Les Autres Conditions and Amputations

Chapter 24

Introduction

• Les autres - “the others” - denotes other locomotor disabilities besides SCI and CP • International Sports Organization for the Disabled • Enormous differences in persons in this category

Physical Activity Programming

• Participation in general physical education • Adaptations for mobility and other assistive devices may be needed • Selection of activities depends on condition

Sport Governing Bodies

• National Disability Sports Alliance – Individuals of all ages whose disability requires motorized chairs, crutches, or canes for participation • Dwarf Athletic Association of America – Children and adults with dwarfism • Disability Sports/USA – Winter sports for all disabilities – All sports for athletes with amputations and les autres conditions

Muscular Dystrophies

• Genetically determined conditions • Progressive muscular weakness - changes in muscle fibers • Common in school-age children • More prevalent in boys • Increase susceptibility to heart disease

Duchenne Muscular Dystrophy

• Most common and most severe • Generally has early onset and premature death • Various indicators • Hypertrophy • Within 7 to 10 years contractures occur • Ages 10 to 15 lose capacity to walk

Facio-Scapular-Humeral Type

• Most common form in adults • Same prevalence in males and females • Average lifespan and condition may arrest • Various indicators – Progressive weakness of shoulder and arm – Progressive weakness of face muscles – May affect hip and thigh muscles

Limb Girdle Type

• Occurs any time after age 10 • Same prevalence in males and females • Earliest symptom difficulty raising arms • May occur first in hips and thigh muscles • Both upper and lower extremities involved • Slow progression

Progressive Muscle Weakness

• Daily upright posture and walking is critical • For nonambulatory stretching and breathing exercises are essential • Generally follows rapid deterioration • Level of intensity is controversial • Full participation in early stages • Adjustment to wheelchair activities

Stages of Muscular Dystrophy

• Ambulate with mild waddling gait and lordosis • Ambulate with moderate waddling gait and lordosis • Ambulate with moderately severe waddling gait and lordosis • Ambulate with severe waddling gait and lordosis • Wheelchair independence • Wheelchair with dependence • Wheelchair with dependence and back support • Bed patient, can do no ADL without maximum assistance

Multiple Sclerosis

• Inflammatory disease of the CNS • Variable symptoms and patterns • Cause is unknown • Scar tissue replaces disintegrating myelin • More frequent in females • Heat and humidity intensifies problems

Course of MS and Programming

• Advanced stages - loss of bladder or bowel control occurs as well as difficulties of speech and swallowing • Severe intention tremors interfere with writing, using eating utensils, and motor tasks • Prognosis varies greatly • Optimal amount of exercise is unknown • Water exercises (cool temperatures, walking, and slow gentle stretching

Friedreich’s Ataxia

• Inherited condition - progressive degeneration of the sensory nerves of the limbs and trunk resulting in diminished kinesthetic input • First occurs between ages 2 and 25 • Degeneration may be slow or rapid • Primary indicators include ataxia (poor balance), clumsiness, and slurred speech

Guillain-Barré Syndrome

• Transient condition of progressive muscle weakness cause by inflammation of the spinal and cranial nerves • Weakness, sometimes followed by paralysis first affects the feet and lower legs, then the upper legs and trunk, and eventually the facial muscles • Most make a complete recovery

Charcot-Marie-Tooth Syndrome

• Most common hereditary neurological disorder that appears between the ages of 5 and 30 • Weakness in the peroneal muscles gradually spreads to the posterior leg and to the small muscles of the hand • Causes foot drop, which characterizes the steppage gait • Caused by demyelination of spinal nerves and motor neurons in the spinal cord • Progressive but may arrest itself

Spinal Muscle Atrophies of Childhood

• Major indicator is flaccid muscle tone floppy baby • Progressive degeneration of the spinal cord’s motor neurons • Severe cases result in loss of muscle strength, tightening of muscles, contractures, and nonuse • Stretching exercises are essential

