title: Health-related quality of life and unmet healthcare needs in Huntington's disease.
abstract: BACKGROUND
  Huntington's disease (HD) is a rare neurodegenerative disorder with a prevalence of 6 per 100.000. Despite increasing research activity on HD, evidence on healthcare utilization, patients' needs for healthcare services and Health-Related Quality of Life (HRQoL) is still sparse. The present study describes HRQoL in a Norwegian cohort of HD patients, and assesses associations between unmet healthcare and social support service needs and HRQoL.
  
  
  METHODS
  In this cross-sectional population-based study, 84 patients with a clinical diagnosis of HD living in the South-East of Norway completed the HRQoL questionnaire EuroQol, EQ-5D-3L. Unmet needs for healthcare and social support services were assessed by the Needs and Provision Complexity Scale (NPCS). Furthermore, functional ability was determined using the Unified Huntington's Disease Rating Scale (UHDRS) Functional assessment scales. Socio-demographics (age, gender, marital status, occupation, residence, housing situation) and clinical characteristics (disease duration, total functional capacity, comorbidity) were also recorded. Descriptive statistics were used to describe the patients' HRQoL. Regression analyses were conducted in order to investigate the relationship between unmet healthcare needs and self-reported HRQoL.
  
  
  RESULTS
  The patients were divided across five disease stages as follows: Stage I: n = 12 (14%), Stage II: n = 22 (27%), Stage III: n = 19 (23%), Stage IV: n = 14 (16%), and Stage V: n = 17 (20%). Overall HRQoL was lowest in patients with advanced disease (Stages IV and V), while patients in the middle phase (Stage III) showed the most varied health profile for the five EQ-5D-3L dimensions. The regression model including level of unmet needs, clinical characteristics and demographics (age and education) accounted for 42% of variance in HRQoL. A higher level of unmet needs was associated with lower HRQoL (β value - 0.228; p = 0.018) whereas a better total functional capacity corresponded to higher HRQoL (β value 0.564; p < 0.001).
  
  
  CONCLUSIONS
  The study findings suggest that patients with HD do not receive healthcare services that could have a positive impact on their HRQoL.
authors.affiliation: Centre for Habilitation and Rehabilitation Models and Services (CHARM), Institute for Health and Society, University of Oslo, P.O. Box 1130, Blindern, 0318, Oslo, Norway. r.m.v.walsem@medisin.uio.no.|Department of Neurohabilitation, Oslo University Hospital, P.O. Box 4950, Nydalen, 0424, Oslo, Norway. r.m.v.walsem@medisin.uio.no.
authors.forename: Marleen R
authors.initials: MR
authors.lastname: van Walsem
authors.affiliation: Centre for Habilitation and Rehabilitation Models and Services (CHARM), Institute for Health and Society, University of Oslo, P.O. Box 1130, Blindern, 0318, Oslo, Norway.|Department of Physical Medicine and Rehabilitation, Oslo University Hospital, P.O. Box 4950, Nydalen, 0424, Oslo, Norway.
authors.forename: Emilie I
authors.initials: EI
authors.lastname: Howe
authors.affiliation: Centre for Rare Disorders, Oslo University Hospital, Rikshospitalet, P.O. Box 4950, Nydalen, 0424, Oslo, Norway.
authors.forename: Gunvor A
authors.initials: GA
authors.lastname: Ruud
authors.affiliation: Institute of Health and Society, University of Oslo, P.O. Box 1130, Blindern, 0318, Oslo, Norway.|Department of Neurology, Oslo University Hospital, P.O. Box 4950, Nydalen, 0424, Oslo, Norway.
authors.forename: Jan C
authors.initials: JC
authors.lastname: Frich
authors.affiliation: Centre for Habilitation and Rehabilitation Models and Services (CHARM), Institute for Health and Society, University of Oslo, P.O. Box 1130, Blindern, 0318, Oslo, Norway.|Department of Physical Medicine and Rehabilitation, Oslo University Hospital, P.O. Box 4950, Nydalen, 0424, Oslo, Norway.
authors.forename: Nada
authors.initials: N
authors.lastname: Andelic
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006301:Health Services Needs and Demand / Q000706:statistics & numerical data*; D054625:Healthcare Disparities / Q000706:statistics & numerical data*; D006801:Humans; D006816:Huntington Disease / Q000523:psychology* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009664:Norway; D011788:Quality of Life / Q000523:psychology*; D012044:Regression Analysis; D057566:Self Report; D012944:Social Support; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: EQ-5D; Health-related Quality of Life; Healthcare needs; Healthcare services; Huntington’s disease; NPCS