One Size Does Not Fit All Mary Catherine Brake Turner MD FACP FAAP brakemecuedu Define cerebral palsy List systems often affected by cerebral palsy List three nonsurgical treatments for spasticity ID: 530689
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Slide1
Cerebral Palsy
One Size Does Not Fit AllMary Catherine Brake Turner, MD, FACP, FAAPbrakem@ecu.eduSlide2
Define
cerebral palsyList systems often affected by cerebral palsyList three non-surgical treatments for spasticity
Name common causes of pain in cerebral palsy List three main roles of the primary care provider
ObjectivesSlide3
Review cerebral palsy and the complexities that accompany this diagnosis
Highlight special considerations for patients with cerebral palsyReview the role of the medical homeDiscuss important transition issues as patients with cerebral palsy become adults
AgendaSlide4Slide5
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 behaviorEpilepsy and secondary MSK problems common
Definition of CPSlide6
Diagnosis is suspected by PCP
Classify based on localization and typeAssessment of associated impairmentsOverall severity
Assessment for InterventionSlide7
Spasticity
Dyskinesia (dystonia and choreoathetosis)Ataxia
HypotoniaType of Motor DisorderSlide8
Diplegia: Lower extremities >> upper extremities
Quadriplegia: Upper and lower extremities are affected equallyHemiplegia: 1 side more involved than its opposite counterpart
LocalizationSlide9
Gross motor – ambulation
Fine motor – self-help skillsOromotor and speech – communication, eating and drinking
Functional Motor AbilitiesSlide10Slide11
Level I – Speed, balance and coordination are limited
Level II- Minimal ability to perform gross motor skills such as running and jumpingLevel III – May ambulate with assistive devices
Level IV – Children may achieve self-mobility using a power wheelchairLevel V – All areas of motor function are limited, no means of independent mobility
Gross Motor Function Classification System for Cerebral Palsy (GMFCS) Slide12
Chorioamnionitis
Birth weight <2000 gmIntracranial hemorrhageNewborn encephalopathy
Periventricular leukomalaciaHydrocephalus
Congenital malformations
Risk Factors for Development of CPSlide13Slide14
All PCPs will encounter children with cerebral palsy in their practice
Prevalence of 3.6 per 1000More than 100,000 children in the US are affected
More than 90% of children with severe disabilities survive to adulthoodWe will see them for health maintenance, care coordination, and acute visits
Relevance to UsSlide15
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 awayThis pediatrician has referred the patient to see me due to weight loss.
CaseSlide16
Malnutrition
Obesity
Vitamin D deficiencyGastro-esophageal reflux
All of the above
What nutritional issues may arise in patients with cerebral palsy?Slide17
Affected by
dysphagia, GERD, delayed gastric motility, constipationMay have to rely on gastrostomy or jejunostomy tubes
+/- fundoplicationGrowth/NutritionSlide18
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
Growth Charts for CPSlide19
Protein (grams/kg)
Based on actual weight, DRIHydrationObviously essential, helps reduce constipation
Holliday-Segar method: 100, 50, 20; based on wtCalories
Calculated per the BMR
Growth/NutritionSlide20
WHO (basal needs: BMR)
[W = weight (kg)]Age (yrs) Gender Equation
0-3 Male 60.9W-54 Female 61W-51
3-10
Male 22.7W+495
Female 22.5W+499
10-18
Male 17.5W+651
Female 12.2W+746
Gevena
, 1985
NutritionSlide21
14.7 cal
/cm in children without motor dysfunction13.9 cal/cm in ambulatory patients with motor dysfunction11.1
cal/cm in non-ambulatory patientsUse arm span to estimate height
Height based methodSlide22
Micronutrients
If formula is <1L/day for adolescents/adults, will need to add MVIConsider monitoring vitamin D status
Growth/NutritionSlide23
Malnutrition
Obesity
Vitamin D deficiencyGastro-esophageal reflux
All of the above
What nutritional issues may arise in patients with cerebral palsy?Slide24
Malnutrition
Obesity
Vitamin D deficiencyGastro-esophageal reflux
All of the above
What nutritional issues may arise in patients with cerebral palsy?Slide25
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 sunlightGERD common in CP
Nutrition ExplanationSlide26
Treatment options include:
Decorative scarves and bibsGlycopyrrolate – risk for mucous plugs
Atropine Drops – local effectScopolamine patch
Botulinum
toxin injections – expensive procedure
Removal of salivary glands –
permanent, not recommended
DroolingSlide27
Children with CP often struggle with oral and/or pharyngeal
dysphagiaDiagnose formally with a swallow study with radiology and speech pathologyTreatment may include use of Thick-It or oatmeal thickener, or reliance solely on gastrostomy tube
SwallowingSlide28
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.
