Developmental Disabilities
OVERVIEW AND ASSESSMENT
The spectrum of developmental disabilities is a group of interrelated chronic neurodevelopmental disorders. It includes cerebral palsy, mental retardation, autism, learning disabilities, communication disorders, anxiety disorders, and attention-deficit disorders. These conditions are suspected or noticed at varying stages during childhood. Some conditions, such as Down syndrome, can be recognized prenatally or at birth; other conditions, such as cerebral palsy or autism, may not present themselves until the child fails to meet typical developmental milestones. This phenomenon makes the nurse’s role in developmental assessment crucial. The pediatric nurse who understands that the development of the child occurs in an orderly, predictable manner and who knows what the milestones should be may be the first person to recognize the deviation and raise the suspicion to the parent or pediatrician. Because there are many confusing and overlapping characteristics for many of these conditions, it is important for the health care provider and family to refer to clinicians who are experienced and knowledgeable in typical and atypical child development to arrive at an accurate diagnosis and coordinate an appropriate care plan. Nurses can play a significant role in supporting the family in navigating the complex needs of many of these disorders.
When a known condition exists, the nurse should be aware of the predisposing health issues associated with the condition and the resources available to promote optimal growth and development of the child within the family.
NURSING ALERTNormal child development is predictable and sequential. The nurse who is knowledgeable about normal development may be the first person to raise the question that a developmental disability exists.
Signs of Developmental Delay
Criteria for Referral
Communication and Feeding
Feeding difficulties—weak sucking or poor coordination of suck-swallowing to sustain normal weight gain.
No social smile by age 4 months.
No babbling (ga-ga, da-da) by age 9 months.
No mama, dada (specific) by age 14 months.
No name of object (one word) by age 14 months.
At least 10 words by age 18 months (not just repeating).
Combines words (eg, me outside, more milk) and uses pronouns by age 24 months.
Regression in language or social responses at any age.
Unresponsive to name.
NURSING ALERTDo not simply reassure the family that normal development “should” or “probably will” develop. Make sure that the child is referred for an appropriate evaluation to properly assess the suspected delay and establish a diagnosis, if possible. In the presence of a developmental delay, a comprehensive vision and hearing evaluation should also be included.
Motor Delay
Assymmetrical movements and poor tone are noted.
Not rolling over by age 6 months.
Not sitting by age 9 months.
Not walking by age 15 months.
Not stair-climbing by age 2 years.
See Chapter 40 for normal pediatric growth and development.
Care of the Child with a Developmental Disability
Nursing Assessment
Review the child’s record including the prenatal and birth history to determine existing health problems that may cause or affect the developmental disability. Always rule out a vision or hearing impairment.
Review the family history, if available, to determine if there are any family members with a history of developmental disabilities. If possible, assess when parents and siblings of the patient reached specified developmental milestones.
Assess the family’s understanding of the diagnosis and its ramifications. The parents’ experiences, cultural beliefs, attitudes toward disabilities, cognitive ability, stage of grief, and their own health affect their ability to assimilate information provided. It will be necessary to repeat the information. (For example, a woman who has just given birth to a child with Down syndrome may have difficulty processing much information shortly after birth.) If possible, provide the family with written information that reinforces verbal information given at the visit. Provide resources with careful consideration of information overload. Additional information and resources may be better introduced at subsequent visits.
Determine the developmental age of the child. The pediatric nurse should be familiar with normal developmental milestones (see Chapter 40, page 1370), noting the child’s strengths and areas presenting challenges, for example, the communication skills are at a 12-month level and the gross motor skills are at a 36-month level. Children who are found to be functioning at one half or less of their chronological age have a moderate to severe developmental delay.
NURSING ALERT be aware of the diversity of cultural norms that may impact developmental milestones.
