Modified from Shapiro B, Batshaw M: Mental retardation (intellectual disability). In Kliegman RM, Behrman RE, Jenson HB, editors: Nelson textbook of pediatrics, ed 18, Philadelphia, 2007, Saunders; Wilks T, Gerber J, Erdie-Lalena C: Developmental milestones: cognitive development, Pediatr Rev 31(9):364–367, 2010.

Results of standardized tests are used in making the diagnosis of intellectual disability (or MR) based on cognitive deficits. Tests for assessing adaptive behaviors include the Vineland Social Maturity Scale and the AAMR Adaptive Behavior Scale. Informal appraisal of adaptive behavior may be made by those fully acquainted with the child (e.g., teachers, parents, other care providers). Frequently these observations lead parents to seek evaluation of the child’s development.

A more useful approach for clinical application is classification based on educational potential or symptom severity. For educational purposes, the mildly impaired group (educable MR) constitutes about 85% of all people with CI, and the group with moderate levels of CI (trainable MR) accounts for about 10% of the intellectually disabled population (American Psychiatric Association, 2000; Katz and Lazcano-Ponce, 2008; Walker and Johnson, 2006) (Table 19-1). Although nurses may be familiar with the approximate range of IQ for classifying severity, they should refrain from using numbers as the criterion for assessing or evaluating the child’s abilities because numbers are of little value in counseling parents or training these children.

TABLE 19-1

Classification of Cognitive Impairment

LEVEL (IQ)*	PRESCHOOL (BIRTH–5 YEARS)—MATURATION AND DEVELOPMENT	SCHOOL AGE (6–21 YEARS)—TRAINING AND EDUCATION	ADULT (≥21 YEARS)—SOCIAL AND VOCATIONAL ADEQUACY
Mild—50–55 to ≈70–75	Often not noticed as delayed by casual observer but is slower to walk, feed self, and talk than most children; follows same sequence in development as normal children	Can acquire practical skills and useful reading and arithmetic to a third- to sixth-grade level with special education; can be guided toward social conformity; achieves mental age of 8–12 years	Can usually achieve social and vocational skills adequate to self-maintenance; may need occasional guidance and support when under unusual social or economic stress; can adjust to marriage but not childrearing
Moderate—35–40 to 50–55	Noticeable delays in motor development, especially in speech; responds to training in various self-help activities	Can learn simple communication, elementary health and safety habits, and simple manual skills; does not progress in functional reading or arithmetic; achieves mental age of 3–7 years	Can perform simple tasks under sheltered conditions; participates in simple recreation; travels alone in familiar places; usually incapable of self-maintenance
Severe—20–25 to 35–40	Marked delay in motor development; little or no communication skills; may respond to training in elementary self-care (e.g., self-feeding)	Usually walks, barring specific disability; has some understanding of speech and some response; can profit from systematic habit training; achieves mental age of toddler	Can conform to daily routines and repetitive activities; needs continuing direction and supervision in protective environment
Profound—below 20–25	Gross delay; minimum capacity for functioning in sensorimotor areas; needs total care	Obvious delays in all areas of development; shows basic emotional responses; may respond to skillful training in use of legs, hands, and jaws; needs close supervision; achieves mental age of young infant	May walk; needs complete custodial care; has primitive speech; usually benefits from regular physical activity

IQ, intelligence quotient.

^*Data from American Psychiatric Association: Diagnostic and statistical manual of mental disorders, ed 4 (text rev) (DSM-IV TR), Washington, DC, 2000, Author; and Rittey CD: Learning difficulties: what the neurologist needs to know, J Neurol Neurosurg Psychiatry 74(suppl 1):30–36, 2005.

