#36644: Caring for the Adult with Developmental Disability

Congenital heart conditions occur in about 50% of infants born with Down syndrome [25,26]. Screening by a pediatric cardiologist is essential, and surgical correction of defects is often necessary. Throughout their life span, individuals with Down syndrome should have follow-up care by a cardiologist [27]. Many individuals with Down syndrome will require subacute bacterial endocarditis prophylaxis with antibiotics prior to any invasive procedures or treatments, including routine dental care [25,28]. The American Heart Association recommendation states that antibiotic prophylaxis is not necessary for all individuals with certain heart conditions; the change in recommendation did not specifically address patients with DD [96]. The American Heart Association recommendations indicate that individuals with previous heart surgery should continue to take antibiotics before invasive procedures.

Further medical management may include the use of medications including digoxin, ACE inhibitors, and diuretics to prevent early heart failure [106]. Mitral valve prolapse (MVP) is a common congenital occurrence, and in those with Down syndrome, there is an increase in valve-related problems as the individual enters adulthood. Echocardiograms are often indicated in adulthood to follow up on the impact of congenital heart conditions and to assess changes related to aging [17,28]. Nurses should be alert for changes in fatigue level, shortness of breath, hypertension, or other signs of increasing cardiac difficulties.

A number of problems may occur in individuals with DD, especially in those with Down syndrome, due to the structure of the head and neck. Ear problems often occur because of a shortened eustachian tube, resulting in serious ear infections [24,26]. Because chronic respiratory disease is common with frequent infections, it has been suggested that individuals with Down syndrome receive pneumococcal and influenza vaccines to reduce the risk of life-threatening respiratory infections [24].

The facial structure of people with Down syndrome is characteristic and usually presents with a small mouth, high palate, and short neck. When combined with obesity, these characteristics increase the risk of obstructive airway disease and sleep apnea. Studies have suggested that nearly all persons with Down syndrome have some form of sleep-related obstruction [104]. Daytime sleepiness, irritability due to fatigue, snoring, and periods of apnea during nighttime sleep should be evaluated. Sleep apnea should be treated because it can lead to other serious long-term problems, such as pulmonary hypertension, heart failure, and even death [17,26]. Tonsillectomy and adenoidectomy may be indicated, but often sleep apnea persists after surgical intervention [25,104]. Other surgical interventions may be considered for persistent sleep apnea, including uvulopalatopharyngoplasty, midline posterior glossectomy, lingual tonsillectomy, genioglossus advancement, hyoid advancement, and craniofacial surgery (e.g., mandibular and midface advancements). In some individuals, positioning with pillows or raising the head of the bed may be sufficient. In others, use of a continuous positive airway pressure (CPAP) machine may be beneficial [17,104]. Patience is required on the part of caregivers in order to help individuals learn how to use the CPAP machine. However, the improvement in rest and general feeling of well-being that comes with sleeping well may be an additional motivator. It is important to note that some patients with DD have sensory disorders or are hypersensitive to forms of touch. For these patients, use of a CPAP machine may be intolerable.

Individuals with DD who do not have sleep apnea may have other problems with sleep. For example, they often tend to have more restless sleep with intermittent myoclonic jerks, decreased oxygen saturation, and the potential for restless legs syndrome [17]. Interrupted sleep may contribute to daytime irritability and can be misinterpreted as a mood disorder. Individuals who demonstrate irritability should have their sleep pattern evaluated; behavioral assessments may also be indicated [24].

Atlantoaxial and atlanto-occipital instability are conditions that occur in about 10% to 20% of individuals with Down syndrome; however, actual spinal compression is rare [24,25,26,28]. Atlantoaxial instability is a term that refers to increased mobility and hyperflexibility of the cervical spine at the level of the first and second cervical vertebrae. Atlanto-occipital instability refers to a similar process in the occipital-cervical area [24]. Neurologic symptoms that may require surgical intervention can occur as a complication of this condition. Early onset of progressive arthritis of the spine is also common with aging and may manifest largely as a refusal to move [17,25,28]. A physical therapy evaluation may be helpful to suggest exercises that strengthen muscles and reduce trauma to the cervical spine. Cervical radiographic screening for atlantoaxial instability is no longer recommended routinely in adults, but cervical radiography in neutral, flexed, and extended positions should be considered if any of the signs or symptoms are present (Table 2) [28]. Pain management may become an issue as arthritis develops.

