Clinical, Demographic, and Pharmacologic Features of Nursing Home Residents With Huntington's Disease

JAMDA xxx (2014) 1e6

JAMDA journal homepage: www.jamda.com

Original Study

Clinical, Demographic, and Pharmacologic Features of Nursing Home Residents With Huntington’s Disease Barbara J. Zarowitz PharmD, CGP, FCCP, BCPS a, *, Terrence O’Shea PharmD, CGP, FASCP a, Martha Nance MD b, c a

Omnicare, Inc, Cincinnati, OH Struthers Parkinson’s Center, Golden Valley, MN c Hennepin County Medical Center, Minneapolis, MN b

a b s t r a c t Keywords: Nursing home Huntington’s disease

Background: The purpose of this descriptive, retrospective analysis was to develop a demographic and clinical profile of nursing home residents with a diagnosis of Huntington’s disease (HD). Methods: Queries were made of a large data repository of linked and de-identified Minimum Data Set version 3.0 and prescription claims records, for the time period of October 1, 2010 through March 31, 2012. Results: Of 249,811 residents, 340 (0.14%) had a diagnosis of HD; 61% were female and 77.9% were Caucasian. The age range mode was 55e59 years (15%). Approximately one-half of the residents with HD exhibited communication difficulties, 78% had moderate or severe cognitive impairment, and most have significant functional limitations. Depression, dementia, anxiety, psychosis, and bipolar disease were present in 59.4%, 50.9%, 35.9%, and 23.2%, and 9.7%, respectively. Only 21% of residents with HD exhibited troublesome behavioral symptoms. Comorbidities of diabetes and cancer were uncommon (0.3%). Use of physical restraints (excluding bed rails) was considerably higher in residents with HD than in the general nursing home population. Falls were documented in almost one-half of residents. Antipsychotics were used in 61.6% of residents; 16.2% had psychotic symptoms. One was treated with tetrabenazine. Anxiolytics were received by 59.1% of residents, whereas only 35.9% had anxiety noted on Minimum Data Set records. Conclusions: The prevalence of HD in US nursing homes is very low (0.14%). Affected residents have significant cognitive and functional impairments, but problematic behaviors are present in only a minority. Serious comorbidities such as cancer and diabetes are rare. Antipsychotics, antidepressants, and anxiolytics are the mainstays of treatment. Ó 2014 - American Medical Directors Association, Inc. All rights reserved.

The prevalence of Huntington’s disease (HD) in the nursing home setting is not well-studied, however, analysis of a prospectively obtained, observational database of subjects with HD found that 7.4% resided in skilled nursing facilities (SNFs).1 The average age of these residents was 52 years, and 63% were women. Compared with community-dwelling HD patients, those residing in SNFs had worse motor function (eg, chorea, bradykinesia, gait abnormality, and imbalance); were more likely to have obsessions, compulsions, delusions, and auditory hallucinations; and had more aggressive, disruptive, and irritable behaviors. Bradykinesia, impaired gait, and impaired tandem walking were predictive of nursing home placement.1

This study was sponsored by Lundbeck, LLC. The authors declare no conflicts of interest. * Address correspondence to Barbara J. Zarowitz, PharmD, CGP, FCCP, BCPS, Omnicare, Inc, 33510 Schoolcraft Rd, Livonia, MI 48150. E-mail address: [email protected] (B.J. Zarowitz).

