Consensus Report Review of Scientific Evidence Addressing Prevalence, Documented Needs, and Interdisciplinary Research: Persons in Early Stage Alzheimer’s Dementia Including Recommendations from the Panel of Experts Submitted to: National Alzheimer’s Association Chicago, Illinois January 30, 2007 1 Table of Contents Review of Scientific Literature Addressing Persons in Early-Stage Dementia Consensus Report of the Expert Panel Topic/Section: Page(s): A. Estimates of early-stage AD and future predictions 3-4 B. Documented need for programs, education, and support 5-21 C. Review of interdisciplinary research Introduction and definitions 22-31 1. Delirium as a future focus area 32-36 2. Early stage support groups 37-44 3. Cognitive training and enhancement programs 45-64 4. Exercise programs 65-73 5. Exemplar programs 74-83 6. Health promotion programs i. Sleep disorders management 84-88 ii. Nutritional support 89-93 iii. Falls prevention 94-96 iv. College courses and recommendations 97 v. References 98-100 7. Other domains i. Driving safety 101-107 ii. Volunteer programs 108-109 iii. Writing programs 110-111 iv. Technology support programs 112-114 v. Art and creative activities 115-116 vi. Dance therapies 117-118 vii. Neural stimulation 119-120 viii. Other Interventions 121-122 ix. References 123-126 D. Summary: Table 1 127-129 E. Consensus Statement from Panel of Experts 130-133 F. Sample Protocols: 1. Mild Cognitive Impairment 134 2. Early Stage AD 135 G. Panel of Experts 136-140 2 A. Estimates of early-stage AD and future predictions Approximately 4.5 million people have Alzheimer’s disease (AD) in the United States. It is estimated that by the year 2030, the rate will be 7.7 million, with an anticipated 13.2 million by 2050 (Hebert, et al., 2003). Overall prevalence of AD appears to double for every 5-year age group beyond age 65 (NIA, 2005). The prevalence of early stage AD is not clear. This relates, in part, to a historic lack of clarity in the terms, definitions, diagnostic criteria and methods that have been used for distinguishing early stage AD. Mild cognitive impairment (MCI) has been the most commonly used term to define the transition from normal cognition to probable AD. As the body of literature on MCI has developed, the parameters and limits of this diagnosis have become increasingly ambiguous (Winblad, Palmer, Kivipelto, et al., 2004) and considerable methodologic differences in defining MCI have been demonstrated (Luis, Loewenstein, Acevedo, Barker, & Duara, 2003). Methodological variability in epidemiologic studies has made it difficult to determine prevalence, incidence and conversion rates to dementia of persons diagnosed with cognitive impairments (Luis, et al, 2003). In the last few years, considerable effort has been made to study and identify specific subtypes that will better distinguish and more accurately diagnosis MCI linked to early AD. Overall prevalence of MCI in population-based epidemiological studies has ranged from 3% to 19% in those 65 and older (Ritchie, 2004). More than half of these cases progress to dementia within a five year period. The Amnestic subtype of mild cognitive impairment (MCI with memory problems) has consistently demonstrated a high risk of progression to dementia, particularly dementia of the Alzheimer type (Gauthier, Reisberg, Zaudig, et al., 2006). Some investigators consider MCI to be an early stage of AD (Morris, Storandt, Miller, et al., 2001), contending that in patients with amnestic MCI, followed longitudinally, the early changes of AD are present (Markesbery, Schmitt, Kryscio, Davis, Smith, & Wekstein, 2006). A revision of current diagnostic criteria is being suggested by some investigators to specifically diagnose early AD, including those individuals considered mildly cognitively impaired for whom the cognitive impairment represents the earliest clinical symptoms of AD (amnestic subtype of MCI) (Morris, 2006). There are investigators who believe that a revision of diagnostic criteria would permit the diagnosis of and thereby facilitate earlier intervention for early stage AD (Markesbery, et al, 2006; Morris). Individuals most at risk for developing AD are those 85 and older. Relating to substantial decreases in mortality among the older population, we are seeing more people survive to ages where AD is more common as well as more people surviving after they are diagnosed with AD (NIA, 2005). Familial history is linked to the more uncommon early onset AD, developing in persons 30-60 years of age. For AD occurring in those over age 60 (the more common type), no familial link is evident (NIA, 2006). 