The International Classification of Functioning, Disability and Health, known more commonly as ICF, is a classification of health and health-related domains. The components focus on body function and structure, activity and participation, and additional information including severity and environmental factors. Care providers should be familiar with standardized tools that capture the ICF components with a focus on cognitive and learning, motor, speech–language, and social and adaptive skills. These assessments are used by medical, educational, psychological, and therapeutic care providers to determine and advocate for particular educational and state supports. The focus on the following dialogue will be on assessments of motor and speech function.

Plotting a child’s anthropometric data on a growth chart allows the PCP to track his or her nutritional status. A weight-to-length ratio below the fifth percentile 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 remember that some CSHCN have short stature as part of their disease process or syndrome. Special growth charts are available for children with Down syndrome (11), Turner syndrome (12), achondroplasia, arthrogryposis–amyoplasia, Marfan syndrome, Noonan syndrome, Prader–Willi syndrome, and Williams syndrome.

A nutritional assessment should be completed during routine health maintenance visits. When there is concern about a child’s growth, a more careful investigation 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. Assessment of possible nutritional deficiencies may provide useful information about general health. Following serum ferritin, vitamin D, and essential elements can be very important in individuals with developmental disabilities. The amount of food eaten is important, but the amount of time it takes a child to complete a meal is also essential. It is not unusual for a child with severe cerebral palsy (CP) or an infant with spinal muscular atrophy (SMA) to eat a meal over a prolonged period. The amount of energy to consume a meal may be significant. An assessment of oral motor function during eating should be performed at health supervision visits if there is a concern for feeding problems. Prevalence estimates suggest that 80% to 90% of individuals with developmental disabilities will experience dysphagia during their lifetime (13). Signs and symptoms of feeding problems include coughing or choking while eating, a wet vocal quality during or after the meal, poor sucking, gagging 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 therapist, nutritionist, gastroenterologist, and/or developmental or rehabilitation physician. Assessments used to evaluate feeding in this population include behavioral feeding examinations, oral motor feeding observations, and video fluoroscopic swallow studies.

Routine immunization against childhood diseases is recommended for all children with disabilities. The most current schedule can be obtained through the Centers for Disease Control and Prevention (CDC) and is approved by the AAP and American Academy of Family Physicians (14). Although children with disabilities are not necessarily at higher risk for contracting childhood infections, they may have greater morbidity and mortality when ill. Administration of diphtheria, tetanus, and pertussis (DTaP) and measles, mumps, and rubella (MMR) vaccines to children with a personal or family history of seizures is controversial as these vaccines can increase the risk of seizures (15). 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. It is recommended that the DTaP vaccine be delayed until a complete neurologic evaluation is completed and the cause of the seizure determined. Conversely, the MMR vaccine should not be withheld 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 disabilities, such as those with rheumatologic diseases and Duchenne muscular dystrophy (DMD) who are on chronic corticosteroids, are included in this special population. In general, it is not recommended that children who are immunocompromised from corticosteroid use receive live bacterial or viral vaccines. Although definitive guidelines do not exist, the current Red Book recommendation is that children receiving high doses of systemic corticosteroids given daily or on alternative days for more than 14 days not receive live-virus vaccines until 1 month after the discontinuation of the medications. High-dose corticosteroids are defined as dosages greater than 2 mg/kg per day or greater than 20 mg/day if the child weighs more than 10 kg. In the case of DMD, it is recommended that children receive all of their immunizations prior to the initiation of corticosteroids (16).

Immunization against influenza of CSHCN, families, and medical providers on a yearly basis decreases the potential devastating morbidity and mortality associated with this virus. Influenza immunization of all high-risk children older than 6 months of age and their close contacts should be strongly encouraged each fall (17). Children who are high risk include those with recurrent pneumonias or upper respiratory infections, and those with CP and neuromuscular diseases such as SMA, congenital myopathies, and muscular dystrophies. Chemoprophylaxis during an influenza outbreak is also recommended to decrease the duration the child is infectious. Children who may have increased risk from complications due to pneumococcal disease should receive the pneumococcal conjugate and/or polysaccharide vaccine (15). Children born prematurely or having certain cardiopulmonary diseases often qualify for respiratory syncytial virus (RSV) prophylaxis (15).

