Abstract
Objective: To determine whether motor, behavioral, or psychiatric symptoms in Huntington disease (HD) predict skilled nursing facility (SNF) placement.
Methods: Subjects were participants in the Huntington Study Group’s Unified Huntington Disease Rating Scale Database (Rochester, NY) between January 1994 and September 1999. Specific motor, psychiatric, and behavioral variables in subjects residing at home and in SNF were analyzed using χ2 and Student’s t-tests. For a subset of subjects for whom longitudinal data existed, a Cox proportional hazards model controlling for age, sex, and disease duration was used.
Results: Among 4,809 subjects enrolled, 3,070 had clinically definite HD. Of these, 228 (7.4%) resided in SNF. The SNF residents’ average age was 52 years, average disease duration was 8.6 years, and they were predominantly women (63%). The SNF residents had worse motor function (chorea, bradykinesia, gait abnormality, and imbalance, p < 0.0001); were more likely to have obsessions, compulsions, delusions, and auditory hallucinations; and had more aggressive, disruptive (p < 0.0001), and irritable behaviors (p = 0.0012). For 1,559 subjects, longitudinal data existed (average length of follow-up, 1.9 years), and 87 (5%) moved from home to SNF. In the Cox model, bradykinesia (HR 1.965, 95% CI 1.083 to 3.564), impaired gait (HR 3.004, 95% CI 1.353 to 6.668), and impaired tandem walking (HR 2.546, 95% CI 1.460 to 4.439) were predictive of SNF placement.
Conclusions: Institutionalized patients with HD are more motorically, psychiatrically, and behaviorally impaired than their counterparts living at home. However, motor variables alone predicted institutionalization. Treatment strategies that delay the progression of motor dysfunction in HD may postpone the need for institutionalization.
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the March 25 issue to find the title link for this article.
Huntington disease (HD) is a progressive, autosomal dominant neurodegenerative disease. Dementia, psychiatric symptoms, chorea, and impairment of voluntary movement together result in progressive loss of independence. Unlike the elderly demented population residing in skilled nursing facilities (SNF), people with HD are younger and have unique care needs. A 1996 retrospective study described the features of 97 people with HD who resided in an HD specialty unit at a SNF (n = 83) or in other SNF (n = 14) in Minneapolis–St. Paul between 1976 and 1993.1 These residents were typically single, age 45, of either sex, and high school graduates. One third had serious behavioral problems, most were treated with psychotropic drugs, and 84% were still ambulatory upon admission.
Little additional information exists about the motor and psychiatric characteristics of people with HD who reside in SNF. Because institutionalization is a highly significant event in the course of HD, identification of the predictors of nursing home placement may engender interventions that postpone this outcome. Furthermore, characterization of HD-specific characteristics in SNF residents should optimize their care. We sought to determine specific motor, psychiatric, and behavioral characteristics in a large cohort of people with HD who reside in nursing homes, and to determine which characteristics predict institutionalization. We examined motor dysfunction, psychiatric symptoms, and the presence of aggressive and disruptive behavior or irritability as possible determinants of SNF placement.
Methods.
Subjects.
Subjects included those found in the Huntington Study Group’s (HSG) Unified Huntington Disease Rating Scale (UHDRS) database (Rochester, NY).2 Data were obtained from 43 HSG member sites in North America, Europe, and Australia. Subjects had clinically definite HD. Some subjects had genetic confirmation by CAG expansion, but this was not required. For a subset of subjects follow-up assessments were made about every 6 months.
Assessment.
All subjects were assessed using the UHDRS. The UHDRS consists of five sections. Demographic data are obtained at the baseline visit; motor, cognitive, behavioral, and functional assessment subtests are repeated at each follow-up visit. The motor assessment includes 31 items indicating oculomotor, speech, motor, and gait findings. Each item is rated from 0 to 4, with 0 indicating normal findings and 4 indicating severe abnormality. The cognitive assessment consists of three tests weighted for frontal/executive function. The behavioral subtest assesses 31 items, including mood disorder, disruptive or aggressive behavior, irritability, obsessions, compulsions, delusions, and hallucinations. Both the severity (0 to 4, with 0 = absent and 4 = severe) and the frequency (0 to 4, with 0 = almost never and 4 = almost always) are assessed separately. The functional assessment comprises three subtests: an activities of daily living checklist, an independence scale, and a total functional capacity scale, which includes one item indicating need for skilled nursing care.