3 References Gauthier, S., Reisberg, B., Zaudig, M., Petersen, R., Ritchie, K., Broich, K., Belleville, S., Brodaty, H., Bennett, D., Chertkow, H., Cummings, J., de Leon, M., Feldman, H., Ganguli, M., Hampel, H., Scheltens, P., Tierney, M., Whitehouse, P., & Winblad, B. (2006). Seminar: mild cognitve impairment. Lancet, 367, 1262–70. Hebert, L.E., Scherr, P.A., Bienjas, J.L., Bennett, D.A., & Evans, D.A. (2003). Alzheimer Disease in the US population: prevalence estimates using the 2000 census. Archives of Neurology, 60(8), 1119-1122. Luis, C., Loewenstein, D., Acevedo, A., Barker, W., & Duara, R. (2003). Mild cognitive impairment: directions for future research. Neurology, 61. 438-444. Markesbery, W., Schmitt, F., Kryscio, R., Davis, D., Smith, C., & Wekstein, D. (2006). Neuropathologic substrate of mild cognitive impairment. Archives of Neurology, 63,38-46. Morris, J. (2006). Editorial: Mild cognitive impairment is early-stage Alzheimer disease time to revise diagnostic criteria. Archives of Neurology, 63, 15-16. Morris, J., Storandt, M., Miller, J., McKeel, D., Price, J., Rubin, E. & Berg, L. (2001). Mild cognitive impairment represents early-stage Alzheimer disease. Archives of Neurology, 58, 397-405. National Institutes on Aging. (2005). Progress report on Alzheimer’s Disease 2004-2005, new discoveries, new insights. Retrieved December 19, 2006, from: http://www.nia.nih.gov/Alzheimers/Publications/ADProgress2004_2005/ National Institutes on Aging. (2006). Alzheimer’s fact sheet. Retrieved December 19, 2006, from: http://www.nia.nih.gov/Alzheimers/Publications/adfact.htm. Ritchie, (2004). Mild cognitive impairment: an epidemiological perspective. Dialogues in Clinical Neuroscience, 6, 401–08. Winblad, B., Palmer, K., Kivipelto, M., Jelic, V., Fratiglion, L., Wahlund, L., Nordberg, A., Backman, L., Albert, M., Almkvist, O., Arai, H., Basun, H., Blennow, K., DeLeon, M., Decarli, C., Erkinjuntti, T., Glacobini, E., Graff, C., Hardy, J., Jack, C., Jorm, A., Ritchie, K., VanDuijn, C., Visser, P., & Petersen, R., (2004). Mild cognitive impairment – beyond controversies, towards a consensus: report of the International Working Group on Mild Cognitive Impairment. Journal of Internal Medicine, 256, 240–246. 4 B. Documented Needs of Persons with Early Stage AD Search Method The literature search took place between November 11, 2006 and January 26, 2007. Databases used in the search include PubMed, CINAHL, PsychInfo, Web of Science, SportDISCUS, and the Cochrane Database of Systematic Reviews and included studies back to 1995 unless there were articles of an earlier date that provided insights with no more recent studies. The following terms were used initially: dementia; early stage; Alzheimer’s Disease; dementia, multi-infarct; dementia, senile; limited to research. Mesh terms were cross referenced such as early stage dementia and needs, early stage dementia and interventions, MH program development, MH community programs, MH program evaluation, MH program implementation, MH program planning, MH program instruction and MH psychotherapy. Studies which looked primarily at caregivers were excluded from this search. When a citation was found, links to related studies from a study to be included in the review were also explored. Further searches were done on the names of authors in research articles found in the initial search as well as on the names of their projects. In addition searches were done on noted experts in the field. Professionals working in the field contributed lists of references on early stage Alzheimer’s disease interventions as well. Once articles were included in this search, further references were found using the ancestry method. Our initial search results produced over 500 articles, 334 were selected for review, and 54 were determined to fit inclusion criteria. Inclusion Criteria All research articles addressing need for persons with early stage dementia in someway were included. Since many studies defined their subject group as Mild Cognitive Impairment (MCI), yet upon review their inclusion criteria were the same as or overlapped the inclusion criteria this group used for early stage AD, these studies were included. In this literature review, particularly when using the search term early stage, it became evident that there are few studies that focus exclusively on early stage. More often studies report a diagnostic parameter such as the MMSE or CDR, or combination of tools in their data, but often don’t consider it as a variable in analysis, thus making stage specific interventions and needs difficult to ferret out. Further, frequently studies report the mean MMSE with standard deviation which demonstrates a range that goes