Associations between illness cognitions and health-related quality of life in the first year after diagnosis of amyotrophic lateral sclerosis.

OBJECTIVE: To describe illness cognitions among patients with amyotrophic lateral sclerosis (ALS), to study cross-sectional associations between illness cognitions and health-related quality of life (HRQoL) and to study the predictive value of illness cognitions measured shortly after the diagnosis for HRQoL at follow-up. METHODS: Prospective longitudinal design. We administered Self-report questionnaires at study onset (n = 72) and follow-up (n = 48). Median follow-up period was 10.0 months. At baseline median ALS Functional Rating Scale-Revised was 43, median time since onset of symptoms was 13.6 months, 79% of patients presented with spinal onset. Illness cognitions Helplessness, Acceptance and Disease Benefits were measured with the Illness Cognitions Questionnaire (ICQ) and HRQoL with the ALS Assessment Questionnaire (ALSAQ-40). Correlational and regression analyses were used. RESULTS: Patients experienced more Helplessness at follow-up. We found no significant changes in Acceptance or Disease Benefits at follow-up. In cross-sectional analyses, Helplessness was independently related to worse HRQoL at baseline (ß = 0.44; p = .001) and Acceptance and Disease Benefits were independently related to worse HRQoL at follow-up (ß = -0.17, p = .045) and (ß = -0.186, p = .03 respectively). Longitudinal analyses showed that, adjusted for disease severity at baseline, Helplessness at baseline was a predictor of worse HRQoL at follow-up (ß = 0.43; p = .006). None of the illness cognitions were a significant predictor of HRQoL with adjustment for baseline HRQoL. CONCLUSION: Helplessness was independently associated with HRQoL in the cross-sectional and longitudinal analyses. These results can help us identify patients shortly after diagnosis who might benefit from psychological interventions.

Participation and mental well-being of mothers of home-living patients with spinal muscular atrophy.

Proximal spinal muscular atrophy (SMA) causes severe physical limitations but also has a major impact on the lives of parents. The aim of this study was to investigate participation and mental well-being (burden, emotional distress and satisfaction with participation) of parents of home-living patients with SMA. Caregiver burden was assessed with the Caregiver Strain Index, emotional distress with the Hospital Anxiety and Depression Scale and satisfaction with participation with the Utrecht Scale for Evaluation of Rehabilitation-Participation. Because the majority of parents were mothers of home-living SMA patients (76%), further analyses were restricted to mothers. Seventy-seven percent of mothers of patients with SMA had paid work. A substantial proportion of mothers (76%) perceived high caregiver burden. Burden, emotional distress and satisfaction with participation were comparable between mothers of children and mothers of adults with SMA. Caregivers' participation in leisure activities was significantly related to their perceived level of caregiver burden, emotional distress and satisfaction with participation. Mothers engaging in more social and leisure activities reported lower emotional distress and caregiver burden. Considering the high level of burden attention should be paid to mental well-being of primary caregivers of patients with SMA. Caregivers should be motivated to keep participating in social/leisure activities.