Study Protocol for the Development of a European eHealth Platform to Improve Quality of Life in Individuals With Huntington's Disease and Their Partners (HD-eHelp Study): A User-Centered Design Approach

Background: Huntington's disease (HD) is an autosomal dominant neurodegenerative disease that affects the quality of life (QoL) of HD gene expansion carriers (HDGECs) and their partners. Although HD expertise centers have been emerging across Europe, there are still some important barriers to care provision for those affected by this rare disease, including transportation costs, geographic distance of centers, and availability/accessibility of these services in general. eHealth seems promising in overcoming these barriers, yet research on eHealth in HD is limited and fails to use telehealth services specifically designed to fit the perspectives and expectations of HDGECs and their families. In the European HD-eHelp study, we aim to capture the needs and wishes of HDGECs, partners of HDGECs, and health care providers (HCPs) in order to develop a multinational eHealth platform targeting QoL of both HDGECs and partners at home.Methods: We will employ a participatory user-centered design (UCD) approach, which focusses on an in-depth understanding of the end-users' needs and their contexts. Premanifest and manifest adult HDGECs (n = 76), partners of HDGECs (n = 76), and HCPs (n = 76) will be involved as end-users in all three phases of the research and design process: (1) Exploration and mapping of the end-users' needs, experiences and wishes; (2) Development of concepts in collaboration with end-users to ensure desirability; (3) Detailing of final prototype with quick review rounds by end-users to create a positive user-experience. This study will be conducted in the Netherlands, Germany, Czech Republic, Italy, and Ireland to develop and test a multilingual platform that is suitable in different healthcare systems and cultural contexts.Discussion: Following the principles of UCD, an innovative European eHealth platform will be developed that addresses the needs and wishes of HDGECs, partners and HCPs. This allows for high-quality, tailored care to be moved partially into the participants' home, thereby circumventing some barriers in current HD care provision. By actively involving end-users in all design decisions, the platform will be tailored to the end-users' unique requirements, which can be considered pivotal in eHealth services for a disease as complex and rare as HD.

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Rural Health Care Providers' Row to Hoe: Professional Quality of Life

PsycEXTRA Dataset ◽

10.1037/e518552007-001 ◽

2006 ◽

Author(s):

B. Hudnall Stamm ◽

Debra Larsen ◽

Kelly S. Davis

Keyword(s):

Quality Of Life ◽

Health Care ◽

Rural Health ◽

Health Care Providers ◽

Rural Health Care ◽

Care Providers ◽

Professional Quality Of Life ◽

Professional Quality

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Psychological risk factors that characterize the trajectories of quality of life after a physical trauma: a longitudinal study using latent class analysis

Quality of Life Research ◽

10.1007/s11136-020-02740-x ◽

2021 ◽

Author(s):

Eva Visser ◽

Brenda Leontine Den Oudsten ◽

Taco Gosens ◽

Paul Lodder ◽

Jolanda De Vries

Keyword(s):

Quality Of Life ◽

Latent Class Analysis ◽

Health Care Providers ◽

Psychological Health ◽

Latent Class ◽

Stress Disorder ◽

Class Analysis ◽

Care Providers ◽

Physical Trauma

Abstract Background The course and corresponding characteristics of quality of life (QOL) domains in trauma population are unclear. Our aim was to identify longitudinal QOL trajectories and determine and predict the sociodemographic, clinical, and psychological characteristics of trajectory membership in physical trauma patients using a biopsychosocial approach. Methods Patients completed a questionnaire set after inclusion, and at 3, 6, 9, and 12 months follow-up. Trajectories were identified using repeated-measures latent class analysis. The trajectory characteristics were ranked using Cohen’s d effect size or phi coefficient. Results Altogether, 267 patients were included. The mean age was 54.1 (SD = 16.1), 62% were male, and the median injury severity score was 5.0 [2.0—9.0]. Four latent trajectories were found for psychological health and environment, five for physical health and social relationships, and seven trajectories were found for overall QOL and general health. The trajectories seemed to remain stable over time. For each QOL domain, the identified trajectories differed significantly in terms of anxiety, depressive symptoms, acute stress disorder, post-traumatic stress disorder, Neuroticism, trait anxiety, Extraversion, and Conscientiousness. Discussion Psychological factors characterized the trajectories during 12 months after trauma. Health care providers can use these findings to identify patients at risk for impaired QOL and offer patient-centered care to improve QOL.

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

The Journal of Indian Orthodontic Society ◽

10.1177/03015742211023469 ◽

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.

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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

Journal of Clinical Medicine ◽

10.3390/jcm10051053 ◽

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.

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Patient Suffering in Chronic Digestive Diseases: Will Primary Care-Specialist Collaboration With Effective Interactive Communication and Integrative Medicine in the Plan of Care Improve Quality of Life?

Journal of Patient Experience ◽

10.1177/2374373520967798 ◽

2020 ◽

Vol 7 (6) ◽

pp. 989-993

Author(s):

Andrew Thomas ◽

Annie Thomas

Keyword(s):

Quality Of Life ◽

Primary Care ◽

Health Care ◽

Health Care Providers ◽

The United States ◽

Care Providers ◽

Physician Visits ◽

Digestive Diseases ◽

Care Specialist

Acute and chronic digestive diseases are causing increased burden to patients and are increasing the United States health care spending. The purpose of this case report was to present how nonconfirmatory and conflicting diagnoses led to increased burden and suffering for a patient thus affecting quality of life. There were many physician visits and multiple tests performed on the patient. However, the primary care physician and specialists could not reach a confirmatory diagnosis. The treatment plans did not offer relief of symptoms, and the patient continues to experience digestive symptoms, enduring this burden for over 2 years. The central theme of this paper is to inform health care providers the importance of utilizing evidence-based primary care specialist collaboration models for better digestive disease outcomes. Consistent with patient’s experience, the authors propose to pilot/adopt the integrative health care approaches that are proven effective for treating digestive diseases.

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

International Clinical Psychopharmacology ◽

10.1097/yic.0b013e3283534fa9 ◽

2012 ◽

Vol 27 (4) ◽

pp. 208-214 ◽

Cited By ~ 11

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

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

Disabilities ◽

10.3390/disabilities1020010 ◽

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.

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

Movement Disorders ◽

10.1002/mds.20208 ◽

2004 ◽

Vol 19 (11) ◽

pp. 1341-1348 ◽

Cited By ~ 35

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

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

10.5327/1516-3180.430 ◽

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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