from early stage to moderate stage, blending results as dementia as a whole and not specific to stage. Similar results are reported when the CDR is used. It is our feeling that to ignore this body of research would exclude many articles that have information relative to early stage dementia. Identification of needs for persons with AD (PwAD) in early stages was often implicit in the studies, and we chose to include that information in this review. There is a need to determine whether or not MCI is an accepted diagnostic category or if those persons should be included in early stage discussions, since many of those “MCI” people will eventually be diagnosed with dementia. Awareness in Persons with Early Stage Dementia 5 Although not directly related to a need, the growing body of evidence concerning retained awareness in persons with early stage AD serves as background for the identification of evidenced-based needs. For several decades, it has been assumed that persons with AD, even in the early disease stages, were unaware of their disease, the implications of the disease for their lives, and disease-related losses. However, a growing body of evidence supports the existence of awareness in persons with AD. Awareness is relevant in the identification of needs, as needs associated with retained awareness may be appropriately identified. With retained awareness, a broader range of needs are evident such as needs related to protection of self-esteem, managing responses to the disease, personal control, education, and support. Retained awareness allows persons with AD the opportunity to share their perspective and, in some ways, to help determine their own quality of life (Burgener, Twigg & Popovich, 2005; evidence grade: C1; Clare, Markova, Merhey, & Kenny, 2005; evidence grade: A1). Clare and Wilson (2006; evidence grade: C1) have found that awareness of persons with AD persists long into the dementia process with no association between discrepancy scores (measure of awareness) and MMSE scores over time. Knowledge regarding retained awareness in persons with AD impacts on program design and content, including the target of support programs. The caregiver has traditionally been the identified target for education, support, and intervention trials, based on the longstanding belief that the onset and progression of dementia is accompanied by disintegration of awareness. Although a study by Onor and colleagues (2006; evidence grade: C1) supports some decline in awareness, awareness can still be objectively assessed. Further, studies have indicated that caregiver assessments varied from the person with AD, even regarding awareness of caregiver emotions (Derouesne, 1999; evidence grade: C1; Starkstein, 1997; evidence grade: C1; Vasterling, 1997; evidence grade: C1). The perpetuation of awareness has implications for program development, content, and assessment of outcomes. This recent evidence supports that programs and assessments that rely on caregiver learning and evaluation are limited and may not adequately represent the person with AD. Many programs and studies preclude participation if a person with AD does not have a reliable informant, a practice that is no longer supported. Further support for awareness of persons with AD comes from research targeted at the transition to AD. In a review of studies, Jonker and colleagues (2000; evidence grade: A1) evaluated the predictive value of memory complaints, indicating self-awareness of memory loss. The reviewers found that subjective memory is predictive of cognitive decline and dementia, particularly in individuals who already have some cognitive decline. Findings indicate the greater the subjective memory, the slower the decline and disease progression. These findings are supported by a more recent study by Jorm and colleagues (2005; evidence grade: C1). Higher levels of awareness are also related to better outcomes in cognitive rehabilitation interventions (Clare, Wilson, Carter, Roth, & Hodges, 2004; evidence grade: C1). Need for Early Diagnosis The implications for need for early diagnosis are multifold. The earlier dementia is differentially diagnosed, the earlier both pharmacologic and non-pharmacological interventions are possible. 