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One Size Does Not Fit All Mary Catherine Brake Turner, MD, FACP, FAAP [email protected] Define cerebral palsy List systems often affected by cerebral palsy List three non-surgical treatments for spasticity Name common causes of pain in cerebral palsy List three main roles of the primary care provider Review cerebral palsy and the complexities that accompany this diagnosis Highlight special considerations for patients with cerebral palsy Review the role of the medical home Discuss important transition issues as patients with cerebral palsy become adults A group of permanent disorders of movement and posture that limit activity Non-progressive Insult to the developing brain Disturbances of sensation, perception, cognition, communication, and behavior Epilepsy and secondary MSK problems common Diagnosis is suspected by PCP Classify based on localization and type Assessment of associated impairments Overall severity Spasticity Dyskinesia (dystonia and choreoathetosis) Ataxia Hypotonia Diplegia: Lower extremities >> upper extremities Quadriplegia: Upper and lower extremities are affected equally Hemiplegia: 1 side more involved than its opposite counterpart Gross motor – ambulation Fine motor – self-help skills Oromotor and speech – communication, eating and drinking Level I – Speed, balance and coordination are limited Level II- Minimal ability to perform gross motor skills such as running and jumping Level III – May ambulate with assistive devices Level IV – Children may achieve self-mobility using a power wheelchair Level V – All areas of motor function are limited, no means of independent mobility Chorioamnionitis Birth weight <2000 gm Intracranial hemorrhage Newborn encephalopathy Periventricular leukomalacia Hydrocephalus Congenital malformations All PCPs will encounter children with cerebral palsy in their practice Prevalence of 3.6 per 1000 More than 100,000 children in the US are affected More than 90% of children with severe disabilities survive to adulthood We will see them for health maintenance, care coordination, and acute visits 30 yoM, former 26 week preemie, with CP, GMFCS Level V, mental retardation, seizure disorder, VP shunt, feed formula by a bottle His PCP is a pediatrician, they live 1 hour away This pediatrician has referred the patient to see me due to weight loss. A. Malnutrition B. Obesity C. Vitamin D deficiency D. Gastro-esophageal reflux E. All of the above Affected by dysphagia, GERD, delayed gastric motility, constipation May have to rely on gastrostomy or jejunostomy tubes +/- fundoplication Special growth charts are available for CP ◦ Limitation is charts are not standards for ALL pts Recommend WHO birth - 2 yrs and CDC 2 yrs up Objective of plotting is to monitor trends ◦ Z-scores: variation from the reference and from each child’s own growth pattern Protein (grams/kg) ◦ Based on actual weight, DRI Hydration ◦ Obviously essential, helps reduce constipation ◦ Holliday-Segar method: 100, 50, 20; based on wt Calories ◦ Calculated per the BMR WHO (basal needs: BMR) [W = weight (kg)] Age (yrs) 0-3 3-10 10-18 Gevena, 1985 Gender Male Female Male Female Male Female Equation 60.9W-54 61W-51 22.7W+495 22.5W+499 17.5W+651 12.2W+746 14.7 cal/cm in children without motor dysfunction 13.9 cal/cm in ambulatory patients with motor dysfunction 11.1 cal/cm in non-ambulatory patients Use arm span to estimate height Micronutrients If formula is <1L/day for adolescents/adults, will need to add MVI Consider monitoring vitamin D status A. Malnutrition B. Obesity C. Vitamin D deficiency D. Gastro-esophageal reflux E. All of the above A. Malnutrition B. Obesity C. Vitamin D deficiency D. Gastro-esophageal reflux E. All of the above Malnutrition due to decreased ability to take in adequate calories Obesity can also be an issue due to poor mobility and overfeeding via gastric tube. Poor exposure to sunlight GERD common in CP Treatment options include: ◦ Decorative scarves and bibs ◦ Glycopyrrolate – risk for mucous plugs ◦ Atropine Drops – local effect ◦ Scopolamine patch ◦ Botulinum toxin injections – expensive procedure ◦ Removal of salivary glands – permanent, not recommended Children with CP often struggle with oral and/or pharyngeal dysphagia Diagnose formally with a swallow study with radiology and speech pathology Treatment may include use of Thick-It or oatmeal thickener, or reliance solely on gastrostomy tube 3 yoF with spastic quadriplegic CP is admitted with fever and increased WOB, no increased seizures, tolerating feeds well by g-tube, her mother has been feeding her stage III foods by mouth, she has history of a Nissen fundoplication. A. Video Swallow study B. CT scan of the chest C. Sputum for AFB D. Gastric emptying study ◦ Aspiration (primary or secondary) ◦ Upper airway obstruction ◦ Infections (poor pulmonary clearance) ◦ Restrictive lung disease (scoliosis) Pulmonary clearance techniques may include chest percussion, cough assist, VEST therapy all with the use of bronchodilator therapy May develop OSA or central sleep apnea Over time may progress to need for trach and vent if severe chronic lung disease A. Video Swallow study B. CT scan of the chest C. Sputum for AFB D. Gastric emptying study A. Video Swallow study B. CT scan of the chest C. Sputum for AFB D. Gastric emptying study 5 yoM with history of failure to thrive, had gtube placed one year ago, no fundoplication, no PPI therapy, minimal weight gain since then, transferred to Vidant Medical Center from a regional hospital for intolerance of bolus G-tube feeds and intermittent coffee ground emesis. MGM reports he has intermittent emesis for past year. A. Dental evaluation B. Reflux and gastric