Programming for Muscular Weakness Conditions

• Avoid activities that cause fatigue or pain • Increase rest periods • Use interval training • Allow personal choices • Control temperature and humidity • Be patient • Introduce wheelchair sports early • Use lots of partner activities

Thermal Injuries

• High incidence of injuries • Mortality highest under 5 and over 65 • May result in amputations • Increasing rate of survival • Scarring causes them to look different

Scar Tissue

• May cause contractures across joints limiting ROM • Jobsts may be worn to reduce hypertrophy • Isoprene splints or braces are also common • Should not limit participation

Program Implications

• Learn tolerance of new skin • Considerations for hydration • Emphasis on flexibility • Endurance and strength objectives • Dance and aquatic activities

Arthritis

• Rheumatism – a whole group of inflammatory disorders affecting muscles and joints • Arthritis – inflammation of the joints

Adult Rheumatoid Arthritis

• Rheumatoid arthritis – Affects all ages, usual onset between 20 and 50 years – Three times more common in women than men until age 50 – Most troublesome early in the day – Aching and stiffness are relieved by gentle exercise

Osteoarthritis

• Osteoarthritis – Mainly affects persons age 50+ – Major cause of disability in the older population – Advanced cases treated with joint replacements – Pain is associated with use or weight-bearing and worsens throughout the day – Nonweight-bearing exercises

Arthritis

• Joint problems include pain, swelling, heat, redness, decreased ROM and related muscle weakness • NSAIDS used to reduce inflammation and pain • Exercise using the 2-hr pain principle • Emphasis on flexibility, ROM, and strength

Juvenile Rheumatoid Arthritis

• Average onset is 6 years • Affects girls more than boys • Etiology is unknown • Onset may be sudden or progressive • May be systemic or peripheral • May affect the knee, ankle, foot, wrist, hand, arm, hip, and/or spinal column

Juvenile Rheumatoid Arthritis

• Generally not fatal • May cause severe disability • Functional recovery is possible • May be acute periods of illness followed by partial or total remission

Program Implications

• Goals include – Relief of pain and spasm – Prevention of flexion contractures and other deformities – Maintenance of normal ROM for each joint – Maintenance of strength - extensors • Activities designed to minimize pull of gravity

Program Implications

• Various contraindicated activities – Cause trauma to the joints or increase risks of falls • Swimming and creative movement are recommended - extension activities • Medications may inhibit normal growth • Peer adjustment may be a concern

Arthrogryposis

• Nonprogressive congenital contracture syndrome • Characterized by dominance of fatty and connective tissue at joints in place of normal muscle tissue • Varies in level of severity • Major disability is restricted ROM • Programming similar to arthritis

Dwarfism and Short-Stature Syndromes

• Dwarf Athletic Association of America – Criteria is 5’ or less • Little People of America – Criteria is 4’ 10” or less • Caused by a genetic condition or some kind of pathology

Disproportionate Dwarfs

• Disproportionate dwarfs – typically have average-sized torsos but unusually short arms and legs – Major cause is skeletal dysplasia or chondrodystrophy - the failure of cartilage to develop into bone – Inherited or caused by spontaneous gene mutation

Proportionate Dwarfs

• Proportionate dwarfs – persons whose body parts are proportionate but abnormally short – Main cause is pituitary gland dysfunction, (growth hormone deficiency) – Many causes can now be treated with growth hormones

Achondroplasia and Hypoachondroplasia

• Achondroplasia – the most common form of dwarfism – disproportionate body structure - average-size trunk, short limbs and in many cases a relatively large head – Associated problems include lumbar lordosis, waddling gait, restricted elbow extension, and bowed legs • Hypoachondroplasia - tallest dwarfs

Diastrophic Dysplasia

• Diastrophic dysplasia – most disabling of the common forms of dwarfism – Usually involves spinal deformity, clubfoot, hand deformities, and frequent hip and knee dislocations – Resistant to corrective surgery

