determining which test • When done • With strong rationale Timing of Instrumental Assessment • Need clear information regarding introduction or return to oral feeding after recovery from primary illness/impairment • Baseline • May be prior to admit for surgical intervention (airway, scoliosis) • Neurological: anoxia, Hypoxic Ischemic Encephalopathy (significant secretions) • Need support to begin feeding process to assure safety and not affect status • Botulism • ICU: head injury • Chronic pulmonary • High flow nasal cannula – less than 2 L Videofluoroscopy and/or FEES? • VSS • FEES • Infants using chain • Anatomy questions swallowing sequences • Secretion management with bottle feeding concerns • Observe phases of • VERY limited acceptance swallowing: effect on • Breastfeeding each other • Positioning • Patient without issue of anatomy or • Body limitations secretion concern • Positioning limitations • Adequate volume of • NICU: PRS, intake for reasonable mylomeningocele imaging Goals of Study • Goals are dependent on individual patient • Determine swallow function/airway protection • Not just aspiration • Not Pass/Fail • Determine specific functional recommendations for oral feeding interventions: • Guides therapeutic approach • Assures moving on “pathway to oral feeding” if at all possible Scheduling Considerations

• Considerations: assure results • Parent/caretaker attend – are valid and reliable powerful learning • NOT just moment in opportunity time • Observing helps • Best environment and • Increase support of typical skills understanding of function • State/alertness/sleep • Rationale for • Feeding interventions schedule/hunger • F/U studies – determine need FEES in the NICU • Collaborative effort – MD/SLP/RN • Consideration of anatomy and function • Population adjusts to FEES • Typically calm and feed • Done at bedside • Possible to do during breastfeeding • Exam helps with education of family CCHMC Protocol for NICU FEES Examination

• Establish reason for FEES – (secretion management, readiness to transition to po, confirm airway protection during swallowing, etc) • Confirm physiologic readiness for exam, respiratory status • Perform baseline sensorimotor assessment and/or review existing data • Prepare environment, assemble supplies, prepare equipment/scope CCHMC Protocol for NICU FEES

• Incorporate neurobehavioral supports • Maintain typical support (O2) • Patient’s nurse is present – monitoring autonomic stability, behavioral organization throughout • Trial therapeutic interventions during the FEES examination • Positioning, elevated side-lying • Non-nutritive stimulation →presentation of nutritive tastes, progressing with volume as tolerated

Aquadeks • Multivitamin mixture – used in NICU • Food color contraindicated in context of increased gut permeability • Green food color – propylene glycol • AAP recommendation < 300 mg per day CCHMC Protocol for NICU FEES

• Position patient for exam • Assessment of: – for oral feeding or • Ability to manage breast feeding secretions • Non-nutritive sucking • Pass scope and perform patterns assessment – encourage • Ability to manage non-nutritive sucking small volumes of during passage nutritive stimulus • Present via controlled • Introduce strategies as bolus volume, slow needed flow nipple • Remove scope, review • Pacifier/Nipple: images, develop plan syringe drip

NICU FEES Protocol and Interpretation

• Judge appearance of hypopharynx and larynx at rest • Assess vocal fold mobility • Assess secretion management and spontaneous swallow frequency • Judge sensory awareness • Assess swallowing parameters – ability to achieve and sustain airway protection during swallowing • Test response to compensatory strategies Interpretation

• Oral control of bolus, swallow response time, laryngeal penetration (location, depth), aspiration, residue • Swallow Onset Time • Pharyngeal clearance • Airway protection – ability to achieve and sustain • Protective reactions/responsiveness to abnormalities in swallow function • Cough or other reaction to clear • Generation of additional swallows to clear residue • Sensory awareness Recommendations • Recommendations come from objective observations in VSS/FEES in the context of overall knowledge of infant/child • Medical issues, age, development, energy, state (maintenance), caloric needs/volume Use and Safety of FEES in the NICU • CCHMC data and experience – Willging, Myer • Recent publications • Suterwala et al., 2017: Using fiberoptic endoscopic evaluation of swallowing to detect laryngeal penetration and aspiration in infants in the neonatal intensive care unit • N = 25 • No adverse events (epistaxis, laryngospasm) or autonomic instability noted • Reynolds, Carroll, & Sturdivant, 2016: Fiberoptic endoscopic evaluation of swallowing: a multidisciplinary alternative for assessment of infants with dysphagia in the neonatal intensive care unit • Protocol described for breastfeeding and bottle feeding, N = 50 Compensatory Strategies

With NICU Infants: • Positioning alterations – upright v sidelying • Pacing – determine appropriate intervals for pacing • Flow Rate – adjusting flow dynamics, nipple flow options • Viscosity changes (rare in our NICU setting) Rationale for Changes in Liquid Viscosity • Rationale: altering viscosity of liquid reduces flow rate, may facilitate oral control and provide increased time for patient to achieve airway closure and thus airway protection during the swallow • Developmentally appropriate? Changes task at all levels: • Suck, bolus collection and transfer, pharyngeal contraction, esophageal peristalsis, digestion • May affect endurance • Frequently used practice in pediatrics → implications for use • Lack of evidence regarding effect of this strategy on long term outcomes

