chapter 15

1. chapter15 Amputations, Dwarfism, and Les Autres David L. Porretta

2. Amputations • Congenital • Acquired • Educational needs—psychosocial

3. Functional Classification System for Amputations • A1: double above the knee (AK) • A2: single AK • A3: double below the knee (BK) • A4: single BK • A5: double above the elbow (AE) • A6: single AE • A7: double below the elbow (BE) • A8: single BE • A9: combined lower and upper extremities

4. Prosthetic Devices • Team of medical specialists • Technological advances—lower limb devices • Made of carbon graphite • Can store and release energy • Simulates function of normal leg or foot • Fit by computer-generated designs

5. Dwarfism • Condition of short stature (5 feet or less) • Term “little people” preferred by people with dwarfism • Classification • Proportionate • Disproportionate (most common)

6. Achondroplasia • Absence of normal cartilage formation • Waddling gait, lordosis, limited range of motion, bowed legs, overweight or obese, large head, flattened face • Spinal stenosis—structural abnormality of spine • Cervical abnormalities—medical screenings • Disadvantage in certain activities—track and field, tennis, baseball, softball, basketball • Safety—undue stress on joints

7. Selected Les Autres Conditions • Muscular dystrophy • Juvenile rheumatoid arthritis • Osteogenesis imperfecta • Arthrogryposis • Multiple sclerosis • Friedreich’s ataxia • Myasthenia gravis • Guillian-Barré syndrome

8. Duchenne Muscular Dystrophy • Most common and severe form of childhood dystrophy • Pseudohypertrophic muscular dystrophy (excessive accumulation of adipose and connective tissue) • Weakness in thigh, hip, back, shoulder, and respiratory muscles • Inability to walk within 10 years of onset (continued)

9. Duchenne Muscular Dystrophy (continued) • Typical characteristics • Waddling gait • Difficulty in climbing stairs • Tendency to fall • Difficulty in rising from a recumbent position • Physical education activities—muscular strength and endurance, breathing, flexibility activities; aquatic activities

10. Juvenile Rheumatoid Arthritis • Still’s disease or juvenile chronic arthritis (JCA) • Joints inflamed—reduced range of motion • Remissions and exacerbations (attacks) • Three types • polyarticular • pauciarticular • systemic (continued)

11. Juvenile Rheumatoid Arthritis (continued) • Physical education—activities that increase or maintain range of motion; muscular strength and endurance activities • Safety—avoid jarring, twisting activities (e.g., tennis, basketball)

12. Osteogenesis Imperfecta • Defect in protein matrix of collagen fibers • Bones easily broken; multiple fractures result in limb deformities • Four types • Type l (least severe) • Type ll (most severe) • Type lll • Type IV (continued)

13. Osteogenesis Imperfecta (continued) • Physical education and sport—activities that increase joint stability; strength-building activities (e.g., swimming) • Safety—Do not place stress on joints (no power volleyball, basketball, etc., unless modified)

14. Arthrogryposis • Multiple joint contractures • Affects some or all joints; deformities common in limbs • Associated conditions—congenital heart defects, respiratory problems, facial abnormalities • Physical education—activities that focus on flexibility (e.g., swimming [enhances flexibility and strengthens weak muscles around joints]) • Safety—modify activities accordingly

15. Multiple Sclerosis • Changes in white matter of nerve fibers—brain and spinal cord • Slowly progressing disease that might result in total incapacitation • No cure • Common symptoms • Extreme fatigue, general weakness • Heat intolerance • Hand tremors, loss of coordination, staggering gait • Double vision and slurred speech • Partial or complete paralysis (continued)

16. Multiple Sclerosis (continued) • Physical activity—moderate levels can reduce related fatigue and promote increased psychological benefits; swimming activities should be done in warm water. • Safety—provide periodic rest periods.

