Health-Related Quality of Life in Spinal Muscular Atrophy Patients and Their Caregivers-A Prospective, Cross-Sectional, Multi-Center Analysis.

caregiver caregiver burden mental health patient reported outcome measures quality of life spinal muscular atrophy

Journal

Brain sciences
ISSN: 2076-3425
Titre abrégé: Brain Sci
Pays: Switzerland
ID NLM: 101598646

Informations de publication

Date de publication:
07 Jan 2023
Historique:
received: 28 11 2022
revised: 29 12 2022
accepted: 03 01 2023
entrez: 21 1 2023
pubmed: 22 1 2023
medline: 22 1 2023
Statut: epublish

Résumé

Spinal muscular atrophy (SMA) is a disabling disease that affects not only the patient’s health-related quality of life (HRQoL), but also causes a high caregiver burden (CGB). The aim of this study was to evaluate HRQoL, CGB, and their predictors in SMA. In two prospective, cross-sectional, and multi-center studies, SMA patients (n = 39) and SMA patient/caregiver couples (n = 49) filled in the EuroQoL Five Dimension Five Level Scale (EQ-5D-5L) and the Short Form Health Survey 36 (SF-36). Caregivers (CGs) additionally answered the Zarit Burden Interview (ZBI) and the Hospital Anxiety and Depression Scale (HADS). Patients were clustered into two groups with either low or high HRQoL (EQ-5D-5L index value <0.259 or >0.679). The latter group was mostly composed of ambulatory type III patients with higher motor/functional scores. More severely affected patients reported low physical functioning but good mental health and vitality. The CGB (mean ZBI = 22/88) correlated negatively with patients’ motor/functional scores and age. Higher CGB was associated with a lower HRQoL, higher depression and anxiety, and more health impairments of the CGs. We conclude that patient and CG well-being levels interact closely, which highlights the need to consider the health of both parties while evaluating novel treatments.

Identifiants

pubmed: 36672091
pii: brainsci13010110
doi: 10.3390/brainsci13010110
pmc: PMC9857112
pii:
doi:

Types de publication

Journal Article

Langues

eng

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Auteurs

Camilla Wohnrade (C)

Department of Neurology, Hannover Medical School, 30625 Hannover, Germany.

Ann-Kathrin Velling (AK)

Department of Neurology, Hannover Medical School, 30625 Hannover, Germany.

Lucas Mix (L)

Department of Neurology, University of Ulm, 89081 Ulm, Germany.

Claudia D Wurster (CD)

Department of Neurology, University of Ulm, 89081 Ulm, Germany.

Isabell Cordts (I)

Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, 81675 Munich, Germany.

Benjamin Stolte (B)

Department of Neurology and Center for Translational Neuro- and Behavioral Science, University Medicine Essen, 45147 Essen, Germany.

Daniel Zeller (D)

Department of Neurology, University of Wuerzburg, 97080 Wuerzburg, Germany.

Zeljko Uzelac (Z)

Department of Neurology, University of Ulm, 89081 Ulm, Germany.

Sophia Platen (S)

Department of Neurology, University of Ulm, 89081 Ulm, Germany.

Tim Hagenacker (T)

Department of Neurology and Center for Translational Neuro- and Behavioral Science, University Medicine Essen, 45147 Essen, Germany.

Marcus Deschauer (M)

Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, 81675 Munich, Germany.

Paul Lingor (P)

Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, 81675 Munich, Germany.

Albert C Ludolph (AC)

Department of Neurology, University of Ulm, 89081 Ulm, Germany.
German Center for Neurodegenerative Diseases, 89081 Ulm, Germany.

Dorothée Lulé (D)

Department of Neurology, University of Ulm, 89081 Ulm, Germany.

Susanne Petri (S)

Department of Neurology, Hannover Medical School, 30625 Hannover, Germany.

Alma Osmanovic (A)

Department of Neurology, Hannover Medical School, 30625 Hannover, Germany.
Essen Center for Rare Diseases (EZSE), University Hospital Essen, 45147 Essen, Germany.

Olivia Schreiber-Katz (O)

Department of Neurology, Hannover Medical School, 30625 Hannover, Germany.

Classifications MeSH