• Abrams, 2011; Gosa et al., 2011; 2015; Cichero et al., 2013 Thickening Agents

• Thickeners available: • Gel-based (breast milk) • Xanthum gum (Simply Thick, Hydra-Aid) • Carob Bean Gum • Food Based • Rice Cereal • Infant baby foods • Starch based • Corn Starch (Thicken Up, Thick It) • Pectin Altering Flow – Considerations for Changes in Viscosity • Thickening agents (gel) are contraindicated in infants less than 12 months, and infants and children up to 12 years with history of necrotizing entercolitis • Potential harmful side effects associated with use of thickening products containing xanthan gum or similar agents • Viscosity of liquids can vary based on product used to thicken, preparation, base liquid characteristics (acidity) • September, Nicholson, Chichero, 2014 – rheological characteristics of thickened infant formula – impact on practice • Drenckpohl et al.2010- risk factors for developing NEC; Gosa et al. 2011-Systematic Review of TL as tx for dysphagia; American Academy of Pediatrics – caution, 2011; Woods et al., 2013 – NEC in infants receiving ST

30 Precautions re Gel-based Thickeners

31 Special Considerations - Breastmilk • Thickening agents (rice cereal, starch-based) add volume without full range of nutrients • Human milk enzymes (amylase) break down rice cereal and starch-based thickeners mixed with breastmilk, returns fluid to thin liquid • As with formula, gum-based gel thickeners add volume without calories, challenge infant to consume greater volume to achieve same calorie/nutrient intake • *Cautions re gum-based thickeners in infants < 12 months of age – Simply Thick product label

32 Thickened Liquids Protocol

Medical Team Decision by: Collaborative task force lead by • Physician: medical GI and NICU physicians, nutritionists and speech considerations pathologists • Neonatal Nutritionist: • Goal: policy regarding use nutritional effects of thickeners with patients • Speech Pathologist: with swallow dysfunction thickening specification Outcome • Use of other methods to • Simply Thick is not used achieve goal with patients under 1 year of age or pts with history • Slow flow of NEC • Pacing • Rice cereal is used • Positioning under 1 year Infants with Cardiac Conditions – Role of FEES Cardiac Patient Referrals

• Population • Infants status post cardiac surgery • Infants/children status post cardiac transplant • Pediatrics with history of cardiac anomalies (including history of CVA related to cardiac diagnosis) • Syndromes including Trisomy 21, DiGeorge, CHARGE, VATER/ VACTERL Cardiac Patients

• Services Provided • Re-initiation or initiation of oral feeding after cardiac surgery or after cardiac stabilization • Education of caregivers regarding feeding techniques to assist with control of flow, positioning, pacing, etc. • Some populations may have increased risk of swallow dysfunction due to CVA from cardio-pulmonary bypass/ use of ECMO, or laryngeal nerve involvement from cardiac surgery • Speech/language stimulation/ therapy for patients with longer term inpatient stays due to need for transplant, use of VAD while waiting for transplant, or patients s/p CVA Cardiac Diagnoses Cardiac Diagnoses in our population of patients: • Single Ventricle Anomalies: Hypoplastic Left Heart Syndrome, Tricuspid Atresia, Double Inlet Left Ventricle, Heterotaxy, some Double Outlet Right Ventricle • ASD, AV Canal Defect, Coarctation of the Aorta, Interrupted Aortic Arch/VSD, Patent Ductus Arteriosus, Pulmonary Atresia, Pulmonary Valvar Stenosis, Tetralogy of Fallot, Transposition of the Great Arteries, Vascular Rings Cardiac Population – Typical Feeding Issues

• Difficulty coordinating suck/swallow given increased respiratory rate/ state/ endurance. • Neurological involvement may affect oral and pharyngeal phases • Oxygen considerations- waiting to progress to flow until weaned sufficiently off of High-Flow Nasal Canula • Considerations of initiation of enteral feeds in conjunction with oral feeds, tube feeding intolerance, NEC with this population Hypoplastic Left Heart Syndrome

• Congenital heart defect – left side of heart small and underdeveloped • Typically undergo the “Norwood” procedure within the first 1-2 weeks of life • This procedure has a higher degree of involvement of the recurrent laryngeal nerve due to its proximity to the repaired areas • Vocal Cord Paralysis with this population has been reported to be 18.5% (Davis et al, 2008) and also 45% (Srinivasan, 2009) • Protocols s/p repair have changed over time, currently all patients have a VSS when appropriate Vocal Fold Involvement • Any open heart procedure where the repair is in close proximity to the aortic arch could have vocal fold involvement. • Also have seen bilateral vocal fold involvement at times which may be related to just intubation/ prolonged intubation Post-operative Swallow Assessment Protocol for patients s/p Norwood or Arch Advancement

Protocol will be initiated by primary team when deemed ready to feed for first oral feed.

