Acute Flaccid Myelitis (AFM)
from a critical care perspective
Demographics
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Current literature: > 50 % of AFM patients are admitted to an ICU at some point in their disease process.
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Acute mortality is rare
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ICU need is primarily for respiratory support.
Goals of care for the ICU:
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Limit complications through rigorous monitoring and supportive care
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Facilitate a rapid and accurate diagnosis
Symptomatology:
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Weakness
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Respiratory distress
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Feeding dysfunction
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Pain/parasthesias
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Neurogenic bowel/bladder
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Autonomic dysfunction
Progression of symptoms:
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Onset to nadir typically evolves in hrs to days.
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Gordon‐Lipkin, Eliza, et al. "Comparative quantitative clinical, neuroimaging, and functional profiles in children with acute flaccid myelitis at acute and convalescent stages of disease." Dev Med & Child Neuro 61.3 (2019): 366-375
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Usually no second phase of worsening in the acute period
Risk Factors for Respiratory Failure
** Currently no good data
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Weakness leading to hypoventilation
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Weakness of the neck/trunk and/or bilateral UE
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Cervical cord lesion à specific but not sensitive
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Aspiration
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Bulbar weakness
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Encephalopathy
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Apnea
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Brainstem lesion (central)
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Bulbar weakness (obstructive)
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Weakness of the neck/trunk (obstructive)
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Incidence of Respiratory Failure
While respiratory support is the most common reason for ICU admission, it is unclear what percent require invasive mechanical ventilation (reports are highly variable).
References

CDC cohort from 2014 shows high % of cervical involvement but only a fifth of patients developed respiratory failure. Thus there may be reassurance that cervical lesions are a risk factor but do not predict with certainty which patients will need ventilation.
Weakness of 4 extremities and/or CN palsies may be a stronger predictor.
Monitoring for Respiratory Dysfunction
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Hypoventilation
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consider Q4 hr assessment for 1st 48 hours
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NIF/forced vital capacity (age > 6yr, cooperation dependent)
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Counting or singing a song (pick a task the child repeat consistently to assess for changes over time)
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Pulse oximetry - note: hypoxia is a late sign or evidence of secondary process
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Apnea
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CR monitor
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+/- sleep study
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Aspiration
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Identify high risk patients
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Bulbar weakness (hypophonic voice, drooling/copious secretions, dysarthria, fatigue with talking, coughing with drinking, pocketing food)
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Encephalopathy
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Make patient NPO and obtain speech consult for swallowing eval
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Identify aspiration promptly
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Symptoms of fever, hypoxia, and tachypnea/dyspnea
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Modify PO status where applicable
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Assess for infection
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Increase respiratory support as needed
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General supportive care
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Neurological assessments
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Neuro checks
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Any interval < Q4 hours leads to diminishing returns if prolonged
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Sleep deprivation and delirium confuse the picture.
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Ask the family to video the motor exam, speech and facial movements.
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Hemodynamics
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CR monitor
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Hypertension is common
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Etiology is often multi factorial
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Assess for pain, neurogenic bladder, autonomic dysfunction
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Pain management
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Children may not report pain when asked directly.
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Irritability or poor cooperation may be indicators of poorly controlled pain
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Good pain control can normalize vital signs and improve cooperation with assessments and therapies.
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Encourage use of non-narcotic pain medications (e.g. gabapentin)
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Consult psychology and/or Child Life early to assist with anxiety and behavioral challenges
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Promote sleep and maintenance of day/night cycles
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Sedation
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Minimize sedation where possible
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Avoid medications that may exacerbate weakness (e.g. paralytics, steroids)
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Bowel/bladder dysfunction
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Gut dysmotility
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Constipation is common.
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Initiate bowel regimen early
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Neurogenic bladder
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Ask and assess for bladder retention.
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In a child who is continent, ask them to urinate then perform a bladder scan or post void residual.
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For neurogenic bladder, in and out cath preferred over continuous Foley catheterization
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Nutrition
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Avoid gaps in feeding
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Modify diet and encourage enteral feeds when appropriate/safe
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Place NG tube early if risk for aspiration
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Mobility and rehabilitation in the ICU
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Consult PT/OT and PM&R early for assessment
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Plan for mobilization
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establish when mobilization is safe
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Set activity goals
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Skin care (avoidance of pressure palsies)
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DVT prophylaxis for immobile patients (post pubertal patients highest risk)
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Infection risk
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Aggressive screening for infection & treatment where appropriate
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Central line care
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Avoidance of prolonged use of Foley catheters
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Diagnosis
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Refer to AFM diagnostic criteria – labeling is important
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Physical Exam
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LP for CSF studies
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MRI brain and spine
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Laboratory studies
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EMG – most useful at least 2 weeks after symptom onset
Outcome
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Data is incomplete
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Kane et al, 12 month follow up (27/28)
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11 % (3) with tracheostomy (2 placed acutely, 1 later) patients
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15 % (4) with G tube dependence.
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Kane, Miranda S., et al. "Incidence, risk factors and outcomes among children with acute flaccid myelitis: a population-based cohort study in a California Health Network between 2011 and 2016." The Ped infect Ds J 38.7 (2019): 667-672
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