20 
Impact of Cognitive or Sensory
Impairment on the Child and Family
Rosalind Bryant
http://evolve.elsevier.com/wong/ncic
CONCEPTS
• Cognition
• Sensory Perception
COGNITIVE IMPAIRMENT
GENERAL CONCEPTS
Cognitive impairment (CI) is a general term that encompasses any type
of intellectual disability and affects from 2.5% to 3% of the population
(Ageranioti-Belanger, Brunet, D’Anjou, et al., 2012; Bellman, Byrne, &
Sege, 2013; Shapiro & Batshaw, 2016). The term intellectual disability
(formerly mental retardation) has become the most common internation-
ally used term (American Association on Intellectual and Developmental
Disabilities, 2013; American Psychiatric Association, 2013; Tasse,
Luckasson, & Nygren, 2013). In this chapter, the term CI is used syn-
onymously with intellectual disability.
Intellectual disability as defined by the American Association on
Intellectual and Developmental Disabilities consists of three components:
(1) intellectual functioning, (2) adaptive behavior, and (3) age younger
than 18 years at time of diagnosis. Intellectual functioning is measured
by the intelligence quotient (IQ) test score. CI is considered an umbrella
term by the American Association on Intellectual and Developmental
Disabilities that is characterized by significant limitations in both
intellectual functioning and adaptive behavior (i.e., the inability to
reason, plan, solve problems, think abstractly, comprehend complex
ideas, learn from experience that is age appropriate, or unable to meet
the standards for culturally appropriate demands of daily life) (Tasse,
Luckasson, & Nygren, 2013).
The American Psychiatric Association’s Diagnostic and Statistical
Manual of Mental Disorders, Fifth Edition (DSM-5) criteria recommend
moving away from exclusively relying on IQ testing toward using
additional measures of adaptive functioning (American Psychiatric
Association, 2013; Moran, 2013). The DSM-5 is the diagnostic standard
and states that the child with CI must demonstrate deficits in adaptive
functioning that result in failure to meet developmental and sociocultural
standards for personal independence and social responsibility (Moran,
2013).
The American Psychiatric Association’s DSM-5 terminology and
diagnostic criteria are consistent with those terms established by the
American Association on Intellectual and Developmental Disabilities
(Tasse, Luckasson, & Nygren, 2013). Careful evaluation to identify the
632
needs of individuals with CI is focused on promoting habilitation for
each person. It is anticipated that when appropriate supports are given
over a prolonged period, the ability of the person with CI to function
each day will generally improve.
Diagnosis and Classification
The diagnosis of CI is usually made after professionals or the family
suspects that the child’s developmental progress is delayed. In some
cases, it is confirmed at birth because of recognition of distinct syn-
dromes. At the other extreme, the diagnosis is made when problems
such as speech delays or school problems arouse concern. In all cases
a high index of suspicion for developmental delay and behavioral signs
is necessary for early diagnosis (Box 20.1), and routine developmental
screening can assist in early identification. Delays are typically seen in
gross and fine motor and speech development, although the latter is
most predictive. Developmental disability can be described as any
significant lag or delay in a child’s physical, cognitive, behavioral,
emotional, or social development when compared against developmental
norms. CI is an impairment encompassing intellectual ability and adaptive
behavior that are functioning significantly below average (see Box 20.1).
In the absence of clear-cut evidence of CI, it is more appropriate to use
a diagnosis of developmental disability.
Results of standardized tests are helpful in contributing to the
diagnosis of CI. Tests for assessing adaptive behaviors include the Vineland
Adaptive Behavior Scale (Vineland-3) and the Adaptive Behavior
Assessment System (ABS–third edition). 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 constitutes about 85% of all people with
CI, and the group with moderate levels of CI accounts for about 10%
of the intellectually disabled population (Shea, 2012) (Table 20.1).
Etiology
The causes of severe CI are primarily genetic, biochemical, and infectious.
Although the etiology is unknown in the majority of cases, familial,
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family 633

BOX 20.1  Early Signs Suggestive of NURSING CARE OF CHILDREN WITH IMPAIRED
Cognitive Impairment COGNITIVE FUNCTION
Dysmorphic syndromes (e.g., Down syndrome, fragile X syndrome) Nurses play a major role in identifying children with CI. In the newborn
Irritability or nonresponsiveness to environment and early infancy periods, few signs are present, except in such disorders
Major organ system dysfunction (e.g., feeding or breathing difficulties) as Down syndrome (discussed later in this chapter). However, delayed
Gross motor delay developmental milestones are the major clues to CI. In addition, nurses
Fine motor delay must have a high index of suspicion for early behavior patterns that
Language difficulties or delay may suggest CI (see Box 20.1). Parental concerns, such as delayed
Behavior difficulties development compared with siblings, need to be taken seriously. All
children should receive regular developmental assessment, and the nurse
Modified from Shapiro, B., & Batshaw, M. (2016). Intellectual
is often the person responsible for performing such assessments (see
disability. In R. M. Kliegman, B. F. Stantan, J. W. St. Geme III, et al.
(Eds.), Nelson textbook of pediatrics (20th ed.). Philadelphia, PA: Chapter 4). When delays are found, the nurse must use sensitivity and
Elsevier; Wilks, T., Gerber, J., & Erdie-Lalena, C. (2010). discretion in revealing this finding to parents.
Developmental milestones: Cognitive development. Pediatrics in
Review, 31(9), 364-367.
EDUCATE CHILD AND FAMILY
To teach children with CI, one must investigate their learning abilities
TABLE 20.1  Intensity of Sounds
and deficits. This is important for the nurse who may be involved in a
Expressed in Decibels home care program or who may be caring for the child in a school or
Decibels Representative Sound health care setting. The nurse who understands how these children
0 Softest sound normal ear can hear learn can effectively teach them basic skills or prepare them for various
10 Heartbeat, rustling of leaves health-related procedures.
20 Whisper at 1.5 m (5 feet) Children with CI have a marked deficit in their ability to discriminate
30-45 Normal conversation between two or more stimuli because of difficulty in recognizing the
60 Noise in average restaurant relevance of specific cues. However, these children can learn to dis-
70-80 Street noises criminate if the cues are presented in an exaggerated, concrete form
80 Loud radio in home and if all extraneous stimuli are eliminated. For example, the use of
90-100 Train colors to emphasize visual cues or the use of singing or rhymes to stress
120 Thunder, loud music auditory cues can help them learn. Their deficit in discrimination also
140 Jet plane during departure implies that concrete ideas are learned much more effectively than
>140 Pain threshold abstract ideas. Therefore demonstration is preferable to verbal explana-
tion, and learning should be directed toward mastering a skill rather
than understanding the scientific principles underlying a procedure.
social, environmental, and organic causes may predominate. Among Another cognitive deficit is in short-term memory. Whereas children
individuals with CI, a sizable proportion of the cases are linked to of average intelligence can remember several words, numbers, or direc-
Down syndrome, fragile X syndrome (FXS), or fetal alcohol syndrome. tions at one time, children with CI are less able to do so. Therefore they
General categories of events that may lead to CI include the following need simple, one-step directions. Learning through a step-by-step process
(Gilissen, Hehir-Kwa, Thung, et al., 2014; Hoyme, Kalberg, Elliot, et al., requires a task analysis in which each task is separated into its necessary
2016; Katz & Lazcano-Ponce, 2008; Mefford, Batshaw, & Hoffman, 2012): components and each step is taught completely before proceeding to
• Infection and intoxication, such as congenital rubella, syphilis, the next activity.
maternal drug consumption (e.g., fetal alcohol syndrome), chronic One critical area of learning that has had a tremendous impact on
lead ingestion, or kernicterus education for cognitively impaired individuals is motivation or the use
• Trauma or physical agent (e.g., injury to the brain experienced during of positive reinforcement to encourage the accomplishment of specific
the prenatal, perinatal, or postnatal period) tasks or behaviors. Advances in technology have greatly aided in providing
• Inadequate nutrition and metabolic disorders, such as phenylketonuria reinforcement, especially in children with severe disabilities and who
or congenital hypothyroidism may have physical disabilities that limit their range of capabilities. For
• Gross postnatal brain disease, such as neurofibromatosis and tuberous example, with the use of specially designed switches, children are given
sclerosis control of some event in the environment, such as turning on the
• Unknown prenatal influence, including cerebral and cranial malforma- computer (Fig. 20.1). Activation of the computer becomes the reinforce-
tions, such as microcephaly and hydrocephalus ment for pushing the switch. Repetitive use of these switches provides
• Chromosomal abnormalities resulting from radiation; viruses; an early, simplistic association with a technical device that may progress
chemicals; parental age; and genetic mutations that occur in disorders to increasingly complex aids.
such as Down syndrome and FXS Early intervention program is a systematic program of therapy,
• Gestational disorders, including prematurity, low birth weight, and exercises, and activities designed to address developmental delays in
postmaturity children with disabilities to help achieve their full potentials (Bull &
• Psychiatric disorders that have their onset during the child’s devel- Committee on Genetics, 2011; Crnic, Neece, McIntyre, et al., 2017;
opmental period up to age 18 years, such as autism spectrum disorders Guralnick, 2017; National Down Syndrome Society, 2012a). Consider-
(ASDs) able evidence indicates that these programs are valuable for cognitively
• Environmental influences, including evidence of a deprived environ- impaired children. Nurses working with these families need to be aware
ment associated with a history of intellectual disability among parents of the types of programs in their community. Under the Individuals
and siblings with Disabilities Education Act (IDEA) of 1990 (Public Law 101-476),
634 SECTION VIII  Family-Centered Care of the Child with Special Needs
FIG. 20.1  A push panel allows a child with cognitive impairment to turn
a computer on and off.
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 old. Services may be provided under
state programs for Children with Special Health Care Needs (CSHCN) 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.
Early intervention exposure includes structured educational programs,
parent training, and family-intervention training with available family
resources that tend to be associated with more positive developmental
and behavioral outcomes in children with CI (Crnic, Neece, & McIntyre
et al., 2017; Guralnick, 2017; Wallander, Biasini, Thorsten, et al., 2014). 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.
Teach Child Self-Care Skills
When a child with CI is born, parents often need assistance in promot-
ing normal developmental skills that other children learn easily. There
is no way to predict when a child should be able to master self-care
skills, such as feeding, toileting, dressing, and grooming because a wide
age variability exists in the cognitively impaired child who is able to
accomplish such functions.
Teaching self-care skills also necessitates a working knowledge of the
individual steps needed to master a skill. For example, before beginning
*Information on early intervention programs in each state is available
from the National Down Syndrome Society, 666 Broadway, 8th Floor,
New York, NY 10012-2317; 800-221-4602; email: info@ndss.org; http://
www.ndss.org; facebook.com/National Down Syndrome Society; twitter.
com/NDSS.
†233 South Wacker Drive., Suite 2400, Chicago, IL 60606-4802; 800-
221-6827; TTY: 312-726-4258; http://www.easterseals.com; email: info@
easterseals.com; facebook.com/easterseals; twitter.com/EasterSealsON.
‡1825 K Street NW, Suite 1200, Washington, DC 20006; 202-534-3700
or 800-433-5255; fax: 202-534-3731; http://www.thearc.org; email: info@
thearc.org; facebook.com/thearcus; twitter.com/thearcus; youtube.com/
user/thearcoftheus.
a self-feeding program, the nurse performs a task analysis. After a task
analysis, the child is observed in a particular situation, such as eating,
to determine what skills are possessed and the child’s developmental
readiness to learn the task. Family members are included in this process
because their “readiness” is as important as the child’s. Numerous self-help
aids are available to facilitate independence and can help eliminate some
of the difficulties of learning, such as using a plate with suction cups
to prevent accidental spills.*
Promote Child’s Optimal Development
Optimal development involves more than achieving independence. It
requires appropriate guidance for establishing acceptable social behavior
and personal feelings of self-esteem, worth, and security. These attributes
are not simply learned through a stimulation program. Rather, they
must arise from the genuine love and caring that exist among family
members. However, families need guidance in providing an environment
that fosters optimal development. Often the nurse can provide assistance
in these areas of childrearing.
Another important area for promoting optimal development and
self-esteem is ensuring the child’s physical well-being. Any congenital
defects, such as cardiac, gastrointestinal, or orthopedic anomalies, should
be repaired. Plastic surgery may be considered when the child’s appear-
ance can be substantially improved. Dental health is significant, and
orthodontic and restorative procedures can improve facial appearance
immensely.
Encourage Play and Exercise
Children who are cognitively impaired have the same need for play
and exercise as any other child. However, because of the children’s
slower development, parents may be less aware of the need to provide
such activities. Therefore the nurse will need to guide parents toward
selection of suitable play and exercise activities. Because play has been
discussed for children in each age-group in earlier chapters, only the
exceptions are presented here (Fig. 20.2).
The type of play is based on the child’s developmental age, although
the need for sensorimotor play may be prolonged. Parents should use
every opportunity to expose the child to as many different sounds,
sights, and sensations as possible. Appropriate toys include musical
mobiles, stuffed toys, floating toys, a rocking chair or horse, a swing,
bells, and rattles. The child should be taken on outings, such as trips to
the grocery store or shopping center. Other people should be encouraged
to visit in the home, and individuals should relate directly to the child
through means such as cuddling, holding, rocking, and talking to the
child in the face-to-face fashion.
Toys are selected for their recreational and educational value. For
example, a large inflatable beach ball is a good water toy; it encourages
interactive play and can be used to learn motor skills, such as balance,
rocking, kicking, and throwing. Attractive toys encourage a child to
reach, therefore assisting in the development of motor skills (see Fig.
20.2). Musical toys that mimic animal sounds or respond with social
phrases are excellent ways of encouraging speech. A doll with removable
clothes and different types of closures can help the child learn dressing
skills. Toys should be simple in design so that the child can learn to
*A resource for a variety of self-help equipment is Patterson Medical
Corporate Headquarters, 28100 Torch Parkway Suite 700, Warrenville,
IL 60555-3938; 800-323-9742; Customer Service: 800-323-5547; http://
www.pattersonmedical.com; http://www.facebook.com/Patterson-
Medical. In Canada: 800-665-9200; 905-858-6000; http://www.patter-
sonmedical.ca.
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family
FIG. 20.2  Placing an attractive object outside the child’s reach encourages
crawling movements. (Courtesy James DeLeon, Texas Children’s Hospital,
Houston, TX.)
FIG. 20.3  A manual switch allows a child with cognitive impairment to
play with a battery-operated toy.
FIG. 20.4  A favorite toy provides stimulation for a young child.
manipulate them without help. For children with severe cognitive and
physical impairment, electronic switches can be used to allow them to
operate toys (Figs. 20.3 and 20.4).
Suitable activities for physical activity are based on the child’s size,
coordination, physical fitness and maturity, motivation, and health (see
Fig. 20.4). Some children may have physical problems that prevent
participation in certain sports, such as atlantoaxial instability in children
635
FIG. 20.5  A child with cognitive and physical impairments can activate
electronic and communication equipment by moving a device near her
head.
with Down syndrome (discussed later in this chapter). These children
often have greater success in individual and dual sports than in team
sports and enjoy themselves most with children of the same develop-
mental level. The Special Olympics* provides these children with a
unique competitive opportunity.
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 adequate hearing and interpretation (receptive skills)
and facial muscle coordination (expressive skills). Because both
receptive and expressive 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 children with physical
limitations, several adaptations or types of communication devices are
available to facilitate selection of the appropriate picture or word (Fig.
20.5). 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 required. The symbols are typically arranged
on a board, and the person points or uses some type of selector to
convey a message.
*1133 19th St. NW, Washington, DC 20036; 800-700-8585 or 202-628-
3630; http://www.specialolympics.org; info@specialolympics.org (website
includes listing of state offices and 170 countries); http://www.facebook.
com>Places>Washington, District of Columbia; twitter.com/Special
Olympics. In Canada: Special Olympics Canada, 21 St. Clair Ave. E,
Suite 600, Toronto, ON M4T 1L9; 416-927-9050 ext. 4388; 888-888-0608;
http://www.specialolympics.ca.
636 SECTION VIII  Family-Centered Care of the Child with Special Needs
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 evaluation of previous mistakes. Behavior modification, especially
reinforcement of desired actions, and use of time-out procedures 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 self-esteem
and social acceptance. Clothes should be clean, age appropriate, and
well fitted with self-adhering fasteners and elastic openings to facilitate
self-dressing.
Opportunities for social interaction and infant stimulation programs
should began at an early age. As soon as possible, parents should enroll
their child in early intervention or other appropriate preschool programs.
Not only do these programs provide education and training, but they
also offer an opportunity for social interaction with other children
and adults. 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
should assess the child’s abilities and encourage others (e.g., parents,
teachers) to promote developmentally appropriate peer interaction,
such as classroom and school activities, dance classes, clubs, vacations
and family outings (Bull & Committee on Genetics, 2011; National
Down Syndrome Society, 2012b; Sanchack & Thomas, 2016; Shapiro
& Batshaw, 2016).
Provide Information on Sexuality
Adolescence may be a particularly difficult time for parents, especially
in terms of the child’s sexual behavior, possibility of pregnancy, future
plans to marry, and ability to be independent. Frequently, minimal
anticipatory guidance has been offered parents to prepare the child for
physical and sexual maturation. The nurse should 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 they are easy
to persuade and lack judgment, they need a well-defined, concrete code
*Sources of information on sexuality and conception include Planned
Parenthood Federation of America, 123 William St., New York, NY
10038; 212-541-7800 or 800-230-7526; http://www.plannedparenthood.
org; http://www.facebook.com/PlannedParenthood/
of conduct with specific instructions for handling certain situations.
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 the child or adolescent from
sexual abuse, parents must closely observe their child’s or adolescent’s
activities and associates. The question of contraceptive protection for
these adolescents is often a parental concern (Quint, O’Brien, Committee
on Adolescence, et al., 2016).
Parents of these adolescents are often concerned about the advisability
of marriage between two individuals with significant CI. There is no
conclusive answer; each situation must be judged individually. In some
instances, marriage is possible. The nurse should discuss this topic with
parents and with the prospective couple, stressing suitable living accom-
modations and contraceptive methods to prevent pregnancy. If children
are conceived, these parents require specialized assistance in learning
to meet the needs of their offspring (Bull & Committee on Genetics,
2011; Shea, 2012).
Help Family Adjust to Future Care
Not all families are able to cope with home care of children who are
cognitively impaired, especially those who have severe or profound CI
or multiple disabilities. Older parents may not be able to continue care
responsibilities after they reach retirement or older age. The decision
regarding residential placement is a difficult one for families, and the
availability of such facilities varies widely. The nurse’s role includes
assisting parents in investigating and evaluating programs and helping
parents adjust to the decision for placement.
Care for the 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;
it may also be destructive to the child’s sense of self-esteem and optimum
development, and it may impair the parents’ ability to cope with the
stress of the experience. To prevent engaging in this nontherapeutic
approach, nurses are to use the mutual participation model in planning
the child’s care. Parents should stay with their child but 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), with special focus on all self-care abilities. Questions about the
child’s abilities 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 that 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-stimulatory
or 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 (Morano,
Ruiz, Hwang, et al., 2017; Oliver & 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 should treat the child with dignity and
respect in a manner that promotes acceptance and understanding by
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family 637