Bedside oral motor/feeding evaluation by speech language pathologist (SLP)/occupational therapist (OT) (Obtain when physiologically stable*)

Video Swallow Study (Obtain when criteria met**)

Negative Positive

Advance oral feeds per SLP/OT recommendations Management based on findings***

ENT consult for bedside flexible laryngoscopy if recommended by Speech/OT or medical team

*Physiologic Stability: ≤2 Liters high flow O2; Respiratory Rate ≤60; Appropriately awake and alert **Criteria for VSS: Patient consistently taking ≥10ml po with therapist on ≥2 occasions. Any child unable to have VSS will jump straight to FEES. ***Options include but not limited to: FEES prior to feeds, Modified diet, NG feeds & swallowing therapy, VC injection, G-tube consult

Feeding Strategies

Some modifications for these infants may include: • Nipple change (slow flow) • Pacing • Consistency change • Limited volumes • Dipped tastes – no appreciable volume FEES – Trach/Vent patients Questions • Physiologic readiness • Current need for respiratory support • Tracheostomy - suctioning frequency • Medical Stability • Vital signs – parameters for patient • Medical, developmental history • Co-morbidities • Oral sensorimotor skills • Status – can improvement be reasonably expected, or will there be progressive decline secondary to the nature of the condition Respiratory Support & Swallowing

• Ventilator support • Tracheostomy Cuffs - toleration of deflation • Nasal Continuous Positive Airway Pressure • High Flow Nasal Cannula Oxygen • Passy Muir Valve • Capping trials Case AR • 7 month old with Trisomy 18 and myelomeningocele • Hypoxic event during feeding shortly after initial nursery discharge • Intubated→failure to extubate→tracheotomy/ventilation • Gastrostomy tube, Alimentum, 31 mL/hr, 9p – 6a • Hydrocephaly→VP shunt • Transferred to ICU for optimization of ventilation Case AR • SLP consult – airway protection/swallow function, feasibility of oral stimulation • Weaned from vent while awake • Mild to moderate tracheobronchomalacia • Grade II subglottic stenosis • Medically stable with trach/GT • Alert, readiness cues • NNS, some anterior loss of oral secretions, frequent tracheal suctioning • Clinical evaluation results • Question – safe to introduce oral trials?

FEES • Able to generate spontaneous swallows to manage secretions in hypopharynx, • Normal vocal fold mobility • Delayed onset of swallow in response to small (< 1 mL) bolus presentations • Treatment planning & goals FEES Protocol Breastfeeding Tongue Function – Bottle Feeding

• Nipple stabilized intra-orally by caregiver • Efficient sucking created by • Positive pressure: compression of the nipple by the tongue against the palate • Negative pressure: the dorso-cranial movement of posterior lowering of the jaw and tongue • Closed system with tongue cupping and anterior labial seal Tongue Function - Breastfeeding

• Anterior tongue wedges between the nipple areolar complex and lower lip/gum, and moves as a rigid body with mandibular motion • Moves anteriorly (can be limited by tight lingual frenulum) • Posterior tongue creates peristaltic movement to help express milk / transfer backward (can be limited by posterior tongue tie) Sucking Patterns - Bottle Feeding vs Breast Feeding

• Sucking pattern produced without the specific anterior protrusion and elevation required for effective breastfeeding

• Bottle fed infants with (tongue tie) can often bottle feed well because of ability for artificial nipple to be maintained in mouth without infant effort Positioning • Typically position patient upright or in a semi- reclined position to pass the scope transnasally • Re-position infant for breastfeeding while scope is in place Use of Food Color • Typically do not use food color during a breastfeeding assessment by FEES • View is adequate • Contraindications to dye in infants with increased gut permeability, medical fragility References • Cichero, J. A., Nicholson, T. M., & September, C. (2013). Thickened milk for the management of feeding and swallowing issues in infants: a call for interdisciplinary professional guidelines. J Hum Lact, 29(2), 132-135. doi:10.1177/0890334413480561. • Drenckpohl, D., Knaub, L., Schneider, C., McConnell, C., Wang, H., & Macwan, K. (2010). Risk factors that may predispose premature infants to increased incidence of necrotizing enterocolitis. ICAN: Infant, Child, & Adolescent Nutrition, 2(1), 37-44. • Gosa, M., Schooling, T., & Coleman, J. (2011). Thickened liquids as a treatment for children with dysphagia and associated adverse effects: A systematic review. ICAN: Infant, Child, & Adolescent Nutrition, 1941406411407664. • Gosa, M. M., & Corkins, M. R. (2015). Necrotizing Enterocolitis and the Use of Thickened Liquids for Infants With Dysphagia. SIG 13 Perspectives on Swallowing and Swallowing Disorders (Dysphagia), 24(2), 44-49. References

• Lau, C. (2014). Interventions to Improve Oral Feeding Performance of Preterm Infants. SIG 13 Perspectives on Swallowing and Swallowing Disorders (Dysphagia), 23(1), 23-45. • Lau, C., & Schanler, R. (2000). Oral feeding in premature infants: advantage of a self-paced milk flow. Acta Paediatrica, 89(4), 453-459. • Lau, C., & Smith, E. (2012). Interventions to improve the oral feeding performance of preterm infants. Acta Paediatrica, 101(7), e269-e274. • Lau, C., Smith, E., & Schanler, R. (2003). Coordination of suck‐swallow and swallow respiration in preterm infants. Acta Paediatrica, 92(6), 721- 727. • Law-Morstatt, L., Judd, D. M., Snyder, P., Baier, R. J., & Dhanireddy, R. (2003). Pacing as a treatment technique for transitional sucking patterns. Journal of Perinatology, 23(6), 483-488. References

• September, C., Nicholson, T. M., & Cichero, J. A. (2014). Implications of changing the amount of thickener in thickened infant formula for infants with dysphagia. Dysphagia, 29(4), 432- 437. doi:10.1007/s00455-014-9523-z. • Woods, C., Oliver, T., Lewis, K., & Yang, Q. (2012). Development of necrotizing enterocolitis in premature infants receiving thickened feeds using SimplyThick®. Journal of Perinatology, 32(2), 150-152. Role of FEES with Infants with Complex Conditions J. Paul Willging, MD Claire Kane Miller, PhD Objective • Describe the protocols used in FEES and videofluoroscopy in medically fragile infants, and the application of each study in special populations • Review cases, findings, and treatment recommendations Medically Fragile Infants • FEES may be the most appropriate, and initial examination • Oral intake often minimal or nil • Safety of swallowing needs to be established Case Studies Infant J.L.

