12/10/2018

Rebecca Martin, OTR/L, OTD

Acute Flaccid (AFM): The what, the why, and the role of occupational therapy • Manager of Clinical Education and Training at the International Center for Injury (ICSCI) at Kennedy Krieger Institute • Assistant Professor at The Johns Hopkins University School of Medicine in the Department of Physical Medicine and Rehabilitation Rebecca Martin, OTR/L, OTD, CKTP, CPAM • Speaks internationally on topics related to Activity-Based Rehabilitation for AOTA Webinar 12.11.2018 • Research interests are in novel applications of electrical stimulation for the restoration of function lost to spinal cord injury 

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Disclosures Objectives

I, nor any member of my family, receive compensation, At the conclusion of this webinar, participants will: financial or otherwise, for any services or products • Understand the history of AFM discussed herein. • List the diagnostic criteria, epidemiology, and clinical characteristics • Understand the medical management of children with AFM • Describe occupational therapy’s distinct role and contribution to interdisciplinary management of children with AFM • Identify improvements for patient with AFM associated with intensive rehabilitation

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History of AFM

• Acute Flaccid Myelitis (AFM) is not new – is the most common cause

• Acute Flaccid in monitored world wide. – Includes polio, non-polio, GBS, toxic neuropathies WHAT IS ACUTE FLACCID MYELITIS – Expected to occur 1/100,000 children world wide

• AFP not reportable in USA – Understanding of incidence in USA is limited

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Emergence of AFM in US AFM Cases in US per CDC1

• California, 2012 • From August to December 2014, 120 confirmed – Calls to the health department for testing in two adults for polio cases of AFM in 34 states. – Between 2012-2015 with 59 cases identified in CA • In 2015, 22 confirmed cases in 17 states. • Colorado, 2014 – Unusual cluster of neurologic illness presenting as acute limb • In 2016, 149 confirmed cases in 39 states and DC. weakness seen in hospitals in Denver area.1 • In 2017, 35 confirmed cases of AFM in 16 states. – Limb and cranial nerve weakness, , spinal MRI showing signal abnormalities in gray matter…polio? • In 2018 YTD, 134 confirmed cases in 33 states. • Between August and December of 2014, 120 children from 34 – 165 additional patients under investigation states2 • At the same time, there was a nationwide outbreak of a severe respiratory illness among children due to -D68 (EV- D68)1

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Confirmed cases of AFM in the US Diagnostic Criteria by month, as reported to the CDC

Reported enterovirus D68 circulation • Confirmed case 1. Acute onset of focal limb weakness, AND 2. An MRI showing a spinal cord lesion largely restricted to gray matter* and spanning one or more spinal segments

• Probable case 1. Acute onset of focal limb weakness, AND 2. with pleocytosis 3 No reported enterovirus D68 circulation • 2017 update – Added in the word “flaccid” to better describe – Spinal cord lesions may not be present on initial MRI (<72hrs) – Removed age restriction

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Clinical Characteristics

• Prodromal, often febrile, illness preceded onset of • Severity ranged from complete paralysis to mild weakness, neurological symptoms by 5 days in most patients flaccid in nature 2 – Typically asymmetric 1, 2 • Most patients reported clinical improvement of illness – UEs more commonly affected 2 before return of the fever along with headaches, stiff • Cranial nerve dysfunction, may include hypophonia, dysarthria, neck or pain in neck, back or affected limb around the dysphagia, diplopia, facial weakness time of neurological deficit onset • Pulmonary insufficiency to ventilator dependent • Timing, quality, and pattern of limb weakness in AFM • Sensory deficits (varying reports) consistent with acute lower motor neuron disease 2 • Varying reports of altered mental status • Rapid progression from full strength to weakness • very rare (4%) over hours to a few days 1,2 • Varying bowel and bladder dysfunction • Recovery progresses distal to proximal

