The demands on the pediatrician as both advocate and provider of care for the physically disabled child and family are great. Often, there is a natural hesitance on the part of the primary physician to establish a diagnosis of severe handicap (eg, cerebral palsy, spina bifida, bronchopulmonary dysplasia, muscular dystrophy) and even greater hesitance to break the news to the family. Nevertheless, those parents who are informed early and to whom the cause and implications of the diagnosis - social, emotional, and financial - are explained are far more satisfied with their physician.1 Ongoing support over the months following diagnosis is vital to facilitate the family's acceptance and coping. Such support demands time, and pediatricians must organize their office schedules, often leaving time at the end of the day for supportive and anticipatory counseling as well as careful record keeping.
As the child grows, long term follow-up issues arise, perhaps the most important of which involve fostering independence in the child and preventing infantilization by well-meaning but often overprotective parents. In addition to specific school issues to be mentioned later, development of a positive self-image is encouraged by normal patterns of school attendance. Medical services should be coordinated so as not to interfere with school activities. Awareness of the availability of appropriate socialization experiences, such as wheelchair athletics, horseback riding, and swimming, is helpful. As adulthood approaches, the normal transition of care to the internist may be much more difficult, as these "adults with pediatric illnesses" may not be easily referred, and often the pediatrician needs to provide ongoing care and advocacy into the adult years.
The pediatrician must provide the disabled child with timely, comprehensive routine care. In addition, the pediatrician must also look beyond the obvious physical disabilities to recognize a whole host of problems associated with the primary diagnosis, which together can adversely affect the quality of life for these children much more than does the physical impairment. This article will focus on some of these problems, ranging from medical issues such as respiratory infection, oral hygiene, nutrition, and bowel and bladder continence to psychosocial issues of parental response to chronic illness and issues of early intervention and school placement. Much more emphasis needs to be placed on the recognition of these problems and the role of the pediatrician, as primary caretaker, in their management. Although general principles of care will be outlined, it is imperative that the primary pediatrician be aware of the services available - medical, educational, or psychosocial - and serve as a liaison between the patient (family) and the multitude of specialists the child may see. A working knowledge of the relevant local, county, state, and federal laws is equally important.
Cerebral palsy, bronchopulmonary dysplasia, and spina bifida with the Arnold-Chiari malformation are examples of conditions in which respiratory problems are frequent and severe. For such children, up-to-date immunization status is of high priority, with special consideration given to providing both Hemophilus influenzae and influenza vaccines. In addition, for children in day care or school situations, the family should be advised of the need to be particularly aware of respiratory infection in the school and watch the child for early signs of infection. Many underlying abnormalities acting alone or in combination contribute to the frequent upper and lower airway infections seen in such children. These include deficient central control of respiration, upper airway obstruction, lower airway and parenchymal disease, incoordination of glutition, and excessive prone positioning. The child with underlying motor problems, poor nutrition, and a predisposition to aspiration is a prime candidate for repeated respiratory infections. The pediatrician should address infection early and aggressively with antibiotics, decongestants, and humidification. Supplemental oxygen may be indicated during acute infection and, when appropriate, saturation monitoring should be undertaken. If the child has a tracheostomy, cultures should be done, appropriate antibiotic coverage provided, and follow-up done to ensure clearing of the infection.
Adequate oral hygiene is of great importance in this population due to the increased risk of developing problems which then adversely affect the quality of life of the patients and their families. Children with physical disabilities are at greater risk for various reasons. The wide variety of antibiotics and anticonvulsants to which these children are exposed from the neonatal period onward can lead to both gingival and enamel dysplasias. In the child with cerebral palsy, the effect of abnormal oromotor tone and postures are manifest by tonic bite as well as persistent rooting, tongue thrusting, and sucking reflexes. Over time, these lead to deformities of the oropharynx: a high arched palate, enamel dysplasias, and crowding of the teeth. Furthermore, poor handling of secretions, deficient chewing and swallowing coordination, and hypersensitivity to textures and temperatures lead to retention of food in the mouth and subsequent cavity development. Any discomfort in the mouth can cause increased irritability, drooling, and deterioration in feeding. This often results in poor nutrition, compromised general health, and greater difficulty in handling the profoundly involved child. In spina bifida, deformities of the midface secondary to hydrocephalus lead to high arched palate and crowding of teeth.
Proper oral hygiene is more difficult to accomplish with the disabled child because of oromotor hypersensitivity and the lack of understanding by the child of the need for good hygiene. Even in older children, the physical inability to brush their own teeth remains. During the first two years preventive measures should include timely weaning from bottle feeding, initiation of toothbrushing by parents, and adequate fluoride supplementation.2'5 Early referral (no later than age 3) to a dentist versed in dealing with both physically and cognitively impaired children is vital for the prevention and early treatment of problems.