Administer a developmental tool such as the Clinical Adaptive Test-Clinical Linguistic Auditory Milestone Scales (CAT-CLAMS). It is a reliable tool that nurses can be trained to use to assess cognitive and communicative development in children at the 1- to 36-month developmental level. It has been found to be more sensitive than the Denver Developmental Screening Scale because language development is the best early predictor of cognitive abilities.
Evidence Base
Honigfield, L., Chandhok, L., Spiegelman, K. (2012). engaging pediatricians in developmental screening: The effectiveness of academic detailing. Journal of Autism & Developmental Disorders, 42(6), 1175-1182.
Bricker, D., Macy, M., Squires, J., Marks, K. (2013). Developmental Screening in Your Community: An Integrated Approach for Connecting Children with Services. baltimore: brookes.
Assess the functional level of the child. The WeeFIM is a tool used to track functional independence in children. It has had multiple revisions and is reliable and valid. It uses the following categories to describe function: completely dependent, needs some physical assistance, can physically perform the task but needs verbal cues and coaching, and completely independent. Functional areas to assess include:
Feeding.
Grooming and bathing.
Dressing.
Mobility.
Problem solving.
Communication.
Evidence Base
Deveney, S., Hoffman, L., Cress, C. (2012). Communication-based assessment of developmental age for young children with developmental disabilities. Journal of Speech, Language & Hearing Research, 55(3), 695-709.
Assess parents’ perception of the child’s development level and the appropriateness of parental expectations. Use such questions as, “Do you have any concerns regarding things your child is doing or should be doing?” “Is your child progressing like your other children have?”
Assess parent-child interaction. Observe and explore bonding and attachment, ability to set appropriate limits, management of behavioral problems, and methods of discipline.
Assess the need for additional resources, such as financial aid, transportation, and counseling, for long-term support of child and family.
Nursing Diagnoses
Impaired Parenting related to birth and diagnosis of developmentally disabled child.
Interrupted Family Processes related to multiple needs of child and difficult bonding.
Ineffective Infant Feeding Pattern related to protruding tongue, poor muscle tone, and weak sucking.
Delayed Growth and Development related to impaired cognitive and motor functioning.
Social Isolation related to developmental differences from other children.
Risk for Injury related to neurologic impairment and developmental delay.
Nursing Interventions
Promoting Adjustment
Allow the parents access to the infant at all possible times to promote bonding when parents appear ready.
Focus on the positive aspects of the infant and serve as a role model for handling and stimulating.
Be cognizant of the grieving process (loss of the anticipated and planned-for “normal child”) that families experience when a diagnosis is made and be aware that spouses can be at different stages.
Accept all questions and reactions nonjudgmentally, offering verbal and written explanations.
Provide the family a quiet place to discuss their questions with each other and someone knowledgeable about the condition (primary care provider, clinical nurse specialist) to support their concerns.
Offer the family the option to take advantage of counseling. A social worker or psychologist can help families deal with immediate reactions. Many parents benefit from continuous or periodic support of counseling professionals.
NURSING ALERTChildren with developmental disabilities and chronic illness are at greater risk for experiencing divorce, child abuse, and neglect than the general population.
Strengthening the Role of the Family
Help the family to realize what strengths they have in caring for their child. The role of the parents is critical; a nurturing, loving environment gives the child the best chance at maximizing potential. An individual who grows up at home has markedly higher adaptive abilities and an increased life span compared with those raised in residential care facilities.
Enlist the help of family and siblings, who can offer valuable support to the parents and child and assist with stimulation activities. Including siblings in the care can help them feel needed and involved, thus strengthening the family.
If sibling issues arise, suggest family counseling to address the needs of all family members.
Identify resources available to the family, such as parent support groups, early intervention programs, specialty clinics, pediatrician or primary health care provider, respite care, financial support programs, and advocacy groups for individuals with developmental disabilities.
For those parents concerned with their ability to care for the child, explore with them their options of foster care, adoption, and residential placement in a nonjudgmental manner.