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Variable (No. With Information) No. (%) At time of acute illness What we know from CDC… Demographics and Age, median (range; IQR) (n = 119) 7.1 y (0.4–20.8 y; 4.8–12.1 y) Sex (n = 120) Clinical Findings Male 71 (59) Female 49 (41) Among Acute Respiratory or febrile illness preceding limb 105 (90) weakness (n = 117) • Most patients with AFM (more than 90%) had a mild Flaccid Myelitis Limb involvement (n = 119)a Upper extremity(ies) only 41 (34) respiratory illness or fever consistent with a viral infection Cases, 2014 Lower extremity(ies) only 27 (23) Upper and lower extremities, but not all 4 before they developed AFM. 9 21 (18) (N = 120) extremities All 4 extremities involved 30 (25) • AFM is not caused by poliovirus Cranial nerve findings (n = 120) 34 (28) Altered mental status (n = 109) 12 (11) • Some patients with AFM have tested positive for Seizures during illness (n = 116) 5 (4)

Required (n = 109) 26 (20) coxsackievirus A16, EV-D71, and EV-D68 but in MANY of

At time of follow-up (median 4.2 mo after acute 56 (49) the patients no pathogen has been detected illness) Functiond • Most patients have onset between August and October, Complete dependence on caretakers 8 (14) with increases in AFM cases every two years since 2014 Somewhat functionally impaired 38 (68)

Fully functional 10 (18) • Most AFM cases are children (over 90%) and have Strength (compared to initial presentation) occurred in 46 states and DC. As weak 11 (20) Some improvement 41 (73) Full recovery 3 (5) More weak 1 (2)

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Why do some kids develop AFM?

• Not a clear definitive link between virus strains and AFM. CDC working to understand the circulation of .

• Possible causes: ACUTE INTERDISCIPLINARY – Direct infection of a virus on the motor neurons MANAGEMENT – Post-viral inflammatory or immune response directed toward motor neurons – Host genetic factors making certain children more susceptible than others

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Medical Treatment Acute Hospitalization

• No clinical trial data available to guide therapeutic • No randomized controlled data, management based recommendations upon expert opinion • Majority of patients receive IVIG, high-dose intravenous steroids, and some received antiviral therapy • Early aggressive rehabilitation to prevent muscular – No significant clinical improvement or deterioration was noted with any of atrophy, joint contractures, and other sequelae of these therapies severe and persistent limb weakness and may • IVIG may be safest therapy available improve functional outcomes 1 • Fluoxetine is effective against EV-D68 and other enteroviruses • 5% of AFM reported complete recovery of strength in • Vigilant supportive care is central component acute phase and 18% reported being fully functional – Mechanical ventilation 4 months after onset 2 – PT and OT to prevent atrophy and contractures and maximize functional outcomes. – Support for psychological as well as physical effects of disability 2

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OT in Acute Care

• Prevention of atrophy, active exercise or FES – Recruitment of surrounding, functional muscle groups, even in cases with no objective improvement in strength • Positional tolerance, ex: upright, head control • Prevention of contracture or joint deformity – Splinting REHABILITATION – Wheelchair fitting • Promote engagement in meaningful activity, as appropriate – Leisure tasks – Bedside self-care • Assist family with coping, educate on expectations

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Rehabilitation vs. Habilitation Considerations for Rehabilitation Goals

• Habilitation for the developing , guiding •Age children to an anticipated potential • Goals will change as the child develops to continue to • Attain function and health as we know it, but at a address age appropriate needs level unknown to them • Lifelong process • Goals and rehabilitation programs will need to change over • Children don’t always understand the end goal as time as the person progresses and their priorities change they have not completed the task before • Frequent updating prevents boredom, increases motivation, • Don’t have prior learning and skills to fall back on adapts to functional changes • Parents • Goals always changing • How do you prioritize the parents • Easy for them to fall behind their peers goals versus the child's goals?

