Cerebral Palsy One Size Does Not Fit All
Cerebral Palsy One Size Does Not Fit All Mary Catherine Brake Turner, MD, FACP, FAAP brakem@ecu. edu
Objectives � Define � List cerebral palsy systems often affected by cerebral palsy � List three non-surgical treatments for spasticity � Name palsy � List common causes of pain in cerebral three main roles of the primary care provider
Agenda � 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
Definition of CP �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
Assessment for Intervention � Diagnosis � Classify is suspected by PCP based on localization and type � Assessment � Overall of associated impairments severity
Type of Motor Disorder � Spasticity � Dyskinesia � Ataxia � Hypotonia (dystonia and choreoathetosis)
Localization � Diplegia: Lower extremities >> upper extremities � Quadriplegia: Upper and lower extremities are affected equally � Hemiplegia: 1 side more involved than its opposite counterpart
Functional Motor Abilities � Gross � Fine motor – ambulation motor – self-help skills � Oromotor and speech – communication, eating and drinking
Gross Motor Function Classification System for Cerebral Palsy (GMFCS) � 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
Risk Factors for Development of CP � Chorioamnionitis � Birth weight <2000 gm � Intracranial � Newborn hemorrhage encephalopathy � Periventricular leukomalacia � Hydrocephalus � Congenital malformations
Relevance to Us � 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
Case � 30 yo. M, 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.
What nutritional issues may arise in patients with cerebral palsy? A. Malnutrition B. Obesity C. Vitamin D deficiency D. Gastro-esophageal reflux E. All of the above
Growth/Nutrition � Affected by dysphagia, GERD, delayed gastric motility, constipation � May have to rely on gastrostomy or jejunostomy tubes � +/- fundoplication
Growth Charts for CP � Special growth charts are available for CP ◦ Limitation is charts are not standards for ALL pts � Recommend up � Objective WHO birth - 2 yrs and CDC 2 yrs of plotting is to monitor trends ◦ Z-scores: variation from the reference and from each child’s own growth pattern
Growth/Nutrition � 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
Nutrition � WHO (basal needs: BMR) [W = weight (kg)] Age (yrs) 0 -3 3 -10 10 -18 Gevena, 1985 Gender Male Female Equation 60. 9 W-54 61 W-51 22. 7 W+495 22. 5 W+499 17. 5 W+651 12. 2 W+746
Height based method � 14. 7 cal/cm in children without motor dysfunction � 13. 9 cal/cm in ambulatory patients with motor dysfunction � 11. 1 � Use cal/cm in non-ambulatory patients arm span to estimate height
Growth/Nutrition � Micronutrients � If formula is <1 L/day for adolescents/adults, will need to add MVI � Consider monitoring vitamin D status
What nutritional issues may arise in patients with cerebral palsy? A. Malnutrition B. Obesity C. Vitamin D deficiency D. Gastro-esophageal reflux E. All of the above
What nutritional issues may arise in patients with cerebral palsy? A. Malnutrition B. Obesity C. Vitamin D deficiency D. Gastro-esophageal reflux E. All of the above
Nutrition Explanation � 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
Drooling � 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
Swallowing � 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
Case � 3 yo. F 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.
What diagnostic procedure will likely help determine cause of her respiratory distress? A. Video Swallow study B. CT scan of the chest C. Sputum for AFB D. Gastric emptying study
Respiratory ◦ Aspiration (primary or secondary) ◦ Upper airway obstruction ◦ Infections (poor pulmonary clearance) ◦ Restrictive lung disease (scoliosis)
Respiratory � 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
What diagnostic procedure will likely help determine cause of her respiratory distress? A. Video Swallow study B. CT scan of the chest C. Sputum for AFB D. Gastric emptying study
What diagnostic procedure will likely help determine cause of her infection? A. Video Swallow study B. CT scan of the chest C. Sputum for AFB D. Gastric emptying study
Case � 5 yo. M 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.
What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance? 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
GI � Reflux ◦ Positioning upright ◦ H 2 or PPI therapy ◦ Fundoplication � Constipation ◦ Hydration and fiber ◦ Scheduled miralax ◦ Suppositories
GI � Delayed ◦ ◦ gastric motility Slow rate of feeds EES Reglan Pyloroplasty
What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance? 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
What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance? 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
When considering treatment for spasticity, which of the following is not considered a treatment goal? 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
Spasticity � PT, ROM exercises ◦ Enhance skill development, delay contractures ◦ Time required to perform � Orthotics ◦ To improve function, prevent contractures ◦ Possibility of pressure sores or muscle wasting � Systemic medications ◦ Diazepam, baclofen, tizanidine, dantrolene ◦ Decrease pain and muscle spasms ◦ Sedation is adverse side effect
Spasticity � 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
Hip Dysplasia � Pain arising from the hip � Clinically important leg length difference � Deterioration � Increasing hip muscle tone � Deterioration � Increasing in ROM of hip in sitting or standing difficulty with perineal care or hygiene
MSK issues requiring Orthopedics � Contractures ◦ Tendon clipping � Hip dislocation ◦ Surgical stabilization � Scoliosis ◦ Surgical repair
When considering treatment for spasticity, which of the following is not considered a treatment goal? 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
When considering treatment for spasticity, which of the following is not considered a treatment goal? 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
The most common cause of pain in patients with CP is: A. Constipation B. Reflux C. Extremity fracture D. Hip dysplasia E. Muscle spasm
Pain –Evidence Based Medicine � Pain in children with CP is under-recognized and thus undertreated � Affects quality of life � Challenges include difficulty communicating and multiple etiologies of pain
Characteristics 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.
Characteristics of Pain � 92% response rate � 55% reported some pain on the HUI 3, with 24% reporting that their child experienced pain that affected some level of activity � Physicians � Identified reported pain in 39% hip dislocation/subluxation (27%), dystonia (17%), and constipation (15%) as the most frequent causes of pain.
The most common cause of pain in patients with CP is: A. Constipation B. Reflux C. Extremity fracture D. Hip dysplasia E. Muscle spasm
The most common cause of pain in patients with CP is: A. Constipation B. Reflux C. Extremity fracture D. Hip dysplasia E. Muscle spasm
Revisit role of PCP � 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
Transition � 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
References � � 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: e 1321 – 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. )
References Samour PQ, King K. Handbook of Pediatric Nutrition. 3 rd 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: e 407413. � Shaw, TM, De. Laet 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 �
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