Athletes with neuromotor and sensory disorders (Table 35-1) have a wide range of abilities.1–7 The World Health Organization International Classification of Functioning, Disability, and Health (ICF) terminology is summarized in Table 35-2.8–10

The older terminology defined a handicap as a disadvantage for a given individual, resulting from impairment or a disability that limits or prevents the fulfillment of a role that is normal (depending on age, sex, and social as well as cultural factors) for that individual. Americans with Disabilities Act defines a disability as an impairment that limits a major life activity; either a record of such an impairment in the preparticipation or physician’s notes, or a perception by the public that an impairment limits major activity of life, are also considered as evidence of disability.11

The term, adapted sport, refers to a sport that is modified or especially designed for an athlete with disability.4,5 The athlete may either participate with others who have no disabilities (integrated settings) or only with other athletes with disabilities (segregated settings). Paralympic Games (Table 35-3) include athletes who have physical disabilities or visual impairment, whereas Special Olympics is a sports training and competition program for persons with intellectual disability (mental retardation) age 8 years and older, irrespective of their abilities.4,5,12 Athletes with other disabilities such as those with muscular dystrophy, multiple sclerosis, chronic juvenile arthritis, osteogenesis imperfecta, ataxia, are all categorized as les austres (meaning “others”).

Persons with disability should be referred to appropriately so as to maintain respect and dignity. A preferred way to address these individuals is the “person first” approach, e.g. a person with cerebral palsy instead of a cerebral palsy victim; in this manner, one refers to a person with a disability rather than to a disabled person.

An estimated 12% of the school-aged children in the United States are physically challenged; there are approximately 40 million physically challenged persons in the United States.1,13,14 It is estimated that there are more than three million individuals with physical and mental disabilities involved in organized athletic competition in the US; and many more in recreational sports. Participation opportunities for the physically and mentally challenged athletes have increased over the past several decades with between 3000 and 6000 Paralympic athletes (Table 35-4) participating at various levels of competition. Several investigators have analyzed the epidemiology of musculoskeletal injuries in athletes with physical and sensory disabilities and key findings are summarized in Table 35-5.15–30

Pathophysiology and clinical presentations differ depending upon specific disorder or physical disability. For ease of discussion, key elements of pathophysiology, clinical presentations, and major medical concerns (Table 35-6) for specific disorders are reviewed together below with comments on relevant and any unique aspects of management.31–36

Spinal Cord Injury (SCI)

Fortunately, spinal cord injuries in children and adolescents are not common; however, they have significant lifelong consequences for independent living as well as sport participation. These athletes are predisposed to injuries related to use of wheelchairs, prostheses, and other adaptive devices, not unlike other athletes who are wheelchair bound.28,37–39 Individuals with SCI are at risk for specific medical problems related to loss of motor and sensory function as well as lack of control of autonomic function (dysautonomia) below the level of the lesion, including impaired thermoregulation and autonomic dysreflexia.40–56

Thermoregulation

Temperature regulation is impaired in athletes with spinal cord injury, especially with lesions above T8. Both, hyperthermia and hypothermia have been reported to be serious problems in these athletes. Impaired sweating below the lesion level reduces the effective body surface area available for evaporative cooling. There is also venous pooling in lower limbs, and decreased venous return, which also reduces heat loss by convection and radiation. This can lead to increased body temperature and hyperthermia. Certain medications (e.g., anticholinergics) taken by these athletes can also increase the risk of hyperthermia.

On the other hand in cooler conditions, such as swimming, there is an increased risk for hypothermia. Impaired vasomotor and sudomotor neural control, decreased muscle mass below the lesion, and possible impaired central temperature-regulating mechanisms, all contribute to the development of hypothermia. There is a lack of shiver response below the level of the lesion. These athletes also lack sensation below this level and thus may not be aware of wet clothes. Problems with appropriate temperature regulation can occur even within milder ambient temperature ranges. Adequate hydration must be maintained, and the athlete should be removed from sports activity at the first sign of any problem. Awareness of these issues and education of athletes and coaches are key to prevention.

Autonomic Dysreflexia

Autonomic dysreflexia has been known to occur in athletes with spinal cord injuries above T6. There is a loss of inhibition of the sympathetic nervous system, which leads to an acute uncontrolled sympathetic response manifested by sweating above the lesion, chest tightness, headache, apprehension, acute paroxysmal hypertension, hyperthermia, cardiac arrhythmias, and gastrointestinal disturbances. A number of stimuli below the level of the lesion can trigger such a response; these include urinary tract infection, bladder distension, bowel distention, pressure sores, tight clothing, and acute fractures. Awareness of the potential for autonomic dysreflexia is the key to prevention. At the first signs of this syndrome the athlete should be removed from the sports activity, any recognized offending stimulus should be eliminated, and the athlete should preferably be transported to an emergency facility for further management. In most cases this is a self-limited response; however, persistent hypertension and cardiac arrhythmias can occur.

