Informed Decision-Making in Amyotrophic Lateral Sclerosis: Supporting the Patient’s Journey

Amyotrophic lateral sclerosis (ALS) is a devastating, fatal neurodegenerative disease with a high variability of both symptoms and associated life expectancy. People with ALS, and their families, are faced with the prospect of unrelenting, progressive loss of function and a (severely) limited life expectancy. On their journey with ALS, the unrelenting, progressive nature of ALS necessitates many complex, time-sensitive decisions. Shared decision-making allows healthcare professionals (HCPs) to support people with ALS, and their families, to make timely decisions that are in line with their preferences and needs. This thesis investigates informed decision-making from the perspective of people with ALS, their caregivers, and HCPs, to better support them during the course of their disease.
The studies in this thesis provide HCPs with information that will allow them to offer person-centered care that supports the autonomy and control of people with ALS by personalizing information to individual needs and preferences, promoting patient choice, and involving family caregivers. Chapter two describes the development of a communication guide to discuss a more personalized prognosis based on a prediction model. We followed this up with a qualitative study of the experiences of people with ALS, their families and HCPs with discussing a more personalized prognosis (Chapter 3). This study shows that a more personalized prognosis can help alleviate uncertainty in people with ALS – who want to know – and their families, allow them to regain control over their future, and make the most of the time they have left. However, it is important to tailor discussion to individual needs and preferences. Thus, discussion of a more personalized prognosis in ALS helps to support a more person-centered care.
Shared decision-making is the pinnacle of person-centered care and more complex in ALS compared to other chronic or terminal diseases due to a lack of clinical evidence, role of emotional factors, loss of ability to communicate, risk of cognitive deterioration, and – especially – the need for timely decision-making due to the progressive nature of ALS. Chapters 5 and 6 investigate the complexity of decision-making on gastrostomy in ALS. In interviews people with ALS, and their caregivers, describe decision-making about gastrostomy as a complex, continuous process during which they weigh clinical factors versus personal values and feelings about autonomy and loss. Despite people with ALS describing their disease taking away their control, they said being supported to make their own decision in their own time – i.e. patient choice – helped them feel in control over the decision-making process.
In Chapter 7 we show that between 2012-2020 around one in four people with ALS in the Netherlands chose euthanasia. However, despite choosing euthanasia more often compared to other diseases, this high frequency of euthanasia did not appear to negatively affect survival and co-occurred with a high satisfaction with end-of-life care. Reasons to hasten death and choose euthanasia included loss of dignity and increased dependency. Finally, in Chapter 8 we describe the case of a person with ALS who chose euthanasia and the care he received from the start of his journey till the end.