CHAPTER 44

Care of Children With Special Health Care Needs

Clare Kasper, MD

Children and youth with special health care needs (SHCN) have physical, developmental, emotional, or behavioral conditions that require special health-related services. These conditions must last longer than 1 year and result in 1 of 5 consequences: the need for prescription medications; the need for increased medical care; compromised mental health or limited educational ability; the need for special therapy; or the need for counseling. Children with SHCN are defined by the International Classification of Functioning, Disability and Health as disabled if they are limited from doing what children of the same age can do. Most information on children with SHCN combines the group with and the group without disabilities. Conditions experienced by children with SHCN can range from mild to severe, depending on the nature and extent of them and their effect on daily living. Frequently, care requirements of families and health professionals for children with a diagnosis of SHCN are dramatically increased. For parents, the diagnosis of a condition in their child can be initially overwhelming and disappointing. Support of the parents is essential as they transition from disappointment to acceptance and assume the role of facilitator of their child’s treatment plans.

Early identification of a health condition by a physician can result in appropriate, definitive treatment of many diagnoses. In some instances, early intervention may even prevent secondary conditions (eg, early management of hearing loss with hearing aids may minimize speech abnormalities). Even when such corrective treatment is not available, prompt identification improves children’s long-term outcome and allows families to obtain appropriate resources for their children. Through early intervention, newborns, infants, and children with irreversible conditions can be introduced to medical, educational, and psychosocial services available in the community that serve to help these children maximize and reach their full potential.

Epidemiology

According to a national survey, an estimated 11.2 million children in the United States have SHCN, affecting 23% of households with children across all racial, ethnic, and socioeconomic groups. Approximately 65% of children with SHCN reportedly have more than 1 medical condition, including attention-deficit/hyperactivity disorder, asthma, autism spectrum disorder (ASD), cerebral palsy, developmental delay, diabetes, and epilepsy. A single sensory disability, such as deafness, affects approximately 3.5% of children; blindness occurs in 1% of children. The prevalence of cerebral palsy is 1.5 to 2 per 1,000 live births. Advances in medical technology, as well as improved survival of low-birth-weight newborns and children with malformations, have increased the number of children living with such disabilities. Environmental exposures have increased the incidence of chronic medical conditions, such as asthma.

The presence of SHCN has a profound effect on the health and education of affected children. Studies show that children with SHCN have 1.5 times more doctor visits and spend 3.5 more days in the hospital than children without these conditions. Children with SHCN miss twice the number of school days and are twice as likely to repeat a grade compared with children without medical conditions.

In addition, numerous associated conditions occur more commonly in children with SHCN. These include intellectual developmental disorders, growth failure, and nutritional problems. Problems with dentition, respiratory infections, and bowel and bladder continence also may occur. Significant emotional disturbances may occur as children adapt to their condition.

Clinical Presentation

Children with SHCN can present in a variety of ways depending on their diagnosis. Many physical disabilities may be readily apparent at birth on the newborn examination or newborn screening. Some diagnoses, such as cerebral palsy, may be detected later, as the motor impairment becomes more evident. Following the child’s development at routine health maintenance visits is essential for early detection of developmental delay. Implementing the Bright Futures program from the American Academy of Pediatrics (AAP) can help in early detection of many conditions. Children with SHCN also may present with chronic illness (eg, asthma, diabetes) or with specific complaints (eg, poor vision or hearing). Alternatively, they may present with a more general concern, such as growth failure (Box 44.1). Behavioral problems or difficulties in school may precipitate the initial visit.

^a May not be present in all children.

Pathophysiology

Pathophysiology of a medical condition is completely dependent on the specific diagnosis. Conditions may be secondary to an embry-ologic defect such as myelomeningocele, or to an infection such as congenital cytomegalovirus (CMV), which interferes with cochlear development and results in hearing loss. Etiologies are often multifactorial. For some conditions, such as ASD, the pathophysiology is unknown and may have several different etiologies.

Special health care needs may be classified as acquired or congenital and static or progressive. Cerebral palsy, for example, is a group of nonprogressive neuromotor disorders resulting from a central nervous system insult prenatally or within the first 2 years after birth. It is characterized by abnormal motor movements and posturing and may be accompanied by other problems as well. Causes of cerebral palsy include preterm birth, low birth weight, asphyxia, prenatal abnormalities (eg, placental insufficiency), congenital infections (eg, toxoplasmosis, CMV), and biochemical abnormalities (eg, severe hyperbilirubinemia). Other causes are environmental (eg, in utero exposure to alcohol) and genetic (eg, inborn errors of metabolism). Severe postnatal injuries or infections also may result in cerebral palsy (eg, abusive head trauma, meningitis). However, an estimated 25% to 50% of cases of cerebral palsy have no discernible cause.