• Born @ 36 weeks with hypoplastic left heart syndrome • Candidate for the Norwood procedure – performed at DOL 3 • Clinically, demonstrating coordinated non-nutritive sucking, accepting ~ 5mls during oral feeding trials with coordinated suck/swallow pattern, displaying weak cry • FEES study performed at 2 weeks of age to assess vocal fold function, airway protection skills FEES – s/p Norwood Procedure Infant K.R. • Full Term • Arthrogryposis • Laryngomalacia • Multiple anomalies: rocker bottom feet • Bedside scope results: prolapsed arytenoids • Bedside eval: adequate skills to initiate oral feeds • Stridor & desats with oral feeding trials prior to supraglottoplasty K.R. FEES KR Findings

• FEES • Pooled secretions spilling into airway, aspirated • With swallow, minimal clearance of material • No cough response • Recommendations • Oral stimulation • Tastes KR – Follow up VSS Infant L.S. • Born at 31 weeks gestation, C section/exit procedure to trach • Large cervical teratoma with resection at 33 weeks of age • Apnea and bradycardia (positional) • Questionable secretion management with significant collection intraorally • Non-nutritive suck – limited active movement Case Study: LS • History • Feeding Evaluation DOL 16 • Prenatal diagnosis of large cervical • Continuous NJ, HM-15ml/hr teratoma • Oral secretions pool • Birth at 31 weeks: c-section/exit • Reflexes intact procedure with trach • Calms with pacifier • Resection at DOL 3 • Compression, no NP • RDS, Apnea/Bradycardia and • Position sidelying on surgical reflux with g-tube/nissen side fundoplication (DOL 40) Clip: L.S. Infant LS • FEES • Pooled secretions in pharynx (asymmetrical) • Delayed swallow onset • Aspiration with no cough • Weak pharyngeal contraction with remaining material after swallow • Recommendations • Tastes of minimal volumes Infant J. C. • 5 month old with arthrogryposis • Polymicrogyria, dysmorphic features • Respiratory distress • Status post supraglottoplasty • Maternal use of opiate • Questions re swallow function, management of secretions JC Pre-FEES JC Fees Exam JC Findings • Redundant arytenoids, static prolapse left > right • Vocal folds difficult to visualize; appear to be immobile in paramedian position • Frequency of spontaneous swallows – reduced • Appeared to be sensate to tactile stimulation of the endoscope • Impaired management of secretions • Delayed onset of the swallow in response to presentation of Aquadeks • Recommendations? Infant AY • 3 month old with possible VACTERL • Butterfly vertebrae – renal issues • ASD/VSD being followed • Feeding difficulties – but 100% orally fed • VSS – posterior impression on esophagus – likely aberrant right subclavian • Elecare per slow flow nipple • Coughing, choking, vomiting Infant AY • Normal orofacial symmetry and tone • No restrictions in range or strength of oral movements • Rapid feeder Infant AY FEES Findings and Recommendations • Normal vocal fold mobility • Appropriate secretion management • Coordinated suck-swallow, rapid feeding pattern without pause intervals • Other? • Recommendations Infant VC • Born at 30 weeks, now 42 weeks • Stable, off O2 for 3 weeks • Full oral feeder • Decline with oral feeding skills in the absence of any other issues • Pooling oral secretions Infant VC

Infant VC Case AL • 31 week preemie • Hx of complex congenital heart disease – status post repair of partial anomalous pulmonary venous connection • Hypoglycemia • Metopic craniosynostosis • FEES at 4 mos – LVFP, delay in swallow onset Case AL Case AC • VACTERL • Anorectal malformation • Recurrent TEF – has esophageal stent • Chronic congestion/pneumonia • Former preemie – FEES @ 1 y to assess secretion management, VF function, airway protection during swallow Case AC Comparing and Contrasting VSS & FEES

J. Paul Willging, MD Claire Kane Miller, PhD Objectives • Identify clinical indications and contraindications for videofluoroscopy and FEES • Differentiate advantages and disadvantages associated with instrumental assessments of swallowing VSS or FEES

• Decision-Making: Which Test? Both Tests? • Presentation of patient • Considering each exam as an adjunct to one another and not necessarily a “stand alone” exam • Access • Comparison chart Validation & Comparison Studies

• Review: Pilot Study – VSS & FEES comparison, simultaneous studies • Willging, 2000; Hartnick, 2000 • Findings and Recommendations – VSS & FEES, within 6 weeks, agreement penetration/aspiration, FEES superior for anatomic abnormalities, residue, premature spillage • Lack of effect of endoscope on swallow fx • Willging, 1997; Suiter & Moorhead, 2007 • Perception of severity – influence of exam type • Aghdam et al, 2017; Nienstedt, 2017 • Reviews – Hiss & Postma, 2003; Langmore, 2017 • Outcomes - Warnecke et al., 2009; Marques et al., 2010; Sitten et al., 2011 Decision-Making VSS, FEES, or Both?