Procedure.
We hypothesized that advanced motor impairment and frequent and severe psychiatric or behavioral disturbances would predict institutionalization. UHDRS item 79 (care level) was used to denote the independence of living. Subjects who required skilled nursing care (0 response on item 79) were compared with those who still resided at home (1 or 2 on item 79). For each group, 27 variables from the UHDRS were analyzed. In all analyses scores were dichotomized into 0, 1, or 2 response vs 3 or 4 response. For the motor subtest, the presence of a score of 3 (moderate) or 4 (severe or unable) for severity of bradykinesia (item 15), chorea (item 17a-g), gait (item 18), tandem gait (item 19), or retropulsion (item 20) was sought. For psychiatric symptoms, a score of 3 (moderate) or 4 (severe) for severity and 3 (frequently) or 4 (almost always) for frequency were sought. Similarly, for behavioral symptoms, a score of 3 or 4 for severity and 3 or 4 for frequency of aggressive or disruptive behavior (item 34a-b) or irritability (35a-b) were included.
Statistical analysis.
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Cross-sectional: The 27 motor, psychiatric, and behavioral variables at baseline were compared for subjects residing at home with those residing in SNF using χ2 and Student’s t-tests.
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Longitudinal: Motor, psychiatric, and behavioral characteristics were examined for predictive value for institutionalization using a survival analysis with a Cox proportional hazards model, adjusting for age, sex, and disease duration.
Results.
Among 4,809 subjects enrolled in the UHDRS database between January 1994 and September 1999, 3,070 had clinically definite HD. A total of 228 subjects (7.4%) resided in SNF (table 1). Most patients (68.8%) resided in the United States; the country of residence for all subjects is shown in table e1 on the Neurology Web site (go to www.neurology.org). Subjects living at home were equally distributed between the sexes (49% men, 51% women). Of those in SNF, 37% were men and 63% were women (p = 0.001). The SNF residents had a longer duration of disease (8.6 ± 5.1 years) than did those residing at home (3.1 ± 3.4 years). The average age of SNF subjects was 52 years, and of at-home subjects was 48 years (p = 0.0001).
Table 1 Demographics of HD population
Cross-sectional analysis.
The subjects in SNF had a higher frequency of marked chorea (17.7%), bradykinesia (58%), retropulsion (62%), gait (61%), and balance (93%) dysfunction than did those residing at home (p < 0.0001 for all correlates; see table e2 on the Neurology Web site at www.neurology.org). Symptoms of depression and anxiety were reported in about 20% of SNF residents. However, the severity and frequency of symptoms of depression, anxiety, and suicidal ideation did not differ between those living at home and those living in SNF. The examiners rated both groups of subjects as commonly being depressed (71.8% of SNF residents and 64.5% home subjects, p = 0.03) or requiring pharmacotherapy for depression (68.5% SNF, 59.8% home, p = 0.012). Severe (14%, p = 0.009) or frequent (13%, p = 0.03) obsessions and severe compulsions (8%, p = 0.02) were more frequent in the SNF residents. Severe or frequent delusions were twice as common in SNF residents (SNF 4.9%, home 2%, p = 0.007). Severe or frequent auditory hallucinations were more prevalent in the SNF residents (2.5 to 3% SNF, 0.4% home, p < 0.0001). Behavioral analysis revealed that the frequency (18.9% vs 8.9%) and severity (26.8% vs 15.6%) of markedly disruptive and aggressive (p < 0.0001) as well as the frequency (25.8% vs 17.3%, p = 0.0012) and severity (31.8% vs 19%, p = 0.0019) of irritable behavior was significantly higher in the SNF population.
Predictors of skilled nursing home placement.
