To live with amyotrophic lateral sclerosis (ALS): health-related quality of life, and use and satisfaction with care during the course of the disease

Amyotrophic lateral sclerosis (ALS) is characterized by progressive muscular weakness and wasting, eventually leading to respiratory failure and death on average 2-4 years after diagnosis. There is no cure and clinical management focus on care to alleviate symptoms and to improve survival and health-related quality of life (HRQL).


The aims of the project are to describe and explore use and satisfaction with care, factors associated with HRQL in people with ALS and their next of kin during the course of disease, and experiences on mechanical ventilation, in order to facilitate person-centred care. The outcome will also be used for choice of variables in a national ALS quality registry.


The project had a longitudinal design with repeated data collections during home visits every 6 months up to 3 years after enrolment. Structured interviews were used for gathering information on socio-demographic and disease-related factors, and questionnaires for information on HRQL, disease severity, cognition, depression, fatigue, coping capacity and burden of care. Qualitative interviews were used for information on experiences of mechanical ventilation. In addition, a questionnaire based on the taxonomy of Ware was used for evaluation of satisfaction with care and healthcare services. Information on use of care and healthcare services was collected through central registers.


The outcome will provide knowledge needed for facilitation of person-centred care for people with ALS and their next of kin The gained knowledge will be used for continuous learning, quality improvement and management in all healthcare services.

Principal investigator

Marie Kierkegaard

Senior lecturer/physcial therapist

Scientific publications

Healthcare Utilisation and Satisfaction with Care in Patients with Amyotrophic Lateral Sclerosis - An Observational Study.
Kierkegaard M, Gottberg K, Johansson S, Littorin S, Sandstedt P, Ytterberg C, Holmqvist LW
J Neuromuscul Dis 2021 May;():

Experiences of next of kin to patients with amyotrophic lateral sclerosis using invasive ventilation via tracheostomy.
Gottberg K, Ytterberg C, Sandstedt P, Johansson S, Kierkegaard M
Disabil Rehabil 2019 Dec;():1-8

Disability and Contextual Factors in Patients with Amyotrophic Lateral Sclerosis - A Three-Year Observational Study.
Sandstedt P, Littorin S, Johansson S, Gottberg K, Ytterberg C, Kierkegaard M
J Neuromuscul Dis 2018 ;5(4):439-449

Caregiver experience, health-related quality of life and life satisfaction among informal caregivers to patients with amyotrophic lateral sclerosis: A cross-sectional study.
Sandstedt P, Littorin S, Cröde Widsell G, Johansson S, Gottberg K, Ytterberg C, et al
J Clin Nurs 2018 Dec;27(23-24):4321-4330

Predictors of health-related quality of life in people with amyotrophic lateral sclerosis.
Sandstedt P, Johansson S, Ytterberg C, Ingre C, Holmqvist LW, Kierkegaard M
J. Neurol. Sci. 2016 Nov;370():269-273