It is well documented that adults with intellectual disability (ID) experience higher rates of a series of health conditions compared to their peers without disability. These health conditions include cardiovascular disease, obesity, diabetes, gastrointestinal disorders, and psychiatric and behavioral disorders. With life expectancy approximating the general population, adults with ID are also now experiencing health conditions related to aging, further increasing their risk for diminished function and well-being. This increased morbidity poses new challenges in geriatric healthcare planning for this population. Relatively simple health prevention practices, such as the implementation of a health screening tool, can substantially increase disease detection and clinical activities directed toward improved health outcomes for people with ID. This study examines data collected from the District of Columbia Developmental Disabilities Administration's (DC DDA's) health screening component of its Health and Wellness Standards. Findings are presented, along with recommendations and implications for improving preventive health screening practices in the ID population.
Preventive health strategies for adults with intellectual disability (ID) have received increased attention due to the marked inequities they often experience in health and health care access (Haveman et al., 2010), described as “a cascade of disparities” (Krahn, Hammond, & Turner, 2006, p. 72). Accordingly, many communities have undertaken health care and disease prevention efforts directed toward this population. These efforts are in tune with the national public health initiative, Healthy People 2020, which includes the promotion of health and well-being for people with disabilities among its goals. The current study looks at the District of Columbia Developmental Disabilities Administration's (DC DDA), health screening component of its Health and Wellness Standards. Specifically, an investigation of adherence to screening recommendations for people with ID, as well as the barriers and facilitators of community prevention and promotion efforts, was conducted with the goal that study findings would advise future policy development surrounding appropriate preventive health measures and adherence protocols for adults with ID.
Life expectancy of people with ID has increased, so that it now approximates the general population, except for those with multiple and severe disabilities or Down syndrome (Coppus, 2013; Merrick, Kandel, & Morad, 2003). However, compared to the general population, people with ID experience a higher risk of psychiatric and behavioral disorders (Cooper, Smiley, Morrison, Williamson, & Allan, 2007; Melville et al., 2008; Smiley et al., 2007) and poorer overall health (Beasley, Klein, & Weigle, 2016; Draheim, Williams, & McCubbin, 2002; Krahn et al., 2006; van Schrojenstein Lantman-de Valk, Metsemakers, Haveman, & Crebolder, 2000). They have higher rates of serious health conditions including cardiovascular disease, obesity, diabetes, and gastrointestinal disorders (Draheim et al., 2002; Evenhuis, Henderson, Beange, Lennox, & Chicoine, 2001; Janicki, Dalton, Henderson, & Davidson, 1999; Stancliffe et al., 2011); much higher rates of exposure to trauma (Spectrum Institute Disability and Abuse Project, 2013); and vision, dental, and hearing problems (Wilkinson, Culpepper, & Cerreto, 2007). The overall incidence of cancer is similar to the general population with a few exceptions. Reviews have cited reduced risk for cancers of the prostate, urinary tract and lung, but increased rates of cancer of the thyroid and gall bladder (Patja, 2001; Sullivan, Hussain, Threlfall, & Bittles, 2004). The variability of risk is closely linked to lifestyle, underscoring the importance of gathering an accurate medical history. People with Down syndrome present a complex picture of cancer incidence. Although there is a reported 20-fold increase in the incidence of leukeumias, there is a generally lower incidence of most solid tumors with the exception of lymphomas, gonadal and extragonadal germ cell tumors, and possible retinoblastomas and pancreatic bone tumors (Hill et al., 2003; Satge et al., 1998). Early studies conducted on institutionalized populations of people with Down syndrome concluded that risks of atherosclerotic disease were extremely low (Yia-Herttula et al., 1989) and more studies continue to affirm that finding even in people who live in the community (Tansley, Holmes, Lutjohann, Head, & Wellington, 2012). However, clinicians at the Children's Hospital of Philadelphia's Down syndrome and Lipid Heart Clinic continue to research the impact of issues related to cardiac surgery on coronary blood flow, familial hypercholesterolemia and the role of oxidative stress (Children's Hospital of Philadelphia newsletter accessed online http://www.chop.edu/news/down-syndrome-and-lipid-heart-clinic-chop#.Vv2PhtIrIdU).
