ABSTRACT
BACKGROUND: Clinical care for Huntington's disease (HD) is often provided in experienced centers that provide multidisciplinary care. However, the value of these centers and their uptake by HD families remain unknown. OBJECTIVE: To describe the services provided by a new HD center, including estimates of capture of the population served. METHODS: Retrospective review of a HD Center launched in 2015, including quantitative and qualitative data on clinic visits, demographic and clinical data. RESULTS: We observed a rapid and ongoing growth on the annual number of clinic encounters, with high demand for in-clinic multidisciplinary care. Using census data and estimates of HD prevalence, we determined that we served about 20% of local patients with HD. Most HD patients received pharmacological treatment for psychiatric symptoms, and over half were treated for chorea. About 25% of new HD diagnoses were on patients without family history of HD. Finally, the demand for predictive testing in at risk individuals significantly increased following the press release reporting the successful completion of the Ionis-HTTRx (RG 6042) trial. CONCLUSIONS: This report indicates a high demand for multidisciplinary care by HD families, supporting its value, providing a snapshot of the organization and function of a single center. Furthermore, it demonstrates how dissemination of news related to research advances influence clinical behavior. Reporting similar information from other HD centers to would provide us with a more global view of the status of HD care across multiple geographical areas.
Subject(s)
Health Services Needs and Demand/statistics & numerical data , Hospitals, Special , Huntington Disease , Patient Acceptance of Health Care/statistics & numerical data , Clinical Trials as Topic , Humans , Huntington Disease/diagnosis , Huntington Disease/epidemiology , Huntington Disease/therapy , Retrospective StudiesABSTRACT
Research in the previous decade has found a link between amyotrophic lateral sclerosis (ALS) and frontotemporal degeneration (FTD). It is estimated that as many as 50% of all people with ALS will have some degree of cognitive impairment and that approximately 10%-25% of patients will meet the Neary criteria for FTD. For the caregivers of persons with both ALS and FTD, the burden of care can be quite high. Nurses are in a position to help the caregivers cope.
Subject(s)
Amyotrophic Lateral Sclerosis/complications , Amyotrophic Lateral Sclerosis/nursing , Frontotemporal Dementia/etiology , Frontotemporal Dementia/nursing , Specialties, Nursing/methods , Adaptation, Psychological , Amyotrophic Lateral Sclerosis/psychology , Caregivers/psychology , Cognition Disorders/etiology , Cognition Disorders/nursing , Cognition Disorders/psychology , Family Nursing/methods , Frontotemporal Dementia/psychology , Health Education/methods , Humans , Social SupportABSTRACT
Frontotemporal dementia (FTD) is a progressive neurological condition caused by degeneration of the frontal and/or anterior temporal lobes resulting in personality, behavioral, and cognitive changes. Amyotrophic lateral sclerosis (ALS) is caused by degeneration of lower motor and pyramidal neurons, leading to loss of voluntary muscle movement. The common molecular pathological and anatomical overlaps between FTD and ALS suggest that the two disorders are strongly linked. In some patients FTD precedes ALS; in others ALS occurs first, while in still others the two disorders begin simultaneously. The association between ALS and FTD creates unique challenges for family caregivers. This paper provides a guide for healthcare providers caring for patients with FTD-ALS exhibiting behavioral, cognitive, and emotional symptoms. Strategies are suggested to help minimize the impact of negative symptoms.