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Role for Occupational Therapy Treat the Things You See

Impairments Activity Limitations and • Compensatory Training • Decreased range of motion Participation Restrictions – What do they need to do now? • Decreased strength • Decreased independence with – What’s the most efficient way to do it? • Decreased coordination self-care activities • Decreased fine motor skills • Decreased independence with • Environmental Modifications and Adaptations home management tasks • Decreased sensation – How do I get them back to where they want to be? • Decreased participation in • Increased / Decreased muscle school/work/ leisure activities – How can I set them up for the most long term success? tone • Decreased community access • Rehabilitation Strategies for Recovery • Joint contracture • Increased caregiver burden – How to optimize the nervous system for return of function? • Decreased endurance – How to maintain physical integrity while we wait? • Decreased mobility • Limited static/ dynamic balance • Limited cognition • Decreased vision/ visual disturbance

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Compensatory Training Areas of Occupation

• Engagement in valued role-defining activities • Strength based, family centered, occupation based • Intervention aimed at completion of context specific approach motor tasks in meaningful •Self-Care • Training functional task rather than impairment – Sitting balance to reduce caregiver burden • Paired with feedback, repetitious, and goal directed – Rolling for in bed dressing • Modifying task, person, environment • School – Modified utensils and mobile arm support for reaching – Modified computer access • Leisure – Voice activated electronics for social participation – Learn to provide caregiver direction for preferred activities

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Environmental Modifications and Adaptations Rehabilitation Strategies for Recovery

• Adaptive equipment for self-care and school activities • Provide repeated, near-normal input to the nervous • Home modifications system to optimize capacity for recovery – Ramps – Robotics – Wheel-in shower – Electrical Stimulation • Environmental Control Units and Smart Home • Offset chronic complications and maintain physical devices, ex: Alexa integrity • Wheelchair access – Micro-light switches – Proportional joystick with alternative access (ex: chin, toe)

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Activity-Based Rehabilitation Best of Both Worlds

• Patterned activity intended to restore a task • Plan for near-term function while considering long- component, focus on impairment term impact, ex: Tenodesis • Use near normal kinematics and conditions • Addition of an activity-based approach elevates • Often high volume repetition occupation-based interventions to restorative • Aimed at recovery of motor and sensory function – Add FES to assist with grasp during self-feeding with MAS for proximal assist • Very young children have immature brain and spine – Prime nervous system with motor-assist UE cycling then use – Show better recovery elbow flexion in tooth-brushing activity – Continue recovery over many years • Intensive activity-based interventions are safe for all • May have undamaged areas of the nervous system populations masked by immaturity

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Cases of AFM at KKI

Demographics Number % Total number 31 Age Range 7 months – 16 years Sex Male 17 54.8 Female 14 45.2 Year of Diagnosis

Prior to 2014 14 45.1 PROGRAMMATIC OUTCOMES 2014 10 32.3 2016 7 22.6 Referral Source Acute Care Hospital 10 32.3 Rehabilitation Facility 39.7 Admitted from Home 18 58.0

Location of Initial Rehabilitation Services Outpatient 14 45.2 Inpatient 17 54.8

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Initial Presentation Wee-FIM Scores

Numbers Presenting Complaint Admission Discharge Fever 29 17 (5 presented with respiratory failure, 1 in each 2009, 2014, 2015 URI Self-Care 45.64 68.45 and 2 in 2016) GI 3 Mobility 40.73 63.64 26 (15 progressed to a more involved neurologic deficit); 11 Monoplegia involved a lower limb, 15 an upper limb Cognition 92.0 112.09 MRI All 31 had anterior horn involvement; Total WeeFIM® 57.0 78.0 5 of them also had polyradicular extension; in6 cases, there were Spine additional findings c/w demyelination; 5 presented cervico- medullary junction involvement

Brain 5 abnormal: 2 had small infarcts, 1 rhomboencephalitis (dorsal pons, dentate), 1 had bi-hemispheric white matter involvement EMG All showed anterior horn involvement, abnormal or unobtainable late response F waves and H reflex, and normal sensory conduction Etiology EV D-68 confirmed in 5, rhinov in 2, 1 HHV6, 1 Mycoplasma, 1 Epstein Barr (IgG+, not IgM)

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JM

2016 admit 2016 Discharge 2017 Head None Activates cervical ROM Activates cervical ROM with support Control with support at occiput at occiput (stronger) Upper No AROM Gravity eliminated Improved digit strength and emerging Extremity bilaterally in digits, no digit 1 twitches in right hand Status AROM otherwise Lower No AROM Against gravity at knee Against gravity at knee, ankle, Extremity and ankle, twitch hamstrings, and glutes (hip gravity CASE STUDY Status hamstrings and glutes eliminated) W/C Dependent Proportional control Proportional control power mobility tilt-in-space power mobility using a using a foot joystick placed on left foot joystick placed on left foot plate (community distance) foot plate Ability to NPO Trials with therapy only Cleared to eat with family, tolerates eat small trials PAMS 24 38 44