Case Slide29Slide30
Video Swallow study
CT scan of the chest
Sputum for AFB
Gastric emptying study
What diagnostic procedure will likely help determine cause of her respiratory distress? Slide31
Aspiration (primary or secondary)
Upper airway obstructionInfections (poor pulmonary clearance)
Restrictive lung disease (scoliosis)RespiratorySlide32
Pulmonary clearance techniques may include chest percussion, cough assist, VEST therapy all with the use of bronchodilator therapy
May develop OSA or central sleep apneaOver time may progress to need for trach and vent if severe chronic lung disease
RespiratorySlide33
Video Swallow study
CT scan of the chest
Sputum for AFB
Gastric emptying study
What diagnostic procedure will likely help determine cause of her respiratory distress? Slide34
Video Swallow study
CT scan of the chest
Sputum for AFB
Gastric emptying study
What diagnostic procedure will likely help determine cause of her infection? Slide35Slide36
Case
5 yoM with history of failure to thrive, had g-tube 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. Slide37
Dental evaluation
Reflux and gastric emptying studyPlain abdominal films
Plot him on the CP growth chart, determine he is still on the curve, reassure parents
All of the above
None of the above
B and C
What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance?Slide38
Reflux
Positioning uprightH2 or PPI therapyFundoplicationConstipationHydration and fiber Scheduled
miralaxSuppositories
GISlide39
Delayed gastric motility
Slow rate of feedsEESReglanPyloroplasty
GISlide40
Dental evaluation
Reflux and gastric emptying studyPlain abdominal films
Plot him on the CP growth chart, determine he is still on the curve, reassure parents
All of the above
None of the above
B and C
What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance?Slide41
Dental evaluation
Reflux and gastric emptying studyPlain abdominal films
Plot him on the CP growth chart, determine he is still on the curve, reassure parents
All of the above
None of the above
B and C
What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance?Slide42
Reduce muscle spasms
Improve functional ability
Reduce pain
Improve hygiene
Prevent tissue injury
Prevent hip migration
Improve cognitive functioning
When considering treatment for spasticity, which of the following is not considered a treatment goal?Slide43
Modified Ashworth Scale.
Blackburn M et al. PHYS THER 2002;82:25-34
Physical TherapySlide44
PT, ROM exercises
Enhance skill development, delay contracturesTime required to perform
OrthoticsTo improve function, prevent contracturesPossibility of pressure sores or muscle wastingSystemic medications
Diazepam,
baclofen
,
tizanidine
,
dantrolene
Decrease pain and muscle spasms
Sedation is adverse side effect
SpasticitySlide45
Botulinum
toxinImprove pain, improve function, help with hygiene2-3 primary muscle groupsWanes after 3 monthsIntrathecal
baclofen pumpNo central effect of sedationDevice complication
Dorsal
Rhizotomy
Permanent
Improves ambulation for spastic
diplegics
SpasticitySlide46
Pain arising from the hip
Clinically important leg length differenceDeterioration in ROM of hipIncreasing hip muscle tone
Deterioration in sitting or standingIncreasing difficulty with perineal care or hygiene
Hip DysplasiaSlide47
Contractures
Tendon clippingHip dislocationSurgical stabilizationScoliosisSurgical repair
MSK issues requiring OrthopedicsSlide48
Reduce muscle spasms
Improve functional ability
Reduce pain
Improve hygiene
Prevent tissue injury
Prevent hip migration
Improve cognitive functioning
When considering treatment for spasticity, which of the following is not considered a treatment goal?Slide49
Reduce muscle spasms
Improve functional ability
Reduce pain
Improve hygiene
Prevent tissue injury
Prevent hip
migration
Improve cognitive functioning
When considering treatment for spasticity, which of the following is not considered a treatment goal?Slide50Slide51
Constipation
Reflux
Extremity fracture
Hip dysplasia
Muscle spasm
The most common cause of pain in patients with CP is:Slide52
Pain in children with CP is under-recognized and thus undertreated
Affects quality of lifeChallenges include difficulty communicating and multiple etiologies of pain
Pain –Evidence Based MedicineSlide53
Cross-sectional study looked at 252 patients with CP ages 3-19
Questionnaire, including Health Utilities Index 3 pain subset, completed by primary caregiverTreating physician was asked to identify the presence of pain and provide a clinical diagnosis if applicable.
Characteristics of Pain Slide54
92% response rate
55% reported some pain on the HUI3, with 24% reporting that their child experienced pain that affected some level of activityPhysicians reported pain in 39%Identified hip dislocation/subluxation
(27%), dystonia (17%), and constipation (15%) as the most frequent causes of pain.
Characteristics of Pain Slide55
Constipation
Reflux
Extremity fracture
Hip dysplasia
Muscle spasm
The most common cause of pain in patients with CP is:Slide56
Constipation
Reflux
Extremity fracture
Hip dysplasia
Muscle spasm
The most common cause of pain in patients with CP is:Slide57
Provide primary care – preventative and acute
Chronic care Care coordinationSubspecialistsHome nursing
Sign care planOrder suppliesICD code 343.9
Social work, can help with community resources
School
Revisit role of PCPSlide58
Help to identify adult primary care and specialists
School through age 21 with IEPThe ARC - http://www.thearc.org/Vocational rehabilitationDiscuss sexuality
Advance directivesPalliative careAlternative care giversInsurance
Equipment
TransitionSlide59Slide60
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.)
ReferencesSlide61
Samour
PQ, King K. Handbook of P
ediatric Nutrition
. 3
rd
ed. Sudbury, MA. Jones and Bartlett Publishers, Inc. 2005.
V
M
archand
; 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
P
arenter
Enteral Nutrition, published online 25 March 2013.
Penner
M,
Xie
WY. Characteristics of Pain in Children and Youth With Cerebral Palsy.
Pediatrics
. 2013, 132: e407-413.
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
References