Ventilator Dependency in ALS: Management, Disease Progression, and Issues of Coping

Abstract

The natural progression of amyotrophic lateral sclerosis (ALS) leads to respiratory failure and death. Artificial ventilation can prolong the course, leading to extreme degrees of weakness and dependence. Little specific information is available to counsel ALS patients about making the decision for artificial ventilation. In order to gain more information, we visited four ventilator-dependent ALS patients and their primary caregivers. We determined the neurologic state and level of function of the patients and interviewed their primary caregivers to assess medical care and management needs (both social and financial) and how they were being met. We also administrated questionnaires to assess the psychological well-being of both patient and primary caregiver and how the relationship between the patient and primary caregiver changed under these circumstances.

The Dynamics of Quality of Life in ALS Patients and Caregivers

“…For example, despite compromised physical function, the majority of people with ALS rated their QOL as 8 or better on a scale of 1 to 10 [3,4]. Several studies have found that even when people with ALS are supported by mechanical ventilation, they are generally satisfied with their quality of life, are not depressed, and have a positive worldview (e.g., [5,6]). …”

A Literature Review of Studies Using Qualitative Research to Explore Chronic Neuromuscular Disease

“…Assisted ventilation can either be noninvasive (masks or nasal cannula) or invasive (intubation), and it has been shown to improve life expectancy for individuals with DMD (Eagle et al, 2002) and MND (Bromberg, Forshew, Iaderosa, & McDonald, 1996). Hirano, Yamazaki, Shimizu, Togari, and Bryce (2006) found that economic, social, and cultural factors may influence the decision for individuals and families to elect to pursue assisted ventilation.…”

“…However, assisted ventilation can cause a considerable amount of stress and burden for family members (Bromberg et al, 1996;Carnevale, Alexander, Davis, Rennick, & Troini, 2006;Gelinas, O'Connor, & Miller, 1998;Heaton, Noyes, Sloper, & Shah, 2005;Mah et al, 2008;Moss et al, 1996). In fact, some MND caregivers expressed anger and resentment over the assisted ventilation process (Gelinas et al, 1998).…”

“…In particular, families need to provide constant surveillance, and they worry about complications including power failures, mechanical malfunctions, skin breakdown, and choking (Mah et al, 2008). In addition, the financial burden can be tremendous (Bromberg et al, 1996). It is significant that all 13 participants in the Young, Marshall, and Anderson (1994) study endorsed that assisted ventilation negatively impacted their caregivers.…”

Patient participation in decision‐making on the introduction of home respiratory care: who does not participate?

“…Other target groups have been adult patients in health care 17 and child health clinic 18 and maternity clinic clients 19 . The only studies found in Medline, CINAHL and Sociofile on patients' decision‐making on home respiratory care 9,10 , 20,21 were qualitative.…”