Increased morbidity experienced by adults with ID can be partially attributed to specific health risks related to genetic disorders and associated biological vulnerabilities (Evenhius et al., 2001). However, there has been an increased focus on secondary causes of increased morbidity. These include higher rates of obesity, adverse effects associated with heavy exposure to psychotropic medications, limited access to health promotion, and sedentary lifestyle (Beasley et al., 2015; Draheim et al., 2002; Rubin, Rimmer, Chicoine, Braddock, & McGuire, 1998; Stancliffe et al., 2011).
These findings suggest a critical need for early identification and management of health conditions for this population. Despite the demonstrated gaps in health access and health outcomes, health prevention and promotion strategies are also lacking (Fisher, 2004; Lewis, Lewis, Leake, King, & Lindemann, 2002; Ouellette-Kuntz, 2005). In their study of health disparities with regard to determinants of health, Krahn et al. (2006) reported that people with ID receive fewer preventive health care services and have poorer access to health promotion practices than the general population. Relatively simple interventions, however, such as use of a health-screening tool, can substantially increase disease detection and clinical activities directed towards improved health outcomes for people with ID (Lennox et al., 2011).
As life expectancy of the ID population has increased in the past two decades, a new population of aging adults has emerged with complex health needs. Literature on the aging ID population consistently reports particularly high rates of sensory impairments, obesity, epilepsy, skin disorders, oral health conditions, behavioral and mental health problems, and gastrointestinal problems (Perkins & Moran, 2010; Reiss, 1990). Health conditions related to aging potentially compound any preexisting health issues and increase the risk of diminished function and well-being.
Increased morbidity poses new challenges in geriatric healthcare planning for adults with ID (Carmeli & Imam, 2014; Fisher, 2004; Perkins & Moran, 2010). This is especially true for public systems that are responsible for overseeing the coordination of health care and prevention efforts for large numbers of aging adults. One such agency, the DC DDA, implemented Health and Wellness Standards to promote positive health outcomes for people with ID and introduced a tool to monitor preventive screenings recommended by the U.S. Preventive Services Task Force (USPSTF). The present study analyzed available data on completed preventive health screenings to assess the initial implementation of these clinical health guidelines.
In 1991, the District of Columbia was the second jurisdiction in the nation to close its institution for people with developmental disabilities, Forest Haven, and move its residents into community-based settings (The Forum for State Health Policy Leadership, 2000). This was one outcome of a 1976 lawsuit, filed by residents of Forest Haven, against the predecessor of the DC DDA, calling for improved services and supports for people with developmental disabilities. As part of subsequent court orders under Evans v. Bowser (2015), as it is now known, the DC DDA assumed responsibility for overseeing the coordination of all services and supports for individuals with ID in the District of Columbia and monitoring for all Evans class members (cohort of individuals previously residing in Forest Haven).
Unlike some other jurisdictions, the DC DDA serves only adults with intellectual disability (not individuals with other disabilities who are not cognitively disabled). The DC DDA provides services for all adults with ID who are identified and who meet eligibility requirements in the District of Columbia, numbering approximately 2,200 people. Participants were drawn from a convenience sample of Evans class members only, due to the DC DDA's desire to evaluate the agency's health and wellness policies and a requirement to report to the Evans court.
Evans class members who were eligible for this study included those receiving services from community-based agencies contracted by the DC DDA. Those eligible included individuals currently residing in Intermediate Care Facilities for Persons with Intellectual Disabilities (ICFs/IDs) of six or fewer people and those enrolled in a Home and Community-Based Waiver receiving residential habilitation, supported living, or host home services. Evans class members residing in their family homes were excluded, as the Health Form 1 Assessment is not a requirement. As of January 17, 2012, a total of 525 Evans class members were receiving services outside their family home (Department on Disability Services [DDS], 2012). The majority of Evans class members are characterized by profound to severe levels of impairment (62%). Thirty-seven percent are described as having mild to moderate levels of impairment. Evans class members are also predominantly African American (80%).