Spondyloepiphyseal Dysplasia

• Abnormal development of the growth plates within the vertebrae • Disproportionately short trunk with various spinal and limb irregularities • Eye complications are common

Program Implications

• Disadvantage in most sports except powerlifting and tumbling • DAAA - various sports • Concerns include spinal stenosis, atlantoaxial instability, and joint defects • Compete in les autres internationally • Classification is an issue at Paralympics

Short Stature and Average or Better Intelligence

• Intelligence and mental functioning is same as in average-sized population • Syndromes characterized by average or better intelligence – Turner syndrome – Noonan syndrome – Morquio syndrome

Short Stature and Mental Retardation

• Short stature is a characteristic of several mental retardation syndromes – Down syndrome – Cornelia de Lange syndrome – Fetal alcohol syndrome – Hurler’s syndrome – Rubella syndrome

Osteogenesis Imperfecta

• Inherited condition present at birth • Bone and cartilage soft and brittle; skin and ligaments are overly elastic and hyperextensible • Bones easily fracture and joints easily dislocate – Peaks between 2 and 15 years of age – Participation in motorized wheelchairs – After condition arrests participation can increase

Ehlers-Danlos Syndrome

• Inherited condition that predisposes joints to dislocations but not bone breaks • Loose and hyperextensible skin, slow wound healing with inadequate scar tissue, fragility of blood vessel walls • High risk sports are contraindicated • Blister prevention and hand protection

Childhood Growth Disorders

• Osteochondroses – growth plate disorders • Disturbance in the normal growth of the epiphysis • Bony center is softened and may deform • Generally condition arrests itself over a period of several years • May lead to permanent deformity and predisposes individual to arthritis

Osgood-Schlatter Condition

• Temporary degenerative condition of the tibial tuberosity • Partial separation of the growth plate from the tibia brought on by overuse or trauma • Treatment may be temporary immobilization • Avoid explosive knee extension or all knee extension, running, and jumping

Perthes’ Condition

• Destruction of the growth center of the hip joint • Occurs between the ages of 4 and 8 and lasts 2 to 4 years • Hip joint must be protected during the body’s natural repair process to decrease chance of permanent damage

Slipped Femoral Epiphysis

• Hip joint disorder • Attributed to trauma, stress, or overuse • Occurs in 11 to 16-year-olds and is associated with obesity • Generally corrected surgically • Restrict weight-bearing activities • Swimming and upper extremity sports

Scheuermann’s Disease

• Disturbance in growth of thoracic vertebrae • Results from epiphysitis and/or osteochondritis • One or several vertebrae are involved • During the active phase, forward flexion is contraindicated • Condition may be relatively pain-free

Scoliosis and Chest Deformity

• Lateral curvature of the spine • Treated by surgery, casting, and braces • Forward flexion of the trunk is generally contraindicated • May be associated with other conditions • Breathing and ROM exercises

Congenital Dislocation of the Hip

• Dysplasia - abnormal development of the hip socket and/or head of the femur • More common in girls • Partial dislocation to complete dislocation • Nonsurgical treatments • Surgical treatments • Problems may be psychological

Pathological Dislocation of the Hip

• Associated with polio, spina bifida and CP • Head of femur displaced upward and anteriorly • Associated with coxa valga and hip adduction contracture • Average age of occurance is 7 years • Corrected by surgery

Clubfoot

• Talipes equinovarus • Most common orthopedic defect • Foot is inverted, heel is drawn up, and forefoot is adducted • Child walks on outer border of the foot • Treatment is manipulation, bracing, and casting - surgery is a last resort

Other Types of Talipes

• Talipes cavus • Talipes calcaneus • Talipes equinus • Talipes varus • Talipes valgus • Each can also coexist like ‘equinovarus’ • Vary in degrees of severity

Program Implications

• Treatments may delay normal development • Gaits vary widely and may change from early in the day to later in the day • May not impair gait • Eligible for wheelchair sports