other children, parents, and those with whom the child comes into
contact in the hospital.
Explain procedures to the child using methods of communication
that are at the appropriate cognitive level. Generally, explanations should
be simple, short, and concrete, emphasizing what the child will physically
experience. Demonstration either through actual practice or with visual
aids is always preferable to verbal explanation. Include parents in
preprocedural teaching to aid in the child’s 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.
FIG. 20.6  A young child with Down syndrome holding a doll with Down
Assist in Measures to Prevent Cognitive Impairment syndrome.
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 have higher fertility rates (Arumugam, Raja, Venugopalan, et al., 2016;
impairment are preventable. Counseling and education can reduce or Lee, 2016; National Down Syndrome Society, 2012c). About 4% of the
eliminate such factors (e.g., poor nutrition, cigarette smoking, chemical cases may be caused by translocation of chromosomes 15 and 21 or
abuse), which increase the risk of prematurity and intrauterine growth 22. This type of genetic aberration is usually hereditary and is not
restriction. Interventions are directed toward improving maternal health associated with advanced parental age. About 2% to 4% of affected
by educating women regarding the dangers of chemicals, including persons demonstrate mosaicism, which refers to a mixture of normal
prenatal alcohol exposure, which affects organogenesis, craniofacial and abnormal chromosomes in the cells. The degree of cognitive and
development, and cognitive ability. Other preventive strategies that play physical impairment has been related to the percentage of cells with
an important role include adequate prenatal care; optimal medical care the abnormal chromosome makeup. However, numerous other interacting
of high-risk newborns; rubella immunization; genetic counseling; and factors are likely to contribute to individual differences and cognitive-level
prenatal screening, especially in terms of Down syndrome or FXS. The outcomes in Down syndrome, such as early neural development, dietary
use of folic acid supplements prevents neural tube defects during factors, lifestyle, and the environment (Coppede, 2016; Karmiloff-Smith,
pregnancy and during the childbearing years; the use of newborn Al-Janabi, D’Souza, et al., 2016).
screening for treatable inborn errors of metabolism (e.g., congenital
hypothyroidism, phenylketonuria, and galactosemia) is appropriate to Diagnostic Evaluation
prevent developmental disabilities in children. Down syndrome can usually be diagnosed by the clinical manifestations
alone (Box 20.2 and Fig. 20.6), but a chromosome analysis should be
done to confirm the genetic abnormality.
DOWN SYNDROME Several physical problems are associated with Down syndrome. Many
Down syndrome is the most common chromosomal abnormality of a of these children have congenital heart malformations, the most common
generalized syndrome, occurring from 1 in 691 to 733 live births in the being septal defects. Respiratory tract infections are prevalent and, when
United States (Lee, 2016; Weijerman & de Winter, 2010). It occurs in combined with cardiac anomalies, are the chief causes of death, par-
people of all races and economic levels. ticularly during the first year of life. Hypotonicity of chest and abdominal
muscles and dysfunction of the immune system probably predispose
Etiology the child to the development of respiratory tract infection. Other physical
The cause of Down syndrome is not known, but evidence from cyto- problems include thyroid dysfunction, especially congenital hypothyroid-
genetic and epidemiologic studies supports the concept of multiple ism, and an increased incidence of leukemia.
causality. Although the cause is unclear, the cytogenetics of the disorder
is well established. In most cases, Down syndrome is attributable to an Therapeutic Management
extra chromosome 21 (group G), hence the name nonfamilial trisomy Although no cure exists for Down syndrome, a number of therapies
21. Although children with trisomy 21 are born to parents of all ages, are advocated, such as surgery to correct serious congenital anomalies
there is a statistically greater risk in older women, particularly those (e.g., heart defects, strabismus). These children also benefit from evalu-
older than 35 years of age. For example, in women 35 years old, the ative echocardiography soon after birth and regular medical care.
chance of conceiving a child with Down syndrome is about 1 in 350 Evaluation of sight and hearing is essential, and treatment of otitis
live births; but in women 40 years old, it is about 1 in 100. However, media is required to prevent auditory loss, which can influence cognitive
the majority (approximately 80%) of infants with Down syndrome are function. Periodic testing of thyroid function is recommended, especially
born to women younger than 35 years old because younger women if growth is severely delayed.
638 SECTION VIII  Family-Centered Care of the Child with Special Needs

BOX 20.2  Clinical Manifestations of Down Syndrome

Head and Eyes Abdomen and Genitalia
Separated sagittal suture Protruding, lax, and flabby abdominal muscles
Brachycephaly Diastasis recti abdominis
Rounded and small skull Umbilical hernia
Flat occiput Small penis
Enlarged anterior fontanel Cryptorchidism
Oblique palpebral fissures (upward, outward slant)* Bulbous vulva
Inner epicanthal folds
Speckling of iris (Brushfield spots) Hands and Feet
Broad, short hands and stubby fingers
Nose and Ears Incurved little finger (clinodactyly)
Small nose* Transverse palmar crease
Depressed nasal bridge (saddle nose)* Wide space between big and second toes*
Small ears and narrow canals Plantar crease between big and second toes*
Short pinna (vertical ear length) Broad, short feet and stubby toes
Overlapping upper helices
Conductive hearing loss Musculoskeletal and Skin
Short stature
Mouth and Neck Hyperflexibility and muscle weakness*
High, arched, narrow palate* Hypotonia
Protruding tongue Atlantoaxial instability
Hypoplastic mandible Dry, cracked, and frequent fissuring
Delayed teeth eruption and microdontia Cutis marmorata (mottling)
Alignment teeth abnormalities (common)
Periodontal disease Other
Neck skin excess and laxity* Reduced birth weight
Short and broad neck Learning difficulty (average intelligence quotient [IQ] of 50)
Hypothyroidism (common)
Chest and Heart Impaired immune function
Shortened rib cage Increased risk of leukemia
Twelfth rib anomalies Early-onset dementia (in one-third)
Pectus excavatum or carinatum
Congenital heart defects (common; e.g., atrial septal defect, ventricular septal
defect)

*Most common findings in modified chart (Arumugam, Raja, Venugopalan, et al., 2016; Pueschel, 1999).