In addition to the motor, psychiatric, and behavioral features of HD described above, affected individuals can exhibit a wide array of other disease-related symptoms, including depression, anxiety, impulsiveness, apathy, cognitive impairment, sleep disturbance, dysarthria, dysphagia, weight loss, and in the later stages, incontinence, mutism, dystonia, and immobility.2,3 There is little published information about the clinical profile and drug therapy regimens of residents with HD residing in SNFs, related to either the motor symptoms (eg, chorea), or the nonmotor symptoms (eg, behavioral, psychiatric, cognitive). Because of the chronic and debilitating nature of HD, the significant quality of life and safety concerns, and the potential for medication-related adverse consequences, an analysis of residents with this condition in the nursing home setting is an important contribution to the understanding of the complexities of HD in SNF patients. The objective of this retrospective study was to characterize the prevalence of HD in residents of US SNFs and to develop a profile of these residents, including demographic information, concurrent

1525-8610/$ - see front matter Ó 2014 - American Medical Directors Association, Inc. All rights reserved. http://dx.doi.org/10.1016/j.jamda.2014.01.010

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B.J. Zarowitz et al. / JAMDA xxx (2014) 1e6

diagnoses, use of medications, and functional and quality of life parameters.

Methods After obtaining an exemption from Institutional Review Board Review and Waiver of Authorization from Sterling Institutional Review Board, data extracts were obtained from Omnicare’s repository of Minimum Data Set (MDS) version 3.0 and prescription claims records for residents in Omnicare-serviced SNFs for the time period of October 1, 2010 through March 31, 2012. Prescription claims data are collected from about 200 pharmacies serving more than 1.4 million residents in about 19,000 long term care and other healthcare facilities in 48 states (excludes Hawaii and Alaska) and Canada. The database stores over 600 million prescription claims processed within last 6 years. Initially, a count of unique residents for whom MDS data existed in the database was obtained and served as the denominator for determining the prevalence of a HD diagnosis in MDS records. The MDS is a comprehensive, standardized resident assessment instrument that is utilized in SNF to develop care delivery and planning, monitor clinical indicators such as falls, fractures, cognition, and behavior, and for payment from Medicare.4 The MDS is completed on admission to the facility, quarterly, annually, and upon significant changes in patient condition. With the implementation of MDS version 3.0 in October, 2010, “Huntington’s disease” appears as a distinct data field. The MDS data elements have been validated, and there is evidence for inter-rater reliability.5 Over 85% of MDS data elements have been found to be reliable with adequate inter-rater reliability and overall good agreement.5,6 The strongest reliability is with elements related to activities of daily living (ADLs). ADLs, pain, distressed mood, behavioral disturbances, and social engagement elements of the MDS have manifested discriminant validity.7 Although there are limitations to this caregiver-related assessment instrument, it is a standardized tool that provides the “best available evidence” of resident status and outcomes in the SNF setting. To perform the data match, prescription claims data and MDS data were obtained from the warehouse for the time period of interest. Once the 2 data sets were extracted, they were matched using resident demographics, and then the matched records were de-identified using a “safe harbor” method where all 18 identifiable data elements listed in section 164.514(b)(2) of the Health Insurance Portability and Accountability Act Privacy Rule are removed and replaced with internal identifiers. During the study period, many residents had more than 1 MDS assessment completed and received multiple medications. To avoid counting MDS data elements multiple times, if a resident had more than 1 MDS assessment during the study period and had different answers for the same MDS data element, then the answer representing the more severe condition or state was used. All medications prescribed during the study period were included, without determining start or stop dates; thus, an individual listed as taking haloperidol and olanzapine could have taken the drugs sequentially (in either order), or could be taking the 2 drugs concurrently.

Results

Table 1 Demographic Characteristics of SNF Residents With HD Diagnosis

Total Number

Percent

HD Residents in MDS database

340 249,811

0.14%

MDS Data Element

Total Number

Percent

134 206

39.4% 60.6%

1 7 17 15 23 48 51 46 44 20 22 21 14 11

0.3% 2.1% 5.0% 4.4% 6.8% 14.1% 15.0% 13.5% 12.9% 5.9% 6.5% 6.2% 4.1% 3.2%

265 35 29 11

77.9% 10.3% 8.5% 3.2%

Sex Male Female Age category <30 years 30e34 years 35e39 years 40e44 years 45e49 years 50e54 years 55e59 years 60e64 years 65e69 years 70e74 years 75e79 years 80e84 years 85e89 years >90 years Race/ethnicity White Black or African American Hispanic or Latino Other

HD, Huntington’s disease; MDS, Minimum Data Set; SNF, skilled nursing facility.

