Transcript Cerebral Palsy - Health Sciences Center

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