Etiology

The causes of severe CI are primarily genetic, biochemical, and infectious. Although the etiology is unknown in the majority of cases, familial, social, environmental, and organic causes may predominate. Among individuals with CI, a sizable proportion of the cases are linked to Down syndrome, fragile X syndrome, or fetal alcohol syndrome. General categories of events that may lead to CI include (Katz and Lazcano-Ponce, 2008; Walker and Johnson, 2006):

• Infection and intoxication, such as congenital rubella, syphilis, maternal drug consumption (e.g., fetal alcohol syndrome), chronic lead ingestion, or kernicterus

• Trauma or physical agent (i.e., injury to the brain experienced during the prenatal, perinatal, or postnatal period)

• Inadequate nutrition and metabolic disorders, such as phenylketonuria or congenital hypothyroidism

• Gross postnatal brain disease, such as neurofibromatosis and tuberous sclerosis

• Unknown prenatal influence, including cerebral and cranial malformations, such as microcephaly and hydrocephalus

• Chromosomal abnormalities resulting from radiation; viruses; chemicals; parental age; and genetic mutations, such as Down syndrome and fragile X syndrome

• Gestational disorders, including prematurity, low birth weight, and postmaturity

• Psychiatric disorders that have their onset during the child’s developmental period up to age 18 years, such as autism spectrum disorders (ASDs)

• Environmental influences, including evidence of a deprived environment associated with a history of intellectual disability among parents and siblings

Nursing Care of Children with Impaired Cognitive Function

Nurses play a major role in identifying children with CI. In the newborn and early infancy periods, few signs are present, with the exception of Down syndrome (p. 576). After this age, however, delayed developmental milestones are the major clues to CI. In addition, nurses must have a high index of suspicion for early behavior patterns that may suggest CI (see Box 19-2). Parental concerns, such as delayed development compared with siblings, need to be taken seriously. All children should receive regular developmental assessment, and the nurse is often the person responsible for performing such assessments (see Chapter 5). When delays are found, the nurse must use sensitivity and discretion in revealing this finding to parents.

Nursing Care Plan—The Child with Impaired Cognitive Function

Educate Child and Family

To teach children with CI, it is necessary to investigate their learning abilities and deficits. This is important for the nurse who may be involved in a home care program or who may be caring for the child in a health care setting. The nurse who understands how these children learn can effectively teach them basic skills or prepare them for various health-related procedures.

Children with CI have a marked deficit in their ability to discriminate between two or more stimuli because of difficulty in recognizing the relevance of specific cues. However, these children can learn to discriminate if the cues are presented in an exaggerated, concrete form and if all extraneous stimuli are eliminated. For example, the use of colors to emphasize visual cues or the use of singing or rhymes to stress auditory cues can help them learn. Their deficit in discrimination also implies that concrete ideas are learned much more effectively than abstract ideas. Therefore, demonstration is preferable to verbal explanation, and learning should be directed toward mastering a skill rather than understanding the scientific principles underlying a procedure.

Another cognitive deficit is in short-term memory. Whereas children of average intelligence can remember several words, numbers, or directions at one time, children with CI are less able to do so. Therefore, they need simple, one-step directions. Learning through a step-by-step process requires a task analysis in which each task is separated into its necessary components and each step is taught completely before proceeding to the next activity.

One critical area of learning that has had a tremendous impact on education for cognitively impaired individuals is motivation. Programs based on the motivational principles of behavior modification, using positive reinforcement for specific tasks or behaviors, have demonstrated marked improvement in children’s ability to learn. Advances in technology have greatly aided in providing reinforcement, especially in children with severe disabilities and who may have physical disabilities that limit their range of capabilities. For example, with the use of specially designed switches, children are given control of some event in the environment, such as turning on the television (Fig. 19-1). The television picture becomes the reinforcement for activating the switch. Repetitive use of these switches provides an early, simplistic association with a technical device that may progress to increasingly complex aids.

FIG. 19-1 A push panel allows a child with cognitive impairment to turn a computer on and off.