• Consideration of your case - what are the factors present? Define the questions – then your plan of action • Keep in mind • Video swallow study provides overall view of oral, pharyngeal, and cervical esophageal phases of swallowing – *logical starting point in most cases • FEES will provide a closer look at anatomy and function, swallowing abnormalities, rule out certain structural issues, confirm/clarify findings of VSS • Decision may be based on availability • Adjunct, not replacement exams Pediatric VSS

• Delineation of physiology and swallowing function • Decision-making re modifications necessary • Overall recommendations to meet nutrition/hydration needs and to maintain pulmonary stability • Lefton-Greif & Arvedson 1998; Arvedson & Brodsky, 2002; Arvedson, 2007 Pediatric VSS Protocol

• Difficulty with “standardizing” • Modifications – made in context • MBSIMP™ • Martin-Harris, 2008 • Bottle-fed children: Lefton- Greif, et al., 2018 • Implications of *terminology used → consistency of recommendations • Knowledge of anatomy, physiology, and development a must Comparing Visualization VSS & FEES

• Secretion Management • Hyoid/Laryngeal excursion • Velopharyngeal Closure • Vocal Fold Mobility • Pharyngeal Contraction • Function of the UES Comparing VSS & FEES Secretion Management

• VSS – no apparent secretions • FEES – can detect and judge: • Appearance of secretions: normal, foamy, thick • Amount of standing secretions: normal, excessive • Pooling within the laryngeal vestibule • Evidence of leakage into the subglottis • Patient response to secretions: normal spontaneous swallows to clear, no attempt to clear, ineffective attempts to clear Hyoid/Laryngeal Elevation FEES – see laryngeal VSS – Able to visualize elevation prior to directly swallow onset Velopharyngeal Closure During Swallowing • FEES • VSS • Nasopharyngoscopy • Clear visualization of routinely used to assess backward motion of velum adequacy of the against posterior velopharyngeal sphincter pharyngeal wall • VP closure is simultaneous with medial motion of the arytenoids Pharyngeal Contraction

• VSS • FEES • Contraction of the pharynx • As the pharynx contracts during swallowing can be during swallowing, structures directly visualized close around and the tip and deflect light • Adequacy of • As structures resume resting hypopharyngeal clearance position, view re-opens • Can view structures before and after – not during swallow • Can infer adequacy of contraction Function of the UES • FEES • VSS • May see incidental • Clear view of upper episodes of reflux during esophageal sphincter exam, opening of the function UES, patient response to • Restriction in opening reflux events apparent • Opening during swallowing occurs during white-out during • Visualize closed segment following swallows Sensory Assessment • VSS – can infer sensory abnormality with persistent residue, ↓ awareness • Subjective judgment • FEES: provides objective info re laryngopharyngeal sensation • Try to do sensory assessment prior to feeding… Comparing VSS & FEES Vocal Fold Mobility

• Vocal folds - shadowy image on VSS • On FEES: clear view of abduction during respiration • Assess airway protection status • Cough – complete adduction/incomplete • Hold breath – TVC adduct – complete/incomplete, FVC adduct- yes/no • Arytenoid contact medially – normal, reduced • Phonation – Adduction, Abduction • TVC adduction, normal, abnormal • FVC – adducted/hyperadduction • Excessive closing down of arytenoids • Asymmetry of arytenoids • Use of supraglottic compensatory compression Interpretation Comparisons Willging JP & Miller, CK . Pediatric FEES Course 2017 Parameter VSS FEES Velopharyngeal closure excellent excellent Vocal cord mobility and closure Good/fair excellent

Laryngeal elevation excellent good Oral control and transit excellent Fair Pharyngeal squeeze good excellent Pooling of secretions poor excellent Swallow response time excellent excellent Penetration good excellent Aspiration excellent *good (loss of view during swallow, loss of view during consecutive swallows)

Residue excellent excellent Interpreting Swallowing Function Overall Considerations

• Does examination type influence perception of severity?

• Issues with reliability – both exams

• Variability in recommendations - both exams

• Need for evidence – in general For example: • Laryngeal penetration: implications of intermittent “shallow” versus “deep” • Intermittent or rare aspiration events • Relative risk of pneumonia based on results of instrumental exam • Additional Cases

Using VSS & FEES in Clinical Practice Patient N.M. • 2 month old infant with gagging and choking episodes • ALTE BRUE • Scoped and found to have "deep notch" • Referred for swallowing studies for decision making regarding surgical intervention of deep notch Laryngeal Clefts Patient NM Rate Frequency of Laryngeal Penetration A. No episodes B. Occasional C. Intermittent episodes of laryngeal penetration (30- 40%) D. Frequent episodes of laryngeal penetration (50% or greater) Patient NM – FEES Rate Swallow Onset Time A. Timely B. Swallow initiated as bolus enters the valleculae C. Swallow initiated as bolus enters the pyriform sinus Patient LL • 8 month old infant • Nasal regurgitation with feeds intermittently • Coughing and choking with bottle feeds • Respiratory illnesses/pneumonia Case LL VSS Clip Case LL FEES Laryngeal Cleft Type Two Submucous Cleft Palate Laryngeal Cleft Type Two Case LL FEES VFSS • Confirm location of LP and aspiration • Point of entry – • Sensory awareness penetration/aspiration • Pattern - *consistency • Direct view of anatomy, interarytenoid area is • Response – cough full, vocal fold mobility • Raises suspicion • Need for further assessment to rule out structural deficit • Submucous cleft palate Laryngeal clefts & “deep notches” VSS FEES • Documents pattern of • View of anatomy, dx aspiration – consistent submucous cleft interarytenoid entry point • Cannot definitively for diagnose laryngeal cleft penetration/aspiration? • May identify fullness in the • Frequency of aspiration interarytenoid area – raise • Patient response suspicion • Deep interarytenoid notch • Sensory threshold Case A.M. Summary of events

• Caustic Ingestion – October, 2012 • Circumferential slough of mucosa through esophagus • NG placed, continued to eat orally, took contrast orally during esophagrams to assess esophageal function • NG – dilations (Oct 2012 – May 2013), continued to take some oral intake in addition to NG feeds Case A.M.

