Why do we need national guidelines for adults with intellectual disability and dementia?

Abstract

The World Health Organization's (WHO) report, Dementia: A Public Health Priority [1] drew attention to the growing impact that Alzheimer's disease and related dementias are having on the world's older population. The report noted the need for more effective early detection and screening, diagnostic and medical treatment services, and innovative models of family supports and social care. It also identified “populations with specific needs,” including adults with intellectual disability (ID)3 (and in particular adults with Down syndrome—who “are at a significant risk of developing Alzheimer's disease”) as needing special attention. Others [4], [5] have noted that such adults were at greater risk and had a differing course of dementia, often with earlier onset and shorter duration, and that they posed a diagnostic challenge for many practitioners. Also, the Edinburgh Working Group on Dementia Care Practices [6], an ad hoc international body, proposed that given these factors, service providers and national Alzheimer's organizations provide equity in services at an earlier age to people with ID. The WHO report noted that dementia is often underdiagnosed and that this may be the result of three factors: stigma associated with dementia, beliefs that memory problems are a normal part of aging, and the belief that nothing can be done—these factors also all apply to the underdiagnosis of dementia among adults with ID. The report proposed that to circumvent these factors, detection and diagnosis require coordination among health and social care systems and better attention to symptom presentation among primary and specialist care services. The WHO contended that initial identification is an important function not only of primary care, but also of the social care community which often has the best opportunity to have an impact when noticing behavioral and functional change. With respect to early identification, the WHO proposed that guidelines for practitioners and others involved with day-to-day dementia care “should include guidance on clinical aspects such as diagnosis, assessment and treatment, as well as quality long-term care” (p.62) [1]. Why is there a need for special guidelines for adults with ID? Individuals with ID are living longer including those with Down syndrome, and the risk of manifesting Alzheimer's disease and related dementias is increasing. Most families, community support organizations, and health care practitioners are not familiar with how age-related decline may typically manifest, including pathologic decline from underlying disease. A change or decline in function may be falsely blamed on the underlying ID (i.e., diagnostic overshadowing) and many adults with ID may go undiagnosed or misdiagnosed. These uncertainties and lack of awareness often can create a delay in recognition that dementia is present, and therefore, appropriate evaluation, treatment, and supports may not be provided. Furthermore, generally accepted measures and procedures for diagnosis often are not appropriate for adults with ID because of their variable functional and intellectual premorbid state. Guidelines offering standardized procedures for screening, assessment, and diagnosis would mitigate many problems associated with accurate ascertainment of the presence of dementia. Furthermore, guidelines for the care and support of adults with ID affected by dementia would go far to improve this growing personal, social, and health problem. With this in mind, the Edinburgh Working Group had noted that “in many jurisdictions, practices have been inconsistent with regard to how to provide services and supports to people with [intellectual disability] affected by dementia” and that “providers have not defined workable responses to the increasing presentation of dementia among people with [intellectual disability]” (p. 280) [6]. The Edinburgh Working Group further proposed that “prevailing practices and policies of service provision need to be examined with an eye to adopting universally applicable guidelines which promote continued community care and support of people with [intellectual disability] who are affected by dementia” (p. 280).