EVIDENCE BASED PAEDIATRIC REHABILITATION

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EVIDENCE BASED PAEDIATRIC REHABILITATION

• Dr Remo N Russo
• Director Paediatric Rehabilitation
• Womens and Childrens Hospital
• North Adelaide South Australia, AUSTRALIA

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OVERVIEW

• Summary of Rehabilitation
• Review of evidence for
• Walking ability in Cerebral Palsy
• New technologies in Cerebral Palsy
• Severity of Injury and Therapy in Acquired Brain
  Injury
• Discussion
• Steroids and Orthoses for Duchenne MD
• Dose of therapy in Rehabilitation
• Medical conditions specific to the rehabilitation
  patient
• Other issues

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PHYSICAL FUNCTIONING
REHABILITATION FUNCTION
COGNITIVE FUNCTIONING
TASK IN REHABILITATION
Daverat et. al. Paraplegia 1995 Neurological Exam
(impairment) correlated with disability but NOT
handicap
Identify requirements for independence Identify
what can be provided by the family Support the
difference (i.e.. Improve function) -
Multidisciplinary
DOMAINS OF FUNCTION Disease organ level
function Impairment system level Disability
personal level Handicap societal level
Picture of mother holding child with quadriplegia
on hip.
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Rehabilitation

• Disorders
• Cerebral palsy
• Acquired brain injury
• Spinal cord injury
• Spina bifida
• Neuromuscular
• Limb deficiency
• Other neurological

• Problems
• Mobility
• Seating
• Weight bearing
• Postural alignment
• Spasticity management
• Orthotic prescription
• Prosthetic prescription

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Rehabilitation Evidence Based Practice

• More Recently
• Increasing evidence
• Increasing numbers of Randomised Trials
• Clinical practice adopting evidence base
• Evidence Based Practice

• Historically
• Poor evidence base
• Few Randomised Trials
• Clinical Practice reflected training and other
  biases
• Clinical Principles

Picture of child in wheelchair Pressure sore on
foot due to Poor positioning
Hyperbaric O2 Therapy Hardy D.Med.Child N. 2002
Picture of father child in HBO chamber
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Walking Ability in Cerebral Palsy - 1

• Will my child walk?
• Major focus for physically disabling conditions
• Clinical Principles
• Postural alignment
• Muscle power
• Balance
• Developmental progression

Severe head lag 9 months
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Walking Ability in Cerebral Palsy - 2

• Prospective cohort studies - predictors of
  ambulatory ability
• Topography DiplegiaQuad.
• hand weight bearing and ability to roll by 18
  months predictive
• Head balance by 9 months
• Sitting by 1.5 - 2 years
• No clinical tools developed from this body of
  work

Picture of child in equinus
Fedrizzi DMCN 2000 31 Children Sp
diplegia Watt DMCN 1989 74 Children with
CP Campos da Paz DMCN 1994 272 CP Children
Badell-Ribera APMR 1995 50 children - CP
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Walking in Cerebral Palsy - 3
GMFCS Palisano DMCN 1997 Level 5 at 2 years
90 probability wheelchair mobile Stability
little change in level other than for slight
deterioration levels 3 4
GMFCS Diagram ages 6-12
Gross Motor Function Classification System - GMFCS
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GMFCS a Predictive Measure
See Rosenbaum BMJ 2003 326970-974 GMFCS curves
showing predicted abilities over time as
correlated with the GMFM 66
Hip subluxation GMFCS level 4
Predictive also of hip subluxation in children
with CP Levels 12 rare
Levels 45 common Levels 3-5
urveillance
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NEW TECHNOLOGIES IN REHABILITATION Spasticity
Management

• Physical Modalities
• Oral medications
• Botulinum toxin
• Orthopaedic surgery
• Intrathecal baclofen
• Dorsal Rhizotomy
• Others

Hemiplegic Cerebral Palsy Pictures showing
impact of spasticity on bimanual function
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Spasticity Management
Valium
BRAIN
Baclofen Oral Intrathecal
SPINAL CORD
Botulinum toxin A
MUSCLE TENDON
Selective Dorsal Rhizotomy
Orthopaedic Surgery
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SPASTICITY MANAGEMENT

GENERAL
Physiotherapy
SDR
Oral therapy
ITB
REVERSIBLE
PERMANENT
Orthoses
Surgery
BTX-A
Physiotherapy
FOCAL
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NEW TECHNOLOGIES IN REHABILITATION BOTULINUM
TOXIN A

• Protein product of Cl. botulinum
• Chemical block of Ach release from nerve
  terminal
• Effects start 1-3 days
• Peak at 2-6 weeks
• Highly variable
• Age, degree of spasticity, therapy, other