Functional Impairments Communication and comprehension problems affected over onehalf of residents (Table 2). Nearly one-half were unable to complete a cognitive interview (brief interview for mental status), and 44.9% of those who did showed severe cognitive impairments. Significant functional limitations in the performance of ADLs were noted in HD residents (data not shown). Extensive assistance or total dependence on staff was noted in the following domains: bathing (94.8%), personal hygiene (85.9%), dressing (84.7%), toileting (84.1%), transfers (80%), bed mobility (76.5%), and eating (65.9%). Mobility was similarly affected, with 55.9% of residents not walking in their room at all in the look-back assessment period, and only 4.1% of residents able to walk in their room independently. Three-quarters of these residents used a wheelchair as their primary mode of locomotion; only 3.2% used a walker, with the remainder using no device. Almost three-quarters (73.2%) of residents were “always incontinent.”

300

79.4%

78.8%

255 (75%)

250 200 Number of 150 Residents 100 50

Demographics

16.5%

14.7%

13.8% 3.8%

5%

2.4%

0

Of a total of 249,811 unique SNF residents identified in the MDS dataset, there were 340 (0.14%) with a diagnosis of HD. Of these, 61% were female (Table 1), and the majority were white (77.9%). Fifty-six percent of HD residents were between the ages of 50 and 69, with a mode of 15% in the age range of 55e59 (Figure 1).

Physical Behavioral Symptoms Not Exhibited

Verbal Behavioral Symptoms

Exhibited SomeƟmes

Other Behavioral Symptoms

Exhibited Daily

Fig. 1. Frequency of behavioral symptom manifestations in skilled nursing facility (SNF) residents with Huntington’s disease (HD).

B.J. Zarowitz et al. / JAMDA xxx (2014) 1e6 Table 2 Selected MDS Elements of Cognition and Mood MDS Data Element

Total Number

Residents with HD diagnosis Makes self understood sometimes, rarely, or never Ability to understand others sometimes, rarely, or never Cognitive assessment Ability to complete cognitive interview BIMS Summary Score 13e15 (cognitively intact) Score 08e12 (moderate impairment) Score 00e07 (severe impairment) Not able to complete cognitive interview Staff assessment for mental status Short-term memory problem Long-term memory problem Cognitive skills for daily decision making Moderately to severely impaired Resident mood interview (PHQ-9) severity score Total interviews conducted Score 00e09 (no depression to mild depression) Staff assessment of resident mood (PHQ-9-OV) Interviews not conducted Score 00e09 (no depression to mild depression)

340 209 172

Percent

61.5% 50.6%

3

population (14/339) experienced a major injury as a result of a fall. Bed rails were used daily in 11.8%, trunk restraints were used in 8.3%, and daily use of limb restraints occurred in 1.8%. Daily use of a chair that prevents rising was documented in 5.3% of residents with HD (Table 3). Medication Use

185 39 63 83 155

21.1% 34.1% 44.9%

140 133

90.3% 85.8%

148

96.7%

202 178

88.1%

138 118

85.5%

BIMS, brief interview for mental status; HD, Huntington’s disease; MDS, Minimum Data Set; PHQ-9, Patient Health Questionnaire-9; PHQ-9-OV, Patient Health Questionnaire-9-Office Visit.

There were 164 residents with HD for whom both MDS and prescription claims data were available. Only 1 resident received tetrabenazine in the study period (Table 4). Antipsychotics were received by 61.6% of residents. Agents most often used included haloperidol (40.6%), quetiapine (31.7%), risperidone (27.7%), and olanzapine (25.7%). Clozapine was used by 3% of residents with HD. Antidepressants were prescribed for 61% of residents. Agents most often used were selective serotonin reuptake inhibitors (SSRI) (69%), mirtazapine (28%), and serotonin-norepinephrine reuptake inhibitors (SNRI) (16%). The use of anxiolytics and antipsychotics far exceeded the percent of residents noted with diagnoses of anxiety or psychosis, whereas the number of residents prescribed antidepressants approximately equaled the number who had depression. About 50% of residents with HD also had a diagnosis of nonAlzheimer dementia, and 24 (7.1%) had a diagnosis of Alzheimer’s disease. A diagnosis of Parkinson’s disease was documented in 22 residents.

