Transcript Active, Healthy Lifestyles for All: Thinking About Philosophy

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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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