Children with special health care needs see their PCP more frequently than a typical child. (5) Many of the visits are for routine care, such as well-child checks, immunizations, and school and sports physi- cals. Routine visits, however, can often be redirected quickly toward a disease-specific focused discussion.
Although important, the basic well-child visit is no longer the focus. It is important for the PCP to ensure that routine health care needs are being addressed, even if it means that a second appointment is sched- uled. The following are examples of health care topics that should be addressed during routine visits.
Growth and Nutrition
Assessment of growth is a basic element of the rou- tine physical examination of children. Routine care in a typical child becomes a challenge when deal- ing with a child with a disability. Length, weight, and head circumference should be obtained at each health maintenance visit in the very young child, and length and weight should be obtained for the older child. Obtaining a weight on a child who is unable to stand on the office scale is often accomplished by
having parents hold the child while stepping on the scale themselves. This is more effective in a smaller child, but is more difficult in a larger child or one with severe spasticity or hypotonia. It is recommended that an office who cares for large numbers of children who are nonambulatory obtain a wheelchair scale, which allows the child to be weighed easily in his or her own wheelchair. Assessment of the length of a child is also problematic when the ability to stand is limited. Use of arm span as a substitution for height may be an acceptable option. Alternatives to standing height also include individual measurements of lower extremity segments when significant joint contractures are pre- sent. To obtain segmental measurements, the child is placed in the supine position on the examination table and the assessment is done by adding all of the mea- surements obtained from the head to pelvis, the pelvis to knees, and knees to feet. Use of knee height has also been used as another means of monitoring a child’s growth (6–8).
Plotting a child’s anthropometric data on a growth chart will allow the PCP to track a child’s nutritional status. A weight-to-length ratio below the fifth percen- tile may represent failure to thrive. However, growth velocity is the more important piece of information.
Many children with disabilities will be below the fifth percentile for their age, but as long as their weight and length increase in parallel to a normal curve, growth may be appropriate. A child’s age should be corrected for prematurity until 2 years of age. One must remem- ber that some children with special health care needs have short stature as part of their disease process or syndrome. Special growth charts are available for children with Down’s syndrome and Turner syndrome (9,10).
A nutritional assessment should be completed dur- ing routine health maintenance visits. When there is concern about a child’s growth, a more careful inves- tigation into the food intake is necessary. The amount, variety, and consistency of food eaten may provide the examiner with information regarding the caloric intake of the child. The amount of food eaten is important, but the amount of time it takes a child to complete a meal also is essential. It is not unusual for a child with severe cerebral palsy or an infant with spinal muscu- lar atrophy to eat a meal over a prolonged period. The amount of energy that is expended during this lengthy meal may cost more in energy expenditure than is actually gained in caloric intake. Additional informa- tion that is important to obtain includes how safe the child appears while eating. This is best achieved by watching an infant or child eat or drink during their routine visit. Signs and symptoms of feeding problems include coughing or choking while eating, a wet vocal quality during or after the meal, poor sucking, gag- ging easily, and vomiting after a meal. A referral to
a comprehensive feeding clinic should be considered if there is a concern about the weight of the child or his or her safety while eating. An interdisciplinary clinic may include an occupational and speech ther- apist, nutritionist, physiatrist, gastroenterologist, and/
or developmental or rehabilitation physician.
Immunizations
Routine immunization against childhood diseases should be recommended for all children with dis- abilities. The most current schedule can be obtained through the Centers for Disease Control and Prevention (CDC) and is approved by the American Academy of Pediatrics and American Academy of Family Physicians. (11) Special consideration must be given to children with special health care needs. Although chil- dren with disabilities are not necessarily at higher risk for contracting childhood infections, they may have greater morbidity when ill with one of these infections.
One of the more controversial subjects is administra- tion of the diphtheria and tetanus toxoids and acel- lular pertussis (DTaP) or measles, mumps, rubella (MMR) vaccine to children with a personal or family history of seizures. Administration of these vaccines can increase the risk of seizures in this group of chil- dren (12). The seizures are typically short in duration, generalized, self-limited, and associated with a fever.
Because the pertussis immunization is given during infancy, the onset of a seizure after the vaccine can be confusing. Frequently, parents implicate the vaccine as the cause of a new-onset seizure disorder, such as infantile spasms, when in fact, the association is coin- cidental. It is recommended that the DTaP be delayed until a complete neurologic evaluation is completed and the cause of the seizure determined. The MMR, on the other hand, is not recommended to be with- held, even with a recent history of seizure, because it is typically first given after the onset of infantile seizures and the etiology of the seizure is generally already known.
