CHAPTER 16 MUSCULAR DYSTROPHY AND JUVENILE RHEUMATOID

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CHAPTER 16MUSCULAR DYSTROPHYANDJUVENILE
RHEUMATOID ARTHRITIS (JRA)
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MUSCULAR DYSTROPHY(ADD THE CASE STUDY FROM PAGE
237 HERE.)
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• I. Definition Skeletal neuromuscular disease is
  characterized by persistent deterioration of
  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

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• 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.)
• Physical Characteristics as the disease
  progresses include
• Muscle weakness
• Fatigue
• Respiratory/heart complications

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• 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 fatigue
  easily reduced strength/endurance habitual
  activity, mild contractures and possible overwork
  weakness ambulation with assistance.

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• 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.

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• Planning the Physical Activity Program
• Individuals with muscular dystrophy commonly
  demonstrate low muscle endurance and fatigue
  quickly while walking and climbing stairs.
• Guidelines for Intervention
• Medical Approval - determine type and state
  recommendations for intervention.
• Assessment - Determine residual strength
  flexibility and functional capabilities.
• Intensity - Avoid all out bouts of exercise. Use
  alternative resistance and water exercise to
  maintain functional ability.

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• Warm-up Cool-down Critical component to prepare
  weakened muscle for work out. Facilitate
  strength, flexibility and maintaining function.

• 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 alleviate
  contractures. Several concerns are evident in
  developing an excise program. They include

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• 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 essential for 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

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• C. Community Home Based Interventions
• (Insert Table 16.2 on page 244 here.)

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JUVENILE RHEUMATOID ARTHRITIS (JRA)(Insert
Case Study on page 245 here)
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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.

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II. Symptoms and Types

• Juvenile arthritis is characterized by major
  changes in the joints including inflammation,
  contractures and joint damage which affect
  mobility, strength, and endurance.
• Nearly all children experience periods when
  symptoms reduce in severity or disappear but may
  go from symptom free to extreme pain and swelling
• When children are symptom free they should be
  encouraged to participate in developmental
  activities

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• Psychological and social impacts are
  multidimensional as joint pain and stiffness
  becomes a distraction that effect concentration
  medication schedules are also disruptive and have
  side effects subtypes are distinguished by the
  number of joints involved within the first 6
  months of onset
• Systemic arthritis entire body is affected from
  high spiking fever to concomitant joint discomfort

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• 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.

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III. Planning the Physical Activity
ProgramMedication

• Nonsteroidal anti-inflammatory medication
  approved in children 12 and under although side
  effects 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.

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• 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

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• Exercise Considerations! (contd.)
• Improves general fitness and endurance
• Improves self-esteem
• Prevents deconditioning
• Maintains bone density

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IV. IMPLEMENTING THE PHYSICAL ACTIVITY PROGRAM

• Input is needed from the collaborative team and
  alleviating stress on their joints on joint
  protection. The Arthritis Foundation recommends
  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.
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Learning Activity

• Have your class identify several exercise
  programs developed by the Arthritis Foundation
  including the aquatics exercise program and
  walking with ease. Also contact the Center for
  Disease Control concerning the National Arthritis
  Action Plan.