Disability and health-related quality of life in patients with amyotrophic lateral sclerosis, and caregiving experience from the perspective of next of kin

Background: Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease leading to progressive muscle weakness resulting in respiratory failure and death. The average survival time from diagnosis is two to three years. The use of tracheostomy invasive ventilation (TIV) may, however, prolong life by several years. ALS has a major impact on the lives of both the patients and their next of kin/ informal caregivers. There is no cure for ALS and the cornerstone of management is symptomatic treatment to alleviate symptoms and improve health-related quality of life (HRQL). Aims: The aims of this thesis were to explore aspects of disability, contextual fac- tors and HRQL in patients with ALS, and to explore the caregiving experiences and HRQL in their next of kin/informal caregivers. In addition, this thesis aimed to investigate the experiences of being next of kin to patients with ALS undergoing TIV. Material and methods: Sixty patients with ALS were included at baseline (Study I) and followed-up every 6 months for three years, unless participants had deceased or declined participation (Study II). Data on disability, contextual factors and HRQL were collected from medical records, and by study-specific protocols and standardized questionnaires administrated during home visits. Forty-nine next of kin/informal caregivers to patients with ALS were included to explore caregiving experience, HRQL and life satisfaction (Study III). Data were collected by study- specific protocols and standardized questionnaires. Multivariate regression analy- ses were used to explore factors associated with patients’ HRQL (Study I) and informal caregivers’ HRQL and life satisfaction (Study III). Descriptive statistics and correlation analyses were used to present and explore data on disability and contextual factors over three years (Study II). Eight next of kin were included to investigate the experiences of being next of kin to patients with ALS undergoing TIV (Study IV). Semi-structured face-to-face interviews were conducted and ana- lyzed with qualitative content analysis with an inductive approach. Results: Regardless of disease severity, fatigue, anxiety, depression and pain were commonly and concurrently present in patients with ALS. Furthermore, activity limitations and participation restrictions were frequently reported. The health con- dition, i.e. high disease severity; the impairments fatigue, anxiety and/or depres- sion; participation restrictions, i.e. low frequency of social and life style activities; and the contextual factors weak coping capacity and mechanical ventilator use (non-invasive and TIV) were associated with worse HRQL in patients with ALS. Both positive and negative caregiver experiences were reported by the informal caregivers. Positive experience and older age in the informal caregiver were associated with better HRQL, while negative caregiving experiences and anxiety and/or depression in the patient with ALS were associated with worse HRQL in informal caregivers. As for life satisfaction, older age in the informal caregiver and not cohabiting with the patient were associated with being satisfied with “life as a whole”. Being next of kin to patients with ALS undergoing TIV involved experi- ences of a turbulent care process aiming to extend life, a struggle to cope with the strains of everyday life, and of conflicting roles as next of kin and carer. Conclusion: Patients with ALS need, throughout the course of the disease, to be regularly screened for commonly present impairments, activity limitations, partici- pation restrictions and perceived HRQL, so that person-centred care can be applied at the right time. There is a need to consider the individual caregiver’s experience when planning services, care and support. Furthermore, it is important to adopt person-centred care, not only for patients but also for their informal caregivers, as factors related to both parties were associated with the informal caregivers’ HRQL and life satisfaction. It is important to involve next of kin to patients with ALS undergoing TIV throughout the whole care process, and to consider the specific needs of the next of kin. Furthermore, specific support interventions for next of kin to facilitate their everyday life and to ease their burden need to be developed