living with cerebral palsy life-span considerations mary rose franjoine, pt dpt ms pcs margo prin...

Living withCerebral Palsy

Life-span Considerations

Mary Rose Franjoine, PT DPT MS PCSMargo Prin Hanynes PT DPT MA PCS

Recommended Reading

Aging in Individuals with Lifelong Disabilities Barbara H. Connolly

Physical and Occupational Therapy in Pediatrics, 2001

– Volume 21, Issue 4; Pages 23-47

Background Resource

Cech and Martin

Functional Movement Development Across the Life Span, 2nd edt.,

WB Saunders, 2002

Learning Objectives

At the conclusion of this lecture the learner will: Describe psychosocial factors that impact on

quality of life in individuals living with developmental disabilities.

Discuss physical factors that impact on the quality of life in individuals living with developmental disabilities.

Identify strategies that can be included in a plan of care to improve the quality of life for individuals living with developmental disabilities.

The Aging Process….• Maturity

• Varies within body systems

• Variability between individuals

• Physical Maturity Intellectual Maturity • Peak Physical Capabilities: 20 – 24 years of age

• Executive Functions: 26 -28 years of age

• Experience shapes the maturation process• Key consideration for individuals living with Cerebral

Palsy and other developmental-based disabilities

Life-span Motor Development

Key considerations:

• Multidirectional

• Efficiency is Gained and/or lost

• Plasticity

• Historical Influences

• Psychosocial Influences

Aging Process

Linier Model Inverted U Model

C B Death

B D

Maturity

C

"Motor development should be seen as a succession of

integrated milestones leading to more complex and

independent function." Bobath

Demographics • 1 out of 8 individuals living in the US is

over the age of 65!!!!!!

• 12% of individuals living in the US over the age of 65 have a developmentally-based disability

When does the Aging Process Begin????

• Physical Changes

• State of Mind

• Senior Citizen status• Typical Adults: 65 years of age• Developmentally Disabled Adults: mid-50’s

Declines in Daily Life Functional Capabilities

• Typical Adult population• 60 to 65 years of age

• Individuals living with Developmental Disabilities

• 35 to 50 years of age

• Individuals living with Cerebra Palsy• Decline in capabilities may begin in early 20’s

Predictors of Successful Aging

• Gender – Women

• Community-dwelling

• Ambulatory

• IQ of 50 or greater (mild)

• Verbal communication with natural or assisted speech

What are the most common changes associated with the

typical aging process?

Age-related changes within the Central Nervous System

Beginning at age 20:• Brain weight decreases• Brain volume decreases• Cortex thins • Glial Cells increase in number• Ventricular volume increases• Cerebral volume decreases (beginning in the 3rd decade of

life)• Conduction velocities decrease• Sensory changes precede motor

• Decline in peripheral nerve conduction velocities precedes

the decline centrally

Age-related changes within the Central Nervous System

MRI studies have shown with increasing age brain tissue proportions decrease• Sulci and Ventricular CSF proportions increase• Cortical gray matter proportions decrease• CSF volume increases• Percentage of gray matter decreases• Protein levels decrease • Lipofuscin increases extracellularly • Percentage of Neurofibrillary Tangles and Neuronal (Senile) Plaques

increase • Cerebral blood flow decreases• Brain metabolism decreases• Dopamine up-take decreases• Decrease in glucose utilization

CT and MRI studies seem to suggest that large neurons in the Temporal and Frontal lobes shrink, while the smaller neurons die off.

PET scans have not confirmed these findings!

Age-related structural changes are not consistent throughout

the Brain!

• Frontal and Temporal lobes are effected greater than the Parietal lobe.

• The Primary Motor and Primary Sensory areas of the Cortex are susceptible to neuronal loss beginning at age 20.

• The Hippocampus, (Limbic System) can sustain a 30 percent loss of neurons by age 30.

Functional ImplicationsLearning • Cortex• Limbic system• Thalamus• Reticular formation• Brain stem

Studies have shown that intellectual abilities peak between 20 and 30 years of age and last until 75 years of age.