• Eventually developed esophageal stricture which was repeatedly dilated • Stricture became unresponsive to dilation – determined esophagus could not be salvaged • Colonic interposition – May 2013 • Esophagram showed satisfactory passage of contrast to stomach, manometry results “normal” • Developed additional stricture→stent placed • Developed pain associated with stent, generalized anxiety related to caustic ingestion event • Complaints of dysphagia escalated, not swallowing secretions Esophageal Manometry - Results

• UES normal with complete relaxation, esophageal body contractions propagate to the point of anastomosis • Normal dry swallows • Normal proximal peristalsis (native esophagus) Video Swallow Clip *watch to rate Pharyngoesophageal Segment Opening

1 = Adequate, bolus flowing through without obstruction 2 = Partial obstruction 3 = Complete obstruction Video Swallow Clip Rate Pharyngoesophageal Segment Opening A. Adequate, bolus flowing through without obstruction B. Partial obstruction C. Complete obstruction FEES Clip Does the Patient demonstrate laryngeal penetration? A. Yes B. No Case A.M.

• Injection of botox in CP muscle (November, 2013) • Recurrent strictures – pain • Endoscopic criopharyngeal myotomy – January, 2014 • Continued strictures • VSS – continued to show persistent abnormal cricopharyngeal function with lack of relaxation with every attempt at swallow Case A.M. • Underwent criopharyngeal myectomy • Pathology results showed – fibrotic changes to tissue • Result of caustic ingestion injury evolution – pattern and progression of injury can be difficult to predict • Follow-up video swallow showed adequate opening of the pharyngoesophageal segment • Patient is eating orally Case J.W. • Duchenne Muscular Dystrophy: type of muscular dystrophy that worsens quickly, death occurs by ~ 25 y • Caused by defective gene for dystrophin (a protein in the muscles) • Only males affected, occurs 1/3,600 male infants • Inherited disorder, risks include family history of Duchenne MD • No cure – future therapy may include stem cells and gene therapy • Uses "sip" ventilator – intermittent positive pressure ventilation (alternative to trach) Case J.W. • Nutrition intake is 100% oral, quality of life issue • Increasing issues with coughing and choking • No prior episodes of pneumonia or respiratory illness • JW underwent a cardiac procedure - worsening problems with eating and drinking • Referred for a video swallow study Case JW VSS PenAsp Rating A. 2 B. 4 C. 6 D. 8 Case JW VSS Primary issue with the swallow appears to be A. Strength of pharyngeal contraction B. Obstruction at the UES C. Both A & B Case JW FEES Does Aspiration Occur? A. Yes B. No Case JW

VSS FEES • Initial look at • Hypopharyngeal swallowing function clearance • Swallow onset time • Location of residue • Airway protection • Effect of compensatory • Residue, effects of swallow strategies ↓strength • Biofeedback for patient • Recommendations • Quality of life References • Aghdam, M.A., Ogawa M., Iwahashi, T, et al. (2017). A comparison of visual recognition of the laryngopharyngeal structures between high and standard frame rate videos of the fiberoptic endoscopic evaluation of swallowing. Dysphagia, 32(5): 617-625. • DaSilva, A., Neto, J. & Santoro, P. (2010). Comparison between videofluoroscopy and endoscopic evaluation of swallowing for the diagnosis of dysphagia in children. Otolaryngology-Head and Neck Surgery, 143:204-209. • Hartnick CK, Miller C, Hartley B, & Willging JP. (2000). Pediatric Fiberoptic Endoscopic Evaluation of Swallowing. Annals of Otology, Rhinology & Laryngology, 109 (11):996-999 • Hiss SG and Postma GN.(2003). Fiberoptic endoscopic evaluation of swallowing. Laryngoscope, 113(8): 1386-1393. References • Langmore, S. (2017). History of fiberoptic endoscopic evaluation of swallowing for evaluation and management of pharyngeal dysphagia: changes over the years. Dysphagia, 32(1): 27-38. • Lefton-Greif MA, McGrattan KE, Carson KA et al. (2018). First steps towards development of an instrument for the reproducible quantification of oropharyngeal swallowing physiology in bottle-fed children. Dysphagia, 33(1): 76-82. • Marques I, et al. (2010). Clinical and fiberoptic endoscopic evaluation of swallowing in Robin sequence treated with nasopharyngeal intubation: the importance of feeding facilitating techniques. The Cleft Palate Craniofacial Journal, 47(5):523-529. • Nienstedt, J.C. (2017). Narrow band imaging enhances the detection rate of penetration and aspiration in FEES. Dysphagia, 32: 443-448. References