Among all Evans class members living outside of their natural homes, a total of 511 eligible participants were identified. Of those, 63.6% were in Independent Living or received Host Home Services, Residential Habilitation Services, or Supported Living Services. The remaining 36.4% received services in Intermediate Care Facilities for Persons with Intellectual Disabilities (ICF/IDs). Over the course of data collection, one Evans class member, who was ventilator-dependent and in a long-term nursing home (which does not require use of the Health Form 1 Assessment) and nine others had to be dropped from the study because they had been transferred, discharged, or had died within the study period. The Health Form 1 Assessment was collected for 444 of the remaining 501 eligible Evans class members, yielding a response rate of 88.6%.
The DC DDA Health and Wellness Standards (2013) laid out guidelines regarding health services and supports. Three areas of emphasis are (1) preventive health, (2) continual assessment for changes in health, and (3) care coordination. A resulting document, the Health Form 1 Assessment, adapted from earlier work in Massachusetts, specifies the DC DDA's requirements for preventative health screening by age and gender. The specific requirements are those that had been recommended by the U.S. Preventive Services Task Force (USPSTF) Guidelines, which is recognized as the standard resource for evidence-based preventative screening guidelines (Wilkinson et al., 2007).
The Health Form 1 Assessment specifies surveillance recommended for several conditions, including various cancers, infectious diseases, sensory issues, and mental health problems. Gender and age is provided on the Health Form 1 Assessment, but further demographic information is not captured. The completed forms, filled out by a registered nurse from the person's service agency, were reviewed by the research team in order to determine whether health screens are being performed according to the recommended guidelines set up by the USPSTF. Requirements of the DC DDA's Health Form 1 Assessment include electronic or written documentation by a registered nurse of all health screenings performed, as well as any screenings that are deferred based on the individual's needs and primary care provider's recommendations. The Health Form 1 Assessment is kept in each person's record and is updated on an ongoing basis.
Data Collection and Processing
The Health Form 1 Assessment was collected by research staff from the community service agency staff on a data disc or flash drive, or electronically through an encrypted zip file. Efforts to increase overall response rate included community education of service providers about the study, repeated follow-up phone calls, and e-mails with agency representatives. Confidentiality and anonymity of study participants were maintained at the data entry stage. Based on USPSTF guidelines, screening activities on the Health Form 1 Assessment were coded and condensed into three categories according to the criteria outlined in Table 1.
The statistical software program IBM SPSS Statistics 21 was used to perform all quantitative analyses. Simple descriptive statistics were calculated for demographic variables and for each activity on the Health Form 1 Assessment. The Mann-Whitney U Test was used to determine gender differences in rates of preventive screenings in our sample. Nonparametric one-sample binomial tests were used to examine differences between our sample population and the general population. Efforts were made to match the age ranges in our study sample to the age ranges used in the calculation of the general population estimates. Screens that were N/A or Deferred due to the patient's age or sex were excluded from the analysis.
Evans class members in the sample were predominantly male (63.5%). The age distribution of the sample ranged from 42 to 90 years with a median age of 59 years, (see Table 2). Just 7.2% of individuals were under 50 years of age whereas 9.2% were 70 years or older.
Screening Rates by Disease Category
Figure 1 summarizes the percentage of individuals for whom applicable prevention screenings were received within the recommended time frame. The calculated frequencies exclude persons in the sample for whom select screenings were not applicable due to their age or sex. Screens performed most often at recommended frequency were cholesterol screening, prostate screening in men, mammography in women, and hypertension screening. Among the least frequent health prevention activities were chlamydia and STD testing, HIV testing, skin cancer screening, and dementia screening, respectively. Depression screening was performed at a comparatively low rate.