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Resources and Next Steps References

• Centers for Disease Control 1. Centers for Disease Control and Prevention. (2016). Acute flaccid myelitis: interim considerations for clinical management. 2. Messacar, K., Schreiner, T. L., Van Haren, K., Yang, M., Glaser, C. A., Tyler, K. L., & Dominguez, S. R. (2016). Acute Flaccid Myelitis: • Webinar on www.occupationaltherapy.com in Spring A Clinical Review of US Cases 2012‐2015. Annals of . • www.spinalcordrecovery.org 3. Case Definitions. (2016, January 22). Retrieved November 15, 2016, from http://www.cdc.gov/acute-flaccid-myelitis/hcp/case- definition.html • www.kennedykrieger.org/abrttraining 4. AFM in the United States. (2016, December 01). Retrieved December 02, 2016, from • Refer to the Children and Youth Evidence Based http://www.cdc.gov/acute-flaccid-myelitis/afm-surveillance.html 5. Greninger, A. L., Naccache, S. N., Messacar, K., Clayton, A., Yu, G., Somasekar, S., ... & Messenger, S. (2015). A novel outbreak Resourceshttp://www.aota.org/Practice/Children-Youth/Evidence-based.aspx enterovirus D68 strain associated with acute flaccid myelitis cases in the USA (2012–14): a retrospective cohort study. The Lancet • Start a conversation on AOTA’s forum Infectious Diseases, 15(6), 671-682. 6. Aliabadi, N., Messacar, K., Pastula, D. M., Robinson, C. C., Leshem, E., Sejvar, J. J., ... & Dominguez, S. R. (2016). Enterovirus D68 CommunOT https://communot.aota.org/home infection in children with acute flaccid myelitis, Colorado, USA, 2014. Emerging Infectious Diseases, 22(8), 1387. • See the AOTA Children and Youth practice resources including FAQs, fact 7. Van Haren, K., Ayscue, P., Waubant, E., Clayton, A., Sheriff, H., Yagi, S., ... & Wadford, D. A. (2015). Acute flaccid myelitis of unknown etiology in California, 2012-2015. JAMA, 314(24), 2663-2671. sheets, occupation toolkit and more http://www.aota.org/Practice/Children- 8. Maloney, J. A., Mirsky, D. M., Messacar, K., Dominguez, S. R., Schreiner, T., & Stence, N. V. (2015). MRI findings in children with Youth.aspx acute and cranial nerve dysfunction occurring during the 2014 enterovirus D68 outbreak. American Journal of Neuroradiology, 36(2), 245-250. • Get involved in the Children and Youth Special Interest Section 9. Sejvar JJ, Lopez AS, Cortese MS, Leshem E, Pastula DM, Miller L, Glaser C, Kambhampati A, Shioda K, Aliabadi N. http://www.aota.org/Practice/Manage/SIS.aspx Acute Flaccid Myelitis in the United States, August–December 2014: Results of Nationwide Surveillance Clinical Infectious Diseases, 2016, 63(6), 737–745. • Join an AOTA pediatric Community of 10. Rybczynski S, Dean J, Melicosta M. Pediatric Spinal Cord Injury Due to Acute Flaccid Myelitis: Epidemiology, Clinical Practice http://www.aota.org/Practice/Children-Youth/PediatricWorkGroups.aspx Management, and Implications for Rehabilitation. Curr Phys Med Rehabil Report (2017), 5:113-120. • Share the link to this recording and discuss with your colleagues 11. Gordon-Likin E, Munoz L, Klein JL, Dean J, Izbudak I, Pardo C. Comparative quantitative clinica, neuroimaging, and functional profiles in children with acute flaccid myelitis at acute and convalescent stages of disease. Dev Med and Child http://www.aota.org/Practice/Children-Youth/Early-Intervention/Resources.aspx Neuro. (2018), published online.

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