Pediatric Annals

A Developmental Perspective for the Rehabilitation of Children with Physical Disability

Gabriella E Molnar, MD

Abstract

In the human life cycle, childhood is the age when both physical and mental abilities undergo the most remarkable evolution.1,2 To prepare a child with a physical disability for optimal functioning in adulthood, the rehabilitation process must consider the changing needs and concerns created by different stages of growth and development.3,4

Discussing theoretical issues of development in motor skills, Wolff stated that "controlled movements are our only means to maintain posture against gravity, for moving in space, changing the environment, and communicating by speech and gesture. Perception ot controlled movement is our only source of concrete knowledge about what others are doing, thinking, or feeling."5 These observations place the significance ot motor behavior in a perspective that surpasses the physical act of movement and provide a broad conceptual rationale for efforts to remediate motor dysfunction.

Despite advances in neurosciences, many aspects of the complex neurophysiologic mechanisms of volitional movement control are unclear. Even less is known about the events that take place in the intact or damaged central nervous system as a result of motor training. Therefore, it is not surprising that therapeutic exercises proposed for neuromuscular dysfunction of central origin are based on empirical observations rather than experimentally established neurophysiologic principles.6-8 The treatment options and methods of choice for cerebral palsy, in particular, remain controversial due to lack of properly controlled clinical trials, despite the evident medical and economic significance of these issues.9-11 As discussion of these issues and specific techniques of motor training are beyond the scope of this article, the reader is referred to other sources of information on the subjects.6-8 This article intends to outline the principles of rehabilitation as they are applied at different ages to children with physical disability.

Achievement of the highest realistically expected physical independence and prevention of musculoskeletal deformities are the two principal aims of treating motor disabilities at any age. In childhood, functional therapeutic goals are adapted to developmental stage and expectations modified by the presenting dysfunction. Preventive intervention is a continuing concern over the growing years with increased emphasis during the rapid phases of skeletal growth and may become a primary aim when functional goals have been achieved. Differences in the objectives of rehabilitation are reflected by variable intensity of therapy and by the changing modes of management at various ages.

In pediatrics, all forms of treatment involve the family. The significance of the triadic relationship of child, family, and physician or other professional care givers, is enhanced when physical disabilities that may have a pervasive influence on all aspects of child rearing are present.12-14 A rehabilitation program cannot succeed without the parents' participation and encouragement of the child's independence in real life situations. An important and often difficult task of the physician is to counsel the parents that undue preoccupation with physical abilities should not distract attention from nurturing the child's cognitive, emotional, and social development15 and that as priorities dictated by these considerations change over the growing years, so do methods of management for motor deficits.

INFANTS AND TODDLERS

In the first two years of life, motor development progresses from complete dependence to efficient bipedal locomotion; from undifferentiated reflex grasp to volitionally coordinated, skillful prehension. Treatment for physical disabilities intends to simulate early motor milestones to the extent feasible. Therapeutic exercises at this age are delivered in the form of handling, moving, and positioning to induce active motion on the part of the infant. It is hoped that in response to these handling techniques the infant will initiate movements necessary for acquiring postural control and coordination or for increase in strength, which will then lead to the…

In the human life cycle, childhood is the age when both physical and mental abilities undergo the most remarkable evolution.1,2 To prepare a child with a physical disability for optimal functioning in adulthood, the rehabilitation process must consider the changing needs and concerns created by different stages of growth and development.3,4

Discussing theoretical issues of development in motor skills, Wolff stated that "controlled movements are our only means to maintain posture against gravity, for moving in space, changing the environment, and communicating by speech and gesture. Perception ot controlled movement is our only source of concrete knowledge about what others are doing, thinking, or feeling."5 These observations place the significance ot motor behavior in a perspective that surpasses the physical act of movement and provide a broad conceptual rationale for efforts to remediate motor dysfunction.