As with individuals with dementia, pain assessment can be difficult among individuals with DD [29]. Response to pain is subjective, and the same person may respond differently in different circumstances, such as when fatigued, in a strange environment, or stressed by some other occurrence. A study of nurses providing care to DD patients has indicated that moaning and crying during manipulation are the most frequent indicators of pain [30]. Facial expression, not using a specific body part, or moving in an unusual way have also been identified as indicators of pain [30]. Assessing pain and appropriately medicating are important nursing interventions in reducing overall discomfort and maintaining activity levels of DD individuals who have developed arthritis.

Subluxation of the hip and knee and various foot disorders also occur frequently in individuals with Down syndrome [17]. In addition, scoliosis commonly occurs in a variety of development disabilities, including Down syndrome, Prader-Willi syndrome, Rett syndrome, and neurofibromatosis [31,32,33]. Some of these conditions may be corrected surgically in childhood. In older individuals who may not have had the benefit of corrective surgical procedures, painful joints and arthritic changes are often a real problem. Efforts to utilize physical therapy interventions and appropriate pain management should be encouraged by the nursing staff [17,30].

Conditions resulting in hearing loss are commonplace among those with DD [7]. Unfortunately, this may lead to further communication problems, particularly if the hearing loss is profound and the individual cannot learn to sign [105]. Individuals with DD and a serious hearing loss may develop their own method of signing and expression with individuals who are familiar to them. This further illustrates the value of having a caregiver or advocate available when medical care is being provided. Many individuals with DD, particularly those with Down syndrome, have small ear canals and examination can be difficult. For adults, use of pediatric equipment for ear exams is beneficial. Because the ear canal is small, accumulations of wax can add to hearing problems. Nurses should be aware of this and clean ears when indicated [105].

According to the College of Family Physicians of Canada, vision and hearing impairments among people with intellectual/developmental disabilities are often underdiagnosed and can result in substantial changes in behavior and in social and adaptive functioning. They recommend performing an annual office-based screening of vision and hearing or when symptoms or signs of visual or hearing problems are noticed. In addition, patients should be referred for optometry assessments to detect glaucoma and cataracts every two years after 40 years of age and for audiology assessments based on screening and for age-related hearing loss every five years after 45 years of age.

(https://www.cfp.ca/content/64/4/254 Last Accessed: June 10, 2022)

Strength of Recommendation: Strong recommendation

Hearing loss may result from conduction problems, sensorineural difficulties, or a combination of both [24]. Screening for hearing problems should occur periodically in childhood because some loss may not be apparent in infancy. The American Academy of Pediatrics and the Down Syndrome Medical Interest Group have recommended audiologic testing at birth and then every six months up to 3 years of age. Testing should thereafter occur annually [24]. Hearing losses that occur in adulthood may lead to behavioral changes that could be mistakenly interpreted as psychiatric problems. Hearing evaluations every three years in adulthood are appropriate.

A number of eye conditions have been associated with developmental disabilities. Estimates of the occurrence of eye conditions range from 25% in the mildly disabled to 60% in the profoundly disabled [7,34]. Among individuals with Down syndrome, dense congenital cataracts occur at a rate of about 3% [25]. Failure to diagnose and remove cataracts will result in blindness. Accommodation problems are common, and correction with glasses may not always be completely successful [17]. Additionally, it may be difficult to get the individual to wear glasses regularly. Consequently, vision may be consistently fuzzy. There is also an increased likelihood of the development of cataracts in adulthood and at an earlier age than is seen in the general adult population [17]. Strabismus and blepharitis are also common, and blepharitis may require ongoing treatment [17,34]. Among patients with tuberous sclerosis, retinal hamartomas or phakomas are more common [35]. Nystagmus, or involuntary eye movement, is associated with hydrocephalus, cerebral palsy, and meningitis [95].