About 15% of children with Down syndrome have atlantoaxial

instability, but almost all of the children are asymptomatic. The American
Academy of Pediatrics no longer recommends screening asymptomatic
children with Down syndrome for atlantoaxial instability with cervical
spine x-rays due to unproven value of detecting patients at risk of
developing spinal cord compression injury (Bull & Committee on
Genetics, 2011; National Down Syndrome Society, 2012d). However,
the Special Olympics continues to require that all athletes with Down
syndrome receive neck x-rays before sports participation because neck
x-ray is the only screen available (Lee, 2016; National Down Syndrome
Society, 2012d). Surveillance helps define the most appropriate level of
physical activity and to identify the small subset of those with either
progressive hyperlaxity or instability (O’Toole & Spiegel, 2016). However,
recommendations for surveillance of the cervical spine in children with
Down syndrome remain varied.
NURSING ALERT
Immediately report any child with the following signs of spinal cord compression:
• Persistent neck pain
• Loss of established motor skills and bladder or bowel control
• Changes in sensation
Prognosis
Life expectancy for those with Down syndrome has improved in recent
years but remains lower than for the general population. The majority
of individuals with Down syndrome survive to approximately 60 years
old and beyond (Englund, Jonsson, Zander, et al., 2013; National Down
Syndrome Society, 2012e; Weijerman & de Winter, 2010). As the prognosis
continues to improve for these individuals, it will be important to provide
for their long-term health care and social and leisure needs.
Nursing Care Management
Support the Family at the Time of Diagnosis
Because of the unique physical characteristics, infants with Down
syndrome are usually diagnosed at birth, and parents should be informed
of the diagnosis at this time. Most parents usually prefer that both of
them be present during the informing interview so that they can support
one another emotionally. Parents appreciate receiving reading material
about the syndrome* and being referred to parent groups and/or profes-
sional counseling.
*For the ARC and National Down Syndrome Society contact information,
see the footnotes earlier in this chapter.
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family
Parental responses to the child may greatly influence decisions
regarding future care. Whereas some families willingly take the child
home, others consider foster care or adoption. The nurse must answer
questions regarding developmental potential carefully because the
responses may influence the parents’ decision. The nurse should share
the available informative sources (e.g., parent groups, professional
counseling, and literature) to help the family learn about Down syndrome
(see Critical Thinking Case Study box).
? CRITICAL THINKING CASE STUDY
Diagnosis of Down Syndrome
The parents of Melissa, a newborn diagnosed as having Down syndrome, ask
the nurse, “What are we supposed to do with her?” They further state that
they already have three other children at home.
1. What evidence should you consider regarding this condition?
2. What additional information is required at this time?
3. List the nursing intervention(s) that have the highest priority.
4. Identify important patient-centered outcomes with reference to your nursing
interventions.
Answers are available at http://evolve.elsevier.com/wong/ncic.
Assist the Family in Preventing Physical Problems
Many of the physical characteristics of infants with Down syndrome
present challenges and nursing problems. The hypotonicity of muscles
and hyperextensibility of joints complicate positioning. The limp, flaccid
extremities resemble the posture of a rag doll; as a result, holding the
infant is difficult and cumbersome. Sometimes parents perceive this
lack of the infant’s molding to their bodies as evidence of inadequate
parenting. The extended body position promotes heat loss because more
surface area is exposed to the environment. Encourage the parents to
swaddle or wrap the infant snugly in a blanket before picking up the
child to provide security and warmth. The nurse also discusses with
parents their feelings concerning attachment to the child, emphasizing
that the child’s lack of clinging or molding is a physical characteristic
and not a sign of detachment or rejection.
Decreased muscle tone compromises respiratory expansion. In
addition, the underdeveloped nasal bone causes a chronic problem of
inadequate drainage of mucus. The constant stuffy nose forces the child
to breathe by mouth, which dries the oropharyngeal membranes,
increasing the susceptibility to upper respiratory tract infections. Measures
to lessen these problems include clearing the nose with a bulb-type
syringe, rinsing the mouth with water after feedings, increasing fluid
intake, and using a cool-mist vaporizer to keep the mucous membranes
moist and the secretions liquefied. Other helpful measures include
changing the child’s position frequently, practicing good hand washing,
and properly disposing of soiled articles, such as tissues. If antibiotics
are ordered, the nurse stresses the importance of completing the full
course of therapy for successful eradication of the infection and preven-
tion of growth of resistant organisms.
Inadequate drainage resulting in pooling of mucus in the nose also
interferes with feeding. Because the child breathes by mouth, sucking
for any length of time is difficult. When eating solids, the child may
gag on the food because of mucus in the oropharynx. Parents are advised
to clear the nose before each feeding; give small, frequent feedings; and
allow opportunities for rest during mealtime.
The protruding tongue also interferes with feeding, especially of
solid foods. Parents need to know that the tongue thrust is not an
indication of refusal to feed but a physiologic response. Parents are
639
advised to use a small but long, straight-handled spoon to push the
food toward the back and side of the mouth. If food is thrust out, it
should be refed.
Dietary intake needs supervision. Decreased muscle tone affects
gastric motility, predisposing the child to constipation. Dietary measures,
such as increased fiber and fluid, promote evacuation. The child’s eating
habits may need careful scrutiny to prevent obesity. Height and weight
measurements should be obtained on a serial basis. Because the previously
used Down syndrome–specific growth charts no longer reflected the
current population styles and body proportions, new growth charts
were developed to provide indications of how growth of an individual
child compares with peers of the same age and sex with Down syndrome
(Centers for Disease Control and Prevention, 2016a; Zemel, Pipan,
Stallings, et al., 2015).
During infancy, the child’s skin is pliable and soft. However, it
gradually becomes rough and dry and is prone to cracking and infection.
Skin care involves the use of minimum soap and application of lubricants.
Lip balm is applied to the lips, especially when the child is outdoors,
to prevent excessive chapping.
Assist in Prenatal Diagnosis and Genetic Counseling
Prenatal diagnosis of Down syndrome is possible through chorionic
villus sampling and amniocentesis because chromosome analysis of
fetal cells can detect the presence of trisomy or translocation. However,
advances in development of noninvasive prenatal testing have resulted
in a measurement of cell-free deoxyribonucleic acid (DNA) from the
plasma of pregnant women, detecting nearly all cases of Down syndrome
(Huang, Zheng, Chen, et al., 2014; Lee, 2016; Lewis, Hill, Silcock, et al.,
2014; Liao, Chan, Jiang, et al., 2012).
Prenatal testing with genetic counseling should be offered to all
women, including those of advanced maternal age (greater than 35
years) and those of younger age (less than 35 years) because most
children with Down syndrome are born to younger mothers due to
their higher overall birth rate (Lee, 2016). If prenatal testing indicates
that the fetus is affected, the nurse must allow the parents to express
their feelings concerning elective abortion and support their decision to
terminate or proceed with the pregnancy. It is important for nurses to
be aware of their own attitudes regarding testing and related decisions.
FRAGILE X SYNDROME
FXS is the most common inherited cause of CI and the second most
common genetic cause of CI or intellectual disability after Down
syndrome. It has been described in all ethnic groups and races; the
incidence of affected boys is 1 in 3600 to 4000, the incidence of affected
girls is 1 in 4000 to 6000, the incidence of carrier girls is 1 in 151, and
the incidence of carrier boys is 1 in 468 worldwide (Mink, 2016; National
Fragile X Foundation, 2017a).
The syndrome is caused by an abnormal gene on the lower end of
the long arm of the X chromosome. Chromosome analysis may dem-
onstrate a fragile site (a region that fails to condense during mitosis
and is characterized by a nonstaining gap or narrowing) in the cells of
affected males and females and in carrier females. This fragile site has
been determined to be caused by a gene mutation that results in excessive
repeats of nucleotide in a specific DNA segment of the X chromosome.
The number of repeats in a normal individual is between 6 and 50. An
individual with 50 to 200 base-pair repeats is said to have a permutation
and is therefore a carrier. When passed from a parent to a child, these
base-pair repeats can expand from 200 or more, which is termed a full
mutation. This expansion occurs only when a carrier mother passes
the mutation to her offspring; it does not occur when a carrier father
passes the mutation to his daughters.
640 SECTION VIII  Family-Centered Care of the Child with Special Needs
The inheritance pattern has been termed X-linked dominant with
reduced penetrance. This is in distinct contrast to the classic X-linked
recessive pattern in which all carrier females are normal, all affected
males have symptoms of the disorder, and no males are carriers.
Consequently, genetic counseling of affected families is more complex
than that for families with a classic X-linked disorder, such as hemophilia.
Both affected sexes are capable of transmitting the fragile X disorder.
Prenatal diagnosis of the fragile X gene mutation is possible with direct
DNA testing in a family with an established history using amniocentesis
or chorionic villus sampling (Finucane, Lincoln, Bailey, et al., 2017;
National Fragile X Foundation, 2017b). The FMR1 mutation testing is
highly accurate and is being researched regarding the incorporation
into the newborn universal screening program (Abrams, Cronister,
Brown, et al., 2012; Bagni, Tassone, Neri, et al., 2012; Finucane, Abrams,
Cronister, et al., 2012; Sorensen, Gane, Yarborough, et al., 2013). A
systematic review of screening technologies found appropriate methods
to screen larger populations of males and females at low cost so that
early interventions may help prevent or delay the disability of fragile
X (Lyons, Kerr, & Mueller, 2015).
Clinical Manifestations
The classic trend of physical findings in adult men with FXS consists
of a long face with a prominent jaw (prognathism); large, protruding
ears; and large testes (macroorchidism). In prepubertal children, however,
these features may be less obvious, and behavioral manifestations may
initially suggest the diagnosis (Box 20.3). In carrier females, the clinical
manifestations are extremely varied.
Therapeutic Management
FXS has no cure. Medical treatment may include the use of serotonin
agents, such as carbamazepine (Tegretol) or fluoxetine (Prozac), to
control violent temper outbursts and the use of central nervous system
stimulants or clonidine (Catapres) to improve attention span and decrease
hyperactivity. Two possible treatments of FXS being investigated are
reactivation of the affected gene and protein replacement (Bagni, Tassone,
Neri, et al., 2012; Kuehn, 2011).
All affected children require referral to early intervention program
that include speech and language therapy, occupational therapy, and
special education assistance. A multidisciplinary assessment of common
medical problems associated with FXS such as cardiac, neurologic, and
BOX 20.3  Clinical Manifestations of
Fragile X Syndrome
Physical Features
Increased head circumference
Long, wide, or protruding ears
Long, narrow face with prominent jaw
Strabismus
Mitral valve prolapse, aortic root dilation
Hypotonia
In postpubertal males, enlarged testicles
Behavioral Features
Mild to severe cognitive impairment
Speech delay; may be rapid speech with stuttering and word repetition
Short attention span, hyperactivity
Hypersensitivity to taste, sounds, touch
Intolerance to change in routine
Autistic-like behaviors, such as social anxiety and gaze aversion
Possible aggressive behavior
orthopedic anomalies and gastrointestinal problems is imperative to
improve comprehensive care that may lead to better quality of life for
these patients and their families (Kidd, Lachiewicz, Barbouth, et al.,
2014).
Prognosis
Individuals with FXS are expected to live a normal life span. Their CI
may be improved by behavioral and educational interventions that
usually begin in preschool-age children.
Nursing Care Management
Because CI is a fairly consistent finding in individuals with FXS, the
care given to these families is the same as for any child with intellectual
disability. Because the disorder is hereditary, genetic counseling is
important to inform parents and siblings of the risks of transmission.
In addition, any male or female with unexplained or nonspecific mental
impairment should be referred for genetic testing and, if needed,
counseling. Families with a member affected by the disorder should be
referred to the National Fragile X Foundation.*
SENSORY IMPAIRMENT
HEARING IMPAIRMENT
Hearing impairment is one of the most common disabilities in the
United States. An estimated 1 to 6 per 1000 well infants have hearing
loss of varying degrees (American Academy of Pediatrics, 2017; Grindle,
2014). For infants admitted to neonatal intensive care units, the incidence
rises sharply to approximately 2 to 4 per 100 neonates (American
Academy of Pediatrics, Joint Committee on Infant Hearing, 2007;
Almadhoob & Ohlsson, 2015; Colella-Santos, Hein, de Souza, et al.,
2014). In the United States there are about 1 million children with
hearing impairment ranging in age from birth to 21 years old, and
almost one-third of these children have other disabilities, such as visual
or cognitive deficits.
Definition and Classification
Hearing impairment is a general term indicating disability that may
range in severity from slight to profound hearing loss. Slight to moderately
severe hearing loss describes a person who has residual hearing sufficient
to enable successful processing of linguistic information through audition,
generally with the use of a hearing aid. Severe to profound hearing loss
describes a person whose hearing disability precludes successful
processing of linguistic information through audition with or without
a hearing aid. Hearing-impaired persons who are speech impaired tend
not to have a physical speech defect other than that caused by the
inability to hear.
Hearing defects may be classified according to etiology, pathology,
or symptom severity. Each is important in terms of treatment, possible
prevention, and rehabilitation.
Etiology
Hearing loss may be caused by a number of prenatal and postnatal
conditions. These may include a family history of childhood hearing
impairment, anatomic malformations of the head or neck, low birth
weight, severe perinatal asphyxia, perinatal infection (cytomegalovirus,
*2100 M St. NW, Ste. 170, PO Box 302, Washington, DC 20037-1233;
800-688-8765; fax: 202-747-6208; http://www.fragilex.org; email:
natlfx@fragilex.org; http://www.facebook.com/natlfragilex; twitter.
com/FragileXnews.
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family
rubella, herpes, syphilis, toxoplasmosis, bacterial meningitis), maternal
prenatal substance abuse, chronic ear infection, cerebral palsy, Down
syndrome, prolonged neonatal oxygen supplementation, or administra-
tion of ototoxic drugs (Colella-Santos, Hein, de Souza, et al., 2014; Gan,
Rowe, Benton, et al., 2016; Haddad & Keesecker, 2016; Singh, 2015).
In addition, high-risk neonates who survive the once fatal prenatal
or perinatal conditions may be susceptible to hearing loss from the
disorder or its treatment. For example, sensorineural hearing loss may
be a result of continuous humming noises or high noise levels associated
with incubators, oxygen hoods, or intensive care units, especially when
combined with the use of potentially ototoxic antibiotics.
Environmental noise is a special concern. Sounds loud enough to
damage sensitive hair cells of the inner ear can produce irreversible
hearing loss. Very loud, brief noise (e.g., gunfire) can cause immediate,
severe, and permanent loss of hearing. Longer exposure to less intense
but still hazardous sounds (e.g., loud persistent music via headphones,
sound systems, concerts, or industrial noises) may also produce hearing
loss (Biassoni, Serra, Hinalaf, et al., 2014; Carroll, Eichwald, Scinicariello,
et al., 2017; Centers for Disease Control and Prevention, 2016b; Guest,
Munro, Prendergast, et al., 2017; Liberman & Kujawa, 2017; Pawlaczyk-
Luszczynska, Zamojska-Daniszewska, Dudarewicz, et al., 2017). Hearing
loss caused by toxic substances (e.g., smoking or secondhand smoke)
or when combined with loud noises, tends to produce a synergistic
effect on hearing that causes hearing dysfunction (Fabry, Davila, Arheart,
et al., 2011; Talaat, Metwaly, Khafagy, et al., 2014).
Pathology
Disorders of hearing are divided according to the location of the defect.
Conductive or middle-ear hearing loss results from interference of
transmission of sound to the middle ear. It is the most common of all
types of hearing loss and most frequently a result of recurrent serous
otitis media. Conductive hearing impairment involves mainly interference
with loudness of sound.
Sensorineural hearing loss involves damage to the inner ear structures
or the auditory nerve. The most common causes are congenital defects
of inner ear structures or consequences of acquired conditions, such
as kernicterus, infection, administration of ototoxic drugs, or exposure
to excessive noise. Sensorineural hearing loss results in distortion of
sound and problems in discrimination. Although the child hears some
of everything going on around him or her, the sounds are distorted,
severely affecting discrimination and comprehension.
Mixed conductive-sensorineural hearing loss results from interference
with transmission of sound in the middle ear and along neural pathways.
It frequently results from recurrent otitis media and its complications.
Central auditory imperception includes all hearing losses that are
not linked to defects in the conductive or sensorineural structures. They
are usually divided into organic or functional losses. In the organic
type of central auditory imperception, the defect involves the reception
of auditory stimuli along the central pathways and the expression of
the message into meaningful communication. Examples are aphasia,
the inability to express ideas in any form, either written or verbal;
agnosia, the inability to interpret sound correctly; and dysacusis, dif-
ficulty in processing details or discriminating among sounds. In the
functional type of hearing loss, no organic lesion exists to explain a
central auditory loss. Examples of functional hearing loss are con-
version hysteria (an unconscious withdrawal from hearing to block
remembrance of a traumatic event), infantile autism, and childhood
schizophrenia.
Symptom Severity
Hearing impairment is expressed in terms of a decibel (dB), a unit of
loudness (see Table 20.1). Hearing is measured at various frequencies,
641
TABLE 20.2  Classification of Hearing
Impairment Based on Symptom Severity
Hearing Level (dB) Effect
Slight: 16-25 Has difficulty hearing faint or distant speech
Usually is unaware of hearing difficulty
Likely to achieve in school but may have problems
No speech defects
Mild to moderate: May have speech difficulties
26-55 Understands face-to-face conversational speech
at 0.9-1.5 m (3-5 ft)
Moderately severe: Unable to understand conversational speech
56-70 unless loud
Considerable difficulty with group or classroom
discussion
Requires special speech training
Severe: 71-90 May hear a loud voice if nearby
May be able to identify loud environmental noises
Can distinguish vowels but not most consonants
Requires speech training
Profound: 91 May hear only loud sounds
Requires extensive speech training
dB, Decibels.
such as 500, 1000, and 2000 cycles/second, the critical listening speech
range. Hearing impairment can be classified according to hearing
threshold level (the measurement of an individual’s hearing threshold
by means of an audiometer) and the degree of symptom severity as it
affects speech (Table 20.2). These classifications offer only general
guidelines regarding the effect of the impairment on any individual
child because children differ greatly in their ability to use residual hearing.
Therapeutic Management
Conductive Hearing Loss
Treatment of hearing loss depends on the cause and type of hearing
impairment. Many conductive hearing defects respond to medical or
surgical treatment, such as antibiotic therapy for acute otitis media or
insertion of tympanostomy tubes for chronic otitis media. When the
conductive loss is permanent, hearing can be improved with the use
of a hearing aid to amplify sound.
The nurse should be familiar with the types, basic care, and handling
of hearing aids, especially when the child is hospitalized.* Types of aids
include those worn in or behind the ear, models incorporated into an
eyeglass frame, and types worn on the body with a wire connection to
the ear (Fig. 20.7). One of the most common problems with a hearing
aid is acoustic feedback, an annoying whistling sound usually caused
by improper fit of the ear mold. Sometimes the whistling may be at a
frequency that the child cannot hear but that is annoying to others. In
this case, if children are old enough, they are told of the noise and
asked to readjust the aid.
As children grow older, they may be self-conscious about the device.
Efforts may be made to make the aid inconspicuous, such as styling
the hair to cover behind-the-ear aids or the use of in-the-ear or miniature
digital models, or encourage the use of attractive frames for glasses
*Information about hearing aids is available from the International
Hearing Society, 16880 Middlebelt Road, Suite 4, Livonia, MI 48154;
800-521-5247 or 734-522-7200; http://www.ihsinfo.org; http://www.
facebook.com/ihsinfo; twitter.com/IHSinfo.
642 SECTION VIII  Family-Centered Care of the Child with Special Needs
FIG. 20.7  On-the-body hearing aids are convenient for young children,
such as this child with severe bilateral hearing loss. Note eye patching
for strabismus.
NURSING ALERT
To reduce or eliminate whistling from a hearing aid, try removing and reinserting
the aid, making certain that no hair is caught between the ear mold and the
ear canal; cleaning the ear mold or ear; or lowering the volume of the aid.
with connected hearing aids. Give children responsibility for the care
of the device as soon as they are able because fostering independence
is a primary goal of rehabilitation.
NURSING ALERT
Stress to parents the importance of storing batteries for hearing aids in a safe
location out of reach of children and teaching children not to remove the
battery from the hearing aid (or supervising young children when they do so).
Battery ingestion requires immediate emergency management.
Sensorineural Hearing Loss
The treatment aim for sensorineural hearing loss is to improve hearing