emptying study C. Plain abdominal films D. Plot him on the CP growth chart, determine he is still on the curve, reassure parents E. All of the above F. None of the above G. B and C Reflux ◦ Positioning upright ◦ H2 or PPI therapy ◦ Fundoplication Constipation ◦ Hydration and fiber ◦ Scheduled miralax ◦ Suppositories Delayed gastric motility ◦ ◦ ◦ ◦ Slow rate of feeds EES Reglan Pyloroplasty A. Dental evaluation B. Reflux and gastric emptying study C. Plain abdominal films D. Plot him on the CP growth chart, determine he is still on the curve, reassure parents E. All of the above F. None of the above G. B and C A. Dental evaluation B. Reflux and gastric emptying study C. Plain abdominal films D. Plot him on the CP growth chart, determine he is still on the curve, reassure parents E. All of the above F. None of the above G. B and C A. Reduce muscle spasms B. Improve functional ability C. Reduce pain D. Improve hygiene E. Prevent tissue injury F. Prevent hip migration G. Improve cognitive functioning Modified Ashworth Scale. Blackburn M et al. PHYS THER 2002;82:25-34 Physical Therapy PT, ROM exercises Orthotics Systemic medications ◦ Enhance skill development, delay contractures ◦ Time required to perform ◦ To improve function, prevent contractures ◦ Possibility of pressure sores or muscle wasting ◦ Diazepam, baclofen, tizanidine, dantrolene ◦ Decrease pain and muscle spasms ◦ Sedation is adverse side effect Botulinum toxin ◦ Improve pain, improve function, help with hygiene ◦ 2-3 primary muscle groups ◦ Wanes after 3 months Intrathecal baclofen pump ◦ No central effect of sedation ◦ Device complication Dorsal Rhizotomy ◦ Permanent ◦ Improves ambulation for spastic diplegics Pain arising from the hip Clinically important leg length difference Deterioration in ROM of hip Increasing hip muscle tone Deterioration in sitting or standing Increasing difficulty with perineal care or hygiene Contractures ◦ Tendon clipping Hip dislocation ◦ Surgical stabilization Scoliosis ◦ Surgical repair A. Reduce muscle spasms B. Improve functional ability C. Reduce pain D. Improve hygiene E. Prevent tissue injury F. Prevent hip migration G. Improve cognitive functioning A. Reduce muscle spasms B. Improve functional ability C. Reduce pain D. Improve hygiene E. Prevent tissue injury F. Prevent hip migration G. Improve cognitive functioning A. Constipation B. Reflux C. Extremity fracture D. Hip dysplasia E. Muscle spasm Pain in children with CP is under-recognized and thus undertreated Affects quality of life Challenges include difficulty communicating and multiple etiologies of pain Cross-sectional study looked at 252 patients with CP ages 3-19 Questionnaire, including Health Utilities Index 3 pain subset, completed by primary caregiver Treating physician was asked to identify the presence of pain and provide a clinical diagnosis if applicable. 92% response rate 55% reported some pain on the HUI3, with 24% reporting that their child experienced pain that affected some level of activity Physicians reported pain in 39% Identified hip dislocation/subluxation (27%), dystonia (17%), and constipation (15%) as the most frequent causes of pain. A. Constipation B. Reflux C. Extremity fracture D. Hip dysplasia E. Muscle spasm A. Constipation B. Reflux C. Extremity fracture D. Hip dysplasia E. Muscle spasm Provide primary care – preventative and acute Chronic care Care coordination ◦ Subspecialists ◦ Home nursing Sign care plan ◦ Order supplies ICD code 343.9 ◦ Social work, can help with community resources ◦ School Help to identify adult primary care and specialists School through age 21 with IEP The ARC - http://www.thearc.org/ Vocational rehabilitation Discuss sexuality Advance directives Palliative care Alternative care givers Insurance Equipment American Academy of Pediatrics, American Academy of Family Physicians, American College of Physicians-Society of Internal Medicine. A consensus statement on health care transitions for young adults with special health care needs. Pediatrics. 2002; 110:1304-1306. Etz, CL, Telfair J. (2007) Health Care Transitions: An Introduction. CL Betz, WM Nehring (Eds.),. Promoting Health Care Transitions for Adolescents with Special Health Care Needs and Disabilities (pp. 1-16). Baltimore: Paul H. Brooks Publishing Co. Fehlings D, Switzer L. Informing evidence-based clinical practice guidelines for children with cerebral palsy at risk of osteoporosis: a systemic review. Developmental Medicine and Child Neurology. 2012, 54: 106-116. Liptak GS, Murphy NA. Clinical Report: Providing a primary Care Medical Home for Children and Youth With Cerebral Palsy. Pediatrics. 2011, 128: e1321 – 1329. National Collaborating Centre for Women's and Children's Health (UK). Spasticity in Children and Young People with Non-Progressive Brain Disorders: Management of Spasticity and Co-Existing Motor Disorders and Their Early Musculoskeletal Complications. London: RCOG Press; 2012 Jul. (NICE Clinical Guidelines, No. 145.) Samour PQ, King K. Handbook of Pediatric Nutrition. 3rd ed. Sudbury, MA. Jones and Bartlett Publishers, Inc. 2005. V Marchand; Canadian Paediatric Society Nutrition and Gastroenterology Committee. Paediatr Child Health 2009;14(6):395-401 Poster: Aug 1 2009 Reaffirmed: Feb 1 2014. Mehta et al.; Defining Pediatric Malnutrition: A Paradigm Shift Toward Etiology-Related Definitions; J Parenter Enteral Nutrition, published online 25 March 2013. Penner M, Xie WY. Characteristics of Pain in Children and Youth With Cerebral Palsy. Pediatrics. 2013, 132: e407413. Shaw, TM, DeLaet DE. Transition of Adolescents to Young Adulthood for Vulnerable Populations. Pediatrics in Review. 2010;31;497-505. Slide from Blackburn M et al. PHYS THER 2002;82:25-34
