Care of the Complex Child by Emily Davidson

Care of the Complex Child by Emily Davidson Laurie Glader Thomas Silva reviewed by Ronald Samuels Wanessa Risko Ellen Elias SUS

Who are the children? Children with: • Physical conditions • Sensory deficits • Cognitive concerns • Emotional disorders SUS

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 SUS

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

What are common special health care needs? • • • CP MR Spina Bifida Down Syndrome Technologically Dependant SUS

Cerebral palsy defined Cerebral palsy is a disorder of movement and tone due to a nonprogressive insult which occurred in the immature brain. SUS

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 SUS

Clinical subtypes • Spastic cerebral palsy – diplegic – hemiplegic – quadriplegic • Dyskinetic cerebral palsy • Ataxic cerebral palsy SUS

Spastic diplegia • • • 40% of all CP 80% of ex-premature infants with CP 10% of infants <1500 g Rare in term infants Periventricular hemorrhagic infarction and periventricular leukomalacia • Course evolves: early hypotonia followed by fluctuations in tone and finally spasticity SUS

Hemiplegia • 20% of all CP • 90% secondary to vascular issues – vaso-occlusive stroke (term) – periventricular venous infarction (pre-term) • 10% secondary to malformations SUS

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) SUS

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 SUS

Ataxic cerebral palsy • 15% of all CP • Usually syndromic – Dandy-Walker – X-linked congenital ataxia – Vermal dysplasia • Initially hypotonic SUS

Treatment goals • Prevention • Limiting disability and improving function • Managing associated medical issues • Managing complications SUS

Orthopedic issues • Manifestations: spasticity, dystonia, weakness and osteopenia • Complications – – – contractures hip subluxation scoliosis fractures pain impaired hygiene SUS

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 SUS

Treatments for spasticity • • Physical therapy Medical agents Neuromuscular injections Therapeutic electrical stimulation • Orthopedic or neurosurgical procedures SUS

Spasticity treatment: medical options • Benzodiazepines • Baclofen – enteral versus pump • Dantrolene SUS

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 SUS

Spasticity treatment: orthopedic and neurosurgery • • Tenotomies Osteotomies Selective dorsal rhizotomy Baclofen pump SUS

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. SUS

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! SUS

Mental retardation: definition • Cognitive functioning significantly below average • Onset within the developmental period • Deficits in adaptive behavior SUS

Subclassification based on cognitive deficit • • Mild retardation: 70 -50 Moderate retardation: 50 -35 Severe retardation: 35 -20 Profound retardation: <20 SUS

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 SUS

The work-up • Hx/PEX • MRI for moderate to profound range retardation • Chromosomal analysis • Hearing/vision assessments • Developmental assessment SUS

Additional work-up • • • EEG Metabolic evaluation Titers for infectious etiology TFT’s Consultation with subspecialists – neurology, genetics, metabolism SUS

Treatment • Highly individualized • Follow/treat associated medical conditions • Emphasis on therapeutic and educational services SUS