Growth failure has been associated with many disabilities; it is particularly prevalent in children with cerebral palsy and bronchopulmonary dysplasia. Factors implicated in poor growth are: higher than normal energy requirements in the child due to high energy expenditures resulting from spasms or athetosis, reduced work load efficiency, feeding disorders due to oromotor dysfunction, and behavior disturbances interfering with feeding and prompting early satiety. To identify the deviations seen in the disabled child it is essential to recognize the normal sequence of oromotor development including: effective suck, progression toward solid versus liquid intake, and effective chewing and swallowing. A careful history should include a detailed diary of typical daily intake, duration of each feeding, and social aspects, including difficulties perceived by the family with regard to the feeding process. For the severely handicapped child, where the severity of involvement and difficulty in feeding lead to development of serious adverse behavioral patterns between the parent and child or where vomiting is a serious problem, supplemental feeding with nasogastric tube or via gastrostomy should be considered.6
Caloric intake should be calculated at a higher than normal rate as there is a 10% to 50% increase in energy requirements above basal levels. A variety of high calorie formulas providing 1 to 2 cal/cc are available (eg, Isocal®, Ensure®). Prior to undertaking gastrostomy, documentation of gastroesophageal reflux, aspiration pneumonia, or severe dehydration is indicated. Additional criteria include departure from a previously followed length and weight curve and serious deterioration in parent-child interaction. Recent evidence indicates that although linear growth does not necessarily accelerate following aggressive feeding, weight gain does improve significantly, pointing toward a need to provide adequate calories. 7
BOWEL AND BLADDER PROBLEMS
The neurogenic bladder and bowel of children with spina bifida is well recognized, and extensive literature is available on current management.8 In general, once an initial episode of bacturia occurs, the bladder is most often managed by clean intermittent catheterization (CIC), at first performed by the family-nurse, then (as early as age 5) by the child. This is supplemented by measures to increase urine acidification (eg, vitamin C, cranberry juice), as well as by antibiotic prophylaxis, most commonly with daily bactrim/ Septra*' or Macrodantin* where appropriate. Urinalysis and culture with sensitivity are required at regular intervals (every 4 to 6 weeks during infancy, increasing to every 3 to 6 months depending on the frequency of urinary tract infection) to guard against infection. The frequency for kidney and bladder imaging is directly correlated with the frequency of infection and evidence of biochemical abnormalities, with renal ultrasound or renal scan preferred to intravenous pyelogram.
The development of the artificial urinary sphincter in the early 1970s, initially hailed as a major breakthrough in the management of the neurogenic bladder and although certainly a welcomed addition to the treatment armamentarium, has not been a panacea for incontinence. Current indications include patients in whom CIC has already tailed but who are appropriately motivated tor continence. Chronic infection cannot be present and an adequate bladder capacity must be ensured. The latter may require surgical bladder augmentation procedures via bowel loop attachment or conversion of an upper to lower motor neuron bladder by surgical neurectomy. Once surgically placed, long term problems have arisen, largely due to either mechanical failure or foreign body reaction to the plastic components causing erosions or other problems necessitating surgical revision. It is hoped that improvements in both design and material will lead to the resolution of these problems. Electrical stimulation of the bladder detrusor is still in the experimental stage, but may offer an additional treatment modality for the neurogenic bladder.
The approach to the bowel is one of timed evacuation with regulation of stool consistency by dietary measures of both appropriately large fluid intake and knowledge by the patient and family (under the guidance of the pediatrician) of "softening" or "hardening" foods for that particular patient. Such training can commence by age 2 but may take many months for adequate control to be achieved. The support and guidance of a committed pediatrician eases the task.
Much less well recognized is the "neurogenic bowel and bladder" of the child with cerebral palsy. Central disinhibition leads to sudden, uncontrolled evacuations, often precipitated by episodes of emotional lability commonly seen in these children (eg, jags of crying or laughing) accompanied by incontinence. Furthermore, tonal deviations and abnormal reflexes in sphincters of skeletal muscle composition can lead to difficulty with initiation of voiding and, even more frequently, constipation. Anticholinergic treatment, at times combined with CIC, leads to marked improvement in almost 80% of involved children.1' The technique of anal stimulation, used so frequently in the spinal cord injured, may be helpful tor the cerebral palsy population.
Success in establishing at least partial continence both during the day and through the night has been achieved in even the severely neurologically impaired population. Behavioral techniques may be coupled with a knowledge of likely times of voiding and bowel evacuation based on diary information specific to the patient. Such techniques include modeling (the use of dolls to "demonstrate" the behavior in the context of play), shaping, repeated exposure to feared situations (eg, sitting on the toilet), and positive reinforcement. 10
The importance of establishing bowel and bladder continence cannot be overemphasized. Bladder incontinence places the child at risk for urinary tract infection and subsequent renal injury. Equally damaging is the potential for decubitus ulcer development caused by irritation of skin by retained urine and feces in the diapered child. Health is compromised and large blocks of time are lost from school whenever a decubitus develops. Finally, the social stigmatization associated with the "outhouse syndrome" can be avoided if continence can be established.
