chapter 16 ms and jra

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CHAPTER 16

MUSCULAR DYSTROPHYAND

JUVENILERHEUMATOID

ARTHRITIS (JRA)



MUSCULAR DYSTROPHY

(ADD THE CASE STUDY FROMPAGE 237 HERE.)



I. Definition – Skeletal neuromuscular disease

is characterized by persistent deteriorationof striated muscle tissue. Muscular

Dystrophy is distinguished from other

Neuromuscular diseases by four criteria:

• 1. Primary myopathy

• 2. Genetic base

• 3. Progressive nature

• 4. Degeneration of muscle fibers



• The disease is a degeneration of muscle tissue in

which muscle cells degenerate and the emergence

of fat and fibrous tissue replaces muscle tissue.

(Insert Figures 16.1 and 16.2 on page 239 here.)

II. Physical Characteristics as the disease

progresses include:

• Muscle weakness

• Fatigue

• Respiratory/heart complications



III. Types and Characteristics of Muscular

Dystrophy

(Insert Table 16.1 on page 238 here.)

Progression through stages of functional ability

include:

1. Low strength/endurance; normal ambulation

with possible overwork weakness; slight

deficiency in function.

2. Reduction in activity; tendency to fatigueeasily; reduced strength/endurance; habitual

activity, mild contractures and possible

overwork weakness; ambulation with assistance.



3. Poor strength/endurance; overwork weakness;

contractures; limited ambulation and decrease in

physical activity and standing.

4. Ambulation significantly decreased;

functional use of wheelchair; severe contractures

and muscular weakness; pulmonary difficulties

and cardiomyopathy.



IV. Planning the Physical Activity Program:

Individuals with muscular dystrophy

commonly demonstrate low muscle enduranceand fatigue quickly while walking and

climbing stairs.

Guidelines for Intervention:• Medical Approval - determine type and state;

recommendations for intervention.

•

Assessment - Determine residual strengthflexibility and functional capabilities.

• Intensity - Avoid all out bouts of exercise. Use

alternative resistance and water exercise to

maintain functional ability.



• Warm-up Cool-down: Critical component to

prepare weakened muscle for work out. Facilitate

strength, flexibility and maintaining function.

V. Implementing the Physical Activity Program -

A. Concerns: Early recognition is essential for early intervention physical activity will help

maintain muscular strength and functional ability.

Recommendation for intervention should promote

range of motion, maintain positive and alleviatecontractures. Several concerns are evident in

developing an excise program. They include:



• Extent of muscle weakness

• Progression of disease

• Degree and intensity of the exercise

• Individual needs

B. Individualized Program Approach:

The components of an exercise program should include

strength, endurance and aerobic power that is essentialfor standing, walking and functional daily tasks. Goals

of the program should include:• Provide short-term realistic goals

•

Focus on maintaining or reducing the rate of deterioration• Focus on submaximal exercises and reduce intensity to

avoid fatigue

• Prevent contractures and provide nutritional counseling

• Provide activities in an enjoyable setting to facilitate

compliance



C. Community Home Based Interventions

(Insert Table 16.2 on page 244 here.)



JUVENILERHEUMATOID

ARTHRITIS (JRA)

(Insert Case Study on page 245 here)



I. Definition - JRA

• JRA is a general term for all types of arthritis

and related conditions occurring (Arthritis

Foundation 2009). The primary pathology of the chronic disease is inflammation of the

connective tissues and is characterized by

swelling and pain.



II. Symptoms and Types

• Juvenile arthritis is characterized by majorchanges in the joints including inflammation,contractures and joint damage which affectmobility, strength, and endurance.

• Nearly all children experience periods whensymptoms reduce in severity or disappear butmay go from symptom free to extreme pain andswelling

• When children are symptom free they should beencouraged to participate in developmentalactivities



• Psychological and social impacts aremultidimensional as joint pain and stiffness

becomes a distraction that effect concentration;medication schedules are also disruptive andhave side effects – subtypes are distinguished bythe number of joints involved within the first 6

months of onset:• Systemic arthritis – entire body is affected from

high spiking fever to concomitant jointdiscomfort



•Polyarticular arthritis – arthritis in five or more

joints with major symptoms of pain in the knees,

ankles, wrist, fingers, elbows and shoulders.

Constant pain is prevalent

•Pauciarticular arthritis - arthritis in four or fewer

joints within the first 6 mo of onset; large joints of

knee, ankle, elbow, and wrist are affected.



III. Planning the Physical Activity

ProgramMedication

• Nonsteroidal anti-inflammatory medication

approved in children 12 and under although sideeffects are apparent

• Glucocorticoid Drugs including cortisone and

prednisone are used for pain and swelling. Side

effects include high blood pressure, osteoporosis, weight gain and slower growth rate.



Exercise Considerations!

Physical activity and exercise are mainstays in

treatment. The Arthritis Foundation recommends participation in physical activity which leads to the

following benefits:

•Maintains joint flexibility

•Maintains muscle strength

•Regains range of motion or strength in joint or

muscle

•Makes functional activities such as walking easier



Exercise Considerations! (contd.)

•Improves general fitness and endurance

•Improves self-esteem

•Prevents deconditioning

•Maintains bone density



IV. IMPLEMENTING THE PHYSICALACTIVITY PROGRAM

Input is needed from the collaborative team andalleviating stress on their joints on jointprotection. The Arthritis Foundationrecommends 3 types of exercises:

Flexibility exercises for joint movement

Strengthening exercise including isometric and

isotonic exercise to support and protect joints

Cardiovascular exercise to control weight and

maintain functional ability

Add Table 16.3 from page 249 here.



Learning Activity

• Have your class identify several exercise

programs developed by the Arthritis

Foundation including the aquaticsexercise program and walking with ease.

Also contact the Center for Disease

Control concerning the National ArthritisAction Plan.

chapter 16 ms and jra

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