Mood and Behavior The Patient Health Questionnaire-9 (PHQ-9) was completed by 202 (60.3%); mood was assessed in the other 132 subjects by the MDS nurse using the Patient Health Questionnaire-9-Office Visit (PHQ-9OV). Only 11.9% of residents were scored by the PHQ-9 as having moderate, moderately severe, or severe depression, whereas no depression (29.7%), minimal depression (35.6%), and mild depression (22.8%) predominated (Table 2). A similar pattern was noted for those assessed with the PHQ-9-OV, with 36.2% judged to have no depression, and only 9.4% with moderate or more depression. Behavioral symptoms were relatively uncommon in residents with HD. Nearly 80% of residents did not exhibit physical behaviors directed toward others, whereas 13.8% did “sometimes” and 3.0% exhibited these types of behaviors daily (Figure 1). Comorbid psychiatric illness was prevalent with nearly 60% having a diagnosis of depression, followed by non-Alzheimer dementia [50.9% (which increased to 58% if Alzheimer’s disease was included)], anxiety disorder (35.9%), psychotic disorder (23.2%), schizophrenia (13.5%), and bipolar disease (9.7%). Other relatively common comorbidities included seizure disorder (14.4%), fractures [8.2% (hip 2.9%, other 5.3%)], and Parkinson’s disease (6.5%). Dysphagia and Nutrition Malnutrition was documented in 6.5% of residents in this study; 20% exhibited significant weight loss (5% of body weight over 30 days or 10% over 180 days) during the assessment look-back period. Swallowing problems were noted in 30% of residents, and two-thirds of residents required extensive assistance or were totally dependent on staff assistance for eating. Twenty-four percent of residents with HD (83/340) used nasogastric or abdominal feeding tubes. Falls; Restraint Use Falls were documented in almost 50% (162/339) of residents over the reporting periods covered by this study, with 41% of fallers experiencing any injury as a result (66/162), and only 9% (14/162) with a major injury. Thus, slightly over 4% of the residential HD

Discussion This study provides the first description of residents with HD in SNFs in the US ascertained through a large national database.1,2 We

Table 3 Active Diagnoses, Other Conditions, and Use of Restraints in SNF Residents With Huntington Disease MDS Data Element

Total Number

Residents with HD diagnosis Active diagnoses Cirrhosis Diabetes Cancer Hip fracture Other fracture Alzheimer disease Dementia other than Alzheimer disease Depression Manic depression (bipolar disease) Anxiety disorder Psychotic disorder (other than schizophrenia) Schizophrenia Parkinson disease Seizure disorder Malnutrition Health conditions Any fall(s) since admission or prior assessment No Yes Restraints Used in bed Bed rail used Trunk restraint used Limb restraint used Other restraint used Used in chair or out of bed Trunk restraint used Limb restraint used Chair prevents rising used Other restraint used