Despite advances in neurosciences, many aspects of the complex neurophysiologic mechanisms of volitional movement control are unclear. Even less is known about the events that take place in the intact or damaged central nervous system as a result of motor training. Therefore, it is not surprising that therapeutic exercises proposed for neuromuscular dysfunction of central origin are based on empirical observations rather than experimentally established neurophysiologic principles.6-8 The treatment options and methods of choice for cerebral palsy, in particular, remain controversial due to lack of properly controlled clinical trials, despite the evident medical and economic significance of these issues.9-11 As discussion of these issues and specific techniques of motor training are beyond the scope of this article, the reader is referred to other sources of information on the subjects.6-8 This article intends to outline the principles of rehabilitation as they are applied at different ages to children with physical disability.

Achievement of the highest realistically expected physical independence and prevention of musculoskeletal deformities are the two principal aims of treating motor disabilities at any age. In childhood, functional therapeutic goals are adapted to developmental stage and expectations modified by the presenting dysfunction. Preventive intervention is a continuing concern over the growing years with increased emphasis during the rapid phases of skeletal growth and may become a primary aim when functional goals have been achieved. Differences in the objectives of rehabilitation are reflected by variable intensity of therapy and by the changing modes of management at various ages.

In pediatrics, all forms of treatment involve the family. The significance of the triadic relationship of child, family, and physician or other professional care givers, is enhanced when physical disabilities that may have a pervasive influence on all aspects of child rearing are present.12-14 A rehabilitation program cannot succeed without the parents' participation and encouragement of the child's independence in real life situations. An important and often difficult task of the physician is to counsel the parents that undue preoccupation with physical abilities should not distract attention from nurturing the child's cognitive, emotional, and social development15 and that as priorities dictated by these considerations change over the growing years, so do methods of management for motor deficits.

INFANTS AND TODDLERS

In the first two years of life, motor development progresses from complete dependence to efficient bipedal locomotion; from undifferentiated reflex grasp to volitionally coordinated, skillful prehension. Treatment for physical disabilities intends to simulate early motor milestones to the extent feasible. Therapeutic exercises at this age are delivered in the form of handling, moving, and positioning to induce active motion on the part of the infant. It is hoped that in response to these handling techniques the infant will initiate movements necessary for acquiring postural control and coordination or for increase in strength, which will then lead to the emergence and eventual mastery of the basic gross and fine motor skills. The central nervous system dysfunction of cerebral palsy is characterized by abnormal tone, posture, and movement patterns that are enhanced by gravity and position in relation to space.16 Treatment of this motor deficit entails avoiding those positions and manners of handling that enhance the abnormal motor behavior, thus enabling the infant to move with greater ease.6 Muscle weakness is the major presenting problem in myopathies and diseases of the lower motor neuron where the aim is to help gain strength through active use. Because movements occur to fulfill an intentional purpose, therapeutic exercises for infants and toddlers are incorporated into functional activities,4 for example, reaching to retrieve a desirable object or crawling or cruising to approach a trusted person.

Feeding difficulties arising from supranuclear incoordination or weakness of the oral-pharyngeal muscles may accompany neuromuscular deficits and occur with greatest frequency in cerebral palsy. Because these muscles also execute phonation and articulation, early feeding problems may herald deficits of speech production at a later age. Intervention for dysfunctional swallowing, chewing, and oral motor coordination has a role equal to gross and fine motor training and constitutes an integral part of early treatment.17

