Background: SCD is a chronic disease characterized by painful vaso-occlusive (VOC) episodes and hemolysis which lead to poor HRQL and premature mortality. As curative treatment with hematopoietic stem cell transplant is only an option for a minority of adult patients, current treatments are palliative with focus on pain management. Clinically, pain assessment is commonly performed by Numeric Rating Scale, where patients rate the intensity of pain from 0 (no pain)-10 (worst imaginable pain). However, the incorporation of HRQL through the use of newer PROs into the assessment of pain and the care of patients with SCD is evolving. Our objective was to better inform the multidimensional impact of pain on adults hospitalized with VOC, as measured by the Patient-Reported Outcomes Measurement Information system (PROMIS) Global Health and the Adult Sickle Cell Quality of Life Measurement System (ASCQ-Me). Reported here are the preliminary results of the first 15 patients of our larger inception cohort with planned enrollment of 140 patients.

Methods: In this study at an urban tertiary care hospital, 15 of 16 eligible adults with SCD completed the PROMIS short form (v1.1) Global Health and ASCQ-Me questionnaires on hospital admission for VOC. These measures yield scores in the domains of physical health, mental health, and sickle-cell related domains of pain-episode frequency, emotional impact, social functioning impact, pain impact, stiffness, and sleep. Seven days post-discharge, 12 of the 15 patients again completed the assessments. Standardized domain scores have a mean of 50 (SD=10) with higher scores indicating better functioning. We calculated means and standard errors (SE) for the domain scores at both assessment periods and mean change scores.

Results: One eligible patient declined enrollment due to poor mental health, stating "I have lost all hope with this disease." Two of the initial 15 patients had hospital readmissions within 7 days and were censored from further analysis; the third did not respond to follow up and was readmitted within 30 days. Sixty percent of patients were female. The mean age was 30.1 years (range 22-36). The most frequent disease genotype was Hb SS (66%), followed by Hb SC (27%) and Hb Sβ+ thalassemia (7%). Eleven patients (73%) identified as black, non-Hispanic; 4 (27%) identified as Hispanic. At hospital admission, 66% of patients had 4 or more pain crises in the last 12 months. The mean score for the most severe pain during the last pain attack was 8.8 (SD = 1.4) on a scale from 0-10. The most recent pain crisis for 66% of patients lasted at least 1 week; 26% reported crisis lasting longer than 2 weeks. Of the 12 patients who completed all assessments, the table presents mean domain scores at each assessment period and the change scores. Of note, 83.3% scored at least 1 SD below the population mean for the PROMIS global physical health subscale on admission. This was unchanged 7 days post-discharge (83.3%). Half scored at least 1 SD below the population mean for PROMIS global mental health subscale, compared to 33% 7 days post-discharge.

Conclusions: Administering disease-specific PRO measures at point of care is feasible. Patients who did not complete assessments following hospital discharge were all readmitted within 30 days, indicating a particularly high-risk group.

At both admission and 7 days post-discharge, a significant number of patients had physical and mental health scores that were 1 SD below population norms, indicating substantial suffering. Compared to admission, scores for global mental health, social functioning and stiffness worsened following discharge on average. Scores in global physical health, emotional impact, pain impact and sleep impact improved. Further study of incorporating PRO measures in the clinical care and improvement in health outcomes among adults with SCD is warranted.


No relevant conflicts of interest to declare.

Author notes


Asterisk with author names denotes non-ASH members.

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