scholarly journals PND59 Cross-Cultural Validation of the Huntington'S Disease Quality of Life Battery for Carers in France and Italy

2012 ◽  
Vol 15 (7) ◽  
pp. A556
Author(s):  
J. Dorey ◽  
A. Aubeeluck ◽  
F. Squitieri ◽  
E. Clay ◽  
E.J.N. Stupple ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cross Cultural ◽  
Cultural Validation

Adaptation and cross-cultural validation of the Spanish version of the Thyroid-Related Quality-of-Life Patient-Reported Outcome (ThyPRO) questionnaire

2018 ◽  
Author(s):  
Lleo Ana Maria Gonzalez ◽  
Mauro Boronat Cortes ◽  
Ulla Feldt-Rasmussen ◽  
Carlos Rodriguez Perez ◽  
AEse Krogh Rasmussen ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Patient Reported Outcome ◽  
Cross Cultural ◽  
Spanish Version ◽  
Patient Reported ◽  
Related Quality ◽  
Cultural Validation

scholarly journals Management of Traumatic Ulcerations of Lips in a Case of Huntington’s Disease: A Novel Application of Essix Retainer

2021 ◽  
pp. 030157422110234
Author(s):  
Mohamed Iqbal J
Keyword(s):  
Quality Of Life ◽  
Characteristic Feature ◽  
Neurodegenerative Disease ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Complete Resolution ◽  
Psychiatric Symptoms

Huntington’s disease is a progressive neurodegenerative disease characterized by motor, cognitive, and psychiatric symptoms. Dystonia of muscles is a characteristic feature of this condition. A case of Huntington’s disease, with orofacial dystonia, leading to severe uncontrolled biting of the lips, was referred by the Department of Neurology. Deep traumatic ulcerations were found in both upper and lower lips. A simple Essix retainer was fabricated and inserted, which acted as a barrier for the teeth from injuring the lips. The ulcers showed complete resolution in 3 to 4 weeks. The vacuum-formed retainers resulted in a good fit and resisted removal by the uncontrolled contortions of the orofacial muscles. The Essix retainer can be effectively used in improving the quality of life of patients, with Huntington’s disease, having such dystonia-related injuries to lips.

scholarly journals Psychometric Properties and Validation of the Polish Version of the 12-Item World Health Organization Disability Assessment Schedule 2.0 in Patients with Huntington’s Disease

2021 ◽  
Vol 10 (5) ◽  
pp. 1053
Author(s):  
Agnieszka Ćwirlej-Sozańska ◽  
Bernard Sozański ◽  
Mateusz Kupczyk ◽  
Justyna Leszczak ◽  
Andrzej Kwolek ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Huntington's Disease ◽  
Psychometric Properties ◽  
Huntington’S Disease ◽  
Scale Score ◽  
World Health ◽  
Disability Assessment ◽  
Whodas 2.0 ◽  
Score Reliability

Background: Huntington’s disease is a progressive neurodegenerative disorder that usually manifests in adulthood and is inherited in an autosomal dominant manner. The main aim of the study was to assess the psychometric properties of the 12-item WHO Disability Assessment Schedule (WHODAS) 2.0 in studying the level of disability in people with Huntington’s disease. Method: This is a cross-sectional study that covered 128 people with Huntington’s disease living in Poland. We examined scale score reliability, internal consistency, convergent validity, and known-group validity. The disability and quality of life of people with Huntington’s disease were also assessed. Results: The scale score reliability of the entire tool for the research group was high. The Cronbach’s α test result for the whole scale was 0.97. Cronbach’s α for individual domains ranged from 0.95 to 0.79. Time consistency for the overall result was 0.99 and for particular domains ranged from 0.91 to 0.99, which confirmed that the scale was consistent over time. All of the 12-item WHODAS 2.0 domains negatively correlated with all of the Huntington Quality of Life Instrument (H-QoL-I) domains. All correlation coefficients were statistically significant at the level of p < 0.001. The results obtained in the linear regression model showed that with each subsequent point of decrease in BMI the level of disability increases by an average of 0.83 points on the 12-item WHODAS 2.0 scale. With each subsequent year of the disease, the level of disability increases by an average of 1.39 points. Conclusions: This is the first study assessing disability by means of the WHODAS 2.0 in the HD patient population in Poland, and it is also one of the few studies evaluating the validity of the WHODAS 2.0 scale in assessing the disability of people with HD in accordance with the recommendations of DSM-5 (R). We have confirmed that the 12-item WHODAS 2.0 is an effective tool for assessing disability and changes in functioning among people with Huntington’s disease.

Validation of the first quality-of-life measurement for patients with Huntington’s disease

2012 ◽  
Vol 27 (4) ◽  
pp. 208-214 ◽  
Author(s):  
Emilie Clay ◽  
Annunziata De Nicola ◽  
Julie Dorey ◽  
Ferdinando Squitieri ◽  
Samuel Aballéa ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Life Measurement ◽  
Quality Of Life Measurement

scholarly journals Co-Design of Social Impact Domains with the Huntington’s Disease Community

Disabilities ◽  
2021 ◽  
Vol 1 (2) ◽  
pp. 116-131
Author(s):  
Natasha Layton ◽  
Natasha Brusco ◽  
Tammy Gardner ◽  
Libby Callaway
Keyword(s):  
Quality Of Life ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Design Process ◽  
Social Life ◽  
Social Inclusion ◽  
Social Impact ◽  
Service Providers ◽  
The Social