and communication with hearing aids or cochlear implants. Sensorineural
hearing loss has been associated with damaged auditory hair cells or
nerve fibers or abnormal development of the inner ear structures. Because
conventional hearing aids only amplify sound that may not be processed
by a damaged inner ear, some children may not benefit from hearing
aids and require a referral for a cochlear implant. A cochlear implant
bypasses the hair cells to directly stimulate surviving auditory nerve
fibers so that they can send signals to the brain. These signals can be
interpreted by the brain to produce sound and sensations (Easwar,
Yamazaki, Deighton, et al., 2017; Gan, Rowe, Benton, et al., 2016; Grindle,
2014). The cochlear implant* consists of an internal surgically implanted
*Hearing Enrichment Language Program of the Hough Ear Institute
as part INTEGRIS Baptist Medical Center Cochlear Implant Clinic,
3300 N.W. Expressway, Oklahoma City, OK 73112; 405-949-3011 or
888-951-2277; http://integrisok.com/baptist-medical-center-oklahoma-
city-ok-services-hearing; facebook.com/integrishealthOK.
prosthetic device (receiver and electrode array) and external device
(microphone, speech processor, and transmitter coil) (Gan, Rowe, Benton,
et al., 2016). The cochlear implant provides a sensation of hearing for
individuals who have severe or profound hearing loss (Farinetti, Gharbia,
Mancini, et al., 2014; Lantos, 2012; Pettinato, De Clerck, Verhoeven,
et al., 2017).
Multichanneled implants are sophisticated devices that stimulate
the auditory nerve at a number of locations with differently processed
signals. This type of stimulation allows a person to use the pitch
information present in speech signals, leading to better understanding
of speech. The trend is toward early use of cochlear implants, usually
by 12 months old, to give the child maximum opportunity to develop
listening, language, and speaking skills.
The cochlear implantation is a safe hearing surgical technique
associated with a low complication rate. A reported global complication
rate comprised minor complications that were mainly infectious in
children (acute otitis media) and cochleovestibular in adults (tinnitus
and vetigo) and major complications, including mostly reimplantation
after revision surgery or device failure (Farinetti, Gharbia, Mancini,
et al., 2014). However, cases of meningitis, particularly pneumococcal
meningitis, have been reported. Therefore it has been recommended
that all children receiving a cochlear implant must be vaccinated with
the pneumococcal polyvalent vaccine (Haddad & Keesecker, 2016).
Nursing Care Management
Assess for Hearing Concerns
Assessment of children for hearing impairment is a critical nursing
responsibility. Identification of hearing loss before the first 3 months
of age with intervention no later than 6 months old is essential to
improve the language and educational development for children with
hearing impairments (Lammers, Jansen, Grolman, et al., 2015; Rohlfs,
Friedhoff, Bohnert, et al., 2017; World Health Organization, 2012). The
Joint Committee on Infant Hearing issued guidelines on auditory
screening of newborns and infants to detect early hearing loss and
implement intervention programs (American Academy of Pediatrics,
2017; American Academy of Pediatrics, Joint Committee on Infant
Hearing, 2007; Joint Committee on Infant Hearing of the American
Academy of Pediatrics, Muse, Harrison, et al., 2013). Auditory testing
is presented in Chapter 4.
At birth, the nurse can observe the neonate’s response to auditory
stimuli, as evidenced by the startle reflex, head turning, eye blinking,
and cessation of body movement. The infant may vary in the intensity
of the response, depending on the state of alertness. However, a consistent
absence of a reaction should lead to suspicion of hearing loss. Box 20.4
summarizes other clinical manifestations of hearing impairment in
infants.
Children who are profoundly hearing impaired are much more likely
to be diagnosed during infancy than the child who is less severely affected.
If the defect is not detected during early childhood, it likely will become
evident during entry into school, when the child has difficulty learning.
Unfortunately, some of these children are erroneously placed in special
classes for students with learning disabilities or CI. Therefore it is essential
that the nurse suspect a hearing impairment in any child who demon-
strates the behaviors listed in Box 20.4.
NURSING ALERT
When parents express concern about their child’s hearing and speech develop-
ment, refer the child for a hearing evaluation. Absence of well-formed syllables
(da, na, yaya) by 11 months old should result in immediate referral.
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family
BOX 20.4  Clinical Manifestations of
Hearing Impairment
Infants
Lack of startle or blink reflex to a loud sound
Failure to be awakened by loud environmental noises
Failure to localize a source of sound by 6 months old
Absence of babble or voice inflections by 7 months old
General indifference to sound
Lack of response to the spoken word; failure to follow verbal directions
Response to loud noises as opposed to the voice
Children
Use of gestures rather than verbalization to express desires, especially after
15 months old
Failure to develop intelligible speech by 24 months old
Monotone and unintelligible speech; lessened laughter
Vocal play, head banging, or foot stamping for vibratory sensation
Yelling or screeching to express pleasure, needs, or annoyance
Asking to have statements repeated or answering them incorrectly
Greater response to facial expression and gestures than to verbal explanation
Avoidance of social interaction; prefer to play alone
Inquiring, sometimes confused facial expression
Suspicious alertness alternating with cooperation
Frequent stubbornness because of lack of comprehension
Irritability at not making themselves understood
Shy, timid, and withdrawn behavior
Frequent appearance of being “in a world of their own” or markedly inattentive
During early childhood, the primary importance of hearing impair-
ment is the effect on speech development. A child with a mild conductive
hearing loss may speak fairly clearly but in a loud, monotone voice. A
child with a sensorineural defect usually has difficulty in articulation.
Communication may be difficult, leading to frustration when words
are not understood. For example, an inability to hear higher frequencies
may result in the word spoon being pronounced “poon.” Children with
articulation problems need to have their hearing tested.
Lipreading.  Although the child may become an expert at lipreading,
only about 40% of the spoken word is understood, less if the speaker
has an accent, mustache, or beard. Exaggerating pronunciation or
speaking in an altered rhythm further lessens comprehension. Parents
can help the child understand the spoken word by using the suggestions
in the Nursing Care Guidelines box. The child learns to supplement
the spoken word with sensitivity to visual cues, primarily body language
and facial expression (e.g., tightening the lips, muscle tension, eye
contact).
Cued speech.  The cued speech method of communication is an
adjunct to straight lipreading. It uses hand signals to help the hearing-
impaired child distinguish between words that look alike when formed
by the lips (e.g., mat, bat). It is most commonly employed by hearing-
impaired children who are using speech rather than those who are
nonverbal.
Sign language.  Sign language, such as American Sign Language
(ASL) or British Sign Language (BSL), is a visual-gestural language that
uses hand signals that roughly correspond to specific words and concepts
in the English language. Encourage family members to learn signing
because using or watching hands requires much less concentration than
lipreading or talking. Also, a symbol method enables some hearing-
impaired children to learn more and to learn faster.
Speech-language therapy.  The most formidable task in the education
of a child who is profoundly hearing impaired is learning to speak.
643
NURSING CARE GUIDELINES
Facilitating Lipreading
Attract child’s attention before speaking; use light touch to signal speaker’s
presence.
Stand close to child.
Face child directly or move to a 45-degree angle.
Stand still; do not walk back and forth or turn away to point or look
elsewhere.
Establish eye contact and show interest.
Speak at eye level and with good lighting on speaker’s face.
Be certain nothing interferes with speech patterns, such as chewing food or
gum.
Speak clearly and at a slow and even rate.
Use facial expression to assist in conveying messages.
Keep sentences short.
Rephrase message if child does not understand the words.
Speech is learned through a multisensory approach using visual, tactile,
kinesthetic, and auditory stimulation. Encourage parents to participate
fully in the learning process.
Additional aids. Everyday activities present problems for older
children with hearing impairment. For example, they may not be able
to hear the telephone, doorbell, or alarm clock. Several commercial
devices are available to help them adjust to these dilemmas. Flashing
lights can be attached to a telephone or doorbell to signal its ringing.
Trained hearing ear dogs can provide great assistance because they alert
the person to sounds, such as someone approaching, a moving car, a
signal to wake up, or a child’s cry. Special teletypewriters or telecom-
munications devices for the deaf (TDD or TTY) help hearing-impaired
people communicate with each other over the telephone; the typed
message is conveyed via the telephone lines and displayed on a small
screen.*
Any audiovisual medium presents dilemmas for these children, who
can see the picture but cannot hear the message. However, with closed
captioning a special decoding device is attached to the television, and
the audio portion of a program is translated into subtitles that appear
on the screen.†
Socialization.  Socialization is extremely important to children’s
development. If children attend a special school for the hearing impaired,
they are able to socialize with peers in that setting. Classmates become
a potential source of close friendships because they communicate more
easily among themselves. Encourage parents to promote these relation-
ships whenever possible.
*Resources and support network information are provided by the
Alexander Graham Bell Association for the Deaf and Hard of Hearing,
3417 Volta Place NW, Washington, DC 20007; voice: 202-337-5220; TTY:
202-337-5221; http://www.agbell.org; fax: 202-337-8314; http://www.
agbell.org; email: info@agbell.org; facebook.com>…>Washington,District
of Columbia>Social Services; https://twitter.com/AGBellAssoc; and
Canadian Hearing Society, 271 Spadina Road, Toronto, ON M5R 2V3;
voice: 416-928-2500; TTY: 416-964-0023; fax: 416-928-2506; http://
www.chs.ca; email: info@chs.ca; https://www.facebook.com>Places>T
oronto,Ontario>Medical&Health.
†Additional information is available from the National Captioning
Institute, 3725 Concord Pkwy., Suite 100, Chantilly, VA 20151; voice/
TTY: 703-917-7600; http://www.ncicap.org.
644 SECTION VIII  Family-Centered Care of the Child with Special Needs
Children with a hearing impairment may need special help with
school or social activities. For children wearing hearing aids, keep
background noise to a minimum. Because many of these children are
able to attend regular classes, the teacher may need assistance in adapting
methods of teaching for the child’s benefit. The school nurse is often
in an optimal position to emphasize methods of facilitated communica-
tion, such as lipreading (see Nursing Care Guidelines box). Because
group projects and audiovisual teaching aids may hinder the hearing-
impaired child’s learning, carefully evaluate the use of these educational
methods.
In a group setting, it is helpful for the other members to sit in a
semicircle in front of the hearing-impaired child. Because one of the
difficulties in following a group discussion is that the hearing-impaired
child is unaware of who will speak next, someone should point out
each speaker. Speakers can also be given numbers, or their names can
be written down as each person talks. If one person writes down the
main topic of the discussion, the child is able to follow lipreading more
closely. Such practices can increase the child’s ability to participate in
sports, organizations such as Scouts, and group projects.
Support Child and Family
Once the diagnosis of hearing impairment is made, parents need extensive
support to adjust to the shock of learning about their child’s disability
and an opportunity to realize the extent of the hearing loss. If the
hearing loss occurs during childhood, the child also requires sensitive,
supportive care during the long and often difficult adjustment to this
sensory loss. Early rehabilitation is one of the best strategies for fostering
adjustment. Progress in learning communication, however, may not
always coincide with emotional adjustment. Depression or anger is
common, and such feelings are a normal part of the grieving process.
Care for the Child During Hospitalization
The needs of the hospitalized child with impaired hearing are the same
as those of any other child, but the disability presents special challenges
to the nurse. For example, verbal explanations must be supplemented
by tactile and visual aids, such as books or actual demonstration and
practice. Children’s understanding of the explanation needs to be
constantly reassessed. If their verbal skills are poorly developed, they
can answer questions through drawing, writing, or gesturing. For example,
if the nurse is attempting to clarify where a spinal tap is done, ask the
child to point to where the procedure will be done on the body. Because
hearing-impaired children often need more time to grasp the full meaning
of an explanation, the nurse needs to be patient, allowing ample time
for understanding.
When communicating with the child, the nurse should use the same
principles as those outlined for facilitating lipreading. Ideally, nurses
without foreign accents should be assigned to the child. The child’s
hearing aid is checked to ensure that it is working properly. If it is
necessary to awaken the child at night, the nurse should gently shake
the child or turn on the hearing aid before arousing the child. The
nurse should always make certain that the child can see him or her
before any procedures, even routine ones such as changing a diaper or
regulating an infusion. It is important to remember that the child may
not be aware of the nurse’s presence until alerted through visual or
tactile cues.
Ideally, parents are encouraged to room with the child. However, the
nurse must convey to them that this is not to serve as a convenience to
the nurse but as a benefit to the child. Although the parents’ aid can
be enlisted in familiarizing the child with the hospital and explain-
ing procedures, the nurse should also talk directly to the youngster,
encouraging expression of feelings about the experience. If the child’s
speech is difficult to understand, try to become familiar with his or her
pronunciation of words. Parents often can be helpful by explaining the
child’s usual speech habits. Nonverbal communication devices that use
pictures or words that the child can point to are also available. The nurse
can make boards by using pictures or writing the words on cardboard
representing common needs, such as parent, food, water, or toilet.
The nurse has a special role as child advocate and is in a strategic
position to alert other health team members and other patients to the
child’s special needs regarding communication. For example, the nurse
should accompany other practitioners on visits to the child’s room to
ensure that they speak to the child and that the child understands what
is said. Caregivers may forget that the child has the abilities to perceive
and learn despite a hearing loss, and consequently they communicate
only with the parents. As a result, the child’s needs and feelings remain
unrecognized and unaddressed.
Because children with impaired hearing may have difficulty forming
social relationships with other children, introduced the child to room-
mates and encourage them to engage in play activities. The hospital
setting can provide growth-promoting opportunities for social relation-
ships. With the assistance of a child life specialist, the child can learn
new recreational activities, experiment with group games, and engage
in therapeutic play. Playing with puppets or dollhouses, role-playing
with dress-up clothes, building with blocks or legos, finger painting,
and water play can help the child express feelings that previously were
suppressed.
Assist in Measures to Prevent Hearing Impairment
A primary nursing role is prevention of hearing loss. Because the most
common cause of impaired hearing is chronic otitis media, it is essential
that appropriate measures be instituted to treat existing infections and
prevent recurrences. Children with a history of ear or respiratory
infections or any other condition known to increase the risk of hearing
impairment should receive periodic auditory testing.
To prevent the causes of hearing loss that begin prenatally and
perinatally, pregnant women need counseling regarding the necessity
of early prenatal care, including genetic counseling for known familial
disorders; avoidance of all ototoxic drugs, especially during the first
trimester; tests to rule out syphilis, rubella, or blood incompatibility;
medical management of maternal diabetes; strict control of alcohol
intake; adequate dietary intake; and avoidance of smoke exposure. Stress
the necessity of routine immunization during childhood to eliminate
the possibility of acquired sensorineural hearing loss from rubella,
mumps, or measles (encephalitis).
Exposure to excessive noise pollution is a well-established cause of
sensorineural hearing loss. The nurse should routinely assess the pos-
sibility of environmental noise pollution and advise children and parents
of the potential danger. A recent randomized single-blind clinical trial
supported the use of earplugs in preventing temporary hearing loss
associated with loud music exposure (Ramakers, Kraaijenga, Cattani,
et al., 2016). Therefore when individuals engage in activities associated
with high-intensity noise (e.g., flying model airplanes, loud music, target
shooting, or snowmobiling), they should protect their hearing by wearing
ear protection devices, decreasing music volume, and limiting or avoiding
exposure to loud sounds (Centers for Disease Control and Prevention,
2016b). Even common household equipment, such as lawn mowers,
vacuum cleaners, and telephones, can be harmful.
NURSING ALERT
Suspect hazardous noise if the listener experiences (1) difficulty in communication
while hearing the sound, (2) ringing in the ears (tinnitus) after exposure to the
sound, or (3) muffled hearing after leaving the sound.
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family
VISUAL IMPAIRMENT
Visual impairment is a common problem during childhood. In the
United States the prevalence of serious visual impairment in the pediatric
population is estimated to be between 30 and 64 children per 100,000
population. Vision impairment such as refractive error, strabismus, and
amblyopia occur in 5% to 10% of all preschoolers, who are usually
identified through vision screening programs (Alley, 2013; American
Academy of Pediatrics, 2016; Rahi, Cumberland, Peckham, et al., 2010;
US Department of Health and Human Services, Office of Disease Preven-
tion and Health Promotion, 2015; US Preventive Services Task Force,
2011). The nurse’s role is one of assessment, detection, prevention,
referral, and (in some instances) rehabilitation.
Definition and Classification
Visual impairment is a general term that encompasses both partial sight
and legal blindness. Partial sight or partial visual impairment is defined
as a visual acuity between 20/70 and 20/200. The child can generally
use normal-sized print because near vision is almost always better than
distance vision. Legal blindness or severe permanent visual impairment
is defined as a visual acuity of 20/200 or lower or a visual field of 20
degrees or less in the better eye. It is important to keep in mind that
legal blindness is not a medical diagnosis but a legal definition.
BOX 20.5  Types of Visual Impairment
Refractive Errors
Myopia
Nearsightedness: Ability to see objects clearly at close range but not at a
distance
Pathophysiology
Results from eyeball that is too long, causing images to fall in front of the
retina
Clinical Manifestations
Headaches
Dizziness
Excessive eye rubbing
Head tilt or forward head thrusts
Difficulty in reading or doing other close work
Clumsiness; walking into objects
Blinking more than usual or irritability when doing close work
Inability to see objects clearly
Poor school performance, especially in subjects that require demonstration, such
as arithmetic
Treatment
Corrected with biconcave lenses that focus rays on retina
May be corrected with laser surgery
Hyperopia
Farsightedness: Ability to see objects at a distance but not at close range
Pathophysiology
Results from eyeball that is too short, causing image to focus beyond retina
Clinical Manifestations
Because of accommodative ability, child can usually see objects at all ranges
Most children are normally hyperopic until about 7 years old
645
Educational and governmental agencies in the United States use the
legal definition of blindness to determine tax status, eligibility for entrance
into special schools, eligibility for financial aid, and other benefits.
Etiology
Visual impairment can be caused by a number of genetic and prenatal
or postnatal conditions. These include perinatal infections (herpes,
chlamydia, gonococci, rubella, syphilis, toxoplasmosis); retinopathy of
prematurity; trauma; postnatal infections (meningitis); and disorders,
such as sickle cell disease, juvenile rheumatoid arthritis, Tay-Sachs disease,
albinism, and retinoblastoma. In many instances, such as with refractive
errors, the cause of the defect is unknown.
Refractive errors are the most common types of visual disorders in
children. The term refraction means bending and refers to the bending
of light rays as they pass through the lens of the eye. Normally, light
rays enter the lens and fall directly on the retina. However, in refractive
disorders, the light rays either fall in front of the retina (myopia) or
beyond it (hyperopia). Other eye problems, such as strabismus, may
or may not include refractive errors, but they are important because,
if untreated, they result in severe permanent visual impairment from
amblyopia. These, along with other less frequent visual disorders, are
summarized in Box 20.5. In addition to these disorders, other visual
problems can be a result of infection or trauma.
Treatment
When required, corrected with convex lenses that focus rays on retina
May be corrected with laser surgery
Astigmatism
Unequal curvatures in refractive apparatus
Pathophysiology
Results from unequal curvatures in cornea or lens that cause light rays to bend
in different directions
Clinical Manifestations
Depend on severity of refractive error in each eye
Possible clinical manifestations of myopia
Treatment
Corrected with special lenses that compensate for refractive errors
May be corrected with laser surgery
Anisometropia
Different refractive strength in each eye
Pathophysiology
May develop amblyopia because weaker eye is used less
Clinical Manifestations
Depend on severity of refractive error in each eye
Possible clinical manifestations of myopia
Treatment
Treated with corrective lenses, preferably contact lenses, to improve vision in
each eye so that they work as a unit
May be corrected with laser surgery
Continued
646 SECTION VIII  Family-Centered Care of the Child with Special Needs