340

Percent

1 1 1 10 18 24 173 202 33 122 79 46 22 49 22

0.3% 0.3% 0.3% 2.9% 5.3% 7.1% 50.9% 59.4% 9.7% 35.9% 23.2% 13.5% 6.5% 14.4% 6.5%

177 162

52.1% 47.6%

42 5 3 7

12.4% 1.5% 0.9% 2.1%

29 6 19 12

8.6% 1.8% 5.6% 3.5%

4

B.J. Zarowitz et al. / JAMDA xxx (2014) 1e6

Table 4 Medications Used in SNF Residents With HD Medication

Total Number

Percent

Residents with HD and existence of prescription claims Tetrabenazine (Xenazine) Parkinson disease medications Unique residents receiving any PD medication Carbidopa/levodopa (all forms) Carbidopa/levodopa/entacapone (Stalevo) Ropinirole (all forms) Pramipexole (all forms) Amantadine Benztropine Antidepressants Unique residents receiving any antidepressant SSRI* SNRIy TCAz Mirtazapine all other Antipsychotics Unique residents receiving any antipsychotic Haloperidol Risperidone Olanzapine Quetiapine All other Acetylcholinesterase inhibitors Memantine Urinary incontinence medications Unique residents receiving any agent Anxiolytics Unique residents receiving any anxiolytic Lorazepam Diazepam Clonazepam Alprazolam Buspirone Sedative-hypnotics Unique residents receiving any sedative-hypnotic Zolpidem All others

164 1

0.6%

27 8 1 4 2 5 13

16.5% 29.6% 3.7% 14.8% 7.4% 18.5% 48.1%

100 69 16 3 28 24

61.0% 69.0% 16.0% 3.0% 28.0% 24.0%

101 41 28 26 32 24 24 7

61.6% 40.6% 27.7% 25.7% 31.7% 23.7% 14.6% 4.3%

18

11.0%

97 63 13 38 13 1

59.1% 64.9% 13.4% 39.2% 13.4% 1.0%

24 17 4

14.6% 70.8% 16.7%

HD, Huntington’s disease; PD, Parkinson’s disease; SNRI, serotonin-norepinephrine reuptake inhibitors; SSRI, selective serotonin reuptake inhibitors; TCA, tricyclic antidepressants. *Sertraline, fluoxetine, paroxetine, citalopram, escitalopram, fluvoxamine maleate. y Duloxetine, venlafaxine, desvenlafaxine. z Amitriptyline, imipramine, doxepin, nortriptyline, amoxapine, trimipramine.

believe it may provide a more accurate “snapshot” of the “typical” resident with HD in long term care than the previous studies. Our results confirm that nursing home residents with HD are commonly female and Caucasian, and considerably younger than SNF residents in general, whose average age is 84 years.1,3 However, despite their young age, a high proportion of residents with HD have moderate-severe impairments in cognition (about 80%), particularly in the area of decision-making in daily life (95%); communication (60%); and ability to perform self-cares (66%-95%) for domains ranging from feeding to bathing, similar to 80- to 85-year-old SNF residents with chronic obstructive pulmonary disease.8 The diagnosis and treatment of depression was noted in about 60%. Only about 10% of HD residents were judged to have moderate or severe depression by the PHQ-9 or the PHQ-9-OV, suggesting that depression in HD is generally well-recognized and well-addressed. Only 20%e25% of residents with HD exhibited problematic verbal, physical, or other behaviors at all, and in only 2%e5% did these behaviors occur daily. In contrast, a retrospective review of 97 nursing home residents with HD in a single long term care HD facility, Nance and Sanders reported that as many as one-third of these individuals exhibited disruptive symptoms.4 Wheelock et al noted that

