Our goal is to implement an ongoing symptom assessment strategy using a validated palliative care tool in the outpatient management of adult patients with sickle cell disease at a comprehensive sickle cell center.
Sickle cell disease is a chronic lifetime illness with intermittent acute exacerbations to daily symptoms. The extension of palliative care symptom management strategies to patients with sickle cell disease has been proposed. Symptom assessment tools such as the ESAS (Edmonton Symptom Assessment System), validated in the palliative care population, may have a role in sickle cell disease management. Ten symptoms are assessed, with the average 24 hour symptom score reported on a 0 to 10 numerical scale, 10 being the worst possible. In our study, 75 adult patients with sickle cell disease presenting for their outpatient visit at a comprehensive sickle cell center were enrolled; they were asked to complete the ESAS and a survey regarding their opinion of the ESAS at each clinic visit.
Study patients were 100% African American, 56% (42/75) female, ages 19–67, with disease types 71% (53/75) HgbSS, 19% (14/75) HgbSC, and 11% (8/75) HgbS/beta thal. At least 49% (37/75) of patients had one follow up visit after enrollment, for a total of 112 clinic visits. On the initial visit, 92% (65/71) agreed or strongly agreed with the statement “the ESAS is easy to complete”; 97% (71/73) reported instructions as clear; 96% (72/75) reported no words were difficult to understand; 83% (59/71) were satisfied or very satisfied with the ESAS as a way to report symptoms; 93% (68/73) would recommend the ESAS to other patients. 62% (45/73) of patients reported the ESAS helped them remember symptoms they were experiencing; 34% (25/73) were encouraged to discuss medical issues more openly with their health care provider. In a comparison of visit 1 (V1) and 2 (V2) in the 37 patient subset with a single follow up visit, 94% (33/35) on V1 and 94% (33/35) on V2 recommended the ESAS to other patients; 78% (28/36) on V1 and 86% (31/36) on V2 were satisfied or very satisfied with the ESAS. Including all 75 patients on their initial visit, the most intense symptoms (≥ 4 on ESAS scale) recorded were pain 4.6 (± 3.1) and sleep 4.1 (±3). In the 37 patient subset with follow up, V1 pain 4.7 (± 2.7) and V1 sleep 3.5 (± 2.5); V2 pain 4.4 (± 3.2) and V2 sleep 4.3 (± 2.6).
The ESAS is an acceptable, brief, easy to understand symptom assessment tool which can be integrated into an outpatient sickle cell clinic as part of a serial-longitudinal symptom management strategy. Future studies should include measuring the impact of symptom-directed interventions on self-reported symptoms.
No relevant conflicts of interest to declare.
Asterisk with author names denotes non-ASH members.