Another approach to functional rehabilitation is represented by environmental adaptations and assistive devices to facilitate mobility, access to surroundings and compensatory means for adaptive personal skills when a motor disability excludes independence in such activities.4·18 For infants with extensive muscle weakness and no head or trunk control, a seating device should be provided in the second half of the first year. In myelodysplasia or other neurologic lesions with complete lower extremity paralysis, a standing device is considered at around 12 to 18 months of age- ,y An infant with upper limb deficiency is fitted with the first simple passive prosthesis around 4 or 5 months of age to encourage emerging bilateral hand play and leaning on both arms in sitting.20 A functional terminal device that allows grasping is added to the prosthesis around 1 to Wi years. The amazing ability of older infants to learn to use mechanical devices by discovering cause and effect through trial and error, and eventually by mental insight, is illustrated in the reports about successful fitting with myoelectric prosthesis as early as 16 to 18 months of age.21 Also, a recent study demonstrated that developmentally disabled children were able to learn to operate a robotic arm at 6 to 7 months of mental age.22 Using normal motor development as a guideline, infants with lower extremity limb deficiency receive a prosthesis around 8 to 10 months in preparation for pulling to stand.20 Adaptations for mobility come in many forms for infants and toddlers who do not have adequate coordination or strength for walking. Scooter boards, crawling devices, and low hand-propelled carts for playing on the floor can assist in the preambulatory stages of mobility.18,25 Absence of sensation, such as in myelodysplasia, makes the use of these devices especially advisable to prevent trauma to the skin while the body is dragged along on the floor. When the prognosis for ambulation is limited, a wheelchair may be prescribed around 1 year of age. The new lightweight models and improved design features eliminated the previous mechanical problems, and enable children with adequate upper extremity function and cognitive abilities to achieve independent mobility during the second year of life. 18,25 A motorized wheelchair is an option when upper extremity function is compromised. Safe and successful operation of this device has been described during the second year of life, although supervision is required as for any other child at this age.25,24

Preventive intervention should already be attended at this age when the possibility of contractures and deformities is anticipated. Spastic or paralytic imbalance of opposing muscles at first leads to soft tissue contractures and eventually to deformities of the underlying joints and bones, especially while the epiphysial growth plates are not ossified. The risk of such complications exists in all long-standing neurologic diseases of childhood; cerebral palsy and spina bifida represent some notorious examples.16·26 Progressive, and at times insidiously developing, acquired musculoskeletal abnormalities may compromise long range functional outcome if preventive measures are overlooked or neglected. The methods used for preventive management include passive range of motion exercises, splints, or braces for stretching and for maintaining anatomic or corrective joint positions on function or at rest. Custom molded wheelchair and seat inserts are used for postural support in case of trunk weakness or malalignment.18

There are many articles that describe the rationale of early intervention for high risk infants.27'51 Those considerations apply to the infant with evident physical deficit. In concert with the earlier quoted statement by Wolff,1 a well designed program in the motor functional mode serves as a vehicle for developmental stimulation as it is combined with a variety of sensory stimuli, communication, and other forms of interaction. Active physical encounters and exploration offer opportunities for learning about body image, distinction of self from the surrounding world, the rules and components of space, object permanence, and cause and effect, which are accomplished in the sensorimotor stage of development. 32 Early motor training and developmental stimulation gain added importance in view of the increased incidence of associated handicaps in some types of neuromuscular dysfunction. The possibility of visual and hearing deficits that occur with greater frequency among disabled children should be investigated at this age, reassessed periodically, and corrected as necessary.

Instruction of the family in modified handling techniques for routine daily care and in methods for practicing physical activities that the infant or toddler already accomplished or is in the process of learning is an essential part of early treatment.433 It is not the intention to place the parents in the role of a therapist but to ensure that new functional skills are transferred in the child's own environment. Fulfilling the natural desire to help their child and the confidence that they understand the problem are psychological benefits for the family members in their adjustment and coping. A motor deficit that hampers physical separation can also delay the process of emotional separation and individuation. This delay may have a lasting effect on the child's personality development and secondarily on the family's style of child rearing.15,34,35

THE PRESCHOOL CHILD

Learning through play is an apt description of the major occupation of preschool years. Consistent with the preoperational stage of cognitive development, 32 therapeutic exercises are delivered in the form of play and games designed to learn new motor skills and refine those already accomplished.8 Intervention in this mode combines opportunities to practice a variety of developmental tasks with therapy for motor dysfunction.