The USPSTF recommendation for colorectal cancer screening can include a colonoscopy, sigmoidoscopy, or fecal occult blood testing, or a combination of the three. The individually reported percentages of persons receiving a sigmoidoscopy (26%) or fecal occult blood test (36%) were comparatively low; however, a high percentage of participants (87.6%, n = 389) received at least one of the three recommended colorectal cancer screenings. Furthermore, 8.3% (n = 37) of Evans class members received all three colorectal cancer screenings, whereas 12.4% (n = 55) received none.
Gender Variations in the Receipt of Recommended Health Screenings
Men and women did not differ significantly in the receipt of most health screenings intended for their gender. Women received some recommended screenings at significantly higher rates compared to men, specifically for osteoporosis (75% vs. 58%), glaucoma (91% vs. 85%), and dementia (62% vs. 50%). However, men received Hepatitis B and C screenings more frequently than their female counterparts (74% vs. 61%). For both men and women, the health screenings received at the highest level of adherence were cholesterol screening, colonoscopy, hypertension, prostate cancer (men), mammography (women), and TB screening. Screenings that were performed least frequently included chlamydia and STDs, HIV, and skin cancer screening for both men and women.
Missed screenings were examined for each health category. These screenings included those recorded as N/A or Deferred (not age-appropriate or gender-appropriate, not recommended by the PCP, not indicated, or not ordered), refused, or scheduled. Skin cancer screening was deferred most frequently (14.9%) followed by HIV testing (5.0%). Type II diabetes screening, tuberculosis screening, hypertension, and vision assessment were never deferred by the primary care physician. The health screens most often recorded as not applicable were Chlamydia and STD screening (14.2%), followed by HIV testing (13.1%), and pap smear (6.5%). Although recorded refusal rates were low, osteoporosis (2.0%) and glaucoma (1.0%) screenings were most frequently refused.
Comparison to the General Population
Table 3 summarizes screening frequencies in our study population compared to general population norms derived from the following sources: National Health Interview Survey (NHIS); Centers for Disease Control and Prevention, National Center for Health Statistics (CDC/NCHS); National Survey of Family Growth (NSFG); and the National Health and Nutrition Examination Survey (NHANES). Evans Class members in our study sample, age-matched to general population estimates for each screening category, were significantly more likely to receive mammograms and screening for colorectal cancer, hypertension, cholesterol, vision, and hearing compared to the general population. Rates were greater compared to those in the general population for pap smears, but differences did not reach the level of significance. Rates significantly surpassed the screening goals established by Healthy People 2020 for mammography, and surveillance for colorectal cancer, cholesterol, hearing (in older adults) and vision. Screening rates for hypertension and hearing also exceeded Healthy People 2020 goals, but not to the level of significance.
Overall findings show that with clear guidelines and a fairly simple tracking process, it is possible to assure that individuals with ID in public care receive most recommended preventive health screens, and at higher rates than the general population. Whereas previous studies used Medicaid claims data to assess preventive health services received, this study analyzed data from individual health records, yielding more accurate estimates.
In this sample, screening rates were significantly higher than estimates for the general population in six out of seven categories, including mammograms, colorectal cancer screenings, hypertension, cholesterol, hearing assessment (adults and older adults), and vision assessment. They were also higher than the Healthy People 2020 target goals in six areas. High rates of hypertension, cholesterol, and diabetes screening in our sample were particularly noteworthy in light of the predominantly African American sample. Untreated hypertension, which can lead to increased risk of stroke, kidney disease, and diabetes, is a major health concern experienced by African Americans. A systemic review of recent literature on racial and ethnic differences in cardiovascular risk factors found higher rates of hypertension and diabetes in African Americans than in Caucasians (Kurian & Cardarelli, 2007). Another review conducted by Haveman et al. (2010) found that the prevalence of hypertension increases with age, making the high rates of screening found in our study all the more positive in the aging study cohort.