Early intervention program is a systematic program of therapy, exercises, and activities designed to address developmental delays in disabled children to help achieve their full potentials (American Academy of Pediatrics [AAP], Committee on Genetics, 2001; National Down Syndrome Society, 2011a; Weijerman and de Winter, 2010). Considerable evidence indicates that these programs are valuable for cognitively impaired children. Nurses working with these families need to be aware of the types of programs in their community. Under the Individuals with Disabilities Education Act (IDEA) of 1990 (Public Law 101-476), states are encouraged to provide full early intervention services and are required to provide educational opportunities for all children with disabilities from birth to 21 years of age. Services may be provided under state Programs for Children with Special Health Needs or Head Start, or by private organizations such as National Down Syndrome Society,* Easter Seals,^† or the Arc of the United States.^‡ Parents should inquire about these programs by contacting the appropriate agencies. The child’s education should begin as soon as possible. As children grow older, their education should be directed toward vocational training that prepares them for as independent a lifestyle as possible within their scope of abilities.

Suitable activities for physical activity are based on the child’s size, coordination, physical fitness and maturity, motivation, and health (Fig. 19-4). Some children may have physical problems that prevent participation in certain sports, such as atlantoaxial instability in children with Down syndrome (p. 576). These children often have greater success in individual and dual sports than in team sports and enjoy themselves most with children of the same developmental level. The Special Olympics* provides these children with a unique competitive opportunity.

FIG. 19-4 A favorite toy provides stimulation for a young child.

Safety is a major consideration in selecting recreational and exercise activities. For example, toys that may be appropriate developmentally may present dangers to a child who is strong enough to break them or use them incorrectly.

Provide Means of Communication

Verbal skills are typically delayed more than other physical skills. Speech requires hearing and interpretation (receptive skills) and facial muscle coordination (expressive skills). Because both types of skills may be impaired, these children need frequent audiometric testing and should be fitted with hearing aids if indicated. In addition, they may need help in learning to control their facial muscles. For example, some children may need tongue exercises to correct the tongue thrust or gentle reminders to keep the lips closed.

Nonverbal communication may be appropriate for some of these children, and various devices are available. For the child without associated physical disabilities, a talking picture board is helpful. For children with physical limitations, several adaptations or types of communication devices are available to facilitate selection of the appropriate picture or word (Fig. 19-5). Some children may be taught sign language or Blissymbols—a highly stylized system of graphic symbols representing words, ideas, and concepts. Although the symbols require education to learn their meaning, no reading skill is needed. The symbols are usually arranged on a board, and the person points or uses some type of selector to convey a message.

FIG. 19-5 A child with cognitive and physical impairments can activate electronic and communication equipment by moving a device near her head.

Establish Discipline

Discipline must begin early. Limit-setting measures need to be simple, consistently applied, and appropriate for the child’s mental age. Control measures are based primarily on teaching a specific behavior rather than on understanding the reasons behind it. Stressing moral lessons is of little value to a child who lacks the cognitive skills to learn from self-criticism or from a lesson based on previous wrong-doing. Behavior modification, especially reinforcement of desired actions, and time-out are appropriate forms of behavior control.

Encourage Socialization

Acquiring social skills is a complex task, as is learning self-care procedures. Active rehearsals with role-playing and practice sessions and positive reinforcement for desired behavior have been the most successful approaches. Parents should be encouraged early to teach their child socially acceptable behavior: waving goodbye, saying “hello” and “thank you,” responding to his or her name, greeting visitors, and sitting modestly. The teaching of socially acceptable sexual behavior is especially important to minimize sexual exploitation. Parents also need to expose the child to strangers so that he or she can practice manners because there is no automatic transfer of learning from one situation to another.

Dressing and grooming are also important aspects of socialization. A child who is dressed in age-appropriate clothing and is well groomed is much more likely to be accepted and to develop good self-esteem. Clothes should be clean, up-to-date, and well fitted. Many attractive outfits can be adapted with self-adhering fasteners and elastic openings to facilitate self-dressing.