For the preschool child, the desire to move about is a strong motivating force and a means to assert autonomy. Intervention to achieve independent mobility by walking or other compensatory ways is intensified for the same reason in children with physical disabilities. Ambulation training is not restricted to children who have a favorable prognosis for walking as a function in daily life. It is also offered when the potential for walking is limited to partial ambulation, short distances, or is intended as an exercise to increase strength, conditioning, agility, and endurance for physical activities. 19 Walkers, crutches, and braces are well known ambulatory aids. As a general guideline, crutch-walking is a compensatory developmental skill that may be expected of children at 2 to 3 years of mental age with good perceptual integration, physical stamina, and upper extremity coordination. The energy cost of ambulation with braces and crutches is much above that of normal walking. 36 The increased energy expenditure does not seem to be a deterrent for young children who have an intense motivation to walk. In spinal cord injury, for example, they often surpass ambulatory function projected for adults with similar neurologic lesions. Children who rely partially or completely on alternative ways of locomotion are trained to operate their wheelchairs safely and efficiently on various outdoor terrains and in different settings of their expanding sphere of function. Adapted hand-driven or foot-pedaled tricycles are fun to use. These devices provide benefits that cannot be measured in terms of speed alone for children who are otherwise unable to walk fast enough to keep up with their peers.

Development in both understanding and coordination enable the preschool child to learn dressing, eating with utensils, self-care, and other activities of daily life, including those that require manipulation of simple tools and implements. Training in these skills is a focus of treatment and should consider not only upper extremity function but also the possibility of associated deficits in cognitive and perceptual development. Impaired arm and hand control may call for simple adaptations of clothing, feeding utensils, and toys or for more elaborate devices, such as an electric feeder.18,37 Specially constructed seating arrangements for wheelchair or for sitting at a table can improve motor control of the arms by stabilizing the trunk. ,8· 38 In severe movement disorders or paralysis of the upper extremities, a head pointer or mouth stick can substitute for lost function. Turning the pages of a book or operating an electric toy or electric wheelchair are examples of application. Mouth sticks require a properly fitted mold to prevent dental malocclusion in the growing child.18 Inventiveness and technology can create functional aids or, as it has been suggested, a "wardrobe of devices"39 to overcome virtually any physical limitation.

Preventive intervention should continue throughout childhood. A standing table, or in cerebral palsy a prone board, can be set up as a place to play. While fulfilling a functional purpose, standing also helps to prevent lower extremity contractures. Progressive subluxation and eventual dislocation of the hips and scoliosis are two major musculoskeletal complications that can occur in cerebral palsy and in spina bifida, especially with mid to upper lumbar lesions.16,26,40,41 Orthopedic surgical procedures may be necessary at this age for progressive deformities. Elective surgery to improve function and cosmesis are most frequently performed for gait abnormalities.

Although cognitive functioning remains concrete in the preoperational stage of thinking,32 preschool children develop the ability to substitute verbal symbols and pictures for real life events and objects. A functional manifestation of this process is the increasingly complex use of language. In cerebral palsy when spastic or dyskinetic incoordination of suprabulbar origin impedes speech production, training in nonverbal communication should start. Receptive language is a prerequisite for such endeavors. Nonverbal communication devices range from simple pointing boards, which show pictures of objects and actions, to sophisticated electronic devices.42,41 Selection of an appropriate device depends on the child's language ability and communication needs in the context of daily activities. Communication, verbal or nonverbal, may be the only effective way severely disabled children can exercise control and assert themselves.

Home environment remains the primary venue in the young child's life where practice and functional application of achievements take place with encouragement from the family. Toilet training should be accomplished by children who have adequate mental abilities. In spina bifida or spinal cord injury with neurogenic bladder dysfunction and incontinence, teaching intermittent selt-catheterization can begin around 5 years of age. 26

Learning the basic rules of social behavior is a task of the preschool years. Expanding knowledge about the environment, experience with interaction, the ability to exercise control, and learning to accept limits affect the continuing process of individuation. Issues of emotional and personality development should be explored and addressed with the parents as thoroughly as the physical and organic aspects of function.44,45 Enrollment in a preschool program is strongly recommended for both educational and social reasons. A suitable program should offer adequate opportunities for challenge and success and may be a special educational or partially or fully integrated setting.

