Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient’s Perspective
Abstract Background Limited information is available on patients' experience living with Huntington’s disease (HD). The primary objective of this study was to assess the health-related quality of life and well being of patients with HD. Methods A non-interventional, cross-sectional study was co...
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Adis, Springer Healthcare
2024-10-01
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| Series: | Neurology and Therapy |
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| Online Access: | https://doi.org/10.1007/s40120-024-00655-0 |
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| author | Jesús Pérez-Pérez Sofía García-López Tamara Fernández Valle Cèlia Painous Maria Rosa Querol-Pascual Pedro J. García Ruiz Elena Bellosta Diago Esther Cubo Delgado Barbara Vives Pastor María Carmen Peiró Villaplana Idaira Martín Santana Marta Blázquez Estrada Matilde Calopa Garride Pablo Mir Carmen Álvarez Jorge Maurino Anna de Prado José Luis López-Sendón |
| author_facet | Jesús Pérez-Pérez Sofía García-López Tamara Fernández Valle Cèlia Painous Maria Rosa Querol-Pascual Pedro J. García Ruiz Elena Bellosta Diago Esther Cubo Delgado Barbara Vives Pastor María Carmen Peiró Villaplana Idaira Martín Santana Marta Blázquez Estrada Matilde Calopa Garride Pablo Mir Carmen Álvarez Jorge Maurino Anna de Prado José Luis López-Sendón |
| author_sort | Jesús Pérez-Pérez |
| collection | DOAJ |
| description | Abstract Background Limited information is available on patients' experience living with Huntington’s disease (HD). The primary objective of this study was to assess the health-related quality of life and well being of patients with HD. Methods A non-interventional, cross-sectional study was conducted in 17 hospitals-based movement disorders units in Spain. Patients aged ≥ 18 years, genetically HD diagnosed [with a diagnostic confidence level score of 4, and an Independence Scale (IS) score ≥ 70] were included. The primary variables were the Huntington's Disease Health-related Quality of Life (HDQLIFE) scores and results of the Satisfaction with Life Scale (SWLS). Secondary outcomes include the Unified HD Rating Scale (UHDRS), Beck Hopelessness Scale (BHS), Stigma Scale for Chronic Illness (SSCI-8), Beck Depression Inventory-Fast Screen (BDI-FS) and Problem Behaviours Assessment for HD short Version (PBA-S). Results A total of 102 patients were included. The mean age (SD) was 53.1 (12.1) years and 56% were male. Most of the patients (99.0%) showed motor symptoms (87.3%), behavioural and psychiatric disturbances (59.8%), or cognitive impairment (20.6%). HDQLIFE domain score means (SD) includes concern with death and dying 45.97 (9.60) end-of-life planning 37.91 (8.84), and meaning and purpose 44.74 (9.05). SWLS score mean was 24.25 (7.33). Depressive symptoms were found in 37.4% of patients and moderate-to-severe feelings of hopelessness in 32.9%. The prevalence of stigma was 55.9% (n = 57). Conclusion HD impacted quality of life, with prevalent motor, psychiatric symptoms and cognitive impairment. Patient perspectives may provide complementary information to implement specific interventions. |
| format | Article |
| id | doaj-art-0d10aac49baa4256ae6b241a94ffe70c |
| institution | Kabale University |
| issn | 2193-8253 2193-6536 |
| language | English |
| publishDate | 2024-10-01 |
| publisher | Adis, Springer Healthcare |
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| series | Neurology and Therapy |
| spelling | doaj-art-0d10aac49baa4256ae6b241a94ffe70c2025-01-26T12:58:35ZengAdis, Springer HealthcareNeurology and Therapy2193-82532193-65362024-10-011419911510.1007/s40120-024-00655-0Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient’s PerspectiveJesús Pérez-Pérez0Sofía García-López1Tamara Fernández Valle2Cèlia Painous3Maria Rosa Querol-Pascual4Pedro J. García Ruiz5Elena Bellosta Diago6Esther Cubo Delgado7Barbara Vives Pastor8María Carmen Peiró Villaplana9Idaira Martín Santana10Marta Blázquez Estrada11Matilde Calopa Garride12Pablo Mir13Carmen Álvarez14Jorge Maurino15Anna de Prado16José Luis López-Sendón17Department of Neurology, Movement Disorders Unit, Hospital de la Santa Creu I Sant Pau, Universitat Autònoma de BarcelonaMedical Department, Roche PharmaDepartment of Neurology, University Hospital