As soon as possible, parents should enroll the child in appropriate preschool programs. Not only do these programs provide education and training, but they also offer an opportunity for social experiences among the children. As children grow older, they should have peer experiences similar to those of other children, including group outings, sports, and organized activities such as scouts and Special Olympics. Nurses can assess the child’s abilities and encourage others (e.g., parents, teachers) to promote developmentally appropriate peer interaction (Johnson and Walker, 2006; National Down Syndrome Society, 2011a; Shapiro and Batshaw, 2007).

Provide Information on Sexuality

Adolescence may be a particularly difficult time for the family, especially in terms of the child’s sexual behavior, possibility of pregnancy, future plans to marry, and ability to be independent. Frequently, little anticipatory guidance has been offered parents to prepare the child for physical and sexual maturation. The nurse can help in this area by providing parents with information about sexuality education that is geared to the child’s developmental level. For example, adolescent girls need a simple explanation of menstruation and instructions on personal hygiene during the menstrual cycle.

These adolescents also need practical sexual information regarding anatomy, physical development, and conception.* Because of their easy persuasion and lack of judgment, they need a well-defined, concrete code of conduct. The subtleties of social sexual behavior are less beneficial than specific instructions for handling certain situations. For example, an adolescent should be firmly told never to go alone anywhere with any person that he or she does not know well. To protect him or her from abusive sexual activities, parents must closely observe their teenager’s activities and associates. The question of contraceptive protection for these adolescents is often a parental concern.

Parents of these adolescents are often concerned about the advisability of marriage between two individuals with intellectual disabilities. There is no conclusive answer; each situation must be judged individually. In some instances, marriage is possible, but parenthood may not be desirable because of the complexity of childrearing and the potential problem of perpetuating mental deficiency. The nurse should discuss this topic with parents and with the prospective couple, stressing suitable living accommodations and contraceptive methods to prevent pregnancy. If children are conceived, these parents require specialized assistance in learning to meet the needs of their offspring (Johnson and Walker, 2006).

Help Family Adjust to Future Care

Not all families are able to cope with home care of their affected child, especially one who is severely or profoundly impaired or has multiple disabilities. Older parents may not be able to assume care responsibilities after they reach retirement or older age. For these parents, the decision regarding residential placement is a difficult one, and the availability of such facilities varies widely. The nurse working with a family should help them investigate and evaluate various programs in addition to assisting them in adjusting to the decision for placement.

Care for Child During Hospitalization

Caring for the child during hospitalization can be a special challenge. Frequently, nurses are unfamiliar with children who are cognitively impaired, and they may cope with their feelings of insecurity and fear by ignoring or isolating the child. Not only is this approach nonsupportive, but it may also be destructive for the child’s sense of self-esteem and optimal development, and it may hamper the parents’ ability to cope with the stress of the experience. One method that successfully avoids this nontherapeutic approach is the use of the mutual participation model in planning the child’s care. Parents are encouraged to stay with their child but should not be made to feel as if the responsibility is totally theirs.

When the child is admitted, a detailed history is taken (see Chapter 21), especially in terms of all self-care activity. During the interview, the child’s developmental age is assessed. It is best to avoid asking directly about IQ levels because this may make the parents uncomfortable and often tells little about the child’s actual abilities. Questions are approached positively. For example, rather than asking, “Is your child toilet trained yet?” the nurse may state, “Tell me about your child’s toileting habits.” The assessment should also focus on any special devices the child uses, effective measures of limit setting, unusual or favorite routines, and any behaviors that may require intervention. If the parent states that the child engages in self-injurious activities (e.g., head banging, self-biting), the nurse should inquire about events that precipitate them and techniques (e.g., distraction, medication) that the parents use to manage them (Johnson and Walker, 2006; Oliver and Richards, 2010).

The nurse also assesses the child’s functional level of eating and playing; ability to express needs verbally; progress in toilet training; and relationship with objects, toys, and other children. The child is encouraged to be as independent as possible in the hospital.

