Objective:

We systematically evaluated palliative care utilization and mode of delivery in a cohort of patients with prion disease. 

Background:

Timely access to palliative care, a specialty that focuses on improving quality of life for patients with serious or advanced medical conditions, is central to the care of patients with prion disease.

Design/Methods:

Clinical data were extracted from the electronic medical records of patients with definite or probable prion disease diagnosed and managed across a tertiary care health system.

Results:

174 patients with prion disease were identified. 165 (94.8%) patients experienced one or more symptoms/signs likely to benefit from palliative care. 113 (64.9%) patients received palliative care resources during the illness course, including hospice care (39.7%), palliative care consultation (14.9% inpatient, 6.9% outpatient), or both (3.4%). The most common reason that patients did not receive palliative care services was lack of referral by the diagnosing clinician (59.0%). Patients with multiple symptoms/signs likely to benefit from palliative care were more likely to receive palliative care services compared to patients with isolated or no symptoms/signs (OR = 4.24, 95%CI = 1.37, 13.03). Clinical presentations dominated by language impairment (OR = 8.18, 95%CI = 1.01, 66.36), the presence of behavioral changes (agitation/combativeness; OR = 2.53, 95%CI = 1.15, 5.55), and constipation (OR = 4.81, 95%CI = 1.01, 22.91) were independently associated with access to palliative care services.

Conclusions:

More than a third of patients with prion disease did not receive palliative care services, although prion disease is a terminal and rapidly progressive disease and despite a preponderance of clinical features likely to benefit from palliative care in most patients. These findings highlight an opportunity to improve care of patients with prion diseases by improving access to comprehensive palliative care resources.