Limb Deficiencies and Amputations

• Limb deficiencies – Congenital amputations – Classified as les autres conditions • Acquired amputations • Prevalence is difficult to determine

Types of Limb Deficiencies

• Many different types • Two broad categories – Dysmelia - absence of arms or legs – Phocomelia - absence of middle segment of limb, but with intact proximal and distal portions • Unknown causes • Compete with and without prostheses

Prostheses

• Substitute for a missing body part • Age of fitting impacts development of motor skills • Postsurgical prosthetic fitting and training • Numerous models of prostheses depending on needs of individual • Tony Volpentest

Gaits and Movement Patterns

• Double-leg amputations - walk on stumps, use prosthetics, or use wheelchairs • Single-leg amputations - use prosthetics • Above-the-knee - hydraulic device in knee • Running gaits – Hop-skip running – Leg-over-leg running

Balance

• Kicking - use natural limb • Ascending stairs - lead with sound limb • Descending stairs - lead with prosthesis • Above-the-knee – Balance more difficult – Stairs - may need a crutch or use railing • Holding objects may upset balance

Reduced Cooling Surfaces and Perspiration

• Reduced skin surface affects process of cooling • Increases perspiration in the rest of body • Considerations for clothing and temperature • Stump and prosthesis hygiene • Hydration is essential

Skin Breakdown on Stump

• Proper socket fit is essential • Utilize porous materials for covering • Prevent sunburn • Keep clean and dry

Muscle Atrophy, Contractures, and Posture Problems

• Muscle atrophy and contracture prevention – Daily strength training - focus on antagonists – Daily range-of-motion exercises • Correct postures should be emphasized – Distribution of weight equally – Inefficient movements may lead to early-onset arthritis

Increased Energy Expenditure

• Prostheses may be heavy and increase energy requirements • Loss of muscle mass decreases number of muscles available • Obesity complicates the problem • Wheelchairs may requires less energy • Motivation and support are essential

Acquired Amputations

• Most often in adults as a result of injuries, diabetes and circulatory problems • In children trauma and cancer are most common causes • Arnie Boldt • Terry Fox • Chris Coy

Degree of Severity

• Adjustment is difficult • May be concurrent injuries or health problems • Sports - amputations are considered minimal disabilities • Considerations are same as congenital amputations

PE Adaptations for Persons with Amputations

• Adjustment in dress for physical education • Waive shower rules if necessary • Provide privacy for changing • Few adaptations in activities should need to be made

Sports Programming

• As similar to peers as possible • Opportunities for disability sport • Knowledge of prostheses and wheelchairs • ISOD and Disabled Sport/USA • Regulations for use of prostheses and orthoses vary by sport

Amputee Sport Classifications

• General terminology – AK - above or through the knee joint – BK - below the knee, but through or above the ankle joint – AE - above or through the elbow joint – BE - below the elbow, but through or above the wrist joint

Amputee Sport Classifications

• Class A1 = Double AK • Class A2 = Single AK • Class A3 = Double BK • Class A4 = Single BK • Class A5 = Double AE • Class A6 = Single AE • Class A7 = Double BE • Class A8 = Single BE • Class A9 = Combined lower leg + upper limb amputations

Sports

• Track and Field – Compete as ambulatory, with and without prosthesis, and using wheelchairs • Sitting and Standing Volleyball – Amputee sport – Athletes classified as A-B-C (from most to least physically able) – Lower net for sitting volleyball

Sports

• Swimming – Functional classification system - various disabilities compete against each other – No prostheses are permitted – Need to adjust for changes in center of gravity and center of buoyancy that affect swimming strokes

Sports

• Horseback riding – Utilize specially made saddles • Cycling – Propel with and without prosthesis • All-terrain vehicles – Allow access to outdoor areas for camping, fishing, and hunting

Winter Sports

• Skiing learned early • Ski with or without prosthesis or using mono-skis • Alpine events include slalom, giant slalom, super giant slalom, and downhill • Cross-country events include both classic and freestyle events