Ensure that the family has adequate respite services to prevent exhaustion and mental fatigue.
Establishing Effective Feeding Techniques
Evidence BaseCloud, H. (2012). Developmental disabilities. In P. Q. Samour & K. King, Pediatric nutrition (4th ed.), 219-236. Sudbury, MA: Jones & bartlett.
Be aware that the presence of hypotonia, as in children with many genetic syndromes and metabolic disorders, or hypertonia, as in children with cerebral palsy, can interfere with feeding by compromising sucking and swallowing.
Demonstrate proper feeding positioning, with the infant’s head elevated, and encourage the parents to always hold the infant during feedings with his or her head elevated and supported in arms.
Try different nipples and bottles to find the easiest for the infant to use without leakage or danger of aspiration.
Allow adequate time for feeding and increase frequency of feedings if infant tires easily.
Offer support and guidance for breastfeeding. Refer to lactation consultant, if needed.
Consider referral for a feeding evaluation to a speech therapist or to an occupational therapist who has experience working with children and families.
Be aware of and anticipate the need for initiation of tube feeding (nasogastric or gastrostomy) so that early referral and education of the parents about feeding options can be discussed
Assess for signs of feeding intolerance and gastroesophageal reflux disease, which is common in children with hypotonia; implement management strategies to optimize feeding and minimize risk of aspiration, such as upright position after feedings.
Frequently assess growth (length or height, weight, and head circumference) to ensure adequate dietary intake and be aware that growth in children with neurologic impairment and genetic syndromes may be slower or limited in relation to the general pediatric population and standard growth charts.
Monitor bowel and elimination patterns of children with neurologic impairment, especially those with generalized hypotonia, as they are at risk for poor gastric motility and constipation. Dietary measures, such as increased fiber and fluid intake, or pharmacologic agents to promote adequate elimination may be needed.
Promoting Optimum Growth and Development
Refer parents to the Early Intervention Program administered by their county so that they can take advantage of the educational and support services available for children from birth to age 3.
If the condition is identified after age 3 years, refer parents to the school district in which they reside.
Help the parents to understand the concept of developmental age and identify the functional level of the child.
Determine whether there is consistency between the developmental age of the child and degree of independence. Cognitive and physical limitations may interfere with emerging independence; however, parents may “baby” or overindulge a child who has a disability.
Work with parents to set reasonable expectations and to break down tasks into simple, achievable steps. Care should be taken not to address too many areas at one time so as not to overwhelm the family.
Use appropriate discipline and behavior-modification techniques, such as extinction, time-out, and reward, to achieve cooperation and success.
Demonstrate and encourage play with the child at the appropriate level to provide stimulation and work toward achieving developmental milestones.
Provide parents with information and links to community, regional, or national associations for children with special needs and refer to parent support groups. These resources are frequently found online and can be easily accessed to provide an ongoing source of information and support to families.
Ensure that the child’s physical well-being is optimized to promote optimal development and self-esteem. Any congenital defects should be repaired (ie, orthopedic, cardiac, craniofacial, etc), and attention should be paid to dental health and restorative orthodontic procedures that can improve facial appearance.
Providing Opportunities to Develop Social and Self-Care Skills
Be aware that feeding, toileting, dressing, and grooming are important aspects of socialization and development of selfesteem. These tasks should be taught in a developmentally appropriate manner to children with a focus on maximizing independence. Many adaptive devices are available and
an occupational therapist can guide families in effective techniques to help children with developmental disabilities master these important self-care skills.
Make parents aware that recreational and leisure-time experiences are valuable in building social skills and self-esteem. Rehearse and role-play desirable social behaviors—such as waving goodbye, saying “hello,” taking turns, saying “please” and “thank you,” and responding to their name—will help build social confidence and acceptance within the child’s peer group.
Offer suggestions that will be enjoyable and developmentally appropriate for the child. Interaction with developmentally delayed and nondevelopmentally delayed peers is desirable. The Special Olympics is one example of an adaptive program. Local programs are also available in many areas.
