By following participants for two years we want to map out what changes are taking place in employed gene carriers of Huntington's disease. The research does not solely focus on whether a participant is employed or not, but wants to highlight…
ID
Source
Brief title
Condition
- Movement disorders (incl parkinsonism)
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
- To investigate changes in employment (e.g. a job change, work cessation/early
retirement, changes in working hours, and changes in duties/responsibilities)
in HD, with a focus on improving the understanding of reasons behind work
cessation.
- To relate possible work related changes to psychiatric, behavioural,
cognitive and motor (dys)function.
Outcome measures questionnaires.
- Work assessment: detailed demographics on employment and work cessation.
This questionnaire is filled in by the participant as well as the proxy.
- UBOS: overall total score and score per subscale; exhaustion, emotional
distance and competence score. The test can be used to assess whether someone
has a burn-out or not. This questionnaire is filled in by the participant as
well as the proxy.
- FrSBe: overall total score and total score per subscale; apathy,
disinhibition, and executive dysfunction. This questionnaire is filled in by
the participant as well as the proxy.
- UCL: total scores per coping subscale; active and passive coping (7 * 28),
palliation (8 * 32), avoidance (8 * 32), seeking support (6 * 24) , emotional
expression (3 * 12) and seeking reassurance (5 * 20). This questionnaire is
filled in by the participant as well as the proxy.
- HADS-SIS: score per subscale; anxiety (0 * 21), depression (0 * 21), inward-
and outward irritability (0 * 12). This questionnaire is filled in by the
participant as well as the proxy.
Outcome measures motor assessments
- UHDRS-TMS: total motor score (0 * 124)
- UHDRS-TFC: total functional capacity (0 * 13)
Outcome measures psychiatric and behavioural evaluation.
- PBA-s: total score per symptom is calculated by multiplying severity by
frequency.
Outcome measures neuropsychological assessment.
- SDMT: total number of correct responses after 90 seconds.
- Stroop: number of correct response after 45 seconds per trial.
- TMT trial A and B: total seconds to complete the task.
Secondary outcome
- Determining whether burn out related symptoms are more prevalent in patients
with HD than in the general population and ascertain in how many cases burn-out
related symptoms can be marked as the onset of HD.
- Report if work type and cultural background of participants influences
employment and how this relates to cognitive, psychiatric and motor
(dys)function.
- Identifying whether limited self-insight influences work cessation by means
of proxy measurement.
- To contribute to better supply of information for patients, family,
employers, health and safety officers and offices regarding employment and
Huntingtons disease progression.
- Identifying whether coping influences working capacity.
For outcome measures see primary study parameters.
Background summary
Huntington*s disease (HD) is a rare autosomal dominant inherited
neurodegenerative disorder, clinically characterized by motor, cognitive and
behavioral symptoms. Due to the relative early age at onset patient* quality of
life is impacted, especially by loss of functionality. Patients at our
outpatient clinic often encounter work related changes or become incapable of
working. Previous retrospective research has shown that work cessation is
associated with cognitive dysfunction and apathy. However, patients and
employers do not seem to assign the work related problems to Huntington*s
Disease. This leads to faulty decision making such as not renewing a patients
contract or the patient enters the WW (Dutch unemployment law), instead of
ending up in the Sickness Benefit Act.
Study objective
By following participants for two years we want to map out what changes are
taking place in employed gene carriers of Huntington's disease. The research
does not solely focus on whether a participant is employed or not, but wants to
highlight which changes lead up to unemployment. Therefore we will collect a
lot of demographical information on working status and work problems
participants encounter, consisting of but not limited to the prevalence of
burn-out in HD compared to the general population. We want to relate this
information to cognitive, psychiatric and motor symptoms of HD. This study aims
to provide more insight into the work-related problems that patients encounter
at an early stage of Huntington's disease and will thereby contribute to
finding leads for further research and (better) support at the psychosocial
level.
Study design
The current study is a retrospective and prospective longitudinal observational
study in Huntington*s Disease gene carriers.
Study burden and risks
Since this study is an observational study in which no interventions take
place, there are no risks associated with the study. The burden on the
participants is limited.
Albinusdreef 2
Leiden 2333ZA
NL
Albinusdreef 2
Leiden 2333ZA
NL
Listed location countries
Age
Inclusion criteria
All eligible participants must meet the following inclusion criteria:
- Have a confirmed CAG-expansion of *36 in the HTT gene.
- Be employed, or have been employed 2 years prior to assessment, in any capacity (e.g. full time (paid job for 36 hours or more), part-time (paid job for less than 36 hours) or volunteer (unpaid job)).
- Be at least 18 years of age.
- Be at most 64 years of age, due to the longitudinal set up of the research and the age of retirement in the Netherlands.
Exclusion criteria
All eligible participants must meet none of the following exclusion criteria:
- Major general or neurological comorbidity that is unrelated to HD.
- Any other condition that in the opinion of the investigator warrants exclusion of the study.
- Inability to understand the information about the study.
Design
Recruitment
metc-ldd@lumc.nl
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL67070.058.18 |