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. 2025 Oct;34(10):2731-2744.
doi: 10.1007/s11136-025-04012-y. Epub 2025 Jun 25.

Health-related quality of life of informal carers in ALS: a systematic review of person reported outcome measures

Rosie Bamber  1 , Theocharis Stavroulakis  1 , Christopher McDermott  1 , Jill Carlton  2
Affiliations

Health-related quality of life of informal carers in ALS: a systematic review of person reported outcome measures

Rosie Bamber et al. Qual Life Res. 2025 Oct.

Abstract

Purpose: Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative condition with swift progression. The devastating impact of ALS affects the health-related quality of life (HRQoL) of informal carers. Various person reported outcome measures (PROMs) have been used to assess HRQoL in informal carers in ALS, yet their validity remains unclear. This review aimed to identify and evaluate the content validity of HRQoL PROMs for informal carers in ALS.

Methods: This review was conducted according to best practice COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) methodology. Two literature searches were conducted in November 2023 and April 2024 across MEDLINE, PsycINFO, Embase, CINAHL, the Cochrane Database of Systematic Reviews, CENTRAL and Google Scholar, to identify HRQoL PROMs used with informal carers in ALS, PROM development articles, and psychometric literature. Evidence synthesis followed COSMIN guidance.

Results: 12,276 articles were screened, and 109 PROMs were identified, with 43 undergoing full COSMIN assessment. Content validity ratings were 'Inconsistent' or 'Insufficient' for all PROMs. All PROMs, except the CarerQoL, were rated 'Insufficient' for comprehensiveness. Only 18.6% of PROMs included informal carers in development. Quality of evidence supporting content validity ratings was 'Very Low' for 93% of PROMs.

Conclusion: HRQoL PROMs used with informal carers in ALS lack evidence to support their content validity, restricting their utility for this purpose. Existing literature on the impact of caring in ALS on informal carers' HRQoL should be interpreted cautiously. Further research is required to establish the content validity of HRQoL PROMs used for this cohort.

Keywords: Amyotrophic lateral sclerosis; COSMIN; Carers; Caring; Content Validity; Health-related quality of life; Motor neuron disease; Person reported outcome measure; Quality of life; Systematic review.

Plain language summary

Amyotrophic Lateral Sclerosis (ALS) is an incurable condition that worsens quickly and leads to death. It has a huge impact on the quality of life of those who provide care to someone with ALS. Various questionnaires have been used to measure quality of life in informal carers in ALS, but it is unclear whether these questionnaires truly reflect carers’ experiences. This review examines whether these questionnaires are suitable for measuring informal carers’ quality of life in ALS. Our findings show that there is insufficient evidence for the use of existing questionnaires with ALS carers, which restricts their usefulness in clinical and research contexts. As a result, current research that reports experiences of ALS carers using existing questionnaires is limited and should be interpreted with caution.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: Not applicable, the current review did not require institutional ethical approval. Consent for publication: Not applicable, the current review did not require participant consent to publish data. Competing interests: R. Bamber and C. McDermott have received funding through a National Institute for Health and Care Research (NIHR) Career Development Award awarded to C. McDermott (Award ID: NIHR301648) and the NIHR Sheffield Biomedical Research Centre. T. Stavroulakis and J. Carlton report no relevant disclosures. Consent to participate: Not applicable, the current review did not require participant consent.

Figures

Fig. 1
Fig. 1
PRISMA-COSMIN Diagram Flowchart adapted according to PRISMA-COSMIN template [26] for Search 1, pertaining to full texts meeting eligibility criteria, and Search 2, pertaining to articles filtered for measurement properties.Reasons for record exclusions: (1) Title and abstract not written in English in a peer-reviewed journal. (2) Not a primary research article with full-text available. (3) Participants are not adult informal carers ≥ 18. (4) Participants are not informal carers for individuals with ALS. (5) HRQoL, or domain/s of HRQoL are not assessed by a freely available, multi-item outcome measurement tool. (6) Articles with mixed syndromic groups have < 75% ALS informal carers, or separate data is not available for ALS informal carers. (7) Not a development article of a HRQoL measure used with ALS informal carers, does not report data on the content validity of HRQoL measures, does not use HRQoL measures with ALS carers and report HRQoL scores. (8) HRQoL PROM development article could not be sourced or unavailable in full text in English. CENTRAL = Cochrane Central Register of Controlled Trials. CINAHL = Cumulative Index of Nursing and Allied Health Literature. COSMIN = Consensus-based Standards for the Selection of Health Measurement Instruments. HRQoL = Health-related quality of life. MEDLINE = Medical Literature Analysis and Retrieval System Online. OMI = Outcome Measurement Instrument. PRISMA = Preferred Reporting Items for Systematic Reviews and Meta-Analyses. PROM = Person Reported Outcome Measure
Fig. 2
Fig. 2
Frequency diagram illustrating PROMs with highest overall content validity and highest frequency of use within articles in this review. HADS = Hospital Anxiety & Depression Scale, RAND-36 = Rand 36-Item Health Survey, SF-36 = Short Form-36, SF-36 MCS = Short Form-36 Mental Component Summary, SF-36 V2 = Short Form-36 Version 2, WHOQOL-BREF = World health organisation quality of life-BREF
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