Realizing that the child may be lonely in the hospital, the nurse makes certain that toys and other activities are provided. The child is placed in a room with other children of approximately the same developmental age, preferably a room with only two beds to avoid overstimulation. The nurse discusses with the other parents the child’s abilities and introduces the parents and children to each other. By the nurse’s example of treating the child with dignity and respect, others who may be fearful of what they do not understand are encouraged to accept the child.

Procedures are explained to the child through methods of communication that are at the appropriate cognitive level. Generally, explanations should be simple, short, and concrete, emphasizing what the child will experience physically. Demonstration either through actual practice or with visual aids is always preferable to verbal explanation. The nurse repeats instructions often and evaluates the child’s understanding by asking questions such as “What will it feel like?” “Show me how you must lie,” or “Where will the dressing be?” Parents are included in preprocedural teaching for their own learning and to help the nurse learn effective methods of communicating with the child.

During hospitalization, the nurse should also focus on growth-promoting experiences for the child. For example, hospitalization may be an excellent opportunity to emphasize to parents abilities that the child does have but has not had the opportunity to practice, such as self-dressing. It may also be an opportunity for social experiences with peers, group play, or new educational and recreational activities. For example, one child who had the habit of screaming and kicking demonstrated a definite decrease in those behaviors after he learned to pound pegs and use a punching bag. Through social services, the parents may become aware of specialized programs for the child. Hospitalization may also offer parents a respite from everyday care responsibilities and an opportunity to discuss their feelings with a concerned professional.

Assist in Measures to Prevent Cognitive Impairment

Besides having a responsibility to families with a child with CI, nurses also need to be involved in programs aimed at preventing CI. Many of the familial, social, and environmental factors known to cause mild impairment are preventable. Counseling and education can reduce or eliminate such factors (e.g., poor nutrition, cigarette smoking, chemical abuse), which increase the risk of prematurity and intrauterine growth restriction. Interventions are directed toward improving maternal health by educating women regarding the dangers of chemicals, including prenatal alcohol exposure, which affects organogenesis, craniofacial development, and cognitive ability (Defendi, 2010; Wilton and Plane, 2006). Other preventive strategies that play an important role include adequate prenatal care; optimal medical care of high-risk newborns; rubella immunization; genetic counseling and prenatal screening, especially in terms of Down or fragile X syndrome; use of folic acid supplements to prevent neural tube defects during pregnancy and during the childbearing years; newborn screening for treatable inborn errors of metabolism, such as congenital hypothyroidism, phenylketonuria, and galactosemia; and early appropriate therapies and rehabilitation services for children with developmental disabilities.

Down Syndrome

Down syndrome is the most common chromosomal abnormality of a generalized syndrome, occurring in 1 in 691 to 1000 live births (National Down Syndrome Society, 2011b; Weijerman and de Winter, 2010). It occurs in people of all races and economic levels.

Etiology

The cause of Down syndrome is not known, but evidence from cytogenetic and epidemiologic studies supports the concept of multiple causality. Approximately 95% of all cases of Down syndrome are attributable to an extra chromosome 21 (group G), thus the name nonfamilial trisomy 21 (National Down Syndrome Society, 2011b; Walker and Johnson, 2006). Although children with trisomy 21 are born to parents of all ages, there is a statistically greater risk in older women, particularly those older than 35 years of age. For example, in women 35 years of age, the chance of conceiving a child with Down syndrome is about 1 in 350 live births, but in women age 40 years, it is about 1 in 100. However, the majority (≈80%) of infants with Down syndrome are born to women younger than age 35 years because younger women have higher fertility rates (National Down Syndrome Society, 2011b). About 3% to 4% of the cases may be caused by translocation of chromosomes 15 and 21 or 22. This type of genetic aberration is usually hereditary and is not associated with advanced parental age. From 1% to 2% of affected persons demonstrate mosaicism, which refers to a mixture of normal and abnormal cell types. The degree of cognitive and physical impairment is related to the percentage of cells with the abnormal chromosome makeup.