Treatment teams should always consider the impact of hearing problems and eye conditions when planning care for individuals with DD. Regular exams will identify changes, and as a result, appropriate correction or treatment can be initiated. Behavior problems may result from sensory difficulties, and eye and ear exams should be conducted before assuming that a problem is entirely behavioral in origin. Sensory impairments may reduce the ability of the DD adult to attend medical appointments unaccompanied and may also increase their distress during consultations and physical examinations [7].

Seizures in persons with DD are likely to be severe, occur often, and be difficult to control. The prevalence of seizures increases as the degree of psychomotor retardation increases, and regularly experiencing seizures may decrease DD patients' life expectancy by up to 20 years [29]. A review of more than 2,000 deaths of individuals with DD in residential care found that 57.2% of these individuals had a seizure disorder [36]. Other studies have reported seizure disorder among individuals with DD occurring at 30 times the rate of that of the general population [37].

Assessing for seizures, administering medications appropriately, and managing the physical and psychologic aftermath of seizures are largely nursing responsibilities. Seizures may be difficult to recognize in patients with DD. In addition to generalized tonic-clonic movements, seizures may manifest as drop attacks, laughing spells, subtle tonic deviations of the eyes, or behavioral changes [38]. When one is familiar with an individual who has seizures, it is often possible to note signs that a seizure is imminent. Some individuals will have an aura or sign that they are about to have a seizure. Blurred vision, changes in light perception, or other sensory experience may cue the beginning of a seizure. If individuals can communicate this to those around them, support may be provided immediately. Even in individuals who cannot communicate clearly, it may be possible to recognize irritability or some other sign that may indicate a seizure is likely.

When individuals are experiencing a seizure, those around them should try to protect the individual from harm as much as possible, although holding them is likely to be more harmful than letting them move about. All seizures should be timed. After five minutes of continuous seizure activity, emergency services should be contacted to transport the person to an emergency department, where medication can be given to stop the seizure [38]. It is not unusual for an individual to be incontinent during a seizure, and they may need assistance after they awaken. Individuals may be confused and/or combative when the seizure is over. They should always be approached with caution. Nurses and other staff should clearly identify themselves and communicate the actions that will be undertaken. Periods of confusion can last for hours after a grand mal seizure. Often, the individual will fall into a deep sleep after the seizure is over and may awaken with no memory of it.

Management of seizures in the older adult with DD is challenging. In addition to being commonplace among individuals with DD, seizures are also often poorly controlled despite the use of multiple seizure medications in high doses. Only 40% of DD individuals with seizures remain seizure-free and controlled without toxicity [39]. Anticonvulsants or antiepileptic drugs (AEDs) are the most commonly administered medications [18,38].

The number of AEDs available has more than doubled over the past two decades. Many of the medications have multiple side effects and drug interactions. Older patients may be more prone to side effects due to polypharmacy and changes in oral absorption and renal and hepatic metabolism. Some of the more common and important side effects of the newer AEDs include hyponatremia (with oxcarbazepine or carbamazepine), nephrolithiasis (with topiramate), aplastic anemia (with felbamate), behavioral changes (with levetiracetam or ezogabine), and vision loss (with vigabatrin) [38,40]. All seizure medicines should be used cautiously in the anticoagulated patient, as many AEDs interact with warfarin. However, seizures should be adequately treated because of the inherent risk of brain damage and the risk of fall and hemorrhagic complications in the event of an injury [38].

In 2009, the U.S. Food and Drug Administration (FDA) issued an update following the completion of its analysis concerning the risk of suicidal behavior or ideation observed during clinical trials of various antiepileptic drugs (compared to placebo) in the treatment of epilepsy, psychiatric disorders, and other conditions. The pooled analysis of 199 clinical trials involving 11 antiepileptic drugs (i.e., carbamazepine, divalproex sodium, felbamate, gabapentin, lamotrigine, levetiracetam, oxcarbazepine, pregabalin, tiagabine, topiramate, and zonisamide) as either monotherapy or as adjuvant therapy showed that 0.43% of patients receiving an antiepileptic had suicidal behavior/ideation compared to 0.24% of patients receiving placebo. As a result of the findings, the FDA has required that the product labeling of the entire class of antiepileptics include a warning concerning the risk of suicidal behavior or ideation, and medication guides have been developed informing patients of this risk [40,41].