6 It is important to track changes over time with sensitive tools so that interventions may be targeted at regaining or compensating for failing skills or to minimize losses (Farias, Mungas, Reed, Harvey, Cahn-Weiner, & DeCarli, 2006; evidence grade: C1; Salmon & Lane, 2001; evidence grade: A1). Because of the growing emphasis on early detection and intervention in dementia care, the need for a clear evidence base for cognitive training and cognitive rehabilitation is increasingly needed (Woods, Moniz-Cook, Orrell, & Spector, 2006, Cochrane report; evidence grade: A1). Not only is there a need for early diagnosis, but the need for a more accurate diagnosis has been identified. Several studies have found that there are more than just cognitive changes that are evident in early stage, supporting the need to assess a variety of cognitive and functional domains (Artero & Ritchie, 2003; evidence grade: C1; Backman, Jones, Berger, Laukka, & Small, 2004; evidence grade: A1; Richards, Moniz-Cook, Duggan, Carr, & Wang, 2003; Evidence grade C1; Salmon & Lange, 2001; evidence grade: A1). An early, correct diagnosis and treatment may also have a positive impact on the caregiver. Early interventions often include education and caregiver (CG) training. These types of programs are associated with reduction of CG burden and stressors as well as better outcomes for the person with AD. Both outcomes have been shown to decrease premature institutionalization (Doody, et al, 2001; evidence grade: B1; Farina, et al, 2006; evidence grade: C1; Gaugler, Kane, Kane, Newcomer, 2005; evidence grade: C1: Geldmacher, 2004; evidence grade: D; Zarit, Femia, Watson, & Rice-Oschger, 2004; evidence grade: C1). Changes that progress beyond what is considered normal aging include not only memory impairment, particularly episodic memory, but research points to the occurrence of neuropsychological behavioral symptoms (NPBD) as mild cognitive impairments begin. The study by Lyketsos and colleagues (Lyketsos, Lopez, Jones, Breitner, DeKosky, 2002; evidence grade: C1) demonstrated that with the onset of dementia symptoms, more than 80% of persons with dementia or their caregivers report one or more NPBD symptoms, with about 50% of those with mild cognitive impairments. Along with the occurrence of these symptoms, there is greater report of caregiver depression. As there is more ADL dependence upon caregivers, caregiver burden also increases (Geldmacher, 2004; evidence grade: D; Lyketsos, et al, 2002; evidence grade: C1; Knopman, Boeve, & Peterson, 2003; evidence grade C1). This is further supported by the work of Richards and colleagues (2003; evidence grade: C1) who demonstrated that measures of psychosocial distress were more meaningful than measures of cognition alone when evaluating outcomes for community dwelling persons with dementia. Farias and colleagues (2006; evidence grade: C1) conducted a study of the everyday functioning of three groups; a normal group, a group with MCI, and a group with AD. They found that while there was a change between groups in each variable measured, the changes were significant in the MCI group compared to the normal group. Of primary importance, this study adds to the body of knowledge that indicates a need for early diagnosis and that persons with mild cognitive impairments have sufficient functional impairments as to benefit from interventions. Even when people don’t demonstrate adequate symptoms to meet DSM IV criteria, impairments impact their lives in important areas such as safety, work related functioning, and planning issues. Their functional deficits are significantly worse than unimpaired individuals (Knopman, Boeve, & Peterson, 2003; evidence grade C1). Researchers concur that impairments exhibited early in the 7 disease course affect more than memory. Other abilities affected may include: writing checks; paying bills; balancing the checkbook; assembling tax records; managing business affairs or papers; shopping alone for personal items, household goods, or groceries; heating water; working at the stove; preparing a balanced meal; remembering appointments, family events, or holidays; adhering to medication schedules; and/or traveling out of the neighborhood and driving. The findings of these and other studies point to the needs of persons with MCI and early stage dementia related to memory, planning and language. Many of the tasks identified could be addressed through adaptive interventions. Program development is indicated for learning compensatory tools for memory and language skills, for example, memory tools such as notes, lists, cueing, calendars, simple directions, task breakdown, assistance with money management, long term planning and health care, work implications, driving implications, and safety implications. Professionals must consider that each person is at a different place in their decline and their abilities, so it takes knowing the person to adequately plan interventions. Group interventions are not always effective because of the participants’ different functional levels. In their evidenced-based