Aerobic exercise and congnitive behavioural therapy in ALS: optimzing quality of life

Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal progressive adult-onset neurodegenerative motor neuron disease (MND). In the absence of an effective treatment, ALS care focuses on symptom treatment, optimizing functioning (daily activities and social participation) and maintaining quality of life (QoL). Current evidence based guidelines for the management of ALS lack sufficient evidence regarding psychological interventions and aerobic exercise training in addition to usual care. The general aims of this thesis were to expand the knowledge on optimizing of body functions, activities, participation and QoL in patients with ALS. The first part of this thesis concerns research aimed at measuring changes in aerobic capacity during the disease course, gaining insight in the determinants and prevalence of participation restrictions in ALS patients and reviewing the evidence on psychological factors related to QoL in ALS. Subsequently, in the second part of this thesis, the design and results of a trial to study the effectiveness of aerobic exercise therapy (AET) and cognitive behavioural therapy (CBT) to optimize functioning and health-related QoL (HRQoL) in ambulatory patients with ALS were presented (i.e. the FACTS-2-ALS study). The main conclusions and recommendations are: • Changes in aerobic capacity in ambulatory patients with ALS could not be successfully monitored due to a diminished feasibility of the Åstrand-Ryhming (ÅR) test protocol. During the disease course, it is, therefore, recommended to assess physical activity, using alternative measures, such as accelerometers. • Ambulatory ALS patients perceive participation restrictions, mainly explained by physical functioning, however, psychological factors contributed also to a small part of variance. Participation restrictions may be influenced if early ALS care is directed toward lung capacity, functional mobility, fatigue, and feelings of helplessness. • Higher levels of anxiety and depression are related to a poorer health-related QoL, whereas higher levels of religiosity appeared to be related to better global QoL. Associations might change during the disease course. Further research is needed to supplement the available evidence and to investigate how psychological factors can be modified to improve QoL in ALS patients. • There is currently insufficient evidence to recommend joint patient-carer cognitive behavioural therapy (CBT) sessions to be included in early-stage usual care, since there was too little evidence of CBT improving QoL in in a selected group of psychologically distressed patients with ALS and their caregivers. Future studies might focus more on the differences in needs between patients and caregivers and tailor the CBT intervention to these different needs. • To improve recruitment rates in future ALS interventions, one might consider home based data collection and Internet-based interventions, a broader range of eligibility criteria, a longer study duration and more participating sites. • 16-weeks of moderate-to-vigorous intensity aerobic exercise therapy (AET) was not recommended to be included in usual care (UC) since AET in addition to UC was not superior to UC alone in preserving HRQoL, and perceived as too burdensome in early-stage ambulatory ALS patients. AET may preserve respiratory function, global function, and disease-specific HRQoL in ALS patients with a relative slow disease progression and a longer survival probability at follow up. ALS patients, who wish to participate in AET, can be reassured that AET will not worsen their disease.