1. Chapter 15 Amputations, Dwarfism, and Les Autres
David L. Porretta
Chapter 15 Amputations, Dwarfism, and Les Autres

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 in lower-limb devices 
Made of carbon graphite. 
Can store and release energy.
Simulates function of normal leg and foot. 
Fit by computer-generated designs.

5. Dwarfism 4 ft, 10 in. (147 cm) or less Term little people preferable
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
Guillain-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
(continued)

9. Duchenne Muscular Dystrophy (continued)
Inability to walk within 10 years after onset
Typical characteristics
Waddling gait
Difficulty climbing stairs
Tendency to fall
Difficulty rising from a recumbent position

10. Juvenile Rheumatoid Arthritis
Still’s disease or juvenile chronic arthritis (JCA)
Joints become inflamed—reduced range of motion
Series of remissions and exacerbations (attacks)
(continued)

11. Juvenile Rheumatoid Arthritis (continued)
Three types
Polyarticular
Pauciarticular
Systemic
Physical education activities—increase or maintain range of motion; muscular strength and endurance
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 I (least severe)
Type II (most severe)
Type III
Type IV
(continued)

13. Osteogenesis Imperfecta (continued)
Physical education and sport activities—increase joint stability (strength-building activities; e.g., swimming).
Safety—do not place stress on joints (e.g., power volleyball, basketball) unless modified.

14. Arthrogryposis Multiple joint contractures
Affects some or all joints; limbs commonly exhibit deformities 
Associated conditions—congenital heart defects, respiratory problems, various facial abnormalities 
Physical education activities—focus on flexibility
Swimming (enhances flexibility and strengthens weak muscles around joints)

15. Multiple Sclerosis
Changes in white matter of nerve fibers—brain and spinal cord
Slowly progressing disease that may result in total incapacitation—no cure
Physical activity—moderate levels can reduce related fatigue; increase psychological benefits; provide rest periods; swimming activities done in warm water
(continued)

16. Multiple Sclerosis (continued)
Common symptoms
Extreme fatigue, general weakness
Heat intolerance
Hand tremors, loss of coordination, staggering gait
Double vision
Slurred speech
Partial or complete paralysis

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)
Symptoms (continued)
Prone to seizures
Foot and spinal deformities
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 may go into remission
Nerve impulses prevented from reaching muscles
Easily confused with muscular dystrophy symptoms (back, lower extremities, and respiratory muscles affected)
Abnormal fatigue
(continued)

20. Myasthenia Gravis (continued)
Eye, face, mouth, tongue muscles easily tire; weak back muscles may lead to spinal deformities
Problems with chewing and speaking
Physical activities
Physical fitness—swimming is a good activity
Program for individual tolerance levels
Posture exercises and activities when spinal deformities are present

21. Guillain-Barré Syndrome
Neurological disorder results in acute and progressive paralysis (lower extremities initially affected).
Symptoms reach maximum in a few weeks.
Syndrome 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 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 may 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/obese.
Develop muscular strength and endurance and flexibility for all body parts and remaining muscles surrounding site of amputation.

24. Physical Fitness for Dwarfism
Perform regular physical education and sport activities.
People with Dwarfism tend to be overweight and have reduced range of motion and joint defects.
Avoid or modify activities that place undue stress on weight-bearing joints.
(continued)

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

26. Physical Fitness for Les Autres
Typically have weak muscles; balance and coordination problems.
Maintain or increase muscular strength and endurance.
Avoid performing beyond 50% of maximum resistance for weight training for people with progressive muscle disorders.
(continued)

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

28. Inclusion
Allow children to participate in regular physical education and sport whenever possible.
Most can safely and effectively participate in regular physical education and sport settings when modification and supports (e.g., teacher aides) are available.
IEP will stipulate whether or not children participate in more restricted physical education and sport settings.

29. Adapted Sport
Amputations (Disabled Sports USA; Wheelchair and Ambulatory Sports, USA; National Wheelchair Basketball Association)
Disabled Sports USA
Track and field; basketball; volleyball; bowling; shooting; table tennis; cycling; archery; weightlifting; swimming
(continued)

30. Adapted Sport (continued)
Dwarfism
Dwarf Athletic Association of America
Track and field; swimming; basketball; bocce; equestrian; soccer; volleyball; team bocce; table tennis; powerlifting
(continued)

31. Adapted Sport (continued)
Les autres
BlazeSports National Disability Sports Alliance (BNDSA)—athletes compete among themselves
Track and field; swimming; volleyball; archery; bocce; shooting; cycling; table tennis; wheelchair team handball; powerlifting