THE EARLY SCHOOL YEARS

Concern about physical abilities and the musculoskeletal system persists; however, from this age on there is a shift in emphasis to educational, social, and emotional growth.

In most cases, basic functional goals have been accomplished by the time a child enters school. Experience through consistent use leads to continuing improvement. Applied physical activities are more appropriate and enjoyable to school-aged children than straightforward therapeutic exercises and for the most part replace formal therapy.8 Traditional verbal conceptual instruction becomes an appropriate method of training with the achievement of concrete operational thought.32

By the early school years, children have generally reached the expected functional level and mode of ambulation whether by walking with or without assistive devices, a combination of using wheelchair and limited walking, or only wheelchair. Therapy for ambulation training is no longer needed at this stage as persistent practice in functional situations is the most effective exercise to increase proficiency. In the group with restricted walking capacity, daily schedule at home and school should include opportunities and incentives to maintain partial ambulation for any limited purpose, or as exercise for previously mentioned physiologic reasons. Less mobile children continue to use a standing table for work and play. A thoughtfully planned and adapted physical education program provides the chance to exercise with regularity in the company of friends.46 Temporary reinstitution of therapeutic exercises may become necessary for specific indications, for example to regain ambulation after lower extremity surgical procedures or to prevent loss in joint range of motion.

Rehabilitation engineering led to numerous applications of advanced technology in physical disabilities.47 Portable voice or word communicators,45 computers for written school assignments and for playing popular video games, which can be operated by switch control or other types of interface, allow previously unattainable independence in severe motor deficits. These sophisticated devices can assist in the broad spectrum ot personal, educational, and recreational needs of school-aged children and are appropriate for the child who demonstrates behavioral and cognitive readiness.

Development of the musculoskeletal system requires anticipatory monitoring. Soft tissue and joint deformities are less likely to occur in active children although hip and knee flexion contractures can develop in wheelchair users. The frequency of musculoskeletal complications is much greater in severely disabled dependent children and presents a problem tor which no easy solution exists. Scoliosis, hip dislocation, abnormal posture, and gait are the most common indications for surgical treatment.26,40,41

School is an environment for practicing daily activity skills and usually stimulates a desire to learn new ones. If the child uses adaptive techniques or devices, these should be explained and applied at school. Consultations for problem solving with subsequent practice at home and school or a limited course ot therapy may be necessary to meet the expanding variety and complexity of challenges in gross and fine motor function. Individualized educational plans contain provisions for supportive therapies at school with the intent ot enhancing the effectiveness of education. Inherent in this intent is that therapy should not conflict with educational priorities. Choosing the most suitable class is a decision based on the child's intellectual function. Physical abilities need consideration in order to provide the least restrictive environment but not for planning the academic curriculum. Children with central nervous system dysfunction may have deficits in perceptual organization, attention, and cognitive function calling for remedial education. The structured setting oí classroom and interaction with peers is also a milieu for learning social skills.45,48,49 This aspect of schooling should be stressed for children with physical disability whose social experience is restricted compared with that of their able-bodied peers.

Participation in adapted sports should be encouraged for fun and fitness.50,51 Swimming, skiing, horseback riding, and wheelchair basketball are among the most popular recreational activities, but virtually all sports can be modified to motor deficits and wheelchairs. Success and a sense of accomplishment are as important as the physical benefits derived from these activities. When planning extracurricular and recreational activities for growing children, keep in mind that adapted motor function, even a relatively mild gait abnormality, requires increased energy expenditure because the biomechanics of movement are less efficient.56 Adequate rest and caloric intake are needed to compensate for the different energy demands of growing children with physical disability.

The complex subject of emotional and social development can be touched upon only briefly, nonetheless emphatically. Consistent and appropriate expectations are essential to nurture the child's independence, drive, and determination.45 Disabled children need the same variety of experiences in social interaction as their able-bodied peers to acquire the skills of proper behavior and manners.12''4 Although the family has the central role in creating such opportunities, physicians must not abandon their responsibility to initiate discussions on these issues with the parents and directly with the child. Continuing guidance and sometimes specific remedial intervention may be necessary for the origin of social dysfunction described in some disabled adults stems from childhood.45

PREPUBESCENCE AND ADOLESCENCE

Accelerated growth, profound physiologic changes, development of abstract thinking, 52 a search for identity, and awakening interest in the opposite sex characterize this age.

