Clinical Changes in Patients With Amyotrophic Lateral Sclerosis Admitted to a Home Care Program

There is a lack of information in the literature about patients with amyotrophic lateral sclerosis (ALS) followed at home.

To characterize ALS patient demographics, longitudinal symptom trajectories, cognition, healthcare utilization, and advance care planning of an Italian home-based palliative care program cohort.

New patients with ALS who required home palliative care were recruited for a period of one year and followed up for six months. Demographics, Karnofsky, ALS subtype, date of diagnosis, awareness, as well as ECAS (Edinburgh Cognitive and Behavioral ALS Screen) were recorded, as well as the date of diagnosis and initiation of home palliative care were collected. The use of noninvasive ventilation (NIV), mechanical ventilation by tracheostomy, gastrostomy, nasogastric tube, parenteral nutrition, were recorded at admission and during home care assistance. The existence of advance directives and shared advance care planning (ACP) was also collected. The ALS Functional Rating Scale-Revised (ALS-FRS-R) and symptom burden was measured by Edmonton Symptom Assessment System (ESAS) were measured at two-month intervals for six months.

Data from 34 consecutive patients with ALS admitted to palliative home care were analyzed over the period considered. While Karnofsky level significantly decreased, ECAS did not show significant changes. The need for vital supports, particularly NIV, increased over time. Only one patient provided an ACP decision at admission, and none provided a living will. The involvement of a legal administrator facilitated the use of ACP significantly over the six months. Seven patients died at home during the first six months of home palliative care, and one patient was lost to follow-up, because they were transferred to another region. No patient was admitted to hospice in the first six months of home palliative care.

In patients with ALS admitted to home palliative care Karnofsky level, total ESAS and ALS-FRS-R score slowly but significantly decreased over six months. Only six patients died within this period. The use of NIV increased over time. No patient required hospital or hospice admission. ACP and living wills were minimal, although ACP rate increased during the study period. The program enabled patients to remain in their homes, reducing the need for hospital care.