Circumstances and Signs of Approaching Death in Patients With Amyotrophic Lateral Sclerosis Undergoing Noninvasive Ventilation in Home Care Settings

Abstract

The purpose of this study was to elucidate the circumstances and symptoms of patients with amyotrophic lateral sclerosis (ALS) using noninvasive positive pressure ventilation (NPPV) in whom death was approaching, to understand how to provide palliative care to dying patients with ALS receiving NPPV management. The participants were 6 home visiting nurses who were head or subhead nurses of a home visiting nursing agency. Data were collected conducting individual semistructured interviews and then group interviews. The results were based on qualitative analysis of the circumstances of death and prognostication and showed 5 categories: difficulties with knowing about approaching death, several signs and symptoms of knowing about approaching death, importance of feeling prepared and provision of palliative care to die at home, death caused by accident, and fate determined by the caregiver's ability. Further research is needed to collect rich data about predicting approaching death to improve end-of-life care for NPPV-dependent ALS patients who do not receive sufficient benefits from NPPV.

The Use of Mechanical Ventilation Support at the End of Life in Motor Neurone Disease/Amyotrophic Lateral Sclerosis: A Scoping Review

“…The review identified 31 documents (see Table S1 ) from those 19 papers reporting empirical research, of which 11 used quantitative methods [ 11 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 ] and 8 used qualitative methods [ 13 , 29 , 47 , 48 , 49 , 50 , 51 , 52 ]. The documents which did not report empirical research studies presented discussions based on clinical case examples (n = 6) [ 17 , 18 , 53 , 54 , 55 , 56 ] or provided reviews or opinion pieces (n = 4) [ 57 , 58 , 59 , 60 ].…”

“…Of the 19 empirical papers included, a range of study designs and methods were reported (see Figure 2 ). These were primarily interview studies (n = 10) [ 13 , 29 , 37 , 38 , 47 , 48 , 49 , 50 , 51 , 52 ]. Two used a more structured approach to the interviews [ 37 , 38 ].…”

“…Of the 10 studies employing interview methods, 4 had a focus on patient and/or family caregiver perspectives [ 37 , 38 , 47 , 50 ], 3 had a focus on the views of healthcare professionals [ 29 , 48 , 49 ] and 3 reported findings from both healthcare professionals and family caregivers [ 13 , 51 , 52 ].…”

“…Findings show (see Figure 4 ) that 11 papers focused on NIV [ 13 , 18 , 38 , 42 , 44 , 49 , 52 , 54 , 57 , 58 , 59 ], 7 focused on IV [ 11 , 17 , 45 , 53 , 55 , 56 , 60 ] and a further 13 included both [ 25 , 29 , 37 , 39 , 40 , 41 , 43 , 46 , 47 , 48 , 50 , 51 , 61 ]. However, those patients on ventilation were often only part of the population included, and for those papers covering both forms of ventilation, it was not always possible to distinguish these patients in the findings.…”

A Natural History Study of VCP Associated Vacuolar Myopathy in a Patient with the Common R155H Mutation

“…As myopathy progresses to respiratory muscles in IBMPFD, PFTs are useful in monitoring respiratory function following diagnosis as is the case in other disorders such as late onset Pompe disease, ALS, and IBM [7,[20][21][22][23][24]. The general increase in sleep disruptions and remarkable drop in sleep duration over time was noted or will on monitoring him with a Fitbit.…”