With the establishment of a diagnosis comes the need to develop a plan for the child's education. Placement during infancy in appropriate early intervention programs staffed by teacher specialists and therapists has been shown to improve parental acceptance of, and coping with, the child's handicaps. Early placement also allows the child early socialization experiences and positive self-image development. Less certain is the improvement in motor function brought about by such intervention."12 District provided preschool programs will continue the educational intervention process until the child is ready for kindergarten, at which time decisions have to be made regarding school placement. Options include a mainstreamed classroom, self-contained classroom within a district school, "handicapped" school centers (eg, Cerebral Palsy Centers, Association of Retarded Citizens programs, or in the case of the most severely cognitively involved, Day Training Centers). The role the pediatrician should play in this process is detailed below.
In 1986, Congress passed PL 99-457 making state provided special education mandatory for children from 3 to 5 years of age, and optional tor children from birth through 3 years (Title I). The promise of federal tunding encourages states to develop such optional programs. With the passage of PL 94-142 in 1975, Congress had already mandated each state to provide appropriate education tor the then more than 8 million handicapped children between the ages of 5 and 18. In 1977, the age limits were extended to between 3 and 21 years of age. The purposes of these laws were to:
* enhance the development of handicapped infants and toddlers and minimize their potential for developmental delay;
* reduce the educational costs to our society, including our nation's schools, by minimizing the need for special education and related services after handicapped infants and toddlers reach school age;
* minimize the likelihood of institutionalization of handicapped individuals and maximize their potential for independent living in society; and
* enhance the capacity of families to meet the special needs ot their infants and toddlers with handicaps.
Handicapping conditions as defined by the law include mental retardation, hearing deficits, speech and language impairments, specific learning disabilities, visual handicaps, emotional disturbances, orthopedic impairments, and a variety of other medical conditions categorized as "other health impaired." Because implementation of the law generally becomes the responsibility of individual school districts within each county, pediatricians should become familiar with the classification system in their state, the members of the local child study team and how they function, and the current status of law so that they may most appropriately advocate for the children they treat.13,14
The law requires that an individualized education plan be developed for each child by a team consisting of educational personnel, specialists in related services, and the parents. In New Jersey, the child study team must include a psychologist, learning disabilities teacher consultant, and school social worker. Additional team members may be physical, occupational, or speech therapists. Medical input, other than attesting to the diagnosis and general health of the child, is not required but often appreciated, especially for the profoundly involved child requiring intensive health professional (ie, nursing) services.
With the deinstitutionalization of the profoundly physically impaired population, district day training centers have seen a shift in the demographics of their clients. Previously serving the sevetely mentally retarded but ambulatory, medically stable population, these centers are being faced with an increasing number of medically fragile children. These children are often spastic and quadriplegic with complex, intractable seizure disorders. Many also have chronic respiratory incompetence and require frequent suctioning and chest physical therapy. They are poor feeders, often at risk for aspiration, or must be fed via gastrostomy tubes; many have tracheostomies. In addition, large, wheelchair bound, severely spastic children need frequent repositioning in and out of their chairs, placing tremendous physical burdens on staff. Pediatricians must be in the forefront with regard to advocacy not only for their patients but also for these centers to ensure proper funding and staffing by the state agencies.
Even for the child with less profound, but nevertheless significant, physical impairment such as mild to moderate cerebral palsy, spina bifida, or marginally ambulatory muscular dystrophy, physician input into child study team decisions is most appropriate in advocating for the "least restrictive" setting. Examples include allowing the marginally ambulatory child to walk from class to class rather than use a wheelchair, allowing the wheelchair bound patient to function within a mainstreamed school setting, and allowing all physically disabled children to participate in appropriate physical education activities.15
Teacher familiarity with the nature of the child's disability is vital for the prevention of undue restrictions. Eiser and Town16 studied teachers' concerns about chronically ill children placed in a mainstreamed setting in British schools. There was a general lack of knowledge about these chronic diseases and how to integrate affected children with their "normal" peers in the class. Activities were unnecessarily restricted due to teachers' fears of what chronically ill children might not be able to do. However, the diseases studied, (asthma, diabetes, epilepsy) were not even those with obvious physical disabilities and the authors suggest that "other disabilities, for example spina bifida, may well create greater difficulties for the teacher."
With advances in medical technology, survival of previously nonviable neonates and children has dramatically improved. This has resulted in an unfortunate increase in the number of physically disabled children. Assessing the problems and coordinating the care of these children and their families has become more time consuming because of* the availability of more sophisticated diagnostic and therapeutic modalities as well as more educational opportunities. Yet it is the pediatrician who is in the best position both to coordinate that care and serve as an important source of comfort and support. It is impetative that the pediatrician fill that role.
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11. Leib SA. et al: Effects of early intervention and stimulation on the preterm infant. Pediatrics 1980; 66:83-90.
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13. Downey WS: PL 94-142 and PL 99-457: Challenges for pediatrics. J Flo Med Assoc 1987: 74(10):778-782.
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15. Silver LB: Learning disabilities: The primary care role in mu Indisciplinan management. Postgrad Med 1986: 79(8):285-296.
16. Eiser C. Town C: Teachers' concerns about chronically sick children: Implications for pediatncians. Dei' Med Child Neurol 1987; 29:56-63.