Praise the child for participation in activities, regardless of whether the child succeeds.
Maintaining Safety
When handling the infant, provide adequate support with a firm grasp because the infant may be floppy due to poor muscle tone.
If hypotonia is accompanied by poor head control and neck strength, position the infant to prevent aspiration should vomiting occur.
Support the infant with a diaper roll, if needed, to maintain position.
Change the infant’s position frequently.
Continuously check the environment for the safety needs of the child.
Be aware that hypotonia can also lead to increased heat loss as more surface area is exposed; therefore, infants may need close attention paid to swaddling and environmental control to maintain thermoregulation.
Advise the parents to:
Maintain appropriate surveillance of the child when cooking or exposing him or her to other potential hazards that he or she may be able to get into but not understand.
Help the child to read words, such as danger and stop.
Teach the child how to call and ask for help.
Teach the child to say no to strangers.
Teach the child appropriate and socially acceptable sexual behaviors to prevent exploitation.
Teach sex education, with practical information about anatomy, physical development, and contraception, in a way the child and adolescent can understand.
Ensure that parents and caregivers have the knowledge and resources to transport their child safely.
NURSING ALERTTeach caregivers to base safety needs on the developmental, not the chronological, age of the child.
Family Education and Health Maintenance
Remind the parents to recognize the child’s routine health care needs and maintain regular follow-up with a primary care provider.
Immunizations.
Regular dental checkups.
Vision and hearing examinations.
Anticipatory guidance related to developmental stages, safety, behavior, and transition planning.
Provide guidance and support to parents with their child’s multidimensional treatment plan. Make sure parents know whom to contact when problems arise. Encourage parents to develop and maintain a notebook with a calendar to keep track of agencies, subspecialists, and professionals who will be involved with their child. It should include such information as business cards, names, addresses, phone numbers, appointment dates, health providers’ orders, and insurance information.
Teach parents the benefits of a therapeutic home environment.
Develop optimum sleeping, eating, working, and playing routines.
Ensure adequate nutrition.
Divide tasks and expectations into small, manageable parts. Give only one or two instructions at a time.
Set firm but reasonable limits on behavior and carry through with consistent discipline.
Avoid situations that cause excessive excitement, stimulation, or fatigue.
Provide an energy outlet through physical activity and outdoor play, as well as vocal outlet.
Channel the need for movement into safe, appropriate activities.
Discuss preparation for independent living, when appropriate.
Help the parents identify areas of home responsibilities that may be delegated to the cognitively impaired child.
Guide development of social skills that will be an asset to later vocational life.
Help the child to develop a set of attitudes and behaviors that will increase motivation.
Refer for vocational assistance to ARC (formerly Association of Retarded Citizens) or other rehabilitative agencies.
Begin planning for transition from pediatric care providers to adult providers well in advance of when eligibility for services will run out to ensure appropriate provider(s) can be established in a timely way and to avoid gaps in health care.
Refer for genetic consultation for information about genetics of the disorder, risk in subsequent pregnancies (parents) or risk to offspring (patient), or risk to other relatives as it is warranted by the diagnosis.
Evidence BaseWang, G., McGrath, b., & Watts, C. (2010). Health care transitions among youth with disabilities or special health needs: An ecological approach. Journal of Pediatric Nursing, 25(6), 505-550.
Richmond, N., Tran, T., berry, S. (2012). Can the medical home eliminate racial and ethnic disparities for transition services among youth with special health care needs? Maternal & Child Health Journal, 16(4), 824-833.
Evaluation: Expected Outcomes
Parents hold infant frequently and seek information from health care provider.
Family members feed, assist with care, and bond with child.
Parents and health care providers monitor nutrition, growth, and development closely, with ongoing assessment of most effective feeding techniques and management of any feedingrelated problems.