BOX 20.5  Types of Visual Impairment—cont’d

Amblyopia Cataracts
Lazy eye: Reduced visual acuity in one eye Opacity of crystalline lens

Pathophysiology Pathophysiology
Results when one eye does not receive sufficient stimulation Prevents light rays from entering eye and refracting on retina
Each retina receives different images, resulting in diplopia (double vision)
Brain accommodates by suppressing less intense image Clinical Manifestations
Visual cortex eventually does not respond to visual stimulation, with resultant Gradual decrease in ability to see objects clearly
loss of vision in that eye Possible loss of peripheral vision
Nystagmus (with permanent visual impairment)
Clinical Manifestations Gray opacities of lens
Poor vision in affected eye Strabismus
Absence of red reflex
Treatment
Preventable if treatment of primary visual defect, such as anisometropia or Treatment
strabismus, begins before 6 years old Requires surgery to remove cloudy lens and replace lens (with intraocular lens
implant, removable contact lens, prescription glasses)
Strabismus Must be treated early to prevent permanent visual impairment from amblyopia
“Squint” or malalignment of eyes
Esotropia: Inward deviation of eye Glaucoma
Exotropia: Outward deviation of eye Increased intraocular pressure

Pathophysiology Pathophysiology
May result from muscle imbalance or paralysis, poor vision, or congenital defect Congenital type results from defective development of some component related
Because visual axes are not parallel, brain receives two images, and amblyopia to flow of aqueous humor
can result Increased pressure on optic nerve causes eventual atrophy and severe permanent
visual impairment
Clinical Manifestations
Squints eyelids together or frowns Clinical Manifestations
Difficulty in focusing from one distance to another Loss of peripheral vision—mostly seen in acquired types
Inaccurate judgment in picking up objects Possible bumping into objects
Inability to see print or moving objects clearly Perception of halos around objects
Closing one eye to see Possible complaint of pain or discomfort (severe pain, nausea, or vomiting if
Tilting head to one side sudden rise in pressure)
If combined with refractive errors, may see any of the manifestations listed for Eye redness
refractive errors Excessive tearing (epiphora)
Diplopia Photophobia
Photophobia Spasmodic winking (blepharospasm)
Dizziness Corneal haziness
Headaches Enlargement of eyeball (buphthalmos)

Treatment Treatment
Depends on cause of strabismus Requires surgical treatment (goniotomy) to open outflow tracts
May involve occlusion therapy (patching stronger eye) or surgery to increase May require more than one procedure
visual stimulation to weaker eye
Early diagnosis essential to prevent vision loss

examination of the injured eye (with the child sedated or anesthetized

Trauma
Trauma is a common cause of visual impairment in children. Injuries
to the eyeball and adnexa (supporting or accessory structures, such as
eyelids, conjunctiva, or lacrimal glands) can be classified as penetrating
or nonpenetrating. Penetrating wounds are most often a result of sharp
instruments (e.g., sticks, knives, or scissors) or propulsive objects (e.g.,
firecrackers, guns, arrows, or slingshots). Nonpenetrating injuries may
be a result of foreign objects in the eyes, lacerations, a blow from a
blunt object such as a ball (baseball, softball, basketball, racquet sports)
or fist, or thermal or chemical burns.
Treatment is aimed at preventing further ocular damage and is
primarily the responsibility of the ophthalmologist. It involves adequate
in severe injuries); appropriate immediate intervention, such as removal
of the foreign body or suturing of the laceration; and prevention of
complications, such as administration of antibiotics or steroids and
complete bed rest to allow the eye to heal and blood to reabsorb (see
Emergency Treatment box). The prognosis varies according to the type
of injury. It is usually guarded in all cases of penetrating wounds because
of the high risk of serious complications.
Infections
Infections of the adnexa and structures of the eyeball or globe may
occur in children. The most common eye infection is conjunctivitis.
Treatment is usually with ophthalmic antibiotics. Severe infections may
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family 647