• Suiter DM, & Moorhead MK.(2007). Effects of flexible fiberoptic endoscopy on pharyngeal swallow physiology. Otolaryngology- Head and Neck Surgery, 137(6): 956-958. • Warnecke T, Teismann I, Oelenberg S, et al. (2009). The safety of fiberoptic endoscopic evaluation of swallowing in acute stroke patients. Stroke, 40(2):482-486. • Willging, J.P. Endoscopic evaluation of swallowing in children. (1995). Int J Pediatr Otorhinolaryngol, (suppl 32): S107-S108. • Willging, J.P., Miller, C.K., McConnell, K., & Rudolph, C.D. Fiberoptic endoscopic evaluation of swallowing in children: a preliminary report of 100 procedures. Dysphagia, 1996: 11:162. • Willging JP, Miller C, McConnell K & Rudolph C. (1997). Lack of effect of fiberoptic endoscopic passage on swallowing function in children. Dysphagia, 13:131. Complex Case Discussion Claire Kane Miller, PhD J. Paul Willging, MD Learner Outcomes • Discuss the range of medical and instrumental assessments available for the evaluation of the complex Aerodigestive patient • Discuss decision-making regarding the diagnostic protocol Process begins with: New Patient presented at Referral ADEC Grand Rounds

Referral intake coordinator receives Requests pertinent medical records Patient medical history and goals for assessment discussed Reviews lead service criteria and assigns

Intake conducted by lead service Nurse Practitioner Plan formulated for patient’s evaluation with team input Input into EMR Ex: Anesthesia, Chest CT, VSS, FEES, Shared Aerodigestive Clinic, MLB/EGD/Flexible Bronchoscopy, Impedance

Shared Aerodigestive "O" drive history created using ADEC Treatment order set entered into EMR and received by template insurance authorization/surgical scheduling Aerodigestive Evaluation Steps • Intake key to pre-visit planning • Presentation of Case – team discussion and collaboration • Decision making re diagnostic protocol • Workup may include: • High resolution chest CT • Swallowing studies – VSS and FEES • MLB, flexible bronchoscopy with BAL, EGD and impedance • Imaging – MRI, scintigraphy • Manometry Weekly ADEC Rounds Results and Plan Plan for Treatment/Follow up

Results reviewed by individual providers Lead service places plan on shared "O" All results posted on shared aerodigestive Aerodigestive drive drive history by data coordinator

Lead service writes orders for follow up Results presented at ADEC Grand Rounds to testing or surgery and forwards to establish collaborative plan schedulers

Plan formulated i.e. surgical options, Integrated Care Plan created by data medication, recommended treatment coordinator with results of all testing

Plan conveyed via phone to parents by the Integrated Care Plan is routed and reviewed lead service after the Wednesday meeting by all providers, sent to referring physician, PCP and family Case YB • 6 month old with microcephaly, hypotonia, chromosomal abnormalities • Admitted with aspiration pneumonia, hypoxia • Aerodigestive assessment: • Flexible bronchoscopy with bronchoalveolar lavage • Microlaryngoscopy and bronchoscopy • EGD • MRI VSS & FEES Findings • VSS: poor sucking efficiency, • FEES clip late onset of the swallow, penetration/aspiration • FEES: normal vocal fold mobility, significant congestion, coughing throughout the exam, penetration, aspiration MLB • Supraglottis structurally normal, no deep notch or laryngeal cleft • Edematous arytenoids • Normal glottis, edematous • Trachea malacic, with moderate secretions Flexible Bronchoscopy • Upper airway obstruction • Laryngeal edema, poor tone • Lower airways notable for mild malacia and mucopurulent bronchitis • Lavage specimen consistent with ongoing aspiration EGD • Esophageal lining normal in appearance • Biopsies - mild reactive changes in distal esophagus, focal acute esophagitis in the proximal esophagus Head MRI • Mild enlargement of the ventricles and subarachnoid fluid spaces over the cerebral convexities • Findings most likely a reflection of decreased cerebral volume Issues • Airway protection issues • BAL results consistent with ongoing aspiration • Parents resistant to GT • Long term plan Additional Cases • Submitted cases Setting Up a FEES Clinic - Guidelines

J. Paul Willging, M.D. Claire Miller, Ph.D. Learner Outcomes • Explain how to implement the use of pediatric FEES in the clinical setting • Credentialing considerations • Review sterilization procedures and infection control ASHA Guidelines Scope of Practice, Level of Competence • Australia • US: www.asha.org – ASHA Portal • Knowledge & Skills Needed by SLPs Providing Services to Individuals with Swallowing and/or Feeding Disorders • Position Statements - Knowledge/Skills Guidelines: • Videofluoroscopic Swallowing Studies • Position Statement re Endoscopy – within SLP scope of practice • Role of the SLP in the Performance and Interpretation of Endoscopic Evaluation of Swallowing • Knowledge & Skills re Endoscopy Gaining Knowledge & Skills related to Dysphagia Practice • Related to service provision for dysphagia • Completion of graduate level coursework • Practicum with dysphagia focus • Attendance at conferences/ workshops (Dysphagia, Fluoroscopy, Endoscopy), continuing education units • Knowledge evaluation via supervisor at host institution, clinical practice with dysphagia focus • Development of expertise, demonstration of clinical competence • Board certification in swallowing (BCS) Implementing FEES in a Pediatric Setting • Establish credibility in dysphagia • Participate in teams as available, maintain excellent skills in fluoroscopy interpretation, keep current with research in dysphagia • Gain support from M.D. and/or experienced SLP interested in doing FEES • Agreement to collaborate or mentor • Gain support with administration • Investigate the feasibility of equipment purchase, use of existing space/equipment • Check with licensure board re restrictions • Obtain training in FEES • Attend workshop, gain familiarity with literature • Return to site and work with identified mentor • Train at the right level to ensure competency Conducting the Exam