For longitudinal analysis, the Cox proportional hazards model adjusting for age, sex, and disease duration was used. A total of 1,559 subjects were followed longitudinally. A total of 87 (5%) subjects initially living at home were institutionalized during follow-up. Average length of follow-up was 1.89 years (SD 1.2). Analysis revealed that motor correlates alone and not psychiatric or behavioral correlates were predictive of institutional care. Impaired gait (HR 3.004, 95% CI 1.353 to 6.668), impaired tandem walking (HR 2.546, 95% CI 1.460 to 4.439), and bradykinesia (HR 1.965, 95% CI 1.083 to 3.5640) were each predictive of subsequent SNF placement (table 2).
Table 2 Predictors of nursing home placement
Discussion.
At present, only palliative care is available for HD. A significant number of people with HD will require long-term care during the course of their disease, and most patients with HD who enter nursing facilities will stay there until their deaths.1 The average length of institutionalization in HD is 2 to 8 years.1 The UHDRS database contains information about the largest number of people yet described who reside in skilled nursing homes (n = 228), and this is the first study to analyze predictors of nursing home placement in HD.
Demographics.
Within this database, there are more women than men with HD residing in nursing homes. This contrasts with the retrospective study (52% men in Minnesota, vs 37% in UHDRS). A similar sex prevalence in institutionalization has been reported in patients with AD and patients with PD.3,4⇓ The reason for the difference in this study may be that the Minnesota cohort consisted primarily of people referred to a specialty care HD unit, and thus our results may be more representative of people with HD in the general nursing home population. As expected, we found that subjects in SNF have had a longer duration of disease (average 8.9 years, similar to 10 years in the Minnesota cohort) and were somewhat older than those still living at home.
Motor correlates.
We analyzed specific motor characteristics in institutionalized people with HD. In cross-sectional analysis, motor dysfunction (marked chorea, bradykinesia, impaired gait and balance) was significantly more common in the SNF population. Sixty percent of institutionalized HD subjects required assistance with ambulation or were nonambulatory. This finding contrasts with the observation that 84% of subjects in SNF were ambulatory upon admission, and 26% became nonambulatory during their stay, perhaps reflecting a more behaviorally disturbed Minnesota cohort. However, the high rate of gait disturbance found in this study of SNF patients with HD is similar to that found in a large cohort of institutionalized patients with PD.4
In the survival analysis, motor symptoms alone predicted institutionalization. No other studies of the relationship of motor function in HD to nursing home placement exist for comparison. In a prospective analysis of predictors of nursing home admission in patients with probable AD, the presence of extrapyramidal signs was highly predictive of institutionalization (RR = 2.51).5 In contrast, in a case-control study in PD the presence of hallucinations was a better predictor of institutionalization than was the severity of the motor score on the Unified Parkinson’s Disease Rating Scale.6 These findings were confirmed by prospective Australian and Norwegian studies of predictors of SNF placement in PD, both of which demonstrated that dementia and hallucinations rather than motor severity predicted SNF placement.7,8⇓
Psychiatric correlates.
In this study, 68.5% of the subjects residing in SNF were judged to require pharmacotherapy for depression, which is a higher prevalence than that found in the Minnesota study (46% of subjects requiring antidepressant medication).1 The prevalence of moderate or severe depressive symptoms was between 11 and 21% in the SNF subjects. The prevalence of depression in HD is estimated to be approximately 40%; we specifically identified moderate or severe depression.9 Suicidal ideation was fairly rare in both groups (2 to 6%). The prevalence of a history of obsessions and compulsions (8 to 14%) was significantly higher in the SNF residents. Furthermore, the prevalence of delusions and auditory hallucinations, although low, was more than twice as common in SNF residents.
In face of evidence that psychiatric symptoms are predictive of nursing home placement in both the elderly demented and in PD, the failure of psychiatric correlates to predict nursing home placement in HD is surprising.4,6-8,10-12⇓⇓⇓⇓⇓⇓ One possible explanation is that patients with severe psychiatric symptoms may be more likely to be admitted to psychiatric facilities rather than SNF and that these patients may not have the opportunity to continue longitudinal follow-up in this database. Alternatively, psychiatric symptoms in people with HD could be more easily controlled, as patients with HD are much younger and may not have as severe a coexistent dementia as those with AD and PD. Furthermore, dopaminergic medications that may induce psychosis in PD are not necessary in HD.
Behavioral correlates.