Youngsters with mild deficit and high level of motor function sustain or expand their physical capabilities taking advantage of the growth-induced increase in strength and endurance. In more severe disabilities rapid growth enhances the tendency to develop progressive deformities. This risk increases in proportion with the extent of motor dysfunction. Scoliosis deteriorates at a fast pace during the adolescent spinal growth spurt regardless of function. Readaptation to sudden changes in body dimensions poses difficulties, especially in upright mobility, when muscle strength, coordination, and postural alignment are compromised. These factors may lead to regression or loss of marginal physical achievements. Nonfunctional ambulation for the purpose of exercise is usually abandoned at this age and reserved for unexpected emergencies. High energy cost, slow speed of progression, and the necessity to manage longer distances in a routine day also contribute to making limited and adapted ambulation less expethent than at a younger age. In the face of other interests and ambitions of teenagers, and at times to the dismay of parents, adolescents of restricted ambulatory capacity tend to rely increasingly on their wheelchairs or opt to use one in selected circumstances.16-26 When the possibility of this outcome is expected, the physician must prepare the family in advance to avert their disappointment and a potential source of conflict between the parents and teenager.

Prevention and treatment measures for threatening or progressive deformities depend on the extent of the problem and may consist of a maintenance home exercise program, splints, braces, and positional adaptations in sitting and in bed, or a course oí physical therapy for defined indications and duration.8 Surgical correction ot deformities may be elected to make a severely affected child more comfortable and easier to care for, or to prevent decubiti. However, maintenance ot postoperative gains in range of motion and body alignment are fraught with difficulties when active motor function is severely impaired.

The principles of preserving and improving physical abilities are the same as in early school years. They involve daily practice ot achievements, applied functional activities for increasing motor proficiency and fitness, problem solving assessments, or at times a limited course of therapy when new functional demands necessitate learning additional compensatory techniques. Environmental controls to operate light switches, doors, kitchen appliances, and other household items may complement the list of assistive devices in severe disabilities.47 Elimination of architectural barriers in schools, public buildings, and transportation is mandated by law. An accessible home environment is a prerequisite for independent wheelchair functioning.55 Orthopedic procedures to improve function have been generally performed prior to this age. However, surgical correction of scoliosis, stabilization of unstable or painful foot and ankle, or leg length equalization are among the procedures that may be warranted during the prepubescent or adolescent years.

Sports and indoor and outdoor recreational activities are a source of enjoyment and socialization for all adolescents and become the mainstay of needed physical exercise in motor disabilities.50,51 Special interests and hobbies should be stimulated for the sake of personal satisfaction and broadening knowledge and skills.

There is a continued emphasis on education in preparation for adult life and a career.45 Attainment ot the highest possible scholastic competence has increased importance for adolescents with physical disability as their future employment opportunities are contingent on these achievements. Vocational counseling can offer useful information about practical matters of career choice.45 Youngsters with limited intellectual ability need to be prepared for sheltered work and their families should learn about the available options. A major part of the adolescent's life centers around school, which is not only an environment for education but for establishing friendships and socializing. Unfortunately, as some authors observed, geographic integration in a regular class does not necessarily lead to social integration.45,56