Mode of action botulinum toxin A
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Delivery of toxin to target muscle

• Surface anatomy
• Muscle stimulation
• Toxin diffuses
• Can target multiple muscles but limited in
  overall dose

Injection of thumb adductor
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Intrathecal Baclofen - ITB

• Severe spasticity in children with cerebral palsy
• GMFCS level 4 5
• Test dose then implantation

Pictures of ITB pump and kit radiology
troubleshoot
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EVIDENCE BASED PRACTICE IN CP MANAGEMENT - 1
Boyd 2001 Eur J of Neurology
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EVIDENCE BASED PRACTICE IN CP MANAGEMENT - 2
BoNT-A Ade-Hall 2000 (Lower Limb) Wasiak 2004
(Upper Limb) Cochrane Database
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Acquired Brain Injury

• 70/100,000 children 0-17 years olds (Schneier
  2006 Pediatrics)
• Australian Bureau of Statistics
• South Australia 1.6 million (New Zealand 4
  million Christchurch 350,000)
• South Australia has 400,000 children (100,000)
• Approx. 280-300 ABI in South Australia per year
  ( 70 ABI per year)
• WCH (1999-2004) 30-50 per year mod-severe ABI
  needing ICU care
• 2-5 with very severe ABI

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Evidence for Severity of Injury Outcome in
Pediatric ABI

• No randomised trials
• No Cochrane Reviews
• Nearly all trials low numbers (prospective cohort studies with few having
  controls level C evidence
• References cited for interest

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Severity of ABI Initial Trauma

• Guidelines B grade Mansfield 2007 Clin Ped
  Emerg Med
• Treatment of hypotension and hypoxia
• Treatment in a paediatric trauma centre
• Maintain brain blood-flow
• Rehabilitation begins at this stage of recovery,
  once it is felt the child is likely to survive

• PRIMARY
• Focal
• Diffuse
• Secondary
• Cerebral oedema

Child with ABI on ventilator
CT scan contusion and oedema Diffuse axonal
injury
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Severity of Injury

• Post-traumatic amnesia (PTA) Jennett et al 1976
  Ponsford 2004 Brain Injury
• Severe injury PTA 7 days

• Glasgow Coma Scale (Teasdale 1974 Lancet)
• Paediatric Simpson-Reilly scale modified
  motor and verbal response
• Mild GCS 13-15
• Moderate GCS 9-12
• Severe GCS 8 or less
• GCS disability (Koskiniemi 1995 Arch Ped Adol Med )

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Memory and the Brain
Frontal lobe
Temporal lobe
FRONTAL LOBE
Hippocampus
TEMPORAL LOBE
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Several Caveats

• Depth of lesion on MRI can predict severity
  (Grados 2001)
• Mild ABI ? good outcome (Keenan 2006 Dev Neurosci
  Yeates 1999 J Head Trauma Rehabil)
• Injury to the developing brain affects brain
  maturation, myelination, and neuronal development
• Younger age worse prognosis (Michaud 1992
  Neurosurgery Anderson 2000 Pediatr Neurosurg)
• Plasticity theory not validated for diffuse
  injury (Anderson 2005 Pediatrics)
• Difficulties can emerge over time
• Frontal lobe pathology Growing into the
  disability
• (Ewing-Cobbs 2004 Dev Neuropsychology)

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Recovery from ABI

• Generally good physical prognosis Haley et. al.
  2003
• The Hidden Disability

• Prognosis for cognitive and behavioural issues
  generally poor Miller et al 2004
• Worse injury predicts worse outcome
• Cognition
• Physical
• Family
• Emotional/behavioural
• Quality of life
• Public health burden

Picture of lower limbs of child with severe ABI
receiving intensive therapy
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Rehabilitation and the effect on outcomes in
Acquired Brain Injury

• Intensive and coordinated physical rehabilitation
  program gaining evidence of efficacy
• Cognitive and behavioural treatments evidence
  lacking with virtually no class 1 studies
• Practice Guidelines (Laatsch 2007 J Head Trauma
  Rehabil)
• Comprehensive ABI treatment program involving
  family members as active participants
• Memory training in children effective

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Injury Prevention
Has come a long way in 40 years
1960s
Picture of slide design impr- ovements.
All I want for Christmas is A baby
capsule!
Speed limits
2007
Equipment design
Picture of unrestrained infant
Lady Di tragedy lost message for use of
seatbelts
Child restraints
Seatbelts
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Conclusion

• Evidence base for rehabilitation has been lacking
• More recently there has been a growing evidence
  base
• There is a lack of high quality evidence for many
  of the interventions we undertake but this is
  changing
• CP management ABI management

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Questions?

• I wish I had an answer to that because I'm tired
  of answering that question!
• Yogi Berra