Parents describe developmental level of child and have realistic goals for attainment of next milestones in collaboration with their health care team and occupational, speech, and physical therapists.
Parents seek out support and resources that enable them to share their experience in raising a child with special needs and gain information needed to optimize the child’s developmental and health outcomes throughout childhood.
Child remains safe through careful handling and appropriate supervision.
DEVELOPMENTAL DISABILITIES
Cognitive Developmental Delay
Cognitive developmental delay, also known as intellectual disabilities, refers to the most severe, general lack of cognitive and problem-solving skills. The American Association on Intellectual and Developmental Disabilities defines intellectual disabilities as a disability characterized by significant limitations both in intellectual functioning and in adaptive behavior, which covers many everyday social and practical skills. This disability originates before the age of 18 years. There are many causes and a wide range of impairments. Approximately 3% of the United States population— 6.6 million people—are considered mentally retarded. Of every 1,000 infants born, 30 will be classified as mentally retarded before they reach age 18 years.
Pathophysiology and Etiology
No identifiable organic or biologic cause can be found for 50% of children with cognitive delays.
Identifiable causes include:
Genetic causes, such as Down syndrome, fragile X syndrome, Angelman’s syndrome, and inborn errors of metabolism such as phenylketonuria (PKU).
Congenital anomalies, including brain malformations, hydrocephalus, and microcephaly.
Intrauterine influences, such as alcohol and drug exposure, congenital infections, and teratogens.
Perinatal trauma—birth hypoxia, intracranial hemorrhage.
Postnatal trauma—acquired brain injury from falls, automobile accidents, near drownings, child abuse (shaken baby syndrome).
Postnatal infections and diseases (meningitis, encephalitis, sepsis, hypoxia, brain tumors).
Environmental exposure to toxins such as lead; environmental deprivation; neglect.
The malfunctioning brain is poorly understood in most cases, but physiologic alterations identified in some cases include:
Congenital brain malformations, brain tissue damage, or underdevelopment of the brain as shown by normal results of computed tomography (CT) scan or magnetic resonance imaging (MRI).
Biochemical errors or errors of metabolism, in which an absence of an enzyme or hormone produces abnormal brain function or formation, as in PKU or hypothyroidism.
IQ is 75 or below; classification has been recently changed to mild and severe and includes both cognitive and functional ability.
The more severe types of intellectual developmental disability tend to be diagnosed in early infancy, especially when they coexist with an identifiable syndrome or congenital anomaly.
Milder forms of intellectual developmental disability tend to be diagnosed in the preschool years, when language and behavioral concerns call attention to slower development.
Limitations in adaptive ability occur in communication, self-care, home living, social skills, community use, selfdirection, health and safety, functional academics, leisure, and work.
Clinical Manifestations
Developmental delays (failure to achieve age-appropriate skills) are evident to some degree in almost all areas.
Infancy
“Poor feeder”—a weak or uncoordinated suck results in poor breast- or bottle-feeding, leading to poor weight gain.
Delayed or decreased visual alertness and curiosity with poor visual tracking of face or objects.
Decreased or lack of auditory response.
Decreased spontaneous activity or asymmetric activity.
Delayed head and trunk control.
Floppy (hypotonic) or spastic (hypertonic) muscle tone.
NURSING ALERTAlthough 75% of individuals with intellectual developmental disabilities have no physical signs, they typically achieve motor milestones at a slower rate.
Toddler
Delayed independent sitting, crawling, pulling to stand, and independent ambulation.
Delayed communication—failure to develop receptive and expressive language milestones. Almost 50% of children with intellectual developmental disability are identified after age 3 years, when speech delays manifest themselves.
Failure of the child to make progress or show interest in the area of independence in self-feeding, dressing, and toilet training may reflect cognitive impairment.
Short attention span and distractibility.
Behavioral disturbances.
Decreased muscle coordination.
