Cerebral PalsyOne Size Does Not Fit All

Mary Catherine Brake Turner, MD, FACP, FAAPbrakem@ecu.edu

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

Objectives

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

Agenda

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

Definition of CP

Diagnosis is suspected by PCP

Classify based on localization and type

Assessment of associated impairments

Overall severity

Assessment for Intervention

Spasticity

Dyskinesia (dystonia and choreoathetosis)

Ataxia

Hypotonia

Type of Motor Disorder

Diplegia: Lower extremities >> upper extremities

Quadriplegia: Upper and lower extremities are affected equally

Hemiplegia: 1 side more involved than its opposite counterpart

Localization

Gross motor – ambulation

Fine motor – self-help skills

Oromotor and speech – communication, eating and drinking

Functional Motor Abilities

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

Gross Motor Function Classification System for Cerebral Palsy (GMFCS)

Chorioamnionitis

Birth weight <2000 gm

Intracranial hemorrhage

Newborn encephalopathy

Periventricular leukomalacia

Hydrocephalus

Congenital malformations

Risk Factors for Development of CP

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

Relevance to Us

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.

Case

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?

Affected by dysphagia, GERD, delayed gastric motility, constipation

May have to rely on gastrostomy or jejunostomy tubes

+/- fundoplication

Growth/Nutrition

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 CP

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

Growth/Nutrition

WHO (basal needs: BMR)[W = weight (kg)]

Age (yrs) Gender Equation0-3 Male 60.9W-54

Female 61W-513-10 Male 22.7W+495

Female 22.5W+49910-18 Male 17.5W+651

Female 12.2W+746Gevena, 1985

Nutrition

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

Height based method

Micronutrients

If formula is <1L/day for adolescents/adults, will need to add MVI

Consider monitoring vitamin D status

Growth/Nutrition

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

What nutritional issues may arise in patients with cerebral palsy?

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

Nutrition Explanation

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

Drooling

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

Swallowing

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

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 respiratory distress?

◦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

Respiratory

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 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?

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.

A. Dental evaluationB. Reflux and gastric emptying studyC. Plain abdominal filmsD. Plot him on the CP growth chart,

determine he is still on the curve, reassure parents

E. All of the aboveF. None of the aboveG. B and C

What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance?

Reflux◦ Positioning upright◦ H2 or PPI therapy◦ Fundoplication

Constipation◦ Hydration and fiber ◦ Scheduled miralax◦ Suppositories

GI

Delayed gastric motility◦ Slow rate of feeds◦ EES◦ Reglan◦ Pyloroplasty

GI

A. Dental evaluationB. Reflux and gastric emptying studyC. Plain abdominal filmsD. Plot him on the CP growth chart,

determine he is still on the curve, reassure parents

E. All of the aboveF. None of the aboveG. 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 studyC.Plain abdominal filmsD. Plot him on the CP growth chart, determine he

is still on the curve, reassure parentsE. All of the aboveF. 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. 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?

Modified Ashworth Scale.

Blackburn M et al. PHYS THER 2002;82:25-34

Physical Therapy

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

Spasticity

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

Hip Dysplasia

Contractures◦ Tendon clipping

Hip dislocation◦ Surgical stabilization

Scoliosis◦ Surgical repair

MSK issues requiring Orthopedics

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

When considering treatment for spasticity, which of the following is not considered a treatment goal?

A. Constipation

B. Reflux

C. Extremity fracture

D. Hip dysplasia

E. Muscle spasm

The most common cause of pain in patients with CP is:

Pain in children with CP is under-recognized and thus undertreated

Affects quality of life

Challenges include difficulty communicating and multiple etiologies of pain

Pain –Evidence Based Medicine

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 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.

Characteristics of Pain

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

The most common cause of pain in patients with CP is:

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

Revisit role of PCP

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

Transition

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.)

References

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: 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