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Care of the Complex Childby

Emily DavidsonLaurie GladerThomas Silva

reviewed byRonald SamuelsWanessa Risko

Ellen Elias

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Who are the children?

Children with:• Physical conditions• Sensory deficits• Cognitive concerns• Emotional disorders

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How many children?

• 31% of children have one or more chronic physical condition at some point

• 20% have developmental delays, learning difficulties, and/or emotional or behavioral problems

• 6% have a severe chronic condition• 0.2% are assisted by technology

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Family issues

• Lack of services• Multiple providers• Multiple agencies• Complex coordination nightmares• Fatigue/stress• Lack of privacy

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What are common special health care needs?

• CP• MR• Spina Bifida• Down Syndrome• Technologically Dependant

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Cerebral palsy defined

Cerebral palsy is a disorder of movement and tone due to a non-progressive insult which occurred in the immature brain.

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Vital Statistics

• 2-3/1,000 live births• Prevalence: 100,000 patients

less than 18 years old in the US• Cost: $5 billion annually• Survival: 87% reach age 30

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Clinical subtypes

• Spastic cerebral palsy– diplegic– hemiplegic– quadriplegic

• Dyskinetic cerebral palsy• Ataxic cerebral palsy

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Spastic diplegia• 40% of all CP• 80% of ex-premature infants with CP• 10% of infants <1500g• Rare in term infants• Periventricular hemorrhagic infarction

and periventricular leukomalacia • Course evolves: early hypotonia

followed by fluctuations in tone and finally spasticity

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Hemiplegia

• 20% of all CP• 90% secondary to vascular issues– vaso-occlusive stroke (term)– periventricular venous infarction (pre-term)

• 10% secondary to malformations

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Spastic quadriplegia

• 5% of all CP• Most severe form with worst

prognosis• 50% occurs in low birthweight

infants• 45% cerebral dysgenesis• 5% destructive lesions (cystic

encephalomalacia)

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Dyskinetic cerebral palsy• 15-20% of all CP• Etiology secondary to hypoxic

ischemic encephalopathy, historically hyperbilirubinemia

• Initially hypotonic; delayed onset of choreoathetosis or dyskinesia

• Prognosis better for cognition, risk of seizures; oromotor issues significant

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Ataxic cerebral palsy

• 15% of all CP• Usually syndromic– Dandy-Walker– X-linked congenital ataxia– Vermal dysplasia

• Initially hypotonic

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Treatment goals

• Prevention• Limiting disability and

improving function• Managing associated medical

issues• Managing complications

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Orthopedic issues

• Manifestations: spasticity, dystonia, weakness and osteopenia

• Complications– contractures– hip subluxation– scoliosis– fractures– pain– impaired hygiene

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Physiologic effects

• Orthopedic - 25% non-ambulatory• Cognitive deficits - 30% mentally

retarded• Seizure disorders -30%• Visual impairment - 25-60%• Auditory impairment - 8-22%• Growth failure and GI disorders• Chronic lung disease• Oromotor impairment

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Treatments for spasticity

• Physical therapy• Medical agents• Neuromuscular injections• Therapeutic electrical

stimulation• Orthopedic or neurosurgical

procedures

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Spasticity treatment:medical options

• Benzodiazepines• Baclofen– enteral versus pump

• Dantrolene

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Treatments for spasticity:neuromuscular injections

• Botulinum A Toxin – inhibits acetylcholine release at the NMJ– onset < 1 wk; duration up to 6 mos

• Phenol neurolysis– causes demyelination– lasts 3-18 months– useful on larger muscle groups– side effects include muscle necrosis, pain

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Spasticity treatment:orthopedic and neurosurgery

• Tenotomies• Osteotomies• Selective dorsal rhizotomy• Baclofen pump

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Cerebral palsy: the work-up

ALWAYS LOOK FOR AN EXPLANATION

• MRI• ABR/hearing assessment• Ophthalmologic evaluation• As indicated: EEG, chromosomes, metabolic

evaluation, TORCH titers, etc.

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Useful goals• Closely monitor physical health– Use subspecialists– At absolute minimum a physical therapist

and orthopedist will be involved

• Promote independence into adulthood

• Assist family with community resources

• Coordinate care!

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Mental retardation: definition

• Cognitive functioning significantly below average • Onset within the developmental

period• Deficits in adaptive behavior

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Subclassification based oncognitive deficit

• Mild retardation: 70-50• Moderate retardation: 50-35• Severe retardation: 35-20• Profound retardation: <20

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Commonly identified etiologies of retardation

• Prenatal factors– chromosomal abnormalities, toxin

exposure , infection (toxo, CMV, rubella, syphilis)

• Perinatal conditions– infection (HSV, GBS), asphyxia, LBW

• Postnatal causes– infectious meningitis (H. flu, strep,

Neisseria), injury, toxin exposure

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The work-up

• Hx/PEX• MRI for moderate to profound

range retardation• Chromosomal analysis• Hearing/vision assessments• Developmental assessment

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Additional work-up

• EEG• Metabolic evaluation• Titers for infectious etiology• TFT’s• Consultation with subspecialists– neurology, genetics, metabolism

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Treatment

• Highly individualized• Follow/treat associated medical

conditions• Emphasis on therapeutic and

educational services