review, Doody et al (2001; evidence grade: B1) found support for use of cholinesterase inhibitors early in the disease process. Early and accurate diagnoses may impact positively on appropriate medication prescription and usage. Clinical trials indicate that cholinesterase inhibitors have the potential to impact positively on a variety of outcomes, including cognition, global function, and ADLs. Some studies also indicate there is an effect on neuropsychological and behavior symptoms as well (Doody et al, 2001; evidence grade: B1; Gelmacher, 2004; evidence grade: D). While there is a need for early diagnosis to help persons prepare and maintain abilities for as long as possible, it is also critical that the person in early stage AD does not become ‘medicalized’. This type of medicalization of AD results in the person with dementia becoming lost in the diagnosis and pharmacological treatment. When this occurs, the focus of helping the person with dementia to maintain personhood is lost (Penrod, Yu, Kolanowski, Fick, et al., 2007; evidence grade: D). Penrod and colleagues describe the need for a combination of biophysical and personhood interventions based on lived experiences with dementia to assure quality of life and humane care. Need for Accessible Service and Knowledgeable Providers Currently 25% of elders in the U.S. live in a rural community, either alone or with their spouse. Rural health care services are less accessible, less specialized and more costly to deliver than those in urban areas. The access of rural elders to a range of health care services is narrower than in urban areas, and fewer community-based services are available to them. Lack of community based services has resulted in a higher utilization rate of nursing home services for rural elders (Coward, Netzer & Mullins, 1996; evidence grade: C1). Lack of services also results in a less- than-complete assessment and diagnosis with fewer interventions for problems with cognition. The increasing numbers of older persons well increase the demands for mental health services that are already sorely lacking. This increase in demand has led to projections of severe shortages of specialized, highly educated professionals to meet the increasing mental health needs of the 8 changing population. There is a need to have qualified health care professionals to meet the needs of persons in early stages of dementia. Because most of what is the current practice in dementia care was unknown ten years ago, the supply of qualified health professionals has not kept pace with the demand for specialized assessment and treatment services. Need for Assessment of Individual Losses and Strengths to build Individualized Plans of Care Persons with dementia suffer different losses at different stages of the disease and therefore accurate assessment of abilities and losses is critical to assist the person in planning for their future and care needs. There is a need to tailor programs to retained skills and not assume all persons with dementia are the same over a changing and unpredictable clinical course (Garand, Buckwalter & Hall, 2000; evidence grade: D; Salmon & Lange, 2001; evidence grade: A1). The Mini-Mental State Exam (MMSE) is relatively insensitive to very mild, but significant cognitive decline in highly educated persons, and has floor effects in tracking persons with severe dementia. The MMSE provides a valuable screening tool, but is less useful for tracking progression over time. Neuropsychological assessment provides a reliable means of detecting dementia in its earliest stages and of tracking the progression of cognitive decline (Salmon & Lange, 2001). Assessment of everyday behavior is significant for diagnosing and predicting disease progression in AD. Memory, IADLs, mood and social behavior have a linear deterioration as the disease progresses. However, this is not true for behavioral symptoms, which may fluctuate overtime, and their severity does not systematically increase with disease progression (Blasi, Brubacher, Zehnder, Monsch, Berres, et al ,2001; evidence grade: B1). There is a need for accurate assessment to focus on the information provided about impaired and spared abilities so that the retained abilities can be capitalized upon and impaired abilities supplemented. This is especially important for communication. Prediction with the use of standardized tests can be made about functions that are likely to decline and interventions can be implemented to sustain those functions for as long as possible (Tomoeda, 2001; evidence grade: A1). Individual assessment and individualized plans may also identify coping strategies to enhance