Although some individuals will continue to have seizures despite being on medication, the frequency, duration, and severity of seizures may be greatly reduced. Administering the medications precisely as ordered and at the times indicated is very important. In individuals who are reluctant to take medications, all effort should be made to administer seizure medications as scheduled.

A number of urologic conditions may be associated with intellectual disability. For example, individuals with Down syndrome are at heightened risk for several problems, including obstruction in the lower urinary tract, renal hypoplasia, and ureterovesical junction obstruction [17,42]. Persons with Smith-Lemli-Opitz syndrome, a type of developmental disability, have been found to have upper urinary tract abnormalities at a rate of 57% and genital abnormalities at a rate of 71% [43]. Other problems that may occur in individuals with DD include renal hypoplasia, obstructive uropathy, and glomerular microcysts [44,97]. It should also be noted that, among boys and men with Down syndrome, undescended testicles are common and the risk for testicular cancer is increased [44].

Although some urologic problems can be corrected surgically, others cannot. There is also a high likelihood of neurogenic bladders due to both poor muscle tone and, in some cases, the side effects of long-term use of medications [17]. Additionally, some individuals with DD have small bladders, which can result in poor bladder control and difficulty with continence. From a nursing standpoint, it is important to be alert for signs of urinary tract infections (UTIs). Individuals with small bladders, poor bladder tone, and possible partial obstructions in the urinary tract are at high risk for a UTI. Encouraging adequate fluid intake is a significant nursing intervention and should be a priority.

Bladder control programs are effective, and toileting on a regular schedule, usually every two hours while awake, is valuable. A 10-year follow-up study of bladder continence training in individuals with DD found that individuals involved in the study retained bladder control, and cueing as well as reminders were sufficient to help them toilet at regular intervals. The individual training programs were found to be cost effective and significantly reduced staff time [45]. The use of adult diapers may be appropriate in some cases, but this should be a decision made after discussion with the individual and his or her advocate. All intimate and personal care should be undertaken with respect for the individual's privacy and dignity [46].

Hypothyroidism occurs frequently among adults with DD. Among individuals with Down syndrome, frequency estimates range from 13% to 50% [26]. The signs of low levels of thyroid hormone may not be obvious in those with DD. Signs include dry skin, lethargy, constipation, weight gain, and low cognitive functioning, all of which may also be signs of the disability. Most experts recommend screening with a thyroid panel every one or two years, beginning at 21 years of age [17,25,26].

Optimal thyroid functioning is important for cognitive functioning and normal metabolic activity. If results are abnormal, thyroid replacement therapy can be initiated. Nurses in residential and treatment facilities for adults with DD should follow up to ensure laboratory tests are being obtained. Before a diagnosis of depression or dementia is made, thyroid function should be checked [17].

Type 1 diabetes has been found to be more common among individuals with DD, particularly those with Down syndrome, than among the general population [98]. Other studies have found that individuals with DD who practice dietary management and regularly exercise have no higher incidence of type 2 diabetes than the general population [52,53]. This is a significant finding and appears to be in line with the general population's increase in diabetes along with an increase in obesity. Given that individuals with DD often tend to be shorter and stockier in build than the average person, it is particularly significant that a program emphasizing dietary controls and exercise may be helpful in reducing the incidence of diabetes among this population [17]. Although there are no specific recommendations regarding screening for diabetes in individuals with Down syndrome, it may be reasonable to screen these patients for this disease. They should also be counseled about diet, exercise, obesity, smoking, and alcohol use [28].

In some respects, there appears to be good news for adults with DD in the area of cardiovascular problems. Although individuals with DD have a higher rate of congenital heart defects and may have complications that persist into adulthood, the risk of additional cardiovascular problems have been found to be no higher for individuals with DD than they are for the general population [54,55]. Additionally, women with Down syndrome have been found to be less likely to show insulin resistance syndrome, a neuroendocrine disorder that underlies atherosclerosis and diabetes [53,56]. Both men and women with Down syndrome have fewer arteriosclerotic risk factors than a similar group of individuals without Down syndrome [56].