Disruptive, aggressive, and irritable behaviors were significantly more common in the SNF residents than in those living at home. The frequency of marked aggression (26.8%) was somewhat lower than that reported by the previous study (34% had violent behaviors that were classified as often/severe). Studies of predictors of nursing home placement in patients with dementia have identified behavioral difficulties as predictors of institutionalization.3,4,9,10,12⇓⇓⇓⇓ The lack of predictive value of these behaviors in patients with HD may, like psychiatric correlates, be due to under-representation of subjects because of placement in psychiatric facilities or locked facilities rather than SNF.
The most significant limitation of this study is the lack of caregiver data. Multiple studies examining correlates of institutionalization in patients with dementia have identified caregiver burden and psychological stress as important predictors of institutionalization.13-15⇓⇓ This is particularly critical in HD because of the familial nature of the disease. Caregivers may themselves be at risk for HD, and in some cases may be responsible for the care of more than one family member or of family members from two or more generations. Financial burdens for families with HD are likely to be more severe because of the typical onset during high or peak earning years as well as due to the possibility of multiple affected family members. A second limitation of this study is the relatively short duration of longitudinal follow-up. It is also possible that subjects with more severe psychiatric or behavioral disturbances may have been lost to follow-up. A third limitation is the lack of information about dysphagia and weight loss, both of which may precipitate institutionalization for some patients with HD. However, we believe that the inclusion of 228 subjects in nursing homes and longitudinal data of 87 subjects who moved from home to skilled nursing care constitutes an important cohort.
We have demonstrated that the most robust predictor of nursing home placement in HD is advanced motor impairment. Nursing home care teams should anticipate the likelihood of the person with HD having difficulty with ambulation and balance. Care plans should include strategies to prevent falls as well as regular monitoring for depression, aggression, and other psychiatric symptoms that may be successfully managed with pharmacologic and behavioral interventions. Potential therapeutic interventions that delay the progression of motor dysfunction in HD may, like anticholinesterase treatment in AD, reduce the cost of care and delay institutionalization.16 Future studies of predictors of nursing home placement in HD should include longer follow-up as well as measurement of caregiver stress. Given the unique burdens of HD caregivers, caregiver interventions should also be studied as a means of delaying institutionalization.
Appendix
HSG participating investigators and coordinators: Phillipa Hedges, Elizabeth McCusker, MD, Samantha Pearce, and Ronald Trent, PhD, Westmead Hospital, Sydney, NSW Australia; David Abwender, PhD, Peter Como, PhD, Irenita Gardiner, RN, Charlyne Hickey, RN, Elise Kayson, RN, Karl Kieburtz, MD, Frederick Marshall, MD, Nancy Pearson, RN, Ira Shoulson, MD, and Carol Zimmerman, RN, University of Rochester, NY; Elan Louis, MD, Karen Marder, MD, Carol Moskowitz, RN, Carmen Polanco, BA, Stuart Taylor, MD, and Naomi Zubin, BA, Columbia Presbyterian Medical Center, New York, NY; Catherine Brown, RN, Jill Burkeholder, Mark Guttman, MD, Sandra Russell, Dwight Stewart, MD, and Jackie Thomson, RN, Markham Health Center, Toronto, Ontario, Canada; Daniel S. Sax, MD, and Marie Saint-Hilaire, MD, Boston University, MA; Jackie Gray, Cindy Hunter, MS, Nanette Mercado, PhD, Eric Siemers, MD, and Joanne Wojeieszek, MD, Indiana University School of Medicine, Indianapolis; Ted Dawson, MD, Elizabeth Leritz, BS, Adam Rosenblatt, MD, Meeia Sherr, RN, and