[Family caregivers and hereditary muscular disorders: association between burden, quality of life and mental health]. / Les aidants familiaux des personnes myopathes: liaisons entre charge de soins, qualité de vie et santé mentale.

OBJECTIVE: The analysis of caregiver difficulties seems important to define the life of the patient. The aim of the study was to evaluate the burden of perceived care, health-related quality of life, and mental health of family caregivers who care for patients with hereditary neuromuscular disease. MATERIALS AND METHOD: Fifty-nine family caregivers were investigated in the course of multidisciplinary consultations in Reims, France, between April 2002 and February 2005. Burden of perceived care, mental functioning and mental health were measured by answers to an burden interview instrument (Zarit Burden Inventory, ZBI), a health-related quality of life questionnaire (the SF-36 and General Health Questionnaire-12 items [GHQ-12]), and an instrument quantifying anxiety and depression (Hospital Anxiety and Depression scale [HAD]). The domain scores of the SF-36 were compared with those of the general population. Non-parametric correlations between scores were calculated. RESULTS: Thirty-five parents, 20 spouses, a sister, a grandmother and two friends cared for patients with hereditary myopathy. The average age of caregivers was 50+/-11 years; 81% were women. Ten percent of the caregivers had above-normal scores on the HAD scale, One-third perceived a significant reduction in health-related quality of life, and more half expressed a moderate or important burden according to their answers on the ZBI. Depression occurred in a great proportion of the caregivers, and they showed reduced health-related quality of life as compared with the general population. CONCLUSION: Taking into account these results, it appears useful to set up a specific support for family caregivers of patients with hereditary myopathy.

Hereditary neuromuscular disease and multicomposite subjective health status: feasibility, internal consistency and test-retest reliability in the French version of the Nottingham Health Profile, the ISPN.

OBJECTIVE: To evaluate the feasibility, internal consistency and reproducibility of the French version of the Nottingham Health Profile (NHP) completed by adults with hereditary neuromuscular disease. DESIGN: Cross-sectional study with evaluation at 15 +/- 7 days for NHP test-retest. SETTING: Multidisciplinary rehabilitation consultations in Reims. SUBJECTS: Sixty-four neuromuscular disease outpatients completed the NHP consecutively between April 2002 and December 2003. MAIN MEASURES: French version of the Nottingham Health Profile (NHP), Barthel Index and sociodemographic characteristics. RESULTS: The average completion percentages for the different dimensions was 84.2% (range 72-97%). With respect to the completion feasibility of the physical mobility subscale, 7-20% of neuromuscular disease patients failed to complete four items out of eight. In the pain subscale, the same difficulty was encountered for three items out of eight. Internal consistency as assessed by Cronbach's alpha was acceptable for the subscales physical mobility (0.88), emotional reaction (0.74), sleep (0.77), and pain (0.81); it was less reliable for the subscale social isolation (0.61), and poor for the subscale energy (0.47). Test-retest agreement measured by intraclass correlation coefficient was in all instances greater than 0.70. CONCLUSION: Some items in the pain and physical mobility subscales pose problems related to the relevance of the wording for patients confined to wheelchairs. Recoding of the measure makes it possible to avoid missing data from these dependent patients. Scores differ statistically according to the coding used. In study reports, details of such procedures should be provided for comparison of ISPN results with those from other studies in the literature.