Special attention should be given to children who are immunocompromised. Children with physical dis- abilities, such as those with rheumatologic diseases and Duchenne muscular dystrophy who are on chronic corticosteroids, are included in this special popula- tion. In general, it is not recommended that children who are immunocompromised from corticosteroid use receive live bacterial or viral vaccines. Although defin- itive guidelines do not exist, the current Red Book rec- ommendation is that children receiving high doses of systemic corticosteroids given daily or on alternative days for more than 14 days not receive live-virus vac- cines until 1 month after the discontinuation of the medications. High-dose corticosteroids are defined by receiving >2 mg/kg per day or >20 mg/day if the
child weighs more than 10 kg. In the case of Duchenne muscular dystrophy, it is recommended that children receive all of their immunizations prior to the initia- tion of corticosteroids (13).
Immunization against influenza of CSHCN, fami- lies, and medical providers on a yearly basis is critical to decrease the potential devastating morbidity and mortality associated with this virus. Chemoprophylaxis during an influenza outbreak is also recommended to decrease the ongoing spread. Influenza immunization of all high-risk children older than 6 months of age and their close contacts should be strongly encour- aged each fall (14). High-risk children being seen in the rehabilitation clinics should include those with recurrent pneumonias or upper respiratory infections and those with neuromuscular diseases such as spi- nal muscular atrophy (SMA), congenital myopathies, and muscular dystrophies. Children who may have increased risk from complications due to pneumococ- cal disease should receive the pneumococcal conju- gate and/or polysaccharide vaccine (12).
Dental
Tooth decay is one of the most common diseases of childhood (15). Tooth decay and poor dental hygiene in children with disabilities is related to swallow- ing problems, drooling, and gastroesophageal reflux.
The administration of medications with sweeteners to make the taste more palatable or those that cause gingival hyperplasia such as phenytoin also contribute to tooth decay. Routine dental care of a child or ado- lescent with severe developmental disabilities may be challenging for parents and caregivers due to an oral aversion, a tonic bite reflex, or the inability of the child to follow instructions to open his or her mouth. Other daily care activities, such as administration of multi- ple medications or respiratory treatments, may make dental hygiene less of a priority. Once a child takes over the care of his or her own teeth, the quality of cleaning may not be optimal because of cognitive and physical limitations.
Dental health of children with cerebral palsy (CP) compared to children with other disabilities is most frequently described in the literature. The incidence of dental caries in children with CP is similar to the gen- eral population, although the quality of the caries is different. The size of the carious lesions is greater than what is seen in typical children (16–18). Periodontal disease is more prevalent in children with CP com- pared to their typical peers, likely due to the presence of gingival hyperplasia from phenytoin administration (19). Malocclusion and developmental enamel defects were also more common in children with CP (20–24).
Erosion of primary and permanent teeth has been attributed to chronic gastroesophageal (GE) reflux.
association of visual disorders with neurologic dis- eases. The eye evaluation from birth to 3 years should include a vision assessment, which is accomplished by having the infant or young child fix on an object. The examiner assesses the child’s ability to maintain the fixation and follow the object into different gaze posi- tions, a skill that by 3 months of age is developmentally appropriate. Further evaluations of the young child should also include external inspection of the eye and lids, pupillary and red reflex examination, and ocular alignment. Assessment of the child older than 3 years should also include age-appropriate visual acuity mea- surements and an attempt at ophthalmoscopy.
Ophthalmologic disorders frequently seen in chil- dren with cerebral palsy require very close follow-up with an ophthalmologist (38). Annual evaluation for cataracts should be completed in children with myo- tonic dystrophy or those on chronic corticosteroids, such as boys with Duchenne muscular dystrophy or a child with a juvenile rheumatoid arthritis (13,39).
Detailed and accurate documentation of the ophthal- mologic examination of a child with spina bifida can be helpful when assessing possible ventriculoperito- neal (VP) shunt malfunctions. For example, a malfunc- tioning VP shunt may cause papilledema or changes in extraocular movements. These are early indications that may manifest prior to more obvious signs, such as headaches or lethargy.
The eye examination of a child with a disability is best performed by a pediatric ophthalmologist due to the child’s high risk for ophthalmologic problems.
The ophthalmologists have the skill needed to obtain a thorough assessment. A referral to a pediatric oph- thalmologist for specialized tests, such as an electro- retinogram (ERG), may be useful in assisting with the diagnosis of rare neurologic conditions, such as mito- chondrial diseases.
Hearing
Newborn hearing screening is standard of care in the United States. In 1999, the American Academy of Pediatrics endorsed the implementation of a univer- sal newborn hearing screening program (40). Two technologies are used for newborn hearing screening:
brainstem auditory evoked response (BAER) and otoa- coustic emissions (OAEs). It is important that all new- borns be screened and is particularly imperative for children with disabilities. Periodic reassessments of children with disabilities are important, since a hear- ing impairment can significantly affect their develop- mental skills.