behavioral symptoms were not a predictor of nursing home placement for patients with HD, which is supported by our findings.1 Others have suggested that behavioral symptoms are a predictor of nursing home placement for dementia, Parkinson’s disease and Alzheimer’s disease.9e11 Dysphagia, significant weight loss, and even overt malnutrition are important issues that require special nursing and medical attention; 1 study found that it was possible for residents even in the late stages of HD to gain weight.3 The use of both feeding tubes and hospice care were rather high in this group (24% and 15%, respectively), as were the need for substantial or total assistance with many activities of daily living, suggesting that this population was at a very advanced stage of the disease. The high use of antipsychotics (61.6%) is more likely for treatment of HD-associated chorea or for behavioral symptoms, than for management of psychosis, as only 23.2% of residents with HD had psychotic symptoms noted. Antipsychotics have been described as appropriate therapy of chorea by HD experts12,13 and were believed to be a reasonable first-choice treatment of irritability by 21% of experts, by up to 43% if there was also impulsivity, 45% if there was also hypersexuality, and 77% if there was also aggression.14 It has been suggested that neuroleptics that block the dopamine D2 receptors (ie, haloperidol, risperidone, olanzapine) are more likely to be effective in treating chorea, whereas agents such as quetiapine and clozapine do not block dopamine D2 receptors and are less likely to be effective.13e15 The evidence base for use of antipsychotics to treat chorea is insufficient to draw any conclusions about their effectiveness.15 Tetrabenazine was approved for the treatment of chorea in HD in 2008 after a pivotal study demonstrating its efficacy16 but was prescribed for only one of the 340 residents with HD identified in this survey. It is unclear whether this low rate of use is due to waning of chorea in the late stages of HD, a perception that chorea does not impact on quality of life at this stage, physician unawareness of the availability and effectiveness of the drug, concern about side effects, financial costs, or some other factor. The use of physical restraints was more common in HD than in the national nursing home population. Although only 11.8% of residents with HD used bed rails daily compared with 15.9% using MDS version 2.0 in the third quarter of 2010,17 the use of trunk restraints was markedly higher in residents with HD (8.3%) than in the general nursing home population (1.5%). The daily use of limb restraints was substantially higher (1.8%) in comparison to the national nursing home average of 0.1%. It is difficult to imagine a situation where the use of a limb restraint would not increase the risk of injury in a person with HD, as the involuntary limb movement leads to chafing against the restraint. Restraint-related deaths have been reported in HD.18 Finally, we were interested to see that the prevalence of selected severe concomitant illnesses (cirrhosis, diabetes, cancer) was low, with only 1 of the 340 residents with each diagnosis. There are conflicting views about the incidence of diabetes in HD.19,20 The rate was very low in this population of residents with late-stage HD, many of whom were on medications known to increase the risk of diabetes. A recent study utilizing the Swedish Cancer Registry has suggested that the incidence of cancer is in fact reduced in HD.21 “Seizure disorder” occurred in about 15% of HD residents, which is noteworthy. Although seizures can occur in more than one-third of individuals with juvenile-onset HD, they have not been thought to occur frequently in adult-onset HD.22 Further study of people in the later stages of HD may be needed to confirm this observation. Limitations The limitations of this study are characteristic of retrospective database analyses. Claims prescription data accurately reflect