Adolescence brings into sharp focus social and emotional issues with special implications in physical disabilities of early onset.45,54,57,58 Justified or unwarranted reliance on others may perpetuate a dependent personality.4'' The desire for conforming with the prevailing norms of peer group is marred by differences in appearance.58,59 Limited experience may hinder competence in social interaction.4' These influences can prolong or impede the resolution of adolescent identity crisis. Societal attitudes contribute an increasing share from the age when more time is spent outside the home and may act as a potentially negative influence, despite the gradually changing perception of disabled persons. 59,60 However, to the credit of their supportive nurturing families, the majority of adolescents with physical disability achieve constructive adjustment and social survival skills.61,62 Nevertheless, physicians must initiate a dialogue about psychosocial issues even with seemingly well-adjusted adolescents.63,64 Arrangements to enroll in organized leisure activities is a practical way to help overcome low self-esteem, feelings of loneliness, and social isolation reported at increased frequency on interviews of adolescents with physical disabilities.56,6'',66 Poor social skills may require remediation by modeling or formal teaching. 4^ Group counseling provides a forum for sharing experiences and constructive strategies. Individual counseling or, at times, psychotherapy is needed for significant social/emotional dysfunction. Organic central nervous system pathology may contribute, at least partly, to some behavior deviations. A long recognized example is the disinhibited, hyperactive behavior seen in brain damage. Investigations on the neurobiology of emotions and behavior may yield more information with implications for clinical management of these aspects of function in the future.

Knowledge of the medical aspects of disability is a prerequisite for sexual counseling of adolescents and their families.45,67,68 Sexual dysfunction in males with spinal cord injury or spina bifida can affect erection or ejaculation but does not categorically exclude the potential for fathering a child.41 Adolescent girls should be informed about possible complications of pregnancy and labor in neuromuscular disabilities and that these are preventable by proper obstetric care. The risk of recurrence and availability of prenatal diagnosis tor some diseases must be explained in spina bifida and other inheritable conditions. Coping with the task of parenting is a consideration for disabled persons. In severe disability and sexual dysfunction, emotional and physical intimacy can offer satisfaction and pleasure similar to that experienced in customary sexual life.

SPECIAL HEALTH CONSIDERATIONS

Disabled children may have specific health problems that are directly or indirectly related to their physical dysfunction.

In cerebral palsy and some diseases of the motor unit with severe suprabulbar or bulbar dysfunction, poor nutritional intake can lead to underdevelopment in weight and height and necessitate supplemental or exclusive use of substitutive feeding methods, such as gastrostomy. Oral stimulation with or without actual food should continue in these instances to sustain the hope of eventual oral feeding. Localized growth disturbances16 occur in lesions of the upper or lower motor neuron and are more severe in the latter cases. Atrophy of the extremities in poliomyelitis and in hemiparesis of early onset represent some examples. Limited physical activity can lead to obesity of increasing proportions by adolescence and is most frequently seen in spina bifida with high neurologic lesion27 and in severe forms of muscular dystrophy.

Sensory deficit associated with spinal cord injury and spina bifida creates a propensity for accidental burns, skin injuries, and decubiti.26,41,68 The incidence of pressure induced skin ulcers is lower in infants and preschoolers and increases with growth in weight and size. Bony prominences, sacrum, perineum, and trochanter are the sites of predilection among children who use a wheelchair. Ambulatory children are more likely to develop decubiti on their feet from ill-fitting shoes and braces or as a result of deformities, for example over the heel in calcaneus foot deformity.41 Children with sensory deficit must learn proper preventive measures, especially to avoid prolonged sitting in a wheelchair without shifting their weight several times every hour. Inspection of the entire asensory skin surface is an essential part of their daily care or self-care. Slow healing, the need to eliminate weight from the area of the ulcer, inability to use a wheelchair, wear shoes, or walk, attendant infections, and recurrence are serious setbacks to function and a threat to good health.26,41,67

Hydrocephalus occurs in spina bifida associated with Arnold-Chiari type II malformation and less commonly in cerebral palsy following perinatal intraventricular bleeding. Somnolence, irritability, headache, and vomiting are well known signs of shunt malfunction, but slow decompensation may present with more insidious symptoms. Most children undergo shunt revision for obstruction or other complications, or for adjustment on account of growth.