Functional Implications

• Decline of cognitive abilities in individuals living with developmental disabilities is thought to be increased when compared to age-matched peers beginning in 30’s.

• Rate of decline is unknown• Decline of Cognitive/Intellectual capabilities in

individuals living with Cerebral Palsy is unclear• Blurred by psycho-social factors, and co-morbidities • Linked with increase incidence of depression and

social isolation

Memory

Memory Storage• Immediate• Short term• Long term

Types of Memory• Reflexive also known as Procedural

Motor memory

• Declarative Verbal performance

Memory changes occur across the life-span

DementiaAlzheimer's Disease

Prevalence is thought to increase in individuals living with developmentally-based disabilities.

• Individuals living with mild to moderate mental retardation have an increased incidence of AD when compared to age-matched peers.

• Individuals who have Learning Disabilities have a higher than average incidence of early onset dementia and AD.

• Individuals living with developmentally-based disabilities residing in institutional settings have an extremely high incidence of dementia & AD when compared to community-dwelling individuals with similar capabilities.

DementiaAlzheimer's Disease

Individuals living with Down Syndrome• Increased prevalence of memory loss• Extremely high incidence of early-onset dementia

• 40% higher than age-matched peers at age 35 years• 45% of adults with Down Syndrome over the age of

50 years experience moderate to severe dementia

• Overall aging process for individuals living with Down Syndrome is thought to be accelerated• Compared to typical age-matched peers• Compared to individuals with similar cognitive

capabilities

What factors influence your ability to learn, to retain new

information, or to recall previously learned knowledge

or skills?

Age-related Changes within the Sensory System

Decline in Sensory system function• Begins in early adulthood - 20 to 30 years of age• Progresses with increasing age• Functional abilities typically not impacted until

Older adulthood – 70’s, 80’s

Tactile

Superficial Sensation

• Gradual declines have been described in the literature

• Limited impact on functional abilities occurs until late in life

• Studies suggest that beyond middle age, the ability to detect the intensity of a noxious object decreases.

Temperature

• Extreme temperature changes are generally perceived by the elderly, but smaller changes are less readily discerned.

• The ability to detect environmental temperature changes also declines with age.

Pain

Perception and discrimination of a painful stimulus declines with increasing age.

Two-point Discrimination

Decreases with age

Kinesthetic

Vibratory Sense• Most common sensory loss in the elderly• Initially seem in the lower extremities

• Studies have reported as much as a 60% loss in the lower extremities of women in their 50’s.

Passive Movement Sense• Detection thresholds increase with age, especially in

LE• Change in UE detection are not reported

Position Sense• Declines with age, especially in the lower extremities

Passive Movement Sense&

Position Sense

• Detection thresholds are twice as high in individuals with Cerebral Palsy over the age of 50 years

Praxis

Motor planning abilities decline with age beginning at age 30.

Reaction Time

• Perception of Stimulus Motor Response

Developmentally, RT is linked closely to the myelinzation process

Fastest reaction times are seen in the 20's.

Studies have shown a 20 percent increase in response time in a 60 year old when compared to a 20 year old.

Functional Implications • Decline in Tactile, PS, PMS & VS may interfere with an

individual's ability to • Access AT

• Communication devices• Environmental control devices• Mobility devices

• Complete BADLs & IADLs• Interface with the world!

• Increases in RT may interfere with the ability to interact with individuals and the environment

Hearing

Decline is due to loss of sensory cells in the inner ear

• 40 percent of adults age 65 experience a hearing loss which effects discrimination of speech sounds.

• 70 percent of adults over age 70 will experience a hearing loss which effects discrimination of speech sounds.

• Changes may begin as early as age 30 years; progressing up to age 80 years.