Diagnosis

The term SHCN is used in a broad sense to include conditions that require additional medical care and supervision, as defined by the US Department of Health and Human Services Maternal and Child Health Bureau. As previously noted, children with SHCN may have significant physical, sensory, or developmental disabilities that may result from preterm birth, congenital infections (eg, CMV), or exposure in utero to alcohol or illicit substances. Some children are born with congenital malformations or inborn errors of metabolism that mandate special diets and occupational or physical therapy. Other children with SHCN include those with chronic medical conditions, such as asthma, obesity, and sickle cell anemia. All these medical conditions alter lifestyle, require increased medical care (including subspecialty care), increased medication usage, and increased use of community services compared with the general pediatric population.

Evaluation

History

a newly diagnosed SHCN, health professionals should first determine any specific parental concerns. A complete medical history should be obtained, including information about the pregnancy and birth. The history also should include any possible exposures as well as significant infections. General screening questions about development are important to ask to assess the child’s developmental progress (Box 44.2). Specific questioning is warranted if parents are concerned about delayed development or if any of their responses indicate that their child is not attaining age-appropriate developmental milestones.

In cases of children with known sensory or physical disabilities, families should be asked directly at each visit about daily activities and the child’s ability for self-help skills. Because many children with disabilities are also on daily medication for seizures or other chronic conditions, it is important to ask about the presence of any drug side effects.

Any behavioral or emotional problems in the child should be identified. Additionally, an overall assessment of family dynamics should be made. It is important to inquire about the relationships between children with SHCN and their siblings, as well as the effect these children have on the parents’ marriage or relationship.

Physical Examination

In general, a complete physical examination, including an oral health assessment and a detailed neurologic assessment and neuromotor examination, should be performed at each visit. Height, weight, and head circumference should be plotted on the growth chart and compared with previous measurements. Failure of adequate growth as measured by any of these parameters should be examined closely. For example, microcephaly, nutritional problems, and growth failure are not uncommon in children with cerebral palsy. Depending on the specific diagnosis, the examination should focus on physical findings associated with the particular condition. For children with physical disabilities such as congenital or acquired amputa-tions, for instance, assessment of the skin that comes in contact with prosthetic devices is a pertinent aspect of the physical examination. Pressure sores may be found in nonambulatory children with cerebral palsy. In children with sensory disabilities, such as unilateral hearing loss, the evaluation of middle ear effusion or infection in the unaffected ear should be prioritized. If a child has a tracheostomy or gastrostomy tube, inspection of the site is essential at each visit to look for erosions or skin infections.

Overall, for most children with SHCN, the neurologic examination is extremely important (Box 44.3). An age-appropriate developmental assessment is also an essential part of the examination at all health maintenance visits.

Laboratory Tests

Laboratory evaluation of newborns, infants, and children with physical or sensory disabilities depends on their specific conditions. Not all patients need a costly array of diagnostic procedures. A chromosomal karyotype or comparative genomic hybridization using peripheral blood is helpful in children with suspected genetic disorders (eg, abnormal facies, a major anomaly, developmental delay). Routine testing for fragile X syndrome should be strongly considered in all boys with intellectual and developmental disabilities and in girls with a family history of it. Other testing should be specific for the suspected diagnosis (see Chapter 84).

Metabolic screening for inborn errors of metabolism should be performed on children with intellectual and developmental disabilities and any of the following symptoms: intermittent vomiting or lethargy, loss of developmental milestones, or seizures. Such screening is not needed in the routine evaluation of children with developmental delay and no other symptoms.

A screening test for visual acuity (Snellen eye chart) and hearing (ie, audiogram) should be performed in all children with suspected sensory deficits, even mild ones. For infants and toddlers, a brain stem auditory evoked response or behavioral audiogram is a more appropriate screening test for hearing (see Chapter 88). A visual evoked response can be performed to test vision.

Psychometric testing may be helpful in certain school-age children to assess intellectual function. Electroencephalography is indicated in all patients with a history of seizures or seizure-like episodes.

Imaging Studies

Brain imaging studies, such as magnetic resonance imaging of the head, can be informative in the setting of suspected intrauterine infection, intraventricular hemorrhage, or genetic disorders with associated developmental delay and even isolated global developmental delay. Electromyography can be used to differentiate cerebral palsy from a congenital myopathy.

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