There are several limitations in reviewing data from the Health Form 1 Assessment to estimate the rate of preventive health screenings. It is important to note these limitations and interpret the results accordingly. Our study sample consists of a particular subset of the ID population and is not representative of all individuals under DC DDA's purview. Evans class members are also under court monitoring, which may have led to an overestimation of screening frequencies found in our study.
Although we used guidelines current at the time of the study, the USPSTF has since updated their recommendations for appropriate health screenings. Analyses of the Health Form 1 Assessments are based on the recommended guidelines in place at the start of the study and may not reflect these recent changes. Although every attempt was made to match parameters used to estimate screening rates in the study sample and the general population, for example, matching ages for each comparison, discrepancies may remain that limit the capacity to make reliable comparisons.
Information extracted from the Health Form 1 Assessment was used to determine individuals for whom prevention screenings were appropriate based on age, gender, and health care provider notes. Therefore, individuals for whom a screening was indicated as not applicable or deferred due to age or gender on the form were excluded in estimations of screening rates within each health screening category. Screening rates presented in the results section may underestimate or overestimate the true rates of screening in the Evans class population, as the form is used as a guide for practitioners and monitors, rather than a systematic tool. It is also possible that not all information was accurately captured on the form due to recording or transferal errors and missing information. For example, screening rates for mammograms may have overestimated the true rate in the study population as sonograms may have been performed if the woman was not able to tolerate the mammography.
Despite these limitations, this study offers important insight into preventive health services for adults with ID, education and training, and further study.
Changes to improve screening and diagnosis
As a result of this study, the Health Form 1 Assessment was immediately revised to address inconsistencies in the collection of data and incomplete information. Significant changes were made to elicit more accurate reporting of health screenings by the primary care provider and the registered nurse and to reduce the number of reporting and transferal errors. These reporting changes included being prompted, in the electronic version of the form, to enter the date of the screen. Updates to the form were also made to reflect the most current USPSTF guidelines by age and gender. For example, the USPSTF no longer recommends skin cancer screening for the general population, so this health screening was removed from the Health Form 1 Assessment.
Because depression screening in our sample was among the less frequently performed, DC DDA made adaptations to the Glasgow Depression Scale for screening of individuals who are nonverbal. This revised depression scale was distributed to registered nurses and made available on a web page. The link to this document was also included as part of the revision to the Health Form 1 Assessment. Depression screening was determined to be an important activity as evidenced by the greater rates of co-occurring mental illness among people with ID compared to the general population. Prevalence studies estimate between 15% and 50% of adults with ID have a co-occurring mental illness (Clay & Thomas, 2005; Deb, Thomas, & Bright, 2001; Gustafsson & Sonnander, 2004) occurring at rates two to three times higher than their non-disabled peers (Cooper et al., 2007; Krahn et al., 2006). The presence of mental illness is linked to greater risk for a number of health issues, such as cardiovascular disease, diabetes, and obesity, making depression screening a priority.
In response to the low screening rates for dementia found in this study, DDA in collaboration with Georgetown University faculty launched a Dementia Capable Care Initiative. Its focus is on early diagnosis and differential diagnosis to rule out other, perhaps treatable, conditions. Adults with Down syndrome are more likely to experience cognitive decline compared to the general population. Strydom, Hassiotis, King, & Livingston (2009) found dementia was two to three times more prevalent in the ID population compared to peers without disability, and this rate was not influenced by level of ID severity. For the general population, age is among the risk factors for the development of dementia (Chen, Lin, & Chen, 2009), so as the population of adults with ID achieve the same life expectancy as nondisabled peers, the number of adults with ID (without Down syndrome) who will be affected by dementia is likely to increase. This strongly supports continued dementia screening and early identification in this population.
