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

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

3. 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

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

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

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

7. 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

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

9. SUS 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

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

11. 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)

12. 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

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

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

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

16. 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

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

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

19. 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

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

21. 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.

22. 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!

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

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

25. 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

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

27. SUS Additional work-up EEG Metabolic evaluation Titers for infectious etiology TFTs Consultation with subspecialists –neurology, genetics, metabolism

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