A significant and demonstrated factor in the reduced risk for cardiovascular disease for these individuals is lower dietary fat consumption and frequent physical exercise. Those who adopted these habits were one-third less likely to have insulin resistance syndrome and abdominal obesity compared to those who participated in less frequent exercise or who consumed higher intakes of dietary fat [53]. This is important information for caregivers and healthcare providers to adults with DD. Appropriate diet and lifestyle, with lower fat intake and regular exercise, is significant in reducing the risk for both diabetes and cardiovascular conditions. Encouraging participation in sporting events, such as the Special Olympics, may be beneficial for DD patients' health in addition to the benefits of establishing a supportive peer group.

Lower bone density and resulting osteoporosis have been found to be common among people with DD [18,57,58,59]. A study of men institutionalized with DD found that 34% had ultrasound index values two standard deviations below an age-matched control group. Additionally, 51% had values more than two standard deviations below the reference group, demonstrating a very high occurrence of low bone mineral density [57]. A study of institutionalized postmenopausal women with DD found that 82% were at high risk for osteoporosis, with a severe decrease in bone mineral density [58]. This is due to multiple factors, including Down syndrome, smaller body size, endocrine abnormalities, reduced physical activity, reduced muscle strength, use of certain medications, and in women/girls, high phosphate levels [29,47,60].

According to the College of Family Physicians of Canada, osteoporosis and osteoporotic fractures are more prevalent and occur at a younger age among people with intellectual/developmental disabilities than among those in the general population. Therefore, they recommend screening both male and female patients for osteoporosis starting in early adulthood. Clinicians should seek advice from a radiologist regarding alternative methods to assess risk of fragility fractures if the patient cannot be assessed using the usual nuclear bone mineral density test, such as by assessing the patient's forearm only.

(https://www.cfp.ca/content/64/4/254 Last Accessed: June 10, 2022)

Strength of Recommendation: Strong recommendation

Low vitamin D levels have also been reported in both men and women [60]. However, it is unclear if low vitamin D levels are associated with dietary issues or if they may result from other factors. Additionally, the effects of vitamin D on bone health have been difficult to separate from the associated effects of dietary calcium [61]. Certain anticonvulsant medications are known to affect the absorption of vitamin D, and this may also be a factor [62].

Bone density measurement may be difficult because individuals with DD may not be able to achieve the positioning needed for a central dual photon x-ray absorptiometry (DEXA) measurement or may not be able to lie still for the time it takes to do a scan. In addition, differences in bone and body size, shorter stature, anatomical deformities, and behavioral alterations may prevent accurate measurements. Calcaneal (heel) DEXA, peripheral DEXA of the finger, photon absorptiometry of the lumbar sacral spine, central DEXA of the femur, and ultrasound are testing options that may be useful alternatives in these situations. These options have shown good correlation with a central DEXA measurement but are not generally used to monitor treatment [47,63].

From a nursing standpoint, the concern should be for the high risk of falls and subsequent fractures and complications. A study of 268 elderly adults with DD determined that falls and injuries were higher in those who were 70 years of age, ambulatory, on antipsychotic medication, had suboptimal seizure control, and were exhibiting more destructive behaviors. Procedures that may help minimize the risk of falls in ambulatory patients include corrective eyewear, suitable shoes, maintenance of good lighting, and a safe environment. Screening for risk of falls may be different in the elderly population with disabilities compared with the normal elderly population [63].

Case Study

Ms. M, Ms. J, Ms. A, and Ms. C are four women, all older than 50 years of age, who reside in a group home setting. All of the women participate in a sheltered workshop during the week and are active participants in a variety of activities offered to the residents in their group home. Two of the women, Ms. J and Ms. C, have family who live in the area, and they often spend weekends and holidays at a family member's home. Their primary care physician sees all four women for yearly physicals, and they receive mammograms and Pap tests on a yearly basis. All four women receive supplemental calcium with vitamin D (600 mg twice daily). This year during their physicals, their physician refers all four women for a bone density study. Based on the results of the studies, both Ms. M and Ms. C are placed on raloxifene (Evista), 60 mg daily. The reports of the studies are forwarded to the nurse consultant. Ms. M's report shows mild osteopenia in her spinal column. Ms. C's report shows significant osteoporosis in both hips and femur. It also shows osteopenia in the spinal column, indicating a significant risk for fracture.