A phenomenon of self-induced autonomic dysreflexia, known as ‘boosting,’ has been recognized over the past several years, especially in wheelchair athletes seeking to improve their race times. These athletes will knowingly trigger such a response, by inducing a trigger such as distending the bladder. They may drink large amount of fluids, strap legs very tightly, or clamp their catheters to induce bladder distention. Self-induced lower leg fractures have also been reported. The exact mechanism of performance enhancement effects is not known; however, it is hypothesized that it is in part caused by increased blood flow to working muscles, and glycogen sparing caused by increased utilization of adipose tissue which is induced by increased catecholamines. This has been shown to enhance performance and reduce race time and give the athlete an advantage. It is important to recognize that self-induced dysreflexia poses serious health risks for the athlete and this practice is considered an ergogenic aid which is not sanctioned by sport governing bodies.

Meningomyelocele

Children with meningomyelocele are at an increased risk for obesity (prevalence of up to 75%) for whom participation in sports and other physical activities is especially therapeutic.1 Approximately 75% of these lesions occur at the lower lumbar and sacral levels with loss of motor and sensory function below the lesion level. The presence of hydrocephalus can adversely affect cerebral function; increased intraventricular pressure and dilatation can damage the motor cortex and lead to development of spasticity above the level of the lesion.1–3,32 Children with meningomyelocele also have deficits in both hand to eye and foot to eye coordination. They have decreased aerobic power, decreased endurance, decreased peak anaerobic power, and mechanical inefficiency similar to others with neuromuscular disorders.4,51–53 The level of the lesion and severity of hydrocephalus are important factors influencing the ability to participate in sports.2,10 Children with meningomyelocele are categorized according to the functional level of the spinal cord lesion.

Because of poor soft tissue support, increased local pressure and lack of sensation below the lesion level, these children are prone to develop localized skin breakdown, with resultant pressure sores and ulcers. They are also at an increased risk for ligament sprains because of lack of strong musculotendinous units around the involved joints. Decreased muscle strength and strength imbalance increase the risk for muscle strains in these athletes. Children with meningomyelocele lack the appropriate loading of their bones because of the lack of weight-bearing activities; this, often combined with nutritional inadequacy, may lead to osteopenia and increased risk for fractures. Fractures may occur following minimal trauma and may initially be mistaken for localized infection because of erythema and swelling. Because of lack of sensation, these athletes do not feel pain, further delaying the diagnosis of a fracture.

Bowel and Bladder Control

Children with meningomyelocele, spinal cord injuries, and other neuromuscular disabilities have difficulty with bladder control (neurogenic bladder) and bowel control (neurogenic bowel). Different bowel and bladder routines, accidents, and odor may cause embarrassment for the child. In the context of sports participation, the athlete may be too preoccupied with the sport to adhere to a prescribed bladder or bowel regimen. Some athletes may be on a scheduled voiding regimen, requiring clean intermittent catheterization, or may have an indwelling catheter. There is also the problem of access to appropriate facilities in a timely fashion. These factors and others (as inadequate hydration) lead to an increased risk for urinary tract infections in these athletes. A regular regimen of voiding, ensuring adequate hydration (before, during, and after the sports activity), and using appropriate sterile voiding techniques will prevent urinary retention and associated complications. In addition to a neurogenic bladder, these athletes also have problems with constipation and stool retention that require following a regular bowel regimen.

Latex Allergy

The prevalence of latex allergy in children with myelomeningocele is between 60% and 70%.35,36,57 Thus, latex allergy is an important consideration while working with athletes who have spina bifida. This information should be ascertained from the athlete or the family, so that during medical emergency one can avoid using latex gloves with these athletes. Other articles containing natural rubber latex should also be avoided. Sources of latex in the medical setting include gloves, stethoscope tubing, blood pressure cuffs, catheters, wound drains, bandages, bulb syringes and others; household sources include balloons, condoms, shoe soles, erasers, some toys, sport equipment, and others.

Hydrocephalus and Shunt

The presence and severity of hydrocephalus and a ventriculoperitoneal (VP) shunt in children with meningomyelocele are major factors affecting the functional level and ability of these athletes to participate in sports. The VP shunt system is generally protected under the skin; however, it is at risk of injury if the overlying skin sustains sufficient impact to cause a laceration. Such an injury requires immediate evaluation by a neurosurgeon. Athletes with cerebrospinal fluid shunts are not necessarily restricted from sport participation simply because of the presence of this shunt. They should wear appropriate helmet/headgear for protection.