Infections and deterioration of the upper urinary tract are serious health-threatening complications of neurogenic bladder dysfunction in both spina bifida and spinal cord injury.26,41,67 Intermittent clean catheterization to prevent bladder distention and reflux, antibiotic treatment for infections and prophylaxis, especially in the presence of upper tract dilatation, are current methods of medical treatment. Ureteral reimplantation to eliminate vesicoureteral reflux and bladder augmentation or, in selected cases, implantation of an artificial sphincter to control incontinence, are the most commonly performed surgical procedures. Social continence can be achieved in most cases with bladder emptying at regular intervals by voiding or catheterization and at times with the addition of drug treatment to influence bladder dynamjcs 26,41,68

Achievement of social continence in neurogenic bowel dysfunction is based on establishing regular emptying at predictable times.26 Preparation of the parents in the principles of management begins in infancy, whereas actual training of the child commences at the usual age around 2 to 3 years. Fecal stasis with constipation and incomplete emptying are the most common problems. The resultant colonic and rectal distention and atonia are difficult to reverse.26,41 A diet containing adequate residue and laxative quality, cautious use of medications, and application of rectal suppository or digital stimulation may be necessary to accomplish regular, complete evacuation41,68

Osteoporosis is a nearly universal complication in muscle weakness and restricted physical activities, particularly walking.26 Fractures with no apparent history of trauma occur most frequently in lower motor neuron paralysis. Children with spina bifida tend to develop neuropathic fractures after surgical procedures when cast immobilization enhances a pre-existing osteopenia. Fracture healing is not influenced by osteoporosis. Stimulation of bone formation is a physiologic consideration in favor of early standing and walking with assistive devices regardless of the long range functional expectations. However, at present no satisfactory treatment exists for preventing or correcting osteoporosis in physical disabilities of early onset.

Spinal cord injury interrupts the central connections of the autonomic nerves.68 This leads to a sudorimotor paralysis and consequent loss of thermoregulatory perspiration distal to the neurologic lesion. In high thoracic paraplegia or quadriplegia, hyperthermia can develop when the ambient temperature is high. This pathophysiologic response should be kept in mind during outdoor recreational activities and participation in sports. The lack of central inhibitory control also produces a heightening of sympathetic responses to sensory stimuli manifested as autonomic dysreflexia.68 Hypertension, headache, bradycardia, flushing of the face, pupillary constriction, and nonthermoregulatory sweating below the level ot spinal cord injury are clinical signs of these episodes. Distended rectum or bladder, decubiti, or bladder infection are the most common precipitating causes. Identification and elimination of the noxious stimulus is the appropriate treatment.

The possibility of impaired respiratory function arises as a consequence oí poor voluntary control or weakness of the respiratory muscles in cerebral palsy, spinal cord injuries, or malformations with high levels of paralysis, muscular atrophy, and advanced stages of muscle diseases. Scoliosis, a frequent complication in these disabilities, further restricts aeration of the lungs. There is an increased risk of aspirations and pulmonary infections when bulbar or suprabulbar palsy compromises swallowing.

Cardiac problems in physical disabilities include malformations associated with congenital skeletal deformities and myocardial involvement in certain muscle diseases, for example Duchenne muscular dystrophy. Seizure disorders occur in diseases of the central nervous system. Their overall incidence is 30% to 50% in cerebral palsy; spastic quadriparesis and hemiparesis have the highest relative frequency. 16

In addition to the usual dental care, special attention is watranted for gingival hypertrophy resulting from anticonvulsant medications. Prenatal anoxia is one cause of dental enamel dysplasia. Weakness or incoordination of the muscles of the face, neck, and mastication lead to abnormal configuration of the maxilla and mandible. High vaulted palate, open bite, and recessed chin are the most common consequences.

CONCLUSION

The management of children with physical disability is a complex undertaking. It encompasses a spectrum of concerns ranging from organic and functional consequences of the presenting motor deficit to psychosocial adjustment. The goals and methods of intervention are dictated by realistic long range expectations and by the trends of growth and development. The rehabilitation process is a team effort that entails cooperation of the family, physicians, allied health professionals, and educators. However, the leading role and credit for a successful outcome belongs to the family and children.

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