well being and self esteem of PwAD in early stages (Clare, 2002). Awareness of persons with AD in early stages has implications for assessment and individualizing care. As noted earlier, Clare and colleagues (2004, 2006) found that awareness persists long into the dementia process. This awareness affects the caregiver/person with dementia dyad in important ways. The perpetuation of awareness has implications for assessment instruments that rely on caregiver report versus self-report, as the caregiver may introduce bias based on many factors. Using caregiver ratings only may not give a clear picture to health care providers and is an important consideration when doing assessments to meet the needs of the person in early stage AD. Obtaining assessments only from caregivers of the needs and strengths of the person with AD increases the likelihood that educational and support programs may fall short of adequately addressing the actual needs of the person with the disease. The need for individualized, person-centered programming may only be met in the perceptions of the person with AD are taken into account and valued. Need for Psychosocial Support: 9 The need to provide early psychosocial support for persons newly diagnosed with dementia and their caregivers (soon after receiving even the tentative diagnosis) has been identified. Early support has been related to better outcomes (Brodaty, Gresham & Luscombe,1997; evidence grade: C1; Moniz-Cook, Gibson, Win, & Wang, 1998; evidence grade: B2). In the study by Monitz-Cook and colleagues, the intervention group received intensive verbal and written information on the diagnosis. An average of four consecutive contacts was used to “break the news” followed by: 1) information on the interactive role of caregiver and person with dementia, 2) counseling regarding feelings of loss associated with the disability and potential progression of the disease with an emphasis on preserved abilities and skills, 3) prevention of crisis, and 4) maintenance of social activity. Persons in the treatment group had more positive outcomes. Persons in the control group without this intense focus on psychosocial support dropped the occasional support they received, had not read the information, did not follow through with the suggested cognitive exercises, and had increased institutionalization rates. The support is a necessary prerequisite to planning and working on improving or retaining memory ability. Need for Information and Knowledge Information must be given in such a way that persons with dementia will use it. When given brochures with encouragement to read, persons with dementia will not read or use the information. It often takes an extended period of time for persons who have received the diagnosis to begin to process and try to understand the implications of the diagnosis, so timing of the information and the way in which it is shared is critical (Moniz-Cook, et al, 1998; evidence grade: B2; Zarit, Femia, Watson, Rice-Oeschger, & Kakos, 2004; evidence grade: C1). Zarit and colleagues (2004) tested the intervention of a 10-session Memory Club program to provide information about memory loss and resources for coping for person in the early stage of dementia and their care partners. The rationale for doing the intervention what that dementia in the early stages presents an opportunity for examining the immediate and long-term consequences of the illness at a point when the individual with memory loss can participate. In addition to providing information, the group’s purpose was to strengthen both the person with dementia and their care partner’s ability to cope and to support one another. A preliminary evaluation of the project indicated positive response from both members of the dyad. While the primary purpose of the intervention was not health promotion, enhancing coping, support and control promotes improved health. Need for Education of Health Providers to the Perspectives and Maintained Awareness of Persons with Dementia Evidence-based reports have described the perceptions of PwAD participating in early stage support groups (Dyck, 2003; evidence grade: D). Participants shared a feeling that they are being marginalized through their loss of independence, treatment by care providers, and public and media portrayals. Persons with dementia emphasize the need to belong, to feel important, to be a part of something bigger, e.g. some sort of collective action or self advocacy, and the need for support groups. These needs for recognition and participation in care support findings from additional studies by Lawton (1994; evidence grade: D), Burgener and Dickerson-Putman, 10