B.J. Zarowitz et al. / JAMDA xxx (2014) 1e6

dispensed medications as they are adjudicated online and represent information 6 months or longer postreconciliation. However, medication consumption may be over-represented using prescription claims data in nursing home residents, who may refuse doses. In addition, clinically important details about the timing, efficacy, and reason for discontinuation of the drugs cannot be determined from claims data. There are potential inaccuracies in coding of MDS data because of poor quality of historical data, inaccurate coding, and inter-rater variability. However, others have shown that MDS data have moderate to moderately high validity and reliability.23 The linking of these datasets allows investigators to ask questions that can be viewed as hypothesisgenerating for future studies, rather than conclusive or causative. We have some concern about the frequent registration of Alzheimer’s disease and Parkinson’s disease in this patient group; overlapping phenotypes (eg, dementia in HD incorrectly labeled as “Alzheimer’s disease”), or phenotypes induced by medications (eg, levodopa-induced dyskinesia in a person with Parkinson’s disease incorrectly labeled as HD) could potentially lead to misdiagnosis or mislabeling in the MDS. The addition of a specific code for Huntington’s disease in the MDS version 3.0 in 2010 should facilitate the identification of true HD cases. To our knowledge, this is the first HD study to utilize this resource. However, the number of residents with HD identified in this review seems low, in view of the estimated 30,000 individuals in the US with the diagnosis of HD, and their need for skilled care.1,3 Whether this reflects an underdiagnosis of HD, or undercoding of HD in the MDS is unclear. Because of the lack of refinement in the MDS as an outcome tool and the fact that it lacks specific fields related to chorea, we are unable to detect changes over time related either to medication use or disease progression. Conclusions Despite the potential limitations described above, this “real world” examination provides meaningful insight into several key observations. (1) Although residents with HD are younger than the typical SNF resident, they have high care needs in most domains of ADLs, mobility, and communication. Dysphagia and weight loss are common. Nursing care plans and rehabilitation therapy evaluations should help the facility to assess and address these issues optimally as the disease progresses. Hospice can be used in the latest stages of the disease. (2) Depression and antidepressant use in this population are approximately equal, involving about 60% of residents. In contrast, antipsychotic and anxiolytic agents are prescribed for about 60% of residents, despite a lower prevalence of psychosis and anxiety, suggesting that these agents are being used for other purposes. (3) During the period of this study, tetrabenazine was just entering the market, and only one resident received this monoamine depleter. Both expert opinion and guidelines published by the American Academy of Neurology suggest that tetrabenazine may be a good option for the treatment of chorea in residents with HD who are not demonstrating aggressive symptoms, psychosis, or active depression.12,15 (4) Avoiding the use of limb restraints should be a focus of quality improvement in nursing homes that accept HD patients. (5) Care facilities should not overestimate the likelihood that a resident with HD will manifest severe behavior challenges; 80% of residents did not manifest problematic physical behaviors, and similar findings were seen for verbal and other behaviors.

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This study provides a baseline for future comparison, as pharmacologic, rehabilitative, and social interventions are introduced into this population. Future studies utilizing the MDS version 3.0 dataset could also compare HD with other conditions or the general nursing home population. Acknowledgments The authors would like to acknowledge the technical assistance of Mr. Alex Shif who linked, de-identified, and queried the OSHO data repository. References 1. Wheelock VL, Tempkin T, Marder K, et al. Predictors of nursing home placement in Huntington disease. Neurol 2003;60:998e1001. 2. Setter SM, Neumiller JJ, Dobbins EK, et al. Treatment of chorea associated with Huntington’s disease: Focus on tetrabenazine. Consult Pharm 2009;24: 524e537. 3. Nance MA, Sanders G. Characteristics of individuals with Huntington disease in long term care. Movement Dis 1996;11:542e548. 4. U.S. Department of Health and Human Services, Centers for Medicare and Medicaid Services, Minimum Data Set Assessment Instrument, version 2.0. 5. Lawton MP, Casten R, Parmelee PA, et al. Psychometric characteristics of the minimum data set II: Validity. J Am Geriatr Soc 1998;46:736e744. 6. Mor V, Angelelli J, Jones R, et al. Inter-rater reliability of nursing home quality indicators in the U.S. BMC Health Services Res 2003;3:20. Available at: http:// www.ncbi.nlm.nih.gov/pmc/articles/PMC280691/?tool¼pubmed. Accessed May 2, 2012. 7. Mor V. A comprehensive clinical assessment tool to inform policy and practice: Applications of the Minimum Data Set. Med Care 2004;42:50e59. 8. Zarowitz BJ, O’Shea T. Chronic obstructive pulmonary disease: Prevalence, characteristics and pharmacologic treatment in cognitively impaired nursing home residents. JMCP 2012;18:598e606. 9. Laplane KL, Fernandez HH, Friedman JH, and the SAGE Study Group. Prevalence, clinical characteristics and pharmacologic treatment of Parkinson’s disease residents in long term care facilities. Pharmacother 1999;19:1321e1327. 10. Haupt M, Kurz A. Predictors of nursing home placement in patients with Alzheimer’s disease. Int J Geriatr Psych 1993;8:741e746. 11. Knopman DS, Kitto J, Deinard S, Heiring J. Longitudinal study of death and institutionalization in patients with primary degenerative dementia. J Am Geriatr Soc 1988;36:108e112. 12. Burgunder JM, Guttman M, Perlman S, et al. An international survey-based algorithm, for pharmacologic treatment of chorea in Huntington’s disease. Version 2. PLoS Curr. August 30, 2011 [revised October 11, 2011]; 3: RRN1260. doi: 10.1371/currents.RRN1260 Available at: http://www.ncbi.nlm.nih.gov/ pmc/articles/PMC3166256/. Accessed February 4, 2013. 13. Nance M, Paulsen JS, Rosenblatt A, Wheelock V. A Physician’s Guide to the Management of Huntington’s Disease. 3rd ed. New York, NY: Huntington’s Disease Society of America; 2011. 14. Groves M, van Duijn E, Anderson K, et al. An international survey-based algorithm for the pharmacological treatment of irritability in Huntington’s disease. PLOS Currents Huntington Disease; September 2, 2011. http://dx.doi.org/ 10.1371/currents.RRN1259 [Last modified: March 26, 2012]. Edition 1. 15. Armstrong MJ, Miyasaki JM. Evidence-based guideline: Pharmacologic treatment of chorea in Huntington’s disease: Report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurol 2012;79: 597e603. 16. Huntington Study Group. Tetrabenazine as antichorea therapy in Huntington disease; A randomized controlled trial. Neurology 2006;66:366e372. 17. Centers for Medicare and Medicaid Services, United States Department of Health and Human Services. MDS Active Resident Information Report. Available at: http://www.cms.gov/Research-Statistics-Data-and-Systems/ Computer-Data-and-Systems/MDSPubQIandResRpt/activeresreport.html. Accessed May 30, 2012. 18. Miles SH, Irvine P. Deaths caused by physical restraints. Gerontologist 1992;32: 762e766. 19. Farrer LA. Diabetes mellitus in Huntington disease. Clin Genet 1985;27:62e67. 20. Boesgaard TW, Nielsen TT, Josefsen K, et al. Huntington’s disease does not appear to increase the risk of diabetes mellitus. J Neuroendocrinol 2009;21: 770e776. 21. Ji J, Sundquist K, Sundquist J. Cancer incidence in patients with polyglutamine diseases. Lancet Oncol 2012;13:642e648. 22. Cloud LJ, Rosenblatt A, Margolis RL, et al. Seizures in juvenile Huntington’s disease: Frequency and characterization in a multicenter cohort. Mov Disord 2012;27:1797e1800. 23. Shin JH. Scherer. Advantages and disadvantages of using MDS data in nursing research. JOGN 2009;35:7e21.

