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Cerebral Palsy Cerebral Palsy

Cerebral Palsy - PowerPoint Presentation

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Cerebral Palsy - PPT Presentation

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

care pain palsy cerebral pain care cerebral palsy children patients motor growth health nutrition spasticity muscle treatment primary determine

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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

AgendaSlide4
Slide5

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 AbilitiesSlide10
Slide11

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 CPSlide13
Slide14

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 Slide29
Slide30

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? Slide35
Slide36

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?Slide50
Slide51

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

TransitionSlide59
Slide60

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