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Review
. 2025 Jun 25.
doi: 10.1007/s11136-025-04012-y. Online ahead of print.

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
Free article
Review

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. .
Free article

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.

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