disorders such as strabismus. This discussion focuses on clinical mani-

EMERGENCY TREATMENT
festations of various types of visual problems (see Box 20.5). Vision
Eye Injuries testing is discussed in Chapter 4.
Foreign Object Infancy.  At birth, the nurse should observe the neonate’s response
Examine eye for presence of a foreign body (evert upper eyelid to examine to visual stimuli, such as following a light or object and cessation of
upper eye). body movement. The infant may vary in the intensity of the response,
Remove a freely movable object with pointed corner of gauze pad lightly depending on the state of alertness.
moistened with water. Of special importance in detecting visual impairment during infancy
Do not irrigate eye or attempt to remove a penetrating object (see Penetrating are the parents’ concerns regarding visual responsiveness in their child.
Injuries). Their concerns, such as lack of eye contact from the infant, must be
Caution child against rubbing eye. taken seriously. During infancy, the child should be tested for strabismus.
Children with lack of binocularity after 2 to 4 months of age or not
Chemical Burns fixating and following an object by 6 months should be referred to a
Irrigate eye copiously with tap water for 20 minutes. pediatric ophthalmologist or an eye care specialist (American Academy
Evert upper eyelid to flush thoroughly. of Pediatrics, 2016; Rogers & Jordan, 2013).
Hold child’s head with eye under a tap of running lukewarm water.
Take child to emergency department.
NURSING ALERT
Have child rest with eyes closed.
Keep room darkened. Suspect visual impairment in an infant who does not react to light and in a
child of any age if the parents express concern.
Ultraviolet Burns
If skin is burned, patch both eyes (make certain eyelids are completely closed);
secure dressing with Kling bandages wrapped around head rather than with Childhood.  Because the most common visual impairment during
tape. childhood is refractive error, testing for visual acuity is essential. The
Have child rest with eyes closed. school nurse usually assumes major responsibility for vision testing in
Refer to an ophthalmologist. schoolchildren. In addition to assessing for refractive errors, the nurse
should be aware of signs and symptoms that indicate other ocular
Hematoma (“Black Eye”) problems. If the family is given a referral requesting further eye testing,
Use a flashlight to check for gross hyphema (hemorrhage into anterior chamber; the nurse is responsible for follow-up concerning the recommendation.
visible fluid meniscus across iris; more easily seen in light-colored than in Learning that their child is visually impaired precipitates an immense
brown eyes). crisis for families. Encourage the family to investigate appropriate
Apply ice for first 24 hours to reduce swelling if no hyphema is present. early intervention and educational programs for their child as soon
Refer to an ophthalmologist immediately if hyphema is present. as possible. Sources of information include state commissions for the
Have child rest with eyes closed. visually impaired, local schools for children with visual impairments,
the American Foundation for the Blind,* the National Federation of
Penetrating Injuries the Blind,† the National Association for Parents of Children with Visual
Take child to emergency department. Impairments,‡ the National Association for Visually Handicapped,§ the
Never remove an object that has penetrated eye. American Council of the Blind,¶ and CNIB.¶
Follow strict aseptic technique in examining eye.
Observe for the following: Promote Parent-Child Attachment
• Aqueous or vitreous leaks (fluid leaking from point of penetration) A crucial time in the life of visually impaired infants is when the infant
• Hyphema and the parents are getting acquainted with each other. Pleasurable
• Shape and equality of pupils, reaction to light, prolapsed iris (not perfectly
circular)
Apply a Fox shield if available (not a regular eye patch) and apply patch over
unaffected eye to prevent bilateral movement. *Two Penn Plaza, Suite 1102, New York, NY 10021; 800-232-5463 or
Maintain bed rest with child in a 30-degree Fowler position. 212-502-7600; fax: 888-545-8331; http://www.afb.org; email: afbinfo@afb.
Caution child against rubbing eye. net; https://wwwfacebook.com/americanfoundtionfortheblind; https://
Refer to an ophthalmologist. twitter.com/AFB1921.
†200 E. Wells St., Baltimore, MD 21230; 410-659-9314; fax: 410-685-
5653; http://www.nfb.org; office@nfbny.org; http://www.facebook.com/
require systemic antibiotic therapy. Steroids are used cautiously because NationalFederationoftheBlind/; https:twitter.com/NFB.
they exacerbate viral infections such as herpes simplex, increasing the ‡PO Box 317, Watertown, MA 02471; 617-972-7441 or 800-562-6265;
risk of damage to the involved structures. http://www.spedex.com; napvi@perfins.org.
§111 East 59th St., The Sol and Lillian Goldman Building, New York,
Nursing Care Management NY 10022-1202; 212-821-9200 or 800-284-4422; http://www.lighthouse-
Nursing care of the visually impaired child is a critical nursing responsibil- guild.org; email: info@lighthouse.org.
ity. Discovery of a visual impairment as early as possible is essential to ¶1703 N. Beauregard St., Suite 420, Alexandria, VA 22311; 800-424-8666;
prevent social, physical, and psychologic damage to the child. Assessment 202-467-5081; fax: 703-465-5085; http://www.acb.org; email: info@acb.
involves (1) identifying those children who by virtue of their history org; http://www.facebook.com/AmericanCounciloftheBlindOfficial.
are at risk, (2) observing for behaviors that indicate a vision loss, and ¶1929 Bayview Ave., East York, ON M4G 0A1; Canada: 800-563-2642;
(3) screening all children for visual acuity and signs of other ocular fax: 416-480-7700; http://www.cnib.ca.
648 SECTION VIII  Family-Centered Care of the Child with Special Needs
patterns of interaction between the infant and parents may be lacking
if there is not enough reciprocity. For example, if the parent gazes
fondly at the infant’s face and seeks eye contact but the infant fails to
respond because he or she cannot see the parent, a troubled cycle of
responses may occur. The nurse can help parents learn to look for other
cues that indicate the infant is responding to them, such as whether the
eyelids blink; whether the activity level accelerates or slows; whether
respiratory patterns change, such as faster or slower breathing, when
the parents come near; and whether the infant makes throaty sounds
when the parents speak to the infant. In time, parents learn that the
infant has unique ways of relating to them. Encourage the parents to
show affection using nonvisual methods, such as talking or reading,
cuddling, and walking the child.
Promote the Child’s Optimal Development
Promoting the child’s optimum development requires rehabilitation
in a number of important areas. These include learning self-help skills
and appropriate communication techniques to become independent.
Although nurses may not be directly involved in such programs, they
can provide direction and guidance to families regarding the availability
of programs and the need to promote these activities in their child.
Development and independence.  Motor development depends on
sight almost as much as verbal communication depends on hearing.
From earliest infancy, parents are encouraged to expose the infant to
as many visual-motor experiences as possible, such as sitting supported
in an infant seat or swing and being given opportunities for holding
up the head, sitting unsupported, reaching for objects, and crawling.
Despite visual impairment, the child can become independent
in all aspects of self-care. The same principles used for promoting
independence in sighted children apply, with additional emphasis on
nonvisual cues. For example, the child may need help in dressing, such
as special arrangement of clothing for style coordination and braille
tags to distinguish colors and prints.
The child with permanent visual impairment also must learn to
become independent in navigational skills. The two main techniques
are the tapping method (use of a cane to survey the environment for
direction and to avoid obstacles) and guides, such as a sighted human
guide or a guide dog. Children who are partially sighted may benefit
from ocular aids, such as a monocular telescope.
Play and socialization. Children with severe permanent visual
impairments do not learn to play automatically. Because they cannot
imitate others or actively explore the environment as sighted children
do, they depend much more on others to stimulate and teach them
how to play. Parents need help in selecting appropriate play materials,
especially those that encourage fine and gross motor development and
stimulate the senses of hearing, touch, and smell. Toys with educational
value are especially useful, such as dolls with various clothing closures.
Children with severe permanent visual impairments have the same
needs for socialization as sighted children. Because they have little dif-
ficulty in learning verbal skills, they are able to communicate with age
mates and participate in suitable activities. The nurse should discuss
with parents the opportunities for socialization outside the home,
especially regular preschools. The trend is to include these children
with sighted children to help them adjust to the outside world for
eventual independence.
To compensate for inadequate stimulation, these children may develop
self-stimulatory activities, such as body rocking, finger flicking, or arm
twirling. Discourage such habits because they delay the child’s social
acceptance. Behavior modification is often successful in reducing or
eliminating self-stimulatory activities.
Education.  The main obstacle to learning is the child’s total depen-
dence on nonvisual cues. Although the child can learn via verbal lecturing,
he or she is unable to read the written word or to write without special
education. Therefore the child must rely on braille, a system that uses
raised dots to represent letters and numbers. The child can then read
braille with the fingers and can write messages using a braille writer.
However, this system is not useful for communicating with others unless
others read braille. A more portable system for written communication
is the use of a braille slate and stylus or a microcassette tape recorder.
A recorder is especially helpful for leaving messages for others and
taking notes during classroom lectures. For mathematic calculations,
portable calculators with voice synthesizers are available.*
Books on CDs and tapes are significant sources of reading material
in addition to braille books, which are large and cumbersome. The
Library of Congress† has talking books, and braille books, that are
available at many local and state libraries and directly from the Library
of Congress. Currently, there are two types of talking books and book
players: digital and cassette, though new books are only made in the
digital format and recorded cassettes are being phased out (The New
York Public Library, 2017). The talking book machine and tape player
are provided at no cost to families, and there is no postage fee for
returning the materials. Learning Ally (formally known as Recording
for the Blind and Dyslexic)‡ also provides texts and CDs and tapes of
books, which are helpful for secondary and college students who are
visually impaired. A means of writing is learning to use a home computer
with a voice synthesizer that can be adapted to speak each letter or
word typed.
Children with partial sight benefit from specialized visual aids that
produce a magnified retinal image. The basic methods are accommodative
techniques, such as bringing the object closer; and devices such as special
plus lenses, handheld and stand magnifiers, telescopes, video projection
systems, and large-print materials. Special equipment is available to
enlarge print. Information about services for the partially sighted is
available from the National Association for Visually Handicapped and
American Foundation for the Blind. Children with diminished vision
often prefer to do close work without their glasses and compensate by
bringing the object very near to their eyes. This should be allowed. The
exception is children with vision in only one eye, who should always
wear glasses for protection.
Care for the Child During Hospitalization
Because nurses are more likely to care for children who are hospitalized
for procedures that involve temporary loss of vision than for children
who have severe permanent visual impairments, the following discussion
concentrates primarily on the needs of such children. The nursing care
objectives in either situation are to (1) reassure the child and family
throughout every phase of treatment, (2) orient the child to the sur-
roundings, (3) provide a safe environment, and (4) encourage inde-
pendence. Whenever possible, the same nurse should care for the child
to ensure consistency in the approach.
*A catalog of numerous products for people with vision problems is
available from Lighthouse International: https://www.lighthouseguild
.org.
†National Library Service for the Blind and Physically Handicapped,
Library of Congress, 1291 Taylor St. NW, Washington, DC 20542-4962;
202-707-5100; 888-657-7323; TTD: 202-707-0744; http://www.loc.gov/
nls; nis@loc.gov. (State listings of libraries for visually impaired and
physically handicapped readers with severe permanent visual impairments
and physical disabilities, as well as other reference circulars, are available
from this office.)
‡20 Roszel Road, Princeton, NJ 08540; 800-221-4792 or 866-RFBD-585;
http://www.learningally.org; http://www.facebook.com/LearningAlly.org.
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family
When sighted children temporarily lose their vision, almost every
aspect of the environment becomes bewildering and frightening. They
are forced to rely on nonvisual senses for help in adjusting to the visual
impairment without the benefit of any special training. Nurses have a
major role in minimizing the effects of temporary loss of vision. They
need to talk to the child about everything that is occurring, emphasizing
aspects of procedures that are felt or heard. They should always identify
themselves as soon as they enter the room and before they approach
the child. Because unfamiliar sounds are especially frightening, these
are explained. Encourage the parents to room with their child and
participate in the care. Familiar objects, such as a teddy bear or doll,
should be brought from home to help lessen the strangeness of the
hospital. As soon as the child is able to be out of bed, orient the child
to the immediate surroundings. If the child is able to see on admission,
this opportunity is taken to point out significant aspects of the room.
Encourage the child to practice ambulating with the eyes closed to
become accustomed to this experience.
The room is arranged with safety in mind. For example, place a
stool next to the bed to help the child climb in and out of bed. The
furniture is always placed in the same position to prevent collisions.
Remind cleaning personnel to keep the room in order. If the child has
difficulty navigating by feeling the walls, a rope can be attached from
the bed to the point of destination, such as the bathroom. Attention
to details (e.g., well-fitting slippers and robes that do not drag on the
floor) is important in preventing tripping. Unlike the child who is
visually impaired, these children are not familiar with navigating with
a cane.
The child is encouraged to be independent in self-care activities,
especially if the visual loss may be prolonged or potentially permanent.
For example, during bathing, the nurse sets up all of the equipment
and encourages the child to participate. At mealtimes, the nurse explains
where each food item is on the tray, opens any special containers, prepares
cereal or toast, and encourages the child in self-feeding. Favorite finger
foods (e.g., sandwiches, hamburgers, hot dogs, or pizza) may be good
selections. Praise the child for efforts at being cooperative and inde-
pendent. Any improvements made in self-care, no matter how small,
are stressed.
Appropriate recreational activities are provided, and if a child life
specialist is available, such planning is done jointly. Because children
with temporary visual impairment have a wide variety of play experiences
to draw on, they are encouraged to select activities. For example, if they
like to read, they may enjoy listening to books on CD or having someone
read to them. If they prefer manual activity, they may appreciate playing
with clay or building blocks or feeling different textures and naming
them. If they need an outlet for aggression, activities such as pounding
or banging on a drum can be helpful. Simple board and card games
can be played with a “seeing partner” or an opponent who helps with
