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- W2975808625 abstract "Dysferlinopathy presents with heterogeneity in muscle involvement, posing significant challenges selecting outcome measures as well as significant functional and quality of life challenges for patients. In an international natural history study, 204 patients with dysferlinopathy were evaluated annually using 4 Patient reported outcome measures (PROMs)– Individualised quality of life measure for neuromuscular disease (INQOL), ACTIVLIMand International physical activity questionnaire (IPAQ). For non-ambulant patients the Egan Klassifikation Scale (EK) was used. INQOL aims to capture the impact of muscle disease on areas of life including activities, independence, relationships, and body image. The IPAQ assesses physical activity through hours of activity at different levels of intensity. The ACTIVLIM measures activity limitations using a three-point grading system (impossible, difficult and easy). The EK is a patient assessment of their own functioning related to daily tasks. Functional ability was assessed using the North Star Assessment for dysferlinopathy (NSAD), timed rise from floor, 10 metre walk / run and timed UP & Go test. We evaluated the suitability of these PROMs in this cohort over three years and examined their relationship with functional ability. Data were analysed for each visit and the entire study. For rating scales (ACTIVLIM, EK, INQOL) we conducted modern psychometric analysis (RUMM2030 software) to assess item fit, person fit, targeting, dependency and reliability. Not all measures were suitable measurement tools in this disease cohort, particularly the INQOL. The ACTIVLIM was more robust. Several of the measures demonstrated longitudinal change. Regulatory authorities require patient relevant measures that are associated with functional measures. Relationships between PROMS and functional tests were discovered in this cohort. Further work is required to adapt these PROM's or alternative measures need to be selected." @default.
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- W2975808625 date "2019-10-01" @default.
- W2975808625 modified "2023-09-23" @default.
- W2975808625 title "P.177Measuring what matters in dysferlinopathy – linking functional ability to patient reported outcome measures" @default.
- W2975808625 doi "https://doi.org/10.1016/j.nmd.2019.06.232" @default.
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