Diagnostic Evaluation
Federal law (PL 94-142—Education for All Handicapped Children and PL 101-476—Individuals with Disabilities
Education Act [IDEA]) ensures that each child with a delay or suspected of having a delay has a comprehensive evaluation by a multidisciplinary team. Part C of PL 105-17 calls for the creation of statewide, coordinated, multidisciplinary interagency programs for the provision of early intervention services. Each state has its own definitions and organizational framework to provide these services. No single test can diagnose intellectual developmental disabilities. The multidisciplinary evaluation should be individually tailored to the child.
Education Act [IDEA]) ensures that each child with a delay or suspected of having a delay has a comprehensive evaluation by a multidisciplinary team. Part C of PL 105-17 calls for the creation of statewide, coordinated, multidisciplinary interagency programs for the provision of early intervention services. Each state has its own definitions and organizational framework to provide these services. No single test can diagnose intellectual developmental disabilities. The multidisciplinary evaluation should be individually tailored to the child.
Rule out sensory deficits by assessment of vision and hearing, even if neonate hearing screen was normal.
Medical evaluation should include prenatal history, developmental history, sequential developmental assessments, family history including genetic assessment, and physical examination. The positive findings determine the direction of the individual evaluation and any diagnostic testing.
Unusual appearance (dysmorphic features) warrants evaluation by a genetic specialist.
History consistent with loss of developmental miles tones and positive family history warrants workup for presence of an inborn error of metabolism (diagnosed by blood, urine, or DNA analysis) or lead poisoning.
Children with macrocephaly, microcephaly, or neurologic abnormalities may require imaging studies, such as CT scan or MRI.
EEG is indicated for children with seizures.
Psychological testing (appropriate tests selected by a child psychologist):
The Bayley Scales are used to assess children ages 2 months to 3 years. This test is weighted on nonlanguage items and is used to assess fine motor, gross motor, language skills, and visual problem solving.
The McCarthy Scale offers a “general cognitive index” that is roughly equivalent to an IQ score.
The Stanford-Binet Intelligence Scale is used to test the mental abilities of children age 2 and older.
The Wechsler Preschool and Primary Scale of Intelligence—Third Edition (WPPSI-3) measures mental age of 3 to 7 years.
The Wechsler Intelligence Scale for Children—Fourth Edition (WISC-IV) tests children whose functional age is above a 6-year level.
The Vineland Scale tests social-adaptive abilities—selfhelp skills, self-control, interaction with others, cooperation; the Adaptive Behavior Scale is similar to Vineland but also measures adjustment.
Complications and Associated Findings
Seizures.
Cerebral palsy.
Sensory deficits.
Communication disorders (speech and language).
Neurodevelopmental disorders.
Psychiatric illness.
Specific learning disabilities.
Emotional and behavioral problems (self-injury, hyperactivity, aggression).
Management
An interdisciplinary team evaluation by a developmental pediatrician, clinical psychologist, and counselor is usually the initial step in the management of mental retardation. This type of evaluation can be obtained through a state or private diagnostic and evaluation center, public school, or universityaffiliated program.
Associated medical problems, such as seizures, feeding difficulties and poor nutrition, sensory deficits, or dental problems, must be treated to allow the child to maximize his or her potential.
If a treatable cause is identified, such as an inborn error of metabolism, a therapeutic diet can be instituted. Hypothyroidism can be treated with thyroid hormone.
A family assessment is essential to address:
Financial stressors—financial aid may be available through Social Security, Medicaid, or state and local programs to help families with children with developmental delays.
Family functioning and coping abilities.
Support for parents, siblings, and extended family.
Respite services.
Support groups.
Recreational programs, such as the Special Olympics and summer camps.
Identification of other affected or at-risk people and the need for genetic counseling for recurrence risk.
The initial evaluation usually leads to recommendations for more targeted evaluations, such as physical therapy, occupational therapy, and social work assessment.
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
