Predictive testing for inherited prion disease

FOURTH INTERNATIONAL CONFERENCE ON ALZHEIMER’S DISEASE

however, have difficulty assessing competency in dementia, due in pal to the absence of standardized assessmentinstruments. Development of such instruments is necessary 10 ensure that reliable and accurate competency judgments are made for dementia patients. Mclbodsz The investigators developed two specialized clinical vignetks which test an important competency (capacity to consent 10 medical treatmem) under five accepted and well-described legal standards (LSlLSS). These standards concern the capacities 10: “evidence” a treatment choice (LSl); make the “reasonable” treatment choice (when Ihe alternative is unreasonable) (LSZ); “appreciate” the consequences of a choice (LS3); make a choice based on “ralional reasons” (LS4): and “unUerstand” the treatment situation and choices (LS5). Detailed, well operationalized, and highly reliable scoring criteria were developed for each legal standard. Ten normal older controls and 21 patients with probable AD (by NINCDS-ADRDA criteria) (10 mdd and 11 moderate) were Hdminiswed the~two vigneUes. Rcsulu: T-test revealed significant control and AD erou~ differences for LSl and LS3-LSS. with ?.tro”eer effect sizes beine associated khincreasing diflicully of legal standaid. Anova d&onstrated Ihat &“trols. mild AD and moderate AD patients performed differentially upon LS3 (F=4.8, p -z .05), LS4 (F=l?_.Z, p < .001)and LS5 (F= 45.9, p < .@Xll). Preliminary cut-off scores

forcompetency, derived fromcontrol performance, revealed thatmoderate AD patients had difficulty with all five legal slandards. but particularly those requiring

appreciation of consequences, rational reasoning, and factual understanding (LS3. L.55). Mild AD patients, in conuast, showed significant difficulty on only the legal slandards involving rational reasoning and factual understanding (LS4 and LS5). Conclusions: Clinical vignetles with five embedded legal standards demonstrated reliability and face, content, and construct validity as an instrument for assessing competency in AD. Normal elderly, mild and moderate AD palients performed differentially upon three of the five legal standards, which appeared 10 form a hierarchy ofdi!Xculty. Preliminary cwoff scores derived from control performance appeared to validly assign rhe compelency stalus of mild and moderate AD patients under Ihe different standards. Clinical vignettes have potential as an objective instrument which can guide physician competency assessment in AD.

jective inability to attri&te problem behaviors to the disease and their over-appraisalof care-recipients'abilities to control them, contribute substantiallyto these impacts. This 3 year NINR funded study proposes to test the effectiveness of a randomized, inter-disciplinary,family systems-based,non-pharmacological and ps choeducational interventionon families of persons with DAT. f he qoal of the program is to decrease the depression experienced-by caregivers and to decrease their perceptions of behavior problems in persons with OAT. We postulate that the way a family perceives and organizes around the dementing disorder affects the strain the primary caregiver experiences, the behavior of the care-recipient,and the depression experienced by the primary caregiver. This interventionfocuses on increasing the concordance of a family's understanding of DAT, enhancing their understandingand skill in the management of behavior problems and increasing the family's support for the primary caregiver and the person with DAT. Families and persons with DAT attend separate, but concurrent groups 2 hours a week for 7 weeks. While family members learn about the disease and their reactions to it, patients with DAT are videotaped performing tasks adapted to their differing abilities. The videotapes are used with the families to help them understand how the disease affects each individual'sability to function and how reinterpreting the behavior, adapting their approach or modifying the environment increases the person with OAT's ability to function. This paper will discuss the issues related to recruitment and randomization of subjects, describe the content of the group sessions, and report quhlitative outcomes from the first year of the study.

442 440

PARTICIPATORY FAMILY

PREDICTIVE

TESTING

FOR INHERITED PRION D1SEASE.K.A.