17. Friedreich’s Ataxia • Inherited neurological disease—gradual loss of motor coordination and progressive nerve degeneration • Symptoms • Poor balance • Lack of limb and trunk coordination • Clumsy, awkward gait • Impaired fine motor control • Atrophy of distal limbs • Slurred speech (continued)

18. Friedreich’s Ataxia (continued) • Prone to seizures • Foot and spinal deformities common • Heart problems • Visual abnormalities • Physical activities—promote muscle strength and endurance; body coordination and balance • Safety—program for individual tolerance levels for cardiac conditions and those prone to seizures

19. Myasthenia Gravis • Neuromuscular disease—reduced muscular strength; disease might go into remission. • Nerve impulses are prevented from reaching muscles. • Easily confused with muscular dystrophy symptoms (back, lower extremities, and respiratory muscles are affected). • Abnormal fatigue is common. (continued)

20. Myasthenia Gravis (continued) • Eye, face, mouth, and tongue muscles easily tire; weak back muscles might cause spinal deformities. • Problems with chewing and speaking are common. • Physical activities • Physical fitness—swimming is a good activity. • Program for individual tolerance levels. • Emphasize posture exercises and activities when spinal deformities are present. • Safety—program activities in a progressive manner; be aware of drug side effects.

21. Guillain–Barré Syndrome • Neurological disorder—results in acute and progressive paralysis (lower extremities initially affected). • Symptoms reach maximum in a few weeks. • Syndrome is typically preceded by either a viral or bacterial infection. • Some people have complete recovery. • For those who do not completely recover, activities should focus on maintaining or improving cardiorespiratory endurance and muscular strength and endurance.

22. Assessment Areas Prior to Implementing Physical Activity for Weight Reduction in Amputees • Type of amputation • Functional range of motion • Strength • Balance and stability • Skin integrity

23. Physical Fitness for Amputees • Use Brockport Physical Fitness Test. • Depending on site and number of amputations, various test items might need to be chosen. • Persons with bilateral AK or BK amputations typically have low aerobic functioning; unilateral or bilateral AK amputees tend to be overweight or obese. (continued)

24. Physical Fitness for Amputees (continued) • Develop muscular strength and endurance, and flexibility for all body parts; focus on remaining muscles surrounding the site of amputation.

25. Physical Fitness for Dwarfism • Encourage individuals to perform regular physical education and sport activities. • Individuals with dwarfism tend to be overweight, have reduced range of motion, and experience joint defects. • Avoid or modify activities that place undue stress on weight-bearing joints. (continued)

26. Physical Fitness for Dwarfism (continued) • Swimming is an excellent activity, especially for achondroplasia. • Modify implements (e.g., rackets, clubs, bats) according to the individual’s size.

27. Physical Fitness for Les Autres • Weak muscles are typical; balance and coordination problems are common. • Maintain or increase muscular strength and endurance. • Avoid performing beyond 50% of maximum resistance for weight training for individuals with progressive muscle disorders. (continued)

28. Physical Fitness for Les Autres (continued) • If functional strength does not return within 12 hours, then intensity exercise level was too great. • Low level of aerobic fitness is common; many exhibit general body fatigue (e.g., MS). • Proper warm-up activities essential, especially for those with JRA, OI, arthrogryposis, and others with joint limitations.

29. Inclusion • Allow children to participate in general physical education and sport whenever possible. • Most can safely and effectively participate in general physical education and sport settings when modifications and supports (e.g., teacher aids) are available. • IEP will stipulate restricted physical education and sport settings.

30. Adapted Sports • Amputations (Disabled Sports USA; Wheelchair Sports, USA; National Wheelchair Basketball) • Disabled Sports USA • Track and field, basketball, volleyball, bowling, shooting, table tennis, cycling, archery, weight lifting, swimming • Dwarfism • Dwarf Athletic Association of America (DAAA) (continued)

31. Adapted Sports (continued) • Track and field, swimming, basketball, bocce, equestrian, soccer, volleyball, team bocce, table tennis, power lifting • Les Autres • National Disability Sports Alliance (NDSA)—athletes compete among themselves • Track and field, swimming, volleyball, archery, bocce, shooting, cycling, table tennis, wheelchair team handball, power lifting