Our study suggests that promoting positive experience and providing services and support to reduce negative aspects of caregiving might be important strategies for healthcare personnel to improve informal caregivers' health.

Background:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by muscle weakness and wasting. Observational natural history studies can give information on body function/structure impairments, activity limitations and participation restrictions, i.e. disability. Information needed to plan and develop care and support.Objective:To describe and explore disease severity and impairments, activity limitations, participation restrictions and contextual factors over time. In specific, to explore concurrent presence of cognitive impairment, fatigue, anxiety, depression and pain, and whether these impairments were related to disease severity.Methods:In this three-year observational study, 60 patients with ALS were included at baseline. Follow-ups were performed every 6 months unless participants had deceased or declined participation. Data was collected from medical records, and by study-specific and standardized questionnaires administrated during home visits.Results:Regardless of disease severity; fatigue, anxiety, depression and pain were present in patients with ALS at each data collection. Approximately one-third experienced two or more of these impairments concurrently, i.e. at the same time point. Cognitive impairment could not be assessed in many patients due to their physical impairments. Disease severity was not associated with fatigue, anxiety, depression or pain.Conclusions:Patients with ALS need, throughout the course of the disease, to be regularly screened for commonly present impairments, activity limitations and participation restrictions so that person-centered interventions can be applied at the right time.

Aims Welander distal myopathy (WDM) and myotonic dystrophy type 1 (DM1) are conditions characterized by gradually impaired hand function. Measurement of hand function is therefore important in therapy for patients. However, to date reliability of common hand function test instruments for these patients has not been evaluated. The aim of the study was to test intra-rater reliability (test-retest) of the hand function testing instruments: the Grippit®, Grip Ability Test (GAT), hand-held myometer (Microfet2TM) and Purdue Pegboard in patients with WDM and DM1. In addition, inter-rater reliability of these instruments was tested in DM1 patients. Methods For test-retest, the two patient groups (16 patients in each group) were tested on 2 consecutive weeks, on the same day of the week and at the same time of day. During the second week, the DM1 patients were randomly tested either before or after retest for inter-rater reliability. Results Mainly good–very good (intra-class correlation coefficient≥0.61) intra-rater and inter-rater reliability were found in three out of four instruments tested: the Purdue Pegboard, Grippit and the hand-held myometer for both WDM and DM1 patients. Conclusions The instruments are thus considered reliable for evaluating hand function in patients with WDM and DM1. However, the GAT and pinch grip measured with the Grippit® need to be interpreted with caution, as the reliability of these instruments varied from fair to good.

Background: Patients with amyotrophic lateral sclerosis (ALS) need a large amount of healthcare services. Knowledge on use of and satisfaction with healthcare is, however, scarce. Objective: The objectives were to explore use and satisfaction of healthcare in patients with ALS. Methods: The sample consisted of patients with ALS, recruited from the ALS clinic at the Karolinska University Hospital, Stockholm, Sweden, participating in a three-year observational study. Data on healthcare utilisation were retrieved from the computerised register at Region Stockholm, Sweden. Information regarding disability, contextual factors and satisfaction with care was collected by home visits. Results: Over time, half, or less of the patients used inpatient care, whereas all used outpatient care. Half of all outpatient contacts were with providers of advanced healthcare in the home and one-fifth with allied health professionals. Nurses performing home visits composed the largest proportion of outpatient contacts. A small amount of the utilised outpatient care emerged from the ALS clinic. Patients with severe disease and longer time since diagnosis had fewer contacts with the ALS clinic. Satisfaction with care was in general stable over time with around two-thirds or more of patients being satisfied. Most patients wanted to participate in care planning, but few had. Conclusion: Patients with ALS use hospital-based specialist care and other outpatient care in parallel with many healthcare providers involved. Our findings highlight the need for implementation of person-centred care to improve both coordination of care, care transitions and satisfaction with healthcare services.