Quaid,

Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, Indiana 46202-5251. The genetic defects for two different dominantly inherited neurodegenerative diseases, Gerstmann-Straussler-Scheinker disease (GSS) and early onset familial Alzheimer disease (FAD), have been found in two separate Indiana kindreds. The discovery of

these defects has made predictive testing possible for these Testing was initiated in July of 1993 late-onset disorders. using protocols modeled after those developed for Huntington disease The purpose of this project is to examine the psychological and social consequences of predictive testing for this class of diseases. As of February 1, 1994, eight individuals (6 females and 2 The male) at risk for GSS have entered the testing protocol. average age is 37.38(S.D.=4.81). Seven (87.5%) are married and 1 is divorced. The average education if 12.88 years (S.D.-2.48) and the average number of children is 2.00 (S.D.=.93). Five (62.5%) had a” affected father and 3(37.5X) had a” affected

(HD).

mother. The average age 51.38 years (S.D.=7.90).

of onset for The average

the age

affected parent of learning of

was their

risk for GSS was 26.62 (S.D.=11.04). Five of these individuals have a lifetime history of Axis I psychiatric disorder and 3 of these 5 remain currently symptomatic. Results have been given to five individuals with the longest period of followup being six months. Four individuals received a decreased risk and one individual received an increased risk result. Two more individuals have completed the pretest counseling, have been notified that results are available, but have not returned for disclosure of these results. The baseline characteristics of these individuals were compared with 46 individuals at risk for ND requesting predictive No significant differences testing for that late-onset disorder. were found between these two groups in age or education, or on scores for several psychological tests including the Beck Depression Scale, Beck Hopelessness Scale and the Symptom Checklist-90.

CAREGIVERS.

CONTROL

AND

THE

WELL-BEING

OF

M.G. Austrom.

Department of Psychiatry,Indiana UniversitySchool

of Medicine, Indianapolis, IN 46202-5111; and D.W. Reid,Department of Psychology, York University, North York, Ontario, Canada, M3J 1P3. Based upon data collected with 147 primary family caregivers of Alzheimer disease (AD) patients, as well as clinical case studies of specific caregivers, the interaction between the family caregiver and the AD patient’s primary health care professional is examined. How that interaction is perceived and the impact that the caregiver/health professional relationship has on caregiver wellbeing will be discussed. It has been reported that victims of tragedies or persons accommodating to substantial changes in their life situation often use others as a source of regaining strength, personal forbearance and in regaining control over their lives. This process of gaining control via interaction with others has been referred to as participatory control (Reid, 1984; Reid & Stirling, 1989). Participatory controlreflects how one interacts with theirprimary healthcare professional in regaining control over their condition. In

the case of the AD patient and their family caregiver, the caregiver must interact with health care professionals in order to make sense of the situation and gain knowledge in how to cope, for their own needs and on behalf of the AD patient as well. Caregivers, however, have often reported a sense of helplessness and abandonment by health care professionals. Clinically it appears that caregivers do much better when they report a participatory relationship with the health care provider, however, the impact of these experiences has received little empirical attention. This session will discuss the possible mediating or buffering effects of participatory control on family caregiver well-being and explore methods of empowering caregivers by building positive relationships with health care professionals. Also to be considered is the interactive nature of multiple variables such as the caregiver’s sense of commitment to the patient, level of burden, and participatory control on overall well-being.

441 MINNESOTA FAMILY WORKSHOP: A PSYCHOEDUCATIONALAPPROACH FOR INDIVIDUALS WITH MILD TO MODERATE DEMENTIA AND THEIR FAMILIES. S.K. Ostwald, K. Hepburn. W. Caron. T. Burns. R. Mantel1 and

A. Krasnoff.. School of Nursing and Dept of Family and Community Medicine, University of Minnesota, Minneapolis, MN and VA Med Ctr Minneapolis, MN. Alzheimer's disease claims more than one victim: Family members who provide care for persons with dementia of the Alzheimer's Type (DAT) experience serious negative impacts in the areas of physical and psychological health and social functioning. Dementia-relatedbehavior problems, including caregivers' sub-

443 THE MEANING OF CAREGIVING FOR ALZHEIMER'S DISEASE AMONG NATIVE AMERICAN CAREGIVERS: STORIES OF SPIRITUALITY, FATALISM, AND MASTERY. P. Boss, L. Kaplan, Dept. of Family Social Science, University of Minnesota-St. Paul, and M. Gordon, Miller-Dwan Hospital and Counseling Center, Duluth, Minnesota, USA. Previous research with Euro-American caregivers indicated that depression is predicted by their perceptions of mastery. The question is: would findinos be similar for oooulations who ;alue fatalism and spiritiality more than mastery and if