Primary care providers should pay special atten- tion to children with specific disabilities, as they are at greater risk for developing hearing loss. For example, children with Down’s syndrome are at increased risk The severity of erosion has been correlated with the
duration of the GE reflux disease, frequency of vomit- ing, pH of the acid, and the quality and quantity of saliva (25–28). Despite the fact that children with CP don’t participate in high-risk activities as frequently as their able-bodied peers, dental trauma is more com- mon (29,30). These injuries, most commonly to the maxillary incisors, are related to trauma during trans- fers or falls.
There is little information about dental problems for children with spina bifida. An important issue that must be addressed at each visit is to ensure that the dental office or operating room provide a latex-free environment (31). Families may need to remind the dentist and hygienist of the child’s risk for an aller- gic reaction to latex. Latex-free gloves must be avail- able to reduce the risk of an allergic reaction. Boys with Duchenne muscular dystrophy (DMD) can have malocclusion with anterior and posterior open bites, which are associated with lip incompetence, mouth breathing, and macroglossia. Deteriorating oral mus- cle function as the child gets older is associated with increased plaque and calculus formation and gingival inflammation, but not necessarily with the presence of dental caries (32,33). Boys with Duchenne muscular dystrophy have a greater risk of malignant hyperther- mia when anesthesia is used for dental care (34,35).
Routine examinations and cleaning to maintain optimal dental hygiene should be performed by a den- tist comfortable in the care of children with special needs. Some of the dental care may need to be accom- plished under anesthesia in order to obtain the maxi- mum benefit. Combining dental procedures with other necessary procedures, such as a brainstem auditory evoked response (BAER), local intramuscular injec- tions with phenol or botulinum toxin, or certain ortho- pedic procedures, may limit the exposure to anesthetic agents. The American Academy of Pediatrics Policy Statement on oral heath care states that children with special health care needs be referred to a dentist as early as 6 months of age and no later than 6 months after the eruption of their first tooth, or 12 months of age (whichever comes first) (36). Visits will provide the dentist with the opportunity to provide specific educa- tion to the family to allow for optimal dental care.
Vision
Vision screening and eye examination should be a com- ponent of all routine health care visits. The American Academy of Pediatrics recommends that the evalua- tion begin in the newborn period and then at all sub- sequent visits, with the goal of identifying conditions that might result in visual impairments or represent serious systemic diseases (37). In the child with a dis- ability, this is especially important, given the frequent
used when out of the home setting. Use of medica- tions such as glycopyrolate or the scopolamine patch can decrease the volume of secretions. The use of bot- ulinum toxin injections into the submandibular and parotid glands is being recommended more frequently for children with cerebral palsy (47). Surgical ligation of the glands is typically reserved for cases that are unresponsive to medications.
Drooling may indicate that a child is having diffi- culty with eating, drinking, or swallowing. In a child with a degenerative neuromuscular disease, such as spinal muscular atrophy, the development of increased drooling or difficulty managing oral secretions should prompt a further investigation into his or her feeding status. A referral to a feeding team should be made for consideration of performing a modified barium swal- low. Alternative feeding modalities, such as a nasogas- tric tube or a gastrostomy tube, may be necessary. Use of medications to dry secretions in a child with muscle weakness may be counterproductive, as thicker secre- tions may be more difficult to clear.
Respiratory Distress
Children with upper respiratory infections commonly present to their primary care provider with fever, increased work of breathing, and tachypnea. The evaluation and treatment of a child with a disability who presents with these symptoms should be simi- lar to a typical child. However, the deterioration may be accelerated, requiring a rapid diagnosis and ini- tiation of treatment. The assessment should begin with a review of vital signs, including pulse oxim- etry. The physical examination focuses on assess- ing the child’s level of alertness, his or her work of breathing, and a chest examination. Children with neuromuscular diseases will frequently increase their respiratory rate in order to maintain oxygen saturation. Unfortunately, a child can decompen- sate quickly in this situation as a result of significant fatigue. Oxygen saturations can be falsely reassuring in the face of hypoventilation.
Diagnostic testing may include pulse oximetry, chest x-ray, venous or arterial blood gas, sputum cul- ture looking for a bacterial etiology, and viral studies for identification of common viruses such as influenza and respiratory syncytial virus (RSV). Viral etiolo- gies are the most common causes of upper respiratory infections in both disabled and typical children.
Use of antiviral medications should be consid- ered in children with disabilities because of their high risk for significant morbidity. Enteral or par- enteral antibiotics should be reserved for suspected bacterial etiologies. Coverage for anaerobic bacteria should be initiated when aspiration pneumonia is suspected.
of otitis media and concomitant transient conductive hearing loss (41). Children with congenital cytomega- lovirus (CMV), both symptomatic and asymptomatic at birth, are at risk for progressive and late-onset hear- ing loss (42). Children with athetoid cerebral palsy due to kernicterus have a high incidence of hearing loss, as do children who have been treated with ototoxic antibiotics for systemic infections (43,44).