• Suggestions for verifying endoscopy skills (ASHA) via a local mentor: • Three Step Process • Observation • Practice under direct supervision – at least 25 exams • Langmore, et al. • Independent practice with indirect supervision • Considerations with the pediatric population • Medically fragile patients – benefits of collaborative exam with physician Reporting • FEES Epic flowsheet • .slpfees template • Collaborative review • Drop-down boxes of FEES exam • Process for dual note • Agree upon findings and dual signature • Evaluate effectiveness of compensatory strategies • Next steps Billing Considerations Establishing FEES as part of the Diagnostic Protocol • Using a Team approach • Referrals, scheduling, clinic procedures • Communicating indications for and benefits of exam • Asking the right diagnostic questions • Using FEES to help answer: • What is the physiologic pattern of the swallow? • Is the patient aspirating, is he at risk, and most importantly why? Advantages of Team vs. “I” Approach • The value of collaboration - multidisciplinary • Patient education prior to exam: “Welcome to FEES Clinic” patient education program • Patient interaction – prior to, during, and after exam • Effective counseling Communication re the “next” step • Appreciation of specific and overlapping knowledge areas Proper Care of Flexible Endoscopes - Review Parts of the Nasopharyngoscope

• Fiberoptic bundle • Barrel • Articulation knob • Eye piece • Light source cable • Options • Channel • Camera connection unit Integrity of the Endoscope

• Good working order • Integrity of the sheath • Light bundles are intact • Articulation fibers functional • Light adequate • Appropriate focus • Safe • No infection risk to patient or operator Equipment Issues • Processing Scopes • Cleaning Guidelines Reprocessing of Flexible Endoscopes • Must have a policy outlining how endoscopes are to be reprocessed • Avoid non-standardization of processes within an institution • Requirement for standard care delivery • Common areas that utilize / process scopes • Sterile Processing and Distribution; Operating Rooms; Outpatient Clinics; Satellite Facilities Level of Cleaning

• Sterile • Destroys all vegetative microorganisms and bacterial spores • Required of all critical devices – anything that penetrates mucosal or skin barriers • High Level Disinfection • Destroys all vegetative microorganisms, but bacterial spores may remain • Minimum requirement of devices that come in contact with heavily colonized body sites such as mucoa of the respiratory tract • Clean • No debris visually apparent • Contaminated Steps in Reprocessing • Precleaning process • Transportation to cleaning facility • Leakage testing • Manual cleaning • Rinsing • High-Level disinfection • Rinsing • Drying • Storage Pre-cleaning Process

• Occurs in the endoscopy room • Remove gross soiling • Enzymatic detergent solution on cloth Transportation to cleaning facility

• Transport in closed, rigid container • Protection of other patients • Protection of health care workers • Physical protection of endoscope • Must have a designated DIRTY area to receive contaminated endoscopes • Need separate paths for clean and dirty instruments to minimize cross contamination Leakage testing

• Purpose • To ensure the integrity of the external skin of the endoscope • Prevent influx of cleaning materials into the core of the endoscope which severely damages the fiberoptic bundles • A positive leak test requires immediate removal from service of the endoscope and repair Manual cleaning • Considered the most important step in removing bacterial burden from the endoscope • Retained debris interferes with the capability of the germicides to effectively kill or inactivate microorganisms • Enzymatic detergent solution • MUST have full immersion of endoscope Rinsing

• Removes residual debris and detergent • Dry exterior of endoscope to prevent dilution of the liquid – chemical germicide High-Level Disinfection • Germicide must be tested for minimum concentration • Toxic substances • Need personal protective equipment • Gloves • Mask • Eye protection • Impervious gown • Room needs appropriate ventilation / hood High-Level Disinfection

Liquid Chemical Germicide Required Exposure Time

• 0.55% ortho-phthaladehyde 12 minutes • Cidex OPA TM

• 0.2% peracetic acid Automated system • Steris 20 TM

• 2% glutaraldehyde • Cidex TM 20 minutes Rinsing

• Move scope to a designated CLEAN processing area • Thoroughly rinse all surfaces of endoscope with sterile / filtered water • 0.2 micron filters remove potential microbial contaminants from potable water • Prevent potential injury to skin and mucous membranes from chemical residue Drying • Inhibits growth of waterborne organisms • Pseudomonas aeruginosa (a common contaminant of tap water) and fungi multiply rapidly in a moist environment • Dry exterior of endoscope with lint-free towel Storage • Designated cabinet • Adequate ventilation to encourage drying • Prevent moisture buildup • Prohibit soiling by moisture or microbes • Protect from physical impact • Cabinet must be routinely cleaned to prevent contamination of the cabinet and endoscope • Do NOT store in the case supplied by the manufacturer Book Keeping • Patient name and demographics • Procedure • Scope identification number • Log book for recording patient information, endoscope number and procedure • Purpose • Be able to identify patients at risk if a break in disinfection process has been detected