Background: For people living with or affected by Huntington’s Disease (HD) to experience a good quality of life, tailored support is required to meet physical, cognitive-behavioral, psychological, and social support needs. Substantial service and knowledge gaps regarding HD exist across support providers and service systems. Measuring unmet needs and what quality of life looks like is a fundamental step required to determine the social impact of service investment and provision. The objectives of this study were to validate and map a draft set of HD Social Impact Domains (HD-SID) against existing national and international outcome frameworks; and evaluate and finalize the HD-SID set using a co-design approach with people with lived experience of, and expertise in, HD. Methods: This research used a qualitative co-design process, with 39 participants across four stakeholder groups (people who were HD gene-positive, gene-negative family members, academics, peak organizations, and service providers) to: (i) map and verify the social life areas impacted by HD; (ii) undertake a rigorous three-phased, qualitative process to critically evaluate the draft HD-SID; and (iii) seek feedback on and endorsement of the HD-SID through this co-design process, with a final set of HD-SID identified. Results: Endorsed HD-SID comprised risks and safety (including housing stability, and economic sustainability) and social inclusion (including health and symptom management, physical wellbeing, emotional wellbeing, and building resilient relationships). Conclusions: Effective measurement of the impacts and outcomes for people with HD is informed by both extant measures and an understanding of the specific population needs. This qualitative co-design research demonstrates that HD-SID resonate with the HD community.

Health-related quality of life in Huntington's disease: A comparison of two generic instruments, SF-36 and SIP

2004 ◽  
Vol 19 (11) ◽  
pp. 1341-1348 ◽  
Author(s):  
Aileen K. Ho ◽  
Anna O.G. Robbins ◽  
Stephen J. Walters ◽  
Stephen Kaptoge ◽  
Barbara J. Sahakian ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Health Related Quality ◽  
Sf 36 ◽  
Related Quality ◽  
Health Related

scholarly journals Cross-cultural validation of the “DISABKIDS” questionnaire for quality of life among Colombian children with chronic diseases

2018 ◽  
Vol 26 (0) ◽  
Author(s):  
Nadia Carolina Reina-Gamba ◽  
Miguel Richart-Martinez ◽  
Julio Cabrero-García
Keyword(s):  
Quality Of Life ◽  
Children And Adolescents ◽  
Chronic Diseases ◽  
Cross Cultural ◽  
Health Related Quality ◽  
Validation Process ◽  
Related Quality ◽  
Health Related ◽  
Cultural Validation

ABSTRACT Objective: to describe the cross-cultural validation process of the “DISABKIDS” questionnaire in Colombia, for both the children and adolescents’ version and the parents’ version, an instrument intended to measure the health-related quality of life of Colombian children and adolescents with chronic diseases. Method: the cross-cultural validation process was conducted according to an international consensual systematic methodology, called standardized linguistic validation, to ensure linguistic equivalence with the original questionnaire. Results: the pretest’s cognitive interviews revealed a need to adjust the questionnaire, which consisted of asking for “health condition” rather than only asking for “condition”. Due to the cultural context, the word “condition” used in the original version, when translated to Spanish, refers to socioeconomic conditions rather than health conditions. For this reason, 11 items in the children’s version and eight items in the parents’ version were adjusted. Conclusions: the Colombian version of DISABKIDS-37 to measure health-related quality of life among children and adolescents with chronic diseases in both the children’s and parents’ versions is equivalent to the original version and is appropriate for use in Colombia. Future studies can assess the questionnaire’s psychometric properties.

scholarly journals PND46 The Huntington Quality of Life Instrument (H-QOL-I): Cross-Cultural Validation in Germany, Poland and USA

2011 ◽  
Vol 14 (7) ◽  
pp. A325-A326
Author(s):  
E. Clay ◽  
M. Mraidi ◽  
D. Zielonka ◽  
J. Cohen ◽  
M. Toumi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cross Cultural ◽  
Life Instrument ◽  
Cultural Validation

scholarly journals Therapeutic management of movement disorders present in Huntington’s Disease: a literature review

2021 ◽  
Author(s):  
Camila Angelo Vidal de Figueiredo ◽  
Kaline dos Santos Kishishita Castro ◽  
Sílvia Raimunda Costa Leite
Keyword(s):  
Quality Of Life ◽  
Literature Review ◽  
Adverse Effects ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Psychiatric Symptoms ◽  
Therapeutic Management ◽  
Abnormal Gait ◽  
Botulinum Toxin Injections

Background: Huntington’s Disease (HD) is a hereditary neurodegenerative genetic disease with motor, cognitive and behavioral repercussions that interferes in several areas of the patients’ lives. Therefore, to increase the quality of life for patients the therapeutic management of symptoms is necessary. Objective: to elucidate the main forms of treatment that reduces motor disorders present in HD. Methods: an integrative literature review was conducted using scientific articles published between 2016-2020 about this topic found in Pubmed and Google Scholar databases. Results: the chorea treatment in HD can be done using Tetrabenazine, deutetrabenazine or antipsychotics. During a study by the Huntington Study Group (HSG), tetrabenazine proved its efficacy, however, due to several adverse effects, its use was reduced. Thus, deutetrabenazine was created, which consists in a tetrabenazine deuterated version, with a longer half- life and less adverse effects. Studies by the HSG found that besides reducing chorea, it also improves motor function in general in patients. Antipsychotics are used when the patient has behavioral and psychiatric symptoms that prevent him from using the other drugs. The dystonia treatment involves physiotherapy and botulinum toxin injections, which are also used in the bruxism therapy, along with mouth protectors. Abnormal gait and balance problems can be reduced with psychomotor rehabilitation, physiotherapy, and using a walker. Conclusion: the control of HD motor symptoms is an important way to increase patients’ quality of life. Therefore, more studies are necessary to expand the effective therapeutic options.

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