Ms. C is 60 years of age, with cerebral palsy. She ambulates with a wheeled walker. She requires a rocker knife and fork to feed herself but does remain substantially independent in her group home setting. After reading the report, the nurse consultant advises Ms. C's case manager of the findings and recommends discussing the results with Ms. C's sister, who is her advocate, and alerting the direct care staff members to the fall risk. The multidisciplinary team also decides, with Ms. C and her sister's agreement, to place half side rails on Ms. C's bed. Until this time, Ms. C has been very slowly walking the block between the workshop and her home twice a day. This is becoming increasingly difficult for her, and she frequently cries with pain, complaining of feeling too tired for activities after returning home. It is decided to transport her via wheelchair to and from the workshop, with her continuing to use her walker for all other ambulation. She is included in a weekly yoga group and a daily stretching exercise group at the workshop to help increase her agility and muscle strength.

Alzheimer disease is the most common form of dementia. Individuals with Down syndrome develop a clinical syndrome of dementia that is nearly identical to the characteristics of Alzheimer disease described in individuals without Down syndrome. The main difference is the early age of onset (i.e., late 40s or early 50s) in individuals with Down syndrome [17,74,75]. It is not clear why there is an increased likelihood of dementia in this population, but it is clear that the plaque and tangle formations found in the brain of individuals with Alzheimer-type dementia are sometimes found in the brains of individuals with developmental disorders. Additionally, there is an increase in frequency as the person with DD ages [74,75]. Epidemiologic and brain imaging studies of patients with Alzheimer disease and without Down syndrome have led to observations that patients with limited education or diminished baseline cognitive abilities may be at increased risk for Alzheimer disease. These data have led to the cognitive reserve hypothesis, which suggests that patients with better baseline cognitive abilities can tolerate more Alzheimer disease pathology and neuronal loss than patients with worse baseline cognitive abilities. Because most patients with Down syndrome have developmental disabilities and limited baseline cognitive ability, the cognitive reserve hypothesis would suggest that patients with Down syndrome are at increased risk of developing Alzheimer disease [75].

Dementia is more prevalent among adults with intellectual/developmental disabilities compared with the general population, with a statistically significantly increased risk in adults with Down syndrome and at an earlier age. For patients at risk of dementia, the College of Family Physicians of Canada recommends referring to a psychologist for a baseline assessment of cognitive, adaptive, and communicative functioning after 40 years of age and at 30 years of age for people with Down syndrome.

(https://www.cfp.ca/content/64/4/254 Last Accessed: June 10, 2022)

Level of Evidence: Moderate recommendation

In some cases, the signs of dementia are associated with the use of neuroleptic medications, hypothyroidism, or depression. For a diagnosis of dementia to be made, the individual must be found to be functioning at a cognitive level below their baseline and the decline must be progressive. Nurses can make a significant contribution in helping to assess an individual for dementia because they are most likely to observe the individual frequently and be able to provide a clearer picture of changes from the baseline level of functioning. Additional studies of individuals with Down syndrome residing in communities have shown a lower occurrence of dementia than in previous studies of institutionalized populations. It is possible that the more active lifestyle of community residents has helped reduce their chances of having early onset dementia [74].

Depression is a persistent alteration in mood, behavior, and cognition that has biologic and psychologic underpinnings and results in functional decline. It often goes unrecognized and untreated, leading to poor quality of life, increased medical comorbidity, decreased ability to live independently, caregiver burden, and heightened mortality [76]. Despite widespread acceptance that depression can occur in adults with intellectual disability, the difficulties encountered when assessing and diagnosing depression in this population may hamper the individual clinician and make selecting appropriate treatment problematic [77]. Depression may be missed when signs of depression are attributed to other causes or to the disability itself. Although new tools have emerged, there is as yet no gold standard diagnostic tool for assessing depression in this population [72,78,79].

Psychotropic drugs may be of great benefit if they are given for specific diagnoses and symptoms. Drugs of most benefit in psychiatric care of individuals with DD are antidepressants, mood stabilizers, and antipsychotic medications [69]. Antidepressants are widely used in the general population, including in adolescents and children, for the treatment of depression, anxiety disorders, obsessive-compulsive disorders, and PTSD. Although the possibility of side effects is not entirely absent, consideration should be given to the damaging effects of untreated depression, and a balance should be achieved.