Candace Young, RN, Johns Hopkins University, Baltimore, MD; Tetsuo Ashizawa, MD, Jenny Beach, RN, and Joseph Jankovic, MD, Baylor College of Medicine, Houston, TX; Jeana Jaglin, RN, and Kathleen Shannon, MD, Rush Presbyterian/St. Luke’s Medical Center, Chicago, IL; Anders Lundin, MD, Karolinska Hospital, Stockholm, Sweden; Kathleen Francis, MD, and Kim Lane, UMDNJ Robert Wood Johnson Medical School, Camden, NJ; Alexander Auchus, MD, J. Timothy Greenamyre, MD, Steven Hersch, MD, Randi Jones, PhD, and David Olson, MD, Emory University, Atlanta, GA; Jang-Ho John Cha, MD, Merit Cudkowicz, MD, Walter Koroshetz, MD, John Penney, MD, Greg Rudolf, Paula Sexton, MA, and Anne B. Young, MD, Massachusetts General Hospital, Boston; Roger Albin, MD, and Kristine Wernette, RN, University of Michigan, Ann Arbor; Donald S. Higgins, MD, and Carson Reider, MS, Ohio State University, Columbus; Vicki Hunt, RN, and Francis Walker, MD, Bowman Gray School of Medicine, Winston-Salem, NC; Robert Hauscr, MD, Juan Sanchez-Ramos, MD, and Audrey Walker, RN, University of South Florida, Tampa; Martha Nance, MD, Minneapolis Veterans Administration Medical Center, MN; Carol Pantello, RN, Gina Rohs, and Oksana Suchowersky, MD, University of Calgary Medical Clinic, Calgary, Alberta, Canada; Kerry Duncan and Lauren Seeberger, MD, Colorado Neurologic Institute, Englewood; Jody Corey-Bloom, MD, Jane Paulsen, PhD, Michael Swenson, MD, and Neal Swerdlow, MD, University of California, San Diego; Wayne Martin, MD, and Marguerite Wieler, BSC, PT, University of Alberta, Edmonton, Alberta, Canada; Alicia Facca, MD, Gustavo Rey, PhD, and William Weiner, MD, University of Miami, FL; Charles Adler, MD, John Caviness, MD, Cindy Lied, RN, and Stephanie Newman, RN, Mayo Clinic, Scottsdale, AZ; Andrew Feigin, MD, and Jennifer Mazurkiewicz, BA, North Shore University Hospital, Manhasset, NY; Karen Caplan, MSW, Janet Cellar, RN, and Kenneth Marck, MD, Yale University School of Medicine, New Haven, CT; Michael Hayden, MD, Lynn Raymond, MD, and Gina Rohs, University of British Columbia, Vancouver; Leon S. Dure, MD, and Jane Lane, RN, Children’s Hospital of Alabama, Birmingham; Diane Brown, RN, Stewart Factor, DO, and Eric Molho, MD, Albany Medical College, Albany, NY; Madeline Harrison, MD, Carol Manning, PhD, and Elke Rost-Ruffner, RN, University of Virginia, Charlottesville; Teresa Tempkin, RNC, MSN, David Richman, MD, and Vicki Wheelock, MD, University of California Davis; Richard Dubinsky, MD, and Carolyn Gray, RN, University of Kansas Medical Center, Kansas City; Ann Catherine Bachoud-Levi, MD, Höpital Henri Mondor, Creteil, France; Hartmut Meierkord, MD, Universitatsklinikum Charite, Berlin, Germany; Joseph Friedman, MD, and Margaret Lannon, RN, Memorial Hospital of Rhode Island, Pawtucket; Joan Lawrence, MD, Royal Brisbane Hospital, Brisbane, Queensland, Australia; Allen Rubin, MD, and Rose Schwarz, RN, Allegheny University, Philadelphia, PA; William Mallonee, MD, David Palmer, MD, Greg Suter, BA, Hereditary Neurologic Disease Center, Wichita, KS
HSG Biostatistics and Coordination Center staff: Alicia Brocht, BA, Kathy Claude, MS, Joshua Goldstein, Michael McDermott, PhD, David Oakes, PhD, and Constancy Orme, BA, University of Rochester, NY.
Acknowledgments
Supported by the Huntington Study Group, the Huntington Disease Society of America (New York, NY), the Huntington Society of Canada, the Hereditary Disease Foundation (Santa Monica, CA), and the Joseph P. Roberson Foundation (Davis, CA).
Acknowledgment
The data presented in this article were obtained from the Unified Huntington Disease Rating Scale longitudinal database maintained by the Huntington Study Group. The database is compiled from information collected by the participating HSG sites. The authors thank the data management staff at the HSG Coordination Center (Rochester, NY).
- Received November 19, 2001.
- Accepted November 29, 2002.
References
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