Cruces, Neurodegenerative Disease Group Health Research Institute of Bizkaia (BioBizkaia), Neuroscience Department University of the Basque CountryParkinson and Movement Disorders Unit, Neurology Service, Hospital Clínic UniversitariDepartment of Neurology, Hospital de BadajozMovement Disorders Unit, Department of Neurology, Fundacion Jimenez Diaz, Universidad Autonoma de MadridDepartment of Neurology, Hospital Clínico Universitario Lozano BlesaHospital Universitario de Burgos, University of BurgosServicio Neurología, Hospital Universitari Son EspasesHospital Universitario La Fe de ValenciaUnidad de Trastornos del Movimiento, Hospital Universitario Insular de Gran CanariaHospital Universitario Central de AsturiasDepartment of Neurology, Hospital Universitari de Bellvitge, L’Hospitalet de LlobregatUnidad de Trastornos del Movimiento, Servicio de Neurología y Neurofisiología Clínica, Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del Rocío/CSIC/Universidad de SevillaMedical Department, Roche PharmaMedical Department, Roche PharmaReal World Evidence, IQVIA Information S.ADepartment of Neurology, Hospital Ramón y Cajal, IRYCISAbstract Background Limited information is available on patients' experience living with Huntington’s disease (HD). The primary objective of this study was to assess the health-related quality of life and well being of patients with HD. Methods A non-interventional, cross-sectional study was conducted in 17 hospitals-based movement disorders units in Spain. Patients aged ≥ 18 years, genetically HD diagnosed [with a diagnostic confidence level score of 4, and an Independence Scale (IS) score ≥ 70] were included. The primary variables were the Huntington's Disease Health-related Quality of Life (HDQLIFE) scores and results of the Satisfaction with Life Scale (SWLS). Secondary outcomes include the Unified HD Rating Scale (UHDRS), Beck Hopelessness Scale (BHS), Stigma Scale for Chronic Illness (SSCI-8), Beck Depression Inventory-Fast Screen (BDI-FS) and Problem Behaviours Assessment for HD short Version (PBA-S). Results A total of 102 patients were included. The mean age (SD) was 53.1 (12.1) years and 56% were male. Most of the patients (99.0%) showed motor symptoms (87.3%), behavioural and psychiatric disturbances (59.8%), or cognitive impairment (20.6%). HDQLIFE domain score means (SD) includes concern with death and dying 45.97 (9.60) end-of-life planning 37.91 (8.84), and meaning and purpose 44.74 (9.05). SWLS score mean was 24.25 (7.33). Depressive symptoms were found in 37.4% of patients and moderate-to-severe feelings of hopelessness in 32.9%. The prevalence of stigma was 55.9% (n = 57). Conclusion HD impacted quality of life, with prevalent motor, psychiatric symptoms and cognitive impairment. Patient perspectives may provide complementary information to implement specific interventions.https://doi.org/10.1007/s40120-024-00655-0Huntington diseaseHealth-related quality of lifePatient-reported outcomesSatisfaction with LifeDepressionStigma |
| spellingShingle | Jesús Pérez-Pérez Sofía García-López Tamara Fernández Valle Cèlia Painous Maria Rosa Querol-Pascual Pedro J. García Ruiz Elena Bellosta Diago Esther Cubo Delgado Barbara Vives Pastor María Carmen Peiró Villaplana Idaira Martín Santana Marta Blázquez Estrada Matilde Calopa Garride Pablo Mir Carmen Álvarez Jorge Maurino Anna de Prado José Luis López-Sendón Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient’s Perspective Neurology and Therapy Huntington disease Health-related quality of life Patient-reported outcomes Satisfaction with Life Depression Stigma |
| title | Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient’s Perspective |
| title_full | Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient’s Perspective |
| title_fullStr | Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient’s Perspective |
| title_full_unstemmed | Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient’s Perspective |
| title_short | Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient’s Perspective |
| title_sort | huntington disease health related quality of life function and well being the patient s perspective |
| topic | Huntington disease Health-related quality of life Patient-reported outcomes Satisfaction with Life Depression Stigma |
| url | https://doi.org/10.1007/s40120-024-00655-0 |
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