Mood stabilizers include lithium, valproic acid, lamotrigine, oxcarbazepine, and carbamazepine. With the exception of lithium, all of these drugs are also used for seizure control, and a dual benefit may be achieved in some individuals with DD [84]. These drugs are also used to treat bipolar disorder, and in individuals with DD who have a family history of bipolar disorder, it is appropriate to assess for signs of mood instability and treat accordingly [84]. Although there is limited data, clinical experience and some research indicate success in reducing aggression and self-injury in those with DD with the use of valproic acid [81].

Atypical (i.e., second-generation) antipsychotics were developed in the 1990s and are often used because they are thought to have fewer side effects and better efficacy than their predecessors [84]. They include risperidone, quetiapine, olanzapine, ziprasidone, aripiprazole, lurasidone, and paliperidone and are used to treat schizophrenia, severe anxiety, and aggression. These drugs are now more accepted than first-generation antipsychotics, but there is still a potential for extrapyramidal symptoms and weight gain [40,84]. It is important to note that the FDA has issued a Public Health Advisory for atypical antipsychotic medications because it has been determined that death rates are higher for elderly people with dementia when taking these medications. Antipsychotic medications are not FDA-approved for the treatment of behavioral disorders in patients with dementia [84].

In some individuals with DD who are demonstrating psychotic thinking processes and are paranoid, delusional, and/or hallucinating, the use of antipsychotics is indicated. In situations of extreme aggression or self-injurious behavior, they may also be indicated. It is important to note that while aggressive challenging behavior is frequently reported in adults with intellectual disability and often treated with antipsychotic drugs, there is no adequate evidence to support this practice. Antipsychotic drugs should no longer be regarded as an acceptable routine treatment for aggressive challenging behavior in people with intellectual disability [85].

Nursing responsibilities in the use of psychotropic medications are to provide accurate assessment information and monitor for side effects and interactions with other medications. Nurses should ensure that all direct care staff members are aware when a new medication is prescribed and advise them of what to watch for. All individuals receiving antipsychotics should be regularly tested for movement disorders to provide objective data [84]. Because seizure disorders occur so frequently in individuals with DD, nurses should be particularly alert for an advent of seizure or an increase in frequency when psychotropic medications are being used [40].

Case Study

Mr. M is 42 years of age and resides in a group home, where he attends a sheltered workshop. He has been diagnosed with a moderate developmental disorder. Other diagnoses include seizure disorder, hypothyroidism, and hypercholesterolemia. He enjoys a variety of activities, including simple puzzles, baseball games, and going out to eat. His seizures are fairly well controlled with valproic acid 1,000 mg twice daily and topiramate 100 mg twice daily. He seizures at five- to six-week intervals and is often confused after seizures. Generally, he is cooperative with staff members' requests, but in the past two months, he has had five incidents of swearing and striking out at other residents. On two occasions, he has been physically aggressive with staff members.

After a meeting with the case manager, Mr. M's mother, the workshop supervisor, the nurse consultant, and Mr. M, it is decided to ask all staff members to observe Mr. M closely over the next two weeks to attempt to identify the source of his aggression. At the meeting, his mother reports that she has observed Mr. M talking to images on his television. When the group meets again two weeks later, the case manager has collected behavior observation sheets that indicate the activities Mr. M was engaging in at half-hour intervals. It is apparent that he is spending more time in his room. He has been observed on six occasions swearing at the television. There were also four incidents of physical aggression toward staff members and other residents. His mother reports that he seems very focused on characters on a reality show and talks about them as if he knows them. She also reports that he becomes quite angry while talking about the characters. It is decided that a psychiatric consult was necessary.

The psychiatrist evaluates Mr. M. When asked about the television programs, he is reticent to discuss them and reports to the psychiatrist, "They'll come after me." The psychiatrist concludes that Mr. M is experiencing thought insertion and delusions associated with his inability to correctly interpret reality television. This is leading to paranoia and physical aggression. The psychiatrist recommends quetiapine 50 mg twice daily and limiting television watching.