• High frequency hearing loss precedes low frequency loss

Hearing Loss

Suspect high rate of unidentified hearing loss in individuals living with developmentally-based disabilities

• Chronic ear infections• Testing difficulty• Access to testing

Loss of Vestibular Function

Vestibular nerve demyelization begins at age 40 years• 40% loss of fibers by age 75 years

• Viscosity of fluid increases with age• Membranes become brittle with age• Hair cells decrease with age

• Vestibular dysfunction• Commonly seen in individuals over the age of 50

years

Balance Dysfunction

• Functionally there is a decline in balance abilities beginning in mid-50’s

• Dizziness and Vertigo: Two or more episodes reported by 40% of typically aging individuals by age 55 years

• Prevalence in individuals living with Cerebral Palsy is unknown• Suspect that there may be a high rate of

undiagnosed Vestibular Dysfunction

Vision

Visual acuity increases into the 20's and remains stable through early adulthood.

• Decline in visual acuity begins between age 40 and 50 years.

• By age 85 there is an 80 percent loss of the acuity level present at age 40 years.

• Beginning at age 40 years• Loss of color vision – spectrum loss• Dark adaptation slows • Visual fields decrease

Vision Loss

Suspect high rate of unidentified vision loss in individuals living with developmentally-based disabilities

• Chronic eye infections• Testing difficulty• Access to testing

• Decline in functional vision may occur early in life secondary to• Poor eye coordination• Poor gaze stability

• Functional vision may decline early in life • Poor eye coordination and gaze stabilization skills

Taste and SmellSocial and emotional aspects of eating are thought to account for a loss of appetite in the elderly verses structural changes in the system.

• The ability to detect noxious odors declines with age.

What are the life changes that can lead to loss of appetite?

Loss of Appetite

Individuals living with developmental-based disabilities have an:• Increased threshold for taste and smell of food

• Loss of pleasure from eating

• Poor dietary intake

• Loss of weight

• Increased risk of infection

• Compromise of Body Functions & Structures

• Death

Malnutrition

Physical, social and emotional factors associated with eating can lead to states of malnutrition

Calorie intake may need to exceed intake for typical age-matched peers

Limited Caloric intake and poor nutritional status may lead to:

• Decreased ability to repair – normal wear and tear• Decreased ability to fight infection• Decrease in Vitamins C and D: bone and skin health

Decreased Body Mass Index

• A “real” life threatening concern for 25% of individuals living with developmental-based disabilities

• Individuals living with Cerebral Palsy prevalence of decrease BMI is unclear• Need for nutritional supplements, or

supplementation of nutrition intake

Increased Body Mass Index

• New Epidemic in Children• Numerous related health concerns

• Diabetes• Hearth Disease • Integumentary problems• . . .

Age-related changes within the Musculoskeletal System

Skeletal System Changes

Maximal bone mass is obtained by the late 20’s or early 30’s.

Bone reabsorption exceeds bone formation between age 35 and 40 years.

Bone loss appears to vary between races and with gender.• Women more so than men• Black males appear to be least affected.

Architectural changes occur within the bone matrix and in collagen fibers that increase the brittleness of bone.

Age-related Changes within the Musculoskeletal System

Muscle System Changes• Gender differences in force generation ability appear in early adolescence• Grip strength doubles between 6 and 12 years of age• Strength doubles between 10 and 16 years of age • Muscle strength appears to peak in your 20’s • Functional muscle strength begins to decline in your 50’s • Muscle strength declines by 30% from the 50’s to the 70’s• Decline after age 70 is more rapid

• Structural changes within the muscle begin in the 50’s and lead to a decline in the ability to generate power

• Etiology of muscle mass loss is associated with loss of contractile proteins, hormonal changes, and changes in motor units.

• 1% of the total number of motor units is lost annually beginning in the 20’s• Loss of muscle mass is greatest in the lower extremities, with the thigh showing the

greatest loss

Functional Implications

• Alteration of preferred movement patterns or functional movement strategies:

• Osteoarthritis may result in pain, or a fear of falling that can lead to a decrease in activity level.

• Osteoporosis and fear of a fall which may result in a fracture can lead to a decrease in activity level.

• Inactivity can lead to a decrease in a muscle’s ability to generate power, decrease in muscle mass, and a decline in functional muscle strength.

