| Title: |
Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient's Perspective |
| Authors: |
Pérez-Pérez, Jesús; García-López, Sofía; Fernández Valle, Tamara; Painous, Cèlia; Querol-Pascual, Maria Rosa; García-Ruiz, Pedro José; Bellosta Diago, Elena; Cubo, Esther; Vives-Pastor, Bárbara; Peiró Villaplana, María Carmen; Martín Santana, Idaira; Blázquez-Estrada, Marta; Calopa, Matilde; Mir, Pablo; Álvarez, Carmen; Maurino, Jorge; Prado, Anna de; López-Sendón, José Luis |
| Contributors: |
Roche; Pérez-Pérez, Jesús; García-López, Sofía; Mir, Pablo; Prado, Anna de; López-Sendón, José Luis; Consejo Superior de Investigaciones Científicas https://ror.org/02gfc7t72 |
| Publisher Information: |
Springer Nature; Adis International |
| Publication Year: |
2025 |
| Collection: |
Digital.CSIC (Consejo Superior de Investigaciones Científicas / Spanish National Research Council) |
| Subject Terms: |
Depression; Health-related quality of life; Huntington disease; Patient-reported outcomes; Satisfaction with Life; Stigma |
| Description: |
[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. ; This study and the journal’s Rapid Service fee is funded by Roche. ; Peer reviewed |
| Document Type: |
article in journal/newspaper |
| File Description: |
application/pdf |
| Language: |
English |
| Relation: |
The underlying dataset has been published as supplementary material of the article in the publisher platform at DOI https://doi.org/10.1007/s40120-024-00655-0; Pérez-Pérez, Jesús; García-López, Sofía; Fernández Valle, Tamara; Painous, Cèlia; Querol-Pascual, Maria Rosa; García-Ruiz, Pedro José; Bellosta Diago, Elena; Cubo, Esther; Vives-Pastor, Bárbara; Peiró Villaplana, María Carmen; Martín Santana, Idaira; Blázquez-Estrada, Marta; Calopa, Matilde; Mir, Pablo; Álvarez, Carmen; Maurino, Jorge; Prado, Anna de; López-Sendón, José Luis. 2025. Correction: Huntington Disease Health Related Quality of Life, Function and Well Being: The Patient’s Perspective. https://doi.org/10.1007/s40120-024-00683-w. https://hdl.handle.net/10261/391358; https://doi.org/10.1007/s40120-024-00655-0; Sí; https://hdl.handle.net/10261/391344; http://dx.doi.org/10.13039/100004337; https://api.elsevier.com/content/abstract/scopus_id/85205729451 |
| DOI: |
10.1007/s40120-024-00655-0 |
| DOI: |
10.13039/100004337 |
| Availability: |
https://hdl.handle.net/10261/391344; https://doi.org/10.1007/s40120-024-00655-0; https://doi.org/10.13039/100004337; https://api.elsevier.com/content/abstract/scopus_id/85205729451 |
| Rights: |
info:eu-repo/semantics/openAccess |
| Accession Number: |
edsbas.81077420 |
| Database: |
BASE |