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Full Financial Disclosures

Parameter

Zarowitz

O’Shea

Nance

Stock Ownership in medically-related fields Consultancies Advisory Boards Partnerships Honoraria

Omnicare stock

Omnicare stock

Fresca stock

none Health Technology Assessment Council none none

None Sanofi-Aventis Pharmaceuticals None Sanofi-Aventis Pharmaceuticals

Grants

Sanofi-Aventis, Lundbeck, Optimer, Dey, AbbVie, Amgen, Novartis, Healthpoint

Sanofi-Aventis, Lundbeck, Optimer, Dey, AbbVie, Amgen, Novartis, Healthpoint

Intellectual Property Rights Expert Testimony Employment

none none Employed by Omnicare

None None Employed by Omnicare

Contracts

Sanofi-Aventis, Lundbeck, Optimer, Dey, AbbVie, Amgen, Novartis, Healthpoint

Royalties Other

none none

Sanofi-Aventis, Lundbeck, Optimer, Dey, AbbVie, Amgen, Novartis, Healthpoint None None

None Lundbeck Pharmaceuticals None Huntington Disease Society of America, Medscape, Augsburg College, WebMD Park Nicollet Foundation, The National Parkinson Foundation, The Michael J Fox Foundation, NIH/NINDS, Neurosearch A/S, Prana Biotechnology, Huntington Disease Society of America None None Employed by Park Nicollet Clinic and Hennepin County Medical Center None

Oxford University Press None