Functional Implications

Individuals living with Cerebral Palsy report decline in muscle strength which interferes with performance of functional activities

Percentage/degree of strength loss is not clear• At age 30 years: 20% report perceived loss• At age 40 years: 60% report

• Increased time to complete tasks• Decreased proficiency and accuracy in task completion• Require “new level” of assistance: care provider or AT

Osteoarthritis

Individuals living with Cerebral Palsy report early onset of arthritic impaired movement & associated pain syndromes

• Shoulder• Hands• Neck• Back/Low back• Hips • Knees

• Increased incidence of hip fracture in age- matched peers beginning at age 40 years

Balance Dysfunction

• Functionally there is a decline in balance abilities beginning in mid-50’s • Loss of Muscle Strength• Loss of flexibility• Decrease in perception of sensory information

• Increase in reaction time • Increase in sensory processing time

• Fear factor!

Fear of Falling

• Individuals living with Cerebral Palsy report and increased fear of falling impacting activity choices when compared to age-matched peers beginning in mid- 20’s

• Fear of Falling with Injury is increased at all ages – negatively correlated

Age-related Changes within the Cardiopulmonary System

The Cardiac System

• Structural changes occur within the heart that result in an increase stiffness and decrease compliance of the ventricles, which leads to an increase in HR, BP and a decrease in CO.

• Maximal HR decreases with age.

• Heart valves become thicken and calcified decreasing their ability to function efficiently.

• SA node cells significantly decrease in number by age70.

Age-related Changes within the Cardiopulmonary System

The Pulmonary System• Structural changes in the thorax, (shorter and wider) lead to an

increase in the work of breathing.• Beginning in your 20”s:

• Decrease in compliance: elasticity • Decrease in chest wall mobility • Increased resistance within the bronchopulmonary system • Decrease in number of alveoli• Alveolar surface area decreases

• Pulmonary Function Tests• Decrease in Tidal Volume • Decrease in Total Lung Capacity• Increase in Residual Volume• Increase in Functional Residual Capacity• Decrease in Forced Expiratory Volume and FEV1

Functional ImplicationsTarget HRExercise ToleranceAerobic capacityPulmonary Health

Growing Body of Evidence Research supports aerobic exercise program

for individuals living with Cerebral Palsy • Independent or AT assisted community-based

programs

Age-related Changes within the Gastro-Intestinal and Urinary

Systems

• Decreased gastric and intestinal motility • Decreased elasticity of bladder and urinary tract

Functional Implications

• Dietary changes

• Lifestyle changes

Program Planning

• When do we begin to modify our plan of care to impact on the aging process?

• What factors should be considered in OT, PT, & ST to facilitate optimal aging for individuals with living Cerebral Palsy?

References• Balandin, S. Morgan, J. Adults with cerebral palsy; what’s

happening. Journal of Intellectual and Developmental Disability. 1997; 24(2); 109 -124.

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• Bertoti, Dolores, B. Workbook for Functional Neurorehabilitation Through the Life Span. F.A. Davis Company, Philadelphia, Pa. 2004.

• Bonder,B. and Wagner, M. Functional Performance in Older Adults, 2nd edt. F.A. Davis Company, Philadelphia, Pa. 2001.

• Cech, D and Martin, S. Functional Movement Development Across the Life-span, 2nd edt. W.B. Saunders, Philadelphia, PA, 2002.

• Connolly, Barbara H., Aging in Individuals with Lifelong Disability. Physical and Occupational Therapy in Pediatrics , 2001: .21(4); 23- 43/

References• Donkervoort, Murielle, Roebroeck, Marij, et.al. Determinants of functioning of adolescents

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• Hammel, Joy Lai, Jin-Shei, et. Al. The impact of assistive technology and environmental interventions on function and living situations status with people living situation status with people who are aging with developmental disabilities. Disability and Rehabilitation . 2002; 24(1/2/3); 93-105.

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• Nieuwenhuijsen, Channah,, Van Der Laar,, Yvette, et.al. Unmet needs and health care utilization in young adults with cerebral palsy. Disability and Rehabilitation. 2008; 30 (7): 1254-1262.

References

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