Recommendations for training and education
Although this study indicates that a simple system that prompts health providers about the specific health screenings are important for the general population and for people with ID, more will be needed to impact the long-standing health disparities experienced by this population. Ongoing training will be needed for staff within the disability system that improves communication with primary care providers and educates support staff about the importance of health screening and accurate data collection. In addition, it will be important to focus on strategies that engage and support people with ID to improve their health literacy so that they understand the importance of finding a medical home and to understand why preventive health screening is important for them. Unlike in the District of Columbia where there is a high level of involvement by licensed clinicians, in other locations non-clinicians may be responsible for tracking health data. This makes it all the more important to ensure that systematic expectations and procedures are in place, supported by adequate training.
Biases of health care personnel also need to be addressed through ongoing training. Bias may have influenced screening rates found in our study. For example, some community support personnel may not consider that the adults in their care may be sexually active, which has implications for the STD and HIV screenings. Previous studies have found that people with intellectual disability with moderate to severe function engage in less sexual activity compared to their peers with other types of disability or without disability (Servais, 2006). Despite the probable lower rate of sexual activity among this older and more disabled study population compared to the general public, some people who were sexually active may have been overlooked. Additionally, there is increased concern about exposure to other risk factors, including potential for sexual abuse. Given the limited sexual health education afforded people with cognitive disability, and the history of negative attitudes and discrimination regarding their sexuality, it is important to provide instruction for medical and program personnel around sexual health and screenings.
Our study raised important ethical concerns related to self-determination and the decision to participate in medical procedures. Physicians provided limited details when screenings were refused by participants. More information surrounding the circumstances of refused screens is needed, but it can be hypothesized that refusals were recorded when patients exhibited challenging behaviors as a test commenced or when a self-determined judgment was made by the individual. Patient education is critical if people are to make informed decisions about their preventive health care. People with ID should be taught about screening preparations, procedures, and guidelines so that they can become as knowledgeable as possible about tests to which they are consenting. The health care provider has an important role in engaging and supporting people throughout the decision-making and screening process (Stancliffe, 2001; Wong & Wong, 2007). Training is needed for disability service agency staff and people with ID to foster a person-centered approach that promotes enhanced self-determination while providing adequate supports for decision-making when the person with ID requires additional support and advocacy.
Our study suggests robust primary care that includes coordinated input from the person and members of their circle of support, which is essential in order to make thoughtful decisions about the need for preventive screening. This is especially important for more intrusive medical tests such as a colonoscopy. Additionally, health screens lacking robust evidence on the benefits to the individual, as evaluated by the USPSTF, call for the discretion of the health care provider. For example, USPSTF recommends hepatitis B screening for populations considered to be at increased risk compared to the general population. Although the study population currently resides in community-based agencies, all were previously institutionalized; therefore they are at greater risk for infectious diseases including hepatitis B hepatitis C and tuberculosis. The USPSTF has not established screening intervals for hepatitis B virus due to insufficient evidence, so the responsibility to make a clinical determination of screening frequency falls upon the health care provider.
Future investigation should focus not only on early detection of conditions but also the actions taken as a result of the findings. Our study focused on a convenience sample consisting of Evans class members only, but further investigation of populations more representative of the entire ID population would be important, including adults living in their natural home. Most people with ID live with their families and this number is expected to continue to grow.
This study demonstrates that robust rates of preventive health screenings can be achieved in public systems for adults with ID. This achievement requires clear policy and organized efforts to keep a focus on prevention at the core of health services and supports. People with ID deserve to receive a high standard of health care including support to understand screenings, information about the benefits of changes in lifestyle practices, and support to carry them out.
This article was supported by Grant DCJM-2013-C-0001 from the District of Columbia Department on Disability Services. Approval for this study was granted by the Georgetown University Institutional Review Board (2013-0709) and the DC DDA Human Rights Committee.
The authors would like to acknowledge the unwavering support of Laura Nuss, the former Director of the DC Department on Disability Services. Her dedication to systems change on health and other issues important to the lives of people with intellectual disability made our work possible.