Among the general community, concern remains regarding risk of vaccines causing other developmental difficulties including autism spectrum disorder (ASD). Though there is no evidence to support these concerns, families continue to decline immunizations for their children. It is very important for providers to document families’ concerns and attempt to sensitively counsel them regarding the state of the medical evidence and the risks posed to children and communities that do not immunize.

DENTAL CARE

Tooth decay is one of the most common diseases of childhood (18). Almost 80% of CSHCN in the United States are reported to need dental care (19), and as few as 10% of dentists report that they serve this population (20). Tooth decay and poor dental hygiene in children with disabilities are related to swallowing problems, drooling, and gastroesophageal (GE) reflux. Many medications are given with sweeteners to increase palatability, and increase risk of tooth decay; others cause gingival hyperplasia (eg, phenytoin) or decrease saliva production. Routine dental care of a child or adolescent 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 multiple 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.

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 (36). Families may need to remind the dentist and hygienist of the child’s risk for an allergic reaction to latex. Latex-free gloves must be available to reduce the risk of an allergic reaction. Boys with DMD can have malocclusion with anterior and posterior open bites, which are associated with lip incompetence, mouth breathing, and macroglossia. Deteriorating oral muscle 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 (37,38). Boys with DMD have a greater risk of malignant hyperthermia when anesthesia is used for dental care (39,40).

Routine examinations and cleaning to maintain optimal dental hygiene should be performed by a dentist comfortable in the care of children with special needs. Some of the dental care may need to be accomplished under anesthesia in order to obtain the maximum benefit. Combining dental procedures with other necessary procedures, such as a brainstem auditory evoked response (BAER), local injections with phenol or botulinum toxin, or certain orthopedic procedures, may limit the exposure to anesthetic agents. The AAP Policy Statement on oral health care states that CSHCN 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) (41). Visits will provide the dentist with the opportunity to provide specific education to the family to allow for optimal dental care.

VISION

Vision screening and eye examination should be a component of all routine health care visits. The AAP recommends that the evaluation begin in the newborn period and then at all subsequent visits, with the goal of identifying conditions that might result in visual impairments or represent serious systemic diseases (42). In the child with a disability, this is especially important, given the frequent association of visual disorders with neurologic diseases. 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 positions, a skill that by 3 months of age is developmentally appropriate. Further evaluations of infants and young children 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 measurements and an attempt at ophthalmoscopy.

Ophthalmologic disorders frequently seen in children with CP require very close follow-up with an ophthalmologist (43). Annual evaluation for cataracts should be completed in children with myotonic dystrophy or those on chronic corticosteroids, such as boys with DMD or a child with a juvenile rheumatoid arthritis (16,44). Detailed and accurate documentation of the ophthalmologic examination of a child with spina bifida can be helpful when assessing possible ventriculoperitoneal (VP) shunt malfunctions. For example, a malfunctioning 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, lethargy, or vomiting.

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 ophthalmologist for specialized tests, such as an electroretinogram (ERG), may be useful in assisting with the diagnosis of rare neurologic conditions, such as mitochondrial diseases.

HEARING

Newborn hearing screening is the standard of care in the United States. In 1999, the AAP endorsed the implementation of a universal newborn hearing screening program (45). Two technologies are used for newborn hearing screening: BAER and otoacoustic emissions (OAEs). Periodic reassessments of children with disabilities are important, since these children are particularly at risk for hearing impairment, and hearing loss will affect their developmental skills.

PCPs should pay special attention to children with specific disabilities, as they are at greater risk for developing hearing loss. For example, children with Down syndrome are at increased risk of otitis media and concomitant transient conductive hearing loss (46). Children with congenital cytomegalovirus (CMV), both symptomatic and asymptomatic at birth, are at risk for progressive and late-onset hearing loss (47). Children with athetoid CP due to kernicterus have a high incidence of hearing loss, as do children who have been treated with ototoxic antibiotics for systemic infections (48,49). Children who have been placed on extracorporeal membrane oxygenation (ECMO) have ongoing risk for hearing loss and need regular hearing screening. Finally, all children showing signs of speech or communication difficulties should have their hearing screened.