The case manager seeks approval from the facility's human rights committee for the medication and for a right restriction to limit television viewing. The committee, which includes Mr. M's mother, agrees, and the quetiapine is started. Mr. M's behavior plan is amended to identify efforts by the staff members to engage him in other activities and to encourage television watching in the day area. Fortunately, baseball season is starting, and it is possible to engage Mr. M and several other residents in watching baseball games.

After three weeks, Mr. M is spending more time with his peers, and there have been no further incidence of aggression. He appears to be tolerating the quetiapine well, and there has been no increase in seizures. After two months, the quetiapine is reduced to 50 mg at bedtime, and Mr. M continues to do well.

There is beginning to be more consideration of nonpharmacologic treatment for mental health disorders among adults with DD. This is probably due in part to the increase in individuals with DD living in community settings and, in many cases, receiving care at community mental health centers. Unfortunately, there is a lack of specially trained mental healthcare providers available to meet the needs of this population. Advanced practice psychiatric nurses are a group who has the ability to provide services and advocacy for individuals with DD and their families [83,86]. This has been the experience of the author in providing psychiatric nursing consultation to residential care facilities for adults with DD.

Behavior Modification

Behavior modification is based on the work of Skinner and is familiar to those who have taken a basic psychology course. The principle of behavior modification is based on the theory that all behavior is in response to positive and negative reinforcement and can be modified based on that theory. An outgrowth of Skinner's work was the establishment of behavior modification programs to treat the chronically mentally ill and individuals with DD [87]. In the 1950s, when these programs were put into place in institutional settings, considerable progress was made in the area of training institutionalized populations on basic skills. After a time, however, ethical and legal concerns arose about the use of negative reinforcement strategies, and many programs were abandoned [69]. However, there is still some value in the use of behavior modification, such as using a reward system to reinforce a desired behavior. For example, token economies, where the individual receives a token for a positive behavior and the token is later cashed in for a desired reward, have been successful in encouraging desired behaviors. If these interventions are included in a treatment plan and appropriate institutional approval is obtained, they can be very beneficial. The following is an example of the use of behavior modification to solve a health problem for a particular individual.

Case Study

Ms. J is a woman with mild developmental disability, diagnosed with type 2 diabetes, recurrent major depression, and PTSD. She lives in a residential facility. Her diabetes is treated with some success with oral agents, although she is noncompliant with diet restrictions. Her recurrent depression and PTSD are treated with an antidepressant and a mood stabilizer. Ms. J's weight remains around 185 lbs, and she has fat folds below her breasts and in the groin area. Direct care staff members report that Ms. J does not shower daily and often goes five to six days without a shower. The nursing staff notes when doing weekly skin audits that Ms. J has excoriated areas in the fat folds and she is complaining of discomfort. The nursing staff has explained to Ms. J the need to shower and dry the area well each day and that topical medication will then be applied to heal the area. Ms. J continues to refuse to shower regularly, and it is determined that it is a necessity for her own well-being that she do so. The case manager discusses this with Ms. J and sets up a program in which she will receive a portion of her weekly allowance on a daily basis, but only after she has showered for the day and allowed the nurse to apply the medication. Ms. J and her advocate agree to this arrangement, and the information is shared with the facilities human rights committee to obtain permission for a rights restriction. After a few weeks, the skin areas begin to heal, and Ms. J is complying with showers on most days. It is determined by the multidisciplinary team to continue the behavior modification strategy with a review on a quarterly basis by the human rights committee.

Cognitive Therapy

Cognitive therapy has been well-received in psychiatric care as an effective treatment for depression and anxiety. The principle of cognitive therapy is to help the individual change their negative self-view, thus improving his or her self-esteem and coping skills. Emphasis is placed on changing negative thinking patterns rather than just changing behavior [69,87]. Cognitive therapy focuses on learning new behaviors by using techniques introduced by the therapist [88,99]. Cognitive behavioral therapy has been used successfully with DD adults, and emerging studies, specifically on dialectical behavior therapy, have shown promise in effectively treating individuals with DD [67,69,88,100]. Role-playing, modeling, and homework assignments are some of the strategies used to initiate changes in thought and behavior.