the game. They should have familiar toys from home to play with
because familiar items are more easily manipulated than new ones. If
parents want to bring presents, they should be objects that stimulate
hearing and touch, such as a radio, music box, or stuffed animal.
Occasionally, children who are visually impaired come to the hospital
for procedures to restore their vision. Although this is an extremely
happy time, it also requires intervention to help them adjust to sight.
They need an opportunity to take in all that they see. They should not
be bombarded with visual stimuli. They may need to concentrate on
people’s faces or their own to become accustomed to this experience.
They often need to talk about what they see and to compare the visual
images with their mental ones. The children may also go through a
period of depression, which must be respected and supported. Encourage
the children to discuss how it feels to see, especially in terms of seeing
themselves.
649
Newly sighted children also need time to adjust and engage in activities
that were impossible before. For example, they may prefer to use braille
to read rather than learning a new “visual approach” because of familiarity
with the touch system. Eventually, as they learn to recognize letters and
numbers, they will integrate these new skills into reading and writing.
However, parents and teachers must be careful not to push them before
they are ready. This applies to social relationships and physical activities
as well as learning situations.
Assist in Measures to Prevent Visual Impairment
An essential nursing goal is to prevent visual impairment. This involves
many of the same interventions discussed for hearing impairments:
• Prenatal screening for pregnant women at risk, such as those with
rubella or syphilis infection and family histories of genetic disorders
associated with visual loss
• Adequate prenatal and perinatal care to prevent prematurity
• Periodic screening of all children, especially newborns through
preschoolers, for congenital and acquired visual impairments caused
by refractive errors, strabismus, and other disorders
• Rubella immunization of all children
• Safety counseling regarding the common causes of ocular trauma,
including safe practices when working with, playing with, and carrying
objects such as scissors, knives, and balls
NURSING ALERT
A helmet with a face mask should be required for children playing football,
hockey, and baseball.
After detection of eye problems, the nurse should encourage the
family to prevent further ocular damage by undertaking corrective
treatment. For the child with strabismus, this often necessitates occlusion
patching of the stronger eye. Compliance with the procedure is greatest
during the early preschool years. It is more difficult to encourage
school-age children to wear the occlusive patch because the poor visual
acuity of the uncovered weaker eye interferes with school work and the
patch sets them apart from their peers. In school, they benefit from
being positioned favorably (closer to the white board or other visual
media) and allowed extra time to read or complete an assignment. If
treatment of the eye disorder requires instillation of ophthalmic medica-
tion, the family is taught the correct procedure (see Chapter 22).
Children who need glasses to correct refractive errors need time to
adjust to wearing glasses. Young children who often pull off glasses
benefit from temporal pieces that wrap around the ears or an elastic
strap attached to the frames and around the back of the head to hold
the glasses on securely. Once children appreciate the value of clear
vision, they are more likely to wear the corrective lenses.
Glasses should not interfere with any activity. Special protective
guards are available during contact sports to prevent accidental injury,
and all corrective lenses should be made from safety glass, which is
shatterproof. Often, corrective lenses improve visual acuity so dramatically
that children are able to compete more effectively in sports. This in
itself is a tremendous inducement to continue wearing glasses.
Contact lenses are a popular alternative to conventional glasses,
especially for adolescents. Several types are available, such as hard lenses,
including gas-permeable ones, and soft lenses, which may be designed
for daily or extended wear. Contact lenses offer several advantages over
glasses, such as greater visual acuity, total corrected field of vision,
convenience (especially with the extended-wear type), and optimal
cosmetic benefit. Unfortunately, they are usually more expensive and
require much more care than glasses, including considerable practice
650 SECTION VIII  Family-Centered Care of the Child with Special Needs
to learn techniques for insertion and removal. If they are prescribed,
the nurse can be helpful in teaching parents or older children how to
care for the lenses.
Because trauma is the leading cause of visual impairment, the nurse
has the major responsibility of preventing further eye injury until specific
treatment is instituted. The major principles to follow when caring for
an eye injury are outlined in the Emergency Treatment box earlier in
this chapter. Because patients with a serious eye injury fear visual
impairment, the nurse should stay with the child and family to provide
support and reassurance.
HEARING–VISUAL IMPAIRMENT
The most traumatic sensory impairment is loss of both vision and
hearing, which may have profound effects on the child’s development.
These losses interfere with the normal sequence of physical, intellectual,
and psychosocial growth. Although such children often achieve the
usual motor milestones, their rate of development is slower. These
children learn communication only with specialized training. Finger
spelling is one desirable method often taught to these children. Words
are spelled letter by letter into the hearing–visually impaired child’s
hand, and the child spells into the other person’s hand. Some children
with residual hearing or visual impairment can learn to speak. Whenever
possible, encourage speech because it allows communication with other
individuals.
The future prospects for hearing and visually impaired children are,
at best, unpredictable. Congenital hearing and visually impairment are
accompanied by other physical or neurologic problems, which further
diminish the child’s learning potential. The most favorable prognosis
is for children who have acquired hearing and visual impairments with
few, if any, associated disabilities. Their learning capacity is greatly
potentiated by their developmental progress before the sensory impair-
ments. Although total independence, including gainful vocational
training, is the goal, some children with hearing–visual impairment are
unable to develop to this level. They may require lifelong parental or
residential care. The nurse working with such families helps them deal
with future goals for the child, including possible alternatives to home
care during the parents’ advancing years.
COMMUNICATION IMPAIRMENT
AUTISM SPECTRUM DISORDERS
ASDs are complex neurodevelopmental disorders of unknown etiology.
The American Psychiatric Association’s Diagnostic and Statistical Manual
of Mental Disorders, Fifth Edition (DSM-5) revised the definition for
ASD based on two behavior domains that include difficulties in social
communication and social interaction, and unusually restricted, repetitive
behavior, interest, or activities (American Psychiatric Association, 2013;
Brentani, Paula, Bordini, et al., 2013; Lai, Lombardo, & Baron-Cohen,
2014).
ASD is now frequently diagnosed in toddlers because their atypical
development is being recognized early (Lai, Lombardo, & Baron-Cohen,
2014; Zwaigenbaum, Bauman, Stone, et al., 2015). It occurs in 1 in 68
children in the United States; is about four times more common in
boys than in girls; and is not related to socioeconomic level, race, or
parenting style (Christensen, Baio, Braun, et al., 2016; National Autism
Association, 2017a).
Etiology
The cause of ASD is unknown. Researchers are investigating a number of
theories, including a link between hereditary, genetic, medical, immune
dysregulation/neuroinflammation, oxidative stress (damage to cellular
tissue), and environmental factors (De Rubeis, He, Goldberg, et al.,
2014; Lai, Lombardo, & Baron-Cohen, 2014; Ng, de Montigny, Ofner,
et al., 2017; Posar & Visconti, 2016; Wong, Napoli, Krakowiak, et al.,
2016). Individuals with ASD may have abnormal electroencephalograms,
epileptic seizures, delayed development of hand dominance, persistence
of primitive reflexes, metabolic abnormalities (elevated blood serotonin),
cerebellar vermis hypoplasia (part of the brain involved in regulating
motion and some aspects of memory), and infantile abnormal head
enlargement (Raviola, Trieu, DeMaso, et al., 2016; Rutter, 2011).
The strong evidence for a genetic basis in twins is consistent with an
autosomal recessive pattern of inheritance. Twin studies demonstrate
a high concordance (60% to 96%) for monozygotic (identical) twins
and less than 5% concordance for dizygotic (nonidentical) twins. In
addition, between 5% and 16% of boys with ASD are positive for the
fragile X chromosome (Clifford, Dissanayake, Bui, et al., 2007; Finucane,
Lincoln, Bailey, et al., 2017; Grafodatskaya, Chung, Szatmari, et al., 2010).
There is a relatively high risk of recurrence of ASD in families with
one affected child (Chawarska, Shic, Macari, et al., 2014; Rutter, 2011;
Yoder, Stone, Walden, et al., 2009; Zwaigenbaum, Bauman, Stone, et al.,
2015). Several genes have been suggested as possible causative factors
in ASD (Talkowski, Minikel, & Gusella, 2014; Willsey & State, 2015;
Wong, Napoli, Krakowiak, et al., 2016).
The scientific evidence to date shows no link between measles,
mumps, and rubella (MMR) and thimerosal-containing vaccines and
ASDs (Barile, Kuperminc, Weintraub, et al., 2012; Goin-Kochel, Mire,
Dempsey, et al., 2016; Price, Thompson, Goodson, et al., 2010; Taylor,
Swerdfeger, & Eslick, 2014; Uno, Uchiyama, Kurosawa, et al., 2015)
(see Translating Evidence into Practice box). ASD has been reported
in association with a number of conditions, such as FXS, tuberous
sclerosis, Prader-Willie syndrome, metabolic disorders, fetal rubella
syndrome, Haemophilus influenzae meningitis, and structural brain
anomalies (National Autism Association, 2017a; Peterson & Barbel,
2013). Recent reports have retrospectively tied ASD to prenatal and
perinatal events, such as maternal and paternal ages over 40 years
old (for fathers, 1 in 116 births; for mothers, 1 in 123 births), uterine
bleeding during pregnancy, low Apgar score, fetal distress, and neonatal
hyperbilirubinemia (Amin, Smith, & Wang, 2011; Kolevzon, Gross, &
Reichenberg, 2007; Rutter, 2011). These same researchers, however, urge
caution in interpreting these findings.
Clinical Manifestations and Diagnostic Evaluation
Children with ASD demonstrate core deficits primarily in social interac-
tions, communication, and behavior. Failure of social interaction and
communication development is the one of the hallmarks of ASD. Parents
of autistic children have reported that their child showed less interest
in social interaction (e.g., abnormal eye contact, decreased response to
own name, decreased imitation, usual repetitive behavior) and had
verbal and motor delays (Bolton, Golding, Emond, et al., 2012; National
Autism Association, 2017b; Sanchack & Thomas, 2016). Children with
ASD may have significant gastrointestinal symptoms. Constipation is
a common symptom and can be associated with acquired megarectum
in children with ASD (Buie, Campbell, Fuchs, et al., 2010; National
Autism Association, 2017a).
Children with ASD do not always have the same manifestations,
from mild forms requiring minimal supervision to severe forms in
which self-abusive behavior is common. The majority of children with
ASD have some degree of CI, with scores typically in the moderate to
severe range. Despite their relatively moderate to severe disability, some
children with autism (known as savants) excel in particular areas, such
as art, music, memory, mathematics, or perceptual skills, such as puzzle
building.
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family
TRANSLATING EVIDENCE INTO PRACTICE
Thimerosal-Containing Vaccines and Autism Spectrum Disorders
Ask the Question
Is the incidence of autism spectrum disorders (ASDs) increased in children receiving
vaccines containing thimerosal?
Search for the Evidence
Search Strategies
Published studies from 2003 to 2016 focused on the pediatric population and
restricted to the English language
Databases Used
PubMed, Cochrane Collaboration, MD Consult, Vaccine Adverse Events Reporting
System (VAERS) database, American Academy of Pediatrics, Autism Research
Institute
Critically Analyze the Evidence
Grade criteria: Moderate evidence with strong recommendations for practice
(Balshem, Helfand, Schünemann, et al., 2011). Evidence does not support an
association between the increase incidence of autism and mercury exposure
from the pharmaceutical preservative thimerosal.
• A Cochrane systematic review of 64 studies assessing the effectiveness and
adverse effects associated with the trivalent measles, mumps, and rubella
(MMR) vaccine on healthy patients up to 15 years old found no significant
association between MMR with either autism or other conditions (Demicheli,
Rivetti, Debalini, et al., 2012). Previously done studies supported the same
conclusion because the studies found no association between thimerosal-
containing vaccines and ASD (Demicheli, Jefferson, Rivetti, et al., 2005; Hurley,
Tadrous, & Miller, 2010; Parker, Schwartz, Todd, et al., 2004; Schultz, 2010;
World Health Organization, 2012).
• Two large studies in Europe found no evidence that childhood vaccination
with thimerosal-containing vaccines was associated with the development
of ASDs. One longitudinal study evaluated more than 14,000 children in the
United Kingdom. The mercury exposure from thimerosal-containing vaccines
was recorded and calculated at ages 3, 4, and 6 months and compared with
cognitive and behavioral developmental assessments performed from 6 to 91
months old (Heron, Golding, & ALSPAC Study Team, 2004). The second study,
a cohort of 467,450 children in Denmark, compared the incidence of ASDs in
children vaccinated with thimerosal-containing vaccines with the incidence
of ASDs in children vaccinated with a thimerosal-free formulation of the same
vaccine (Hvid, Stellfeld, & Wohlfahrt, 2003). Another study that evaluated
1047 children from early life to 7 to 10 years old and their biologic mothers
found no statistically significant associations between thimerosal exposure
from vaccines early in life. It noted a small but statistically significant association
between early thimerosal exposure and the presence of tics in boys and recom-
mended there be further research in this area (Barile, Kuperminc, Weintraub,
et al., 2012). An evidence-based meta-analysis of case-control and cohort
studies reported the components of the vaccines (thimerosal or mercury) or
multiple vaccines (MMR) are not associated with the development of autism
or ASD (Taylor, Swerdfeger, & Eslick, 2014).
• Case-control studies have also found no relationship between MMR vaccination
and the increased risk of ASDs (Price, Thompson, Goodson, et al., 2010; Uno,
Uchiyama, Kurosawa, et al., 2015; Yau, Green, Alaimo, et al., 2014). Another
small case-control study investigated the mercury level in maternal prenatal
serum and early postnatal newborn serum of children with ASD (n = 84)
compared with children with intellectual disability or developmental delay (n
= 49) and general population (n = 159) and found no significant association
with the risk of ASD (Yau, Green, Alaimo, et al., 2014). A similar finding
651
was concluded in a meta-analysis of evidence on the impact of prenatal
and early infancy exposures to mercury on autism and attention-deficit/
hyperactivity disorder (ADHD) with the recommendation of further study to
be conducted on effects of environmental perinatal mercury exposures and
increase risk of developmental disorders (Yoshimasu, Kiyohara, Takemura, et al.,
2014).
A review study reported 91 studies that examine the potential relationship
between mercury and ASD from 1999 to February 2016. Of these studies, the
majority (74%) suggest that mercury is a risk factor for ASD, revealing both direct
and indirect effects. The evidence indicates that mercury is a risk factor for ASD
(Kern, Geier, Sykes, et al., 2016). Conversely, Goin-Kochel, Mire, Dempsey, and
colleagues (2016) reported in a cohort study that examined 2755 ASD children
based on parental description of ASD onset against vaccine receipt that the
findings did not support a connection between regressive-onset ASD and vaccines.
Another evidence-based meta-analysis of case-control studies and cohort studies