Associated Conditions

Children with the associated Arnold-Chiari type 2 malformation should be restricted from activities that have significant risk of injury to the cervical spine; these include sports such as diving and football. Children with progressively worsening extremity strength, scoliosis, and bowel and bladder function should be evaluated for possible hydromyelia and tethered cord. These athletes should be restricted from further sports participation until after appropriate orthopedic and neurosurgical intervention and reassessment of their functional abilities. Examples of high-risk sports for children with spina bifida include football, cheerleading, scuba diving, water skiing, polo, and bobsledding.

Cerebral Palsy

Cerebral palsy is characterized by spasticity, athetosis, and ataxia. Fifty percent of athletes with cerebral palsy participate in wheelchair sports and the other 50% are ambulatory. Multiple factors affect the ability of athletes with cerebral palsy to optimally perform in sports, influence the risk for injury, and can have implications for developing training programs for athletes with cerebral palsy (Table 35-7).58–73

Athletes with cerebral palsy are at increased risk for overuse syndromes, muscle strains, chronic knee pain, patellofemoral problems, and chondromalacia patellae. Progressively decreased flexibility of hamstrings and quadriceps contributes to proximal patellar migration. Normal hip development is affected because of decreased flexibility and increased spasticity around the hips. This eventually contributes to the development of coxa valga, acetabular dysplasia, and hip subluxation. Hip flexion contractures and tight hamstrings can lead to increased lumbar lordosis, chronic back pain, and spondylolysis. Some athletes find it difficult to control rackets and bats because of impaired hand–eye coordination; athletes with perceptual problems may also have difficulties in throwing and catching. Many will develop ankle and foot deformities affecting sport participation and requiring orthopedic management. The presence of tonic neck reflexes can adversely affect effective development of certain sport skills, such as use of bats, hockey sticks, or rackets.

Athletes with cerebral palsy benefit from carefully designed conditioning programs which should include appropriately supervised strength training and flexibility exercises. Strength training should take into account the differential tone and spasticity in different muscle groups so that the training is directed to appropriate muscle groups to optimize muscle balance. Stretching, started after a period of warm-up, should be slow and sustained to prevent activation of stretch reflex. Specific training can also help improve ataxia and coordination.

Wheelchair Athletes

Wheelchair athletes include those with cerebral palsy, spina bifida, and spinal cord injuries. Sports with descending order of injury risk for wheelchair athletes are track, basketball, road racing, tennis, and field events. In wheelchair athletes, overuse injuries are the most common injuries, shoulders and wrists are the most frequently injured regions and74–76 shoulder pain is a common complaint; specific shoulder injuries include rotator cuff impingement, rotator cuff tendonitis, biceps tendonitis, and tear of the long head of biceps tendon. Soft tissue injuries (most commonly seen in track, road racing, and basketball) include lacerations, abrasions, and blistering affecting arms and hands. Peripheral entrapment neuropathies is common in wheelchair athletes, the most common of which is the carpal tunnel syndrome, reported in 50% to 75% of the athletes.74–76 In athletes with spinal cord injuries and meningomyelocele, painless hip dislocations can occur. Some athletes may develop progressive neuromuscular scoliosis limiting their cardiorespiratory capacity.

Pressure Sores

Wheelchair athletes with spinal cord injuries and myelomeningocele are especially at risk for developing pressure sores. The wheelchair athlete’s knees are at a higher level than the buttocks, a position that leads to increased pressure over the sacrum and ischial tuberosities for prolonged periods of time. Because of lack of pain and touch sensation, skin lesions remain asymptomatic. With delay in recognition, pressure sores can become infected. Thus, frequent, meticulous skin examinations are necessary for early detection of problem pressure areas. Any sores must be promptly treated to prevent complications. There must be adequate local padding to relieve pressure. The athlete should have appropriate chair size and fit, and should be educated to frequently change position.

Athletes with Limb Amputations

Use of assistive/adaptive devices, prostheses, and orthoses is common in athletes with limb amputations; these devises should be of proper fit and checked and adjusted regularly as the physical growth of the child or adolescent progresses.37–39 Sports governing bodies have rules allowing or disallowing participation of athletes with prosthetic devices; currently, high school interscholastic athletics allow athletes to wear these devices in many sports including football, wrestling, soccer, and baseball. The factors considered include the type of amputation and prosthesis as well as the potential for harm to others or unfair advantage for the athlete because of the prosthetic device. Prostheses can increase local skin pressure and contribute to abrasions, blisters, and skin rash. Prepatellar, infrapatellar and pretibial bursitis in the below-knee amputee can result from socket irritation.