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

Amputations Congenital Acquired Educational needs—psychosocial

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

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.

Dwarfism 4 ft, 10 in. (147 cm) or less Term little people preferable Classification Proportionate Disproportionate (most common)

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

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

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)

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

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)

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)

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)

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.

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)

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)

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

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)

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

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)

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

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.

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

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.

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)

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.

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)

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.

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.

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)

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

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