supported the same conclusion; the findings from a large sample of US children
(95,727) suggest that vaccinations are not associated with the development of
autism or ASD (Taylor, Swerdfeger, & Eslick, 2014).
• In 2004 the Institute of Medicine completed an update to review the evidence
and concluded that the epidemiologic evidence supports the rejection of a
causal relationship between thimerosal exposure from childhood vaccines
and the onset of autism. The Institute of Medicine (2013) review of the
evidence reported from January 1990 to May 2013 concluded that the review
revealed no evidence that the US childhood immunization schedule is linked
to learning or developmental disorders or attention-deficit or disruptive
disorders. This was also supported in a report on adverse effects of vaccines
by the Institute of Medicine (2011). Based on guidelines established by the
US Food and Drug Administration (2014) and other government monitoring
agencies, no children will be exposed to excessive mercury from childhood
vaccines.
Apply the Evidence: Nursing Implications
There is moderate-quality evidence with a strong recommendation that there is
no link between vaccines containing thimerosal and ASDs.
Quality and Safety Competencies: Evidence-Based Practice*
Knowledge
Differentiate clinical opinion from research and evidence-based
summaries.
Compare research summaries that provide evidence of the lack of association
between vaccines containing thimerosal and autism or other neurodevelopmental
disorders.
Skills
Base individualized care plan on patient values, clinical expertise, and
evidence.
Integrate evidence into practice by sharing results with parents regarding the
benefits of vaccinating their children and the evidence regarding lack of
association between immunizations and autism disorders.
Attitudes
Value the concept of evidence-based practice as integral to determining
best clinical practice.
Appreciate strengths and weakness of the evidence that confirms the lack of a
link between vaccines containing thimerosal and autism or other neurodevel-
opmental disorders.
Continued
652 SECTION VIII  Family-Centered Care of the Child with Special Needs
TRANSLATING EVIDENCE INTO PRACTICE—cont’d
Thimerosal-Containing Vaccines and Autism Spectrum Disorders
References
Balshem, H., Helfand, M., Schünemann, H. J., et al. (2011). GRADE guidelines: 3. Rating
the quality of evidence. Journal of Clinical Epidemiology, 64(4), 401–406.
Barile, J. P., Kuperminc, G. P., Weintraub, E. S., et al. (2012). Thimerosal exposure in early
life and neuropsychological outcomes 7-10 years later. Journal of Pediatric Psychology,
37(1), 106–118.
Demicheli, V., Jefferson, T., Rivetti, A., et al. (2005). Vaccines for measles, mumps and
rubella in children. Cochrane Database of Systematic Reviews, (4), CD004407.
Demicheli, V., Rivetti, A., Debalini, M. G., et al. (2012). Vaccines for measles, mumps and
rubella in children. Cochrane Database of Systematic Reviews, (2), CD004407.
Goin-Kochel, R. P., Mire, S. S., Dempsey, A. G., et al. (2016). Parental report of vaccine
receipt in children with autism spectrum disorder: Do rates differ by pattern of ASD
onset? Vaccine, 34, 1335–1342.
Heron, J., Golding, J., & ALSPAC Study Team. (2004). Thimerosal exposure in infants and
developmental disorders: A prospective cohort study in the United Kingdom does not
support a causal association. Pediatrics, 114(3), 577–583.
Hurley, A. M., Tadrous, M., & Miller, E. S. (2010). Thimerosal-containing vaccines and
autism: Review of recent epidemiologic studies. The Journal of Pediatric Pharmacology
and Therapeutics, 15(3), 173–181.
Hvid, A., Stellfeld, M., & Wohlfahrt, J. (2003). Association between thimersol-containing
vaccine and autism. Journal of the American Medical Association, 290(13), 1763–1766.
Institute of Medicine. (2004). Immunization safety review:Vaccines and autism. Washington,
DC: National Academies Press.
Institute of Medicine. (2011). Adverse effects of vaccines: Evidence and causality, National
Academies Press. Retrieved from http://www.iom.edu/Reports/2011/Adverse-Effects-
of-Vaccines-Evidence-and-Causality.aspx.
Institute of Medicine. (2013). The childhood immunization schedule and safety: Stakeholders
concerns, scientific evidence, and future studies. Washington, DC: National Academies
Press.
*Based on the Quality and Safety Education for Nurses website at http://www.qsen.org.
NURSING ALERT
Claims of beneficial results from the use of secretin, a peptide hormone that
stimulates pancreatic secretion, have been studied extensively in multiple
randomized controlled trials, denoting no clear evidence of any benefit in the
treatment of ASD (Krishnaswami, McPheeters, & Veenstra-Vanderweele, 2011;
Lee, Oh, & Park, 2014;Williams, Wray, & Wheeler, 2012).*
*Additional information on secretin may be found by contacting the
Autism Society, 4340 East-West Hwy., Suite 350, Bethesda, MD
20814-3067; 800-3AUTISM or 301-657-0881; http://www.autism-
society.org.
Communication impairments are a common sign in children with
ASD that may range from absent to delayed speech. Any child who
does not display language skills such as babbling or gesturing by 12
months old, single words by 16 months old, and two-word phrases by
24 months old is recommended for immediate hearing and language
evaluation. Autism regression is when the child seems to develop normally
but then regresses suddenly; this is a red-flag event that has been fre-
quently displayed in expressive language (Fernell, Eriksson, & Gillberg,
2013; Raviola, Trieu, DeMaso, et al., 2016; Sanchack & Thomas, 2016).
Early recognition, referral, diagnosis, and intensive early intervention
tend to improve outcomes for children with ASD (Adelman & Kubiszyn,
2016; National Autism Association, 2017a; Reichow, Barton, Boyd, et al.,
2012; Peterson & Barbel, 2013; Zwaigenbaum, Bauman, Stone, et al.,
2015). Unfortunately, diagnosis is often not made until 2 to 3 years
after symptoms are first recognized. However, in a retrospective study,
the majority of parents observed atypical development in their ASD
children before 24 months old (Lemcke, Juul, Parner, et al., 2013).
The American Academy of Pediatrics has recommended that
pediatric health care providers administer two ASD screenings at
Kern, J. K., Geier, D. A., Sykes, L. K., et al. (2016). The relationship between mercury and
autism: A comprehensive review and discussion. Journal of Trace Elements in Medicine
and Biology, 37, 8–24.
Parker, S. K., Schwartz, B., Todd, J., et al. (2004). Thimerosal-containing vaccines and autistic
spectrum disorder: A critical review of published original data. Pediatrics, 114(3), 793–804.
Price, C. S., Thompson, W. W., Goodson, B., et al. (2010). Prenatal and infant exposure to
thimerosal from vaccines and immunoglobulins and risk of autism. Pediatrics, 126(4),
656–664.
Schultz, S. T. (2010). Does thimerosal or other mercury exposure increase the risk for autism?
A review of current literature. Acta Neurobiologiae Experimentalis, 70(2), 187–195.
Taylor, L. E., Swerdfeger, A. L., & Eslick, G. D. (2014). Vaccines are not associated with
autism: An evidence-based meta-analysis of case-control and cohort studies. Vaccine,
32(29), 3623–3629.
Uno, Y., Uchiyama, T., Kurosawa, M., et al. (2015). Early exposure to the combined measles-
mumps-rubella vaccine and thimerosal-containing vaccines and risk of autism spectrum
disorder. Vaccine, 33(21), 2511–2516.
US Food and Drug Administration. (2014). Thimerosal in vaccines. Retrieved from http://
www.fda.gov/BiologicsBloodVaccines/SafetyAvailability/vaccineSafety/UCM096228.
World Health Organization. (2012). Global vaccine safety: Global Advisory Committee on
Vaccine Safety, report of meeting held 6-June 7, 2012. Retrieved from http://www.who.
int/vaccine_safety/committee/reports/Jun_2012/en/.
Yau, V. M., Green, P. G., Alaimo, C. P., et al. (2014). Prenatal and neonatal peripheral blood
mercury levels and autism spectrum disorders. Environmental Research, 133, 294–303.
Yoshimasu, K., Kiyohara, C., Takemura, S., et al. (2014). A meta-analysis of the evidence on
the impact of prenatal and early infancy exposures to mercury on autism and attention
deficit/hyperactivity disorder in the childhood. Neurotoxicology, 44, 121–131.
Rosalind Bryant
ages 18 and 24 months using a valid screening tool. The Modified
Checklist for Autism in Toddlers (M-CHAT) as a widely used screening
tool for autism accompanied by a follow-up interview (M-CHAT/F)
was used in a recent study and demonstrated that minimally trained
primary care pediatricians can administer the M-CHAT/F reliably and
efficiently during well-child visits (Sturner, Howard, Bergmann, et al.,
2016). Children whose screening results are concerning subsequently
should receive a comprehensive developmental evaluation from either
a developmental pediatrician, child neurologist, child psychiatrist,
or child psychologist (Centers for Disease Control and Prevention,
2016c).
Prognosis
Although ASD is usually a lifelong condition with often devastating
comorbid conditions, with early and intensive interventions the symptoms
associated with autism can be greatly improved, and in some cases
reported symptoms were completely overcome (National Autism
Association, 2017a; Sanchack & Thomas, 2016; Wodka, Mathy, & Kalb,
2013). Some ultimately achieve independence, but most require lifelong
adult supervision. Aggravation of psychiatric symptoms occurs in about
half of the children during adolescence, with girls having a tendency
for continued deterioration.
Early recognition of behaviors associated with ASD is critical to
implement appropriate interventions and family involvement. There is
a growing body of evidence that parent-delivered interventions are
associated with some improved outcomes, yet further research is needed
in this area incorporating consistent measures (Bearss, Burrell, Stewart,
et al., 2015; Brentani, Paula, Bordini, et al., 2013; Oono, Honey, &
McConachie, 2013). The prognosis is most favorable for children with
higher intelligence, functional speech, and less behavioral impairment
(Orinstein, Helt, Troyb, et al., 2014; Raviola, Trieu, DeMaso, et al., 2016;
Solomon, Buaminger, & Rogers, 2011).
 CHAPTER 20  Impact of Cognitive or Sensory Impairment on the Child and Family
Nursing Care Management
Therapeutic intervention for ASD and associated comorbidities is a
specialized area involving professionals with advanced training. Although
there is no cure for ASD, numerous therapies have been used. The most
promising results have been through highly structured and intensive
behavior modification programs. In general, the objective in treatment
is to promote positive reinforcement, increase social awareness of others,
teach verbal communication skills, and decrease unacceptable behavior.
Providing a structured routine for the child to follow is a key in the
management of ASD.
ASD is associated with comorbidities (e.g., aggression, explosive
outburst, self-injury, asthma, epilepsy, gastrointestinal/digestive disorders,
immune disorders, feeding disorders, anxiety disorder, bipolar disorder,
sleeping disorders) that have been treated not only with early behavioral
modification programs but also with medical management (e.g.,
aripiprazole [Abilify] and risperidone [Risperdal]) and complementary
and alternative medicine (National Autism Association, 2017b; Sanchack
& Thomas, 2016). Complementary and alternative medicine has emerged
as a treatment of ASD ranging from parent-massage and therapeutic
horseback riding to the implementation of elimination diets (e.g.,
gluten-free diet and casein-free diet); vitamin and omega-3 supplementa-
tion; and high-fat, low-carbohydrate ketogenic diet; however, there is
a need for further research to validate these therapeutic approaches
(Cheng, Rho, & Masino, 2017; Gabriels, Pan, Dechant, et al., 2015; Lee,
Oh, & Park, 2014; Lofthouse, Hendren, Hurt, et al., 2012; Ly, Bottelier,
Hoekstra, et al., 2017).
When these children are hospitalized, the parents are essential to
planning care and ideally should stay with the child as much as possible.
Nurses should recognize that not all children with ASD are the same
and that they require individual assessment and treatment. Decreasing
stimulation by using a private room, avoiding extraneous auditory and
visual distractions, and encouraging the parents to bring in possessions
the child is attached to may lessen the disruptiveness of hospitalization.
Because physical contact often upsets these children, minimal holding
and eye contact may be necessary to avoid behavioral outbursts. Take
NCLEX REVIEW QUESTIONS
1. A mother comments to a nurse working on the pediatric unit, “My
second child just does not seem to be acting like or responding the
same way as my first child.” Nursing interventions to respond to
this inquiry should include which of the following? Select all that
apply.
A. Assessment for dysmorphic syndromes (e.g., multiple congenital
anomalies, microcephaly)
B. Inquiring about temperament: irritability or lethargy
C. Explaining that all children are different and that it can be
detrimental to compare them
D. Noting language development appropriate for the child’s age
E. Meeting the siblings to assess similarities that may be familial
rather than problematic
2. When interacting with a parent at her child’s well visit, which state-
ment by the mother would be an indication for a speech referral?
Select all that apply.
A. Failure to speak any meaningful words spontaneously in a 2-year-
old child
B. Using different words or nicknames for certain people
C. Failure to use sentences of three or more words in a
3-year-old
653
care when performing procedures on, administering medicine to, and
feeding these children because they may be either fussy eaters who
willfully starve themselves or gag to prevent eating, or indiscriminate
hoarders who swallow any available edible or inedible items, such as a
thermometer. Eating habits of ASD children may be particularly
problematic for families and may involve food refusal accompanied by
mineral deficiencies, mouthing objects, eating nonedibles, and smelling
and throwing food (Belschner, 2007; Herndon, DiGuiseppi, Johnson,
et al., 2009).
Children with ASD need to be introduced slowly to new situations,
with visits with staff caregivers kept short whenever possible. Because
these children have difficulty organizing their behavior and redirecting
their energy, they need to be told directly what to do. Communication
should be at the child’s developmental level, brief, and concrete.
Family Support
ASD, as with so many other chronic conditions, involves the entire
family and often becomes “a family disease.” Nurses can help alleviate
the guilt and shame often associated with this disorder by stressing
what is known from a biologic standpoint and by providing family
support. It is imperative to help parents understand that they are not
the cause of the child’s condition.
Parents need expert counseling early in the course of the disorder
and should be referred to the Autism Society website. The society provides
information about education, treatment programs and techniques, and
facilities such as camps and group homes. Other helpful resources for
parents of children with ASD are the local and state departments of
mental health and developmental disabilities; these organizations provide
important programs and in-school programs throughout the United
States for children with ASD.
As much as possible, the family is encouraged to care for the child
in the home. With the help of family support programs in many states,
families are often able to provide home care and assist with the edu-
cational services the child needs. As the child approaches adulthood
and the parents become older, the family may require assistance in
locating a long-term placement facility.
D. Stuttering or any other type of dysfluency
E. Omission of word endings (e.g., plurals, tenses of verbs) in a
3-year-old
F. Frequent omission of final consonants in a 3-year-old
3. A mother of a child born with Down syndrome is overwhelmed
with the future and asks many questions. Which of the following
facts should the nurse be aware of? Select all that apply.
A. Eighty percent of infants with Down syndrome are born to women
younger than 35 years old because younger women have higher
fertility rates.
B. When feeding infants and young children, use a small, straight-
handled spoon to push food to the side and back of the mouth.
Feeding difficulties occur due to a protruding tongue and
hypotonia.
C. Parents generally believe the experience of having this special
child makes them stronger and more accepting of others.
D. Although some placement in the regular classroom has occurred
more recently, this has been found to be detrimental to the child
with Down syndrome due to lack of one-on-one teaching.
E. The child’s lack of clinging or molding is a physical characteristic,
not a sign of detachment or rejection.