Examining Mental Health, Education, Employment, and Pain in Sickle Cell Disease.

Importance: Pain related to sickle cell disease (SCD) is complex and associated with social determinants of health. Emotional and stress-related effects of SCD impact daily quality of life and the frequency and severity of pain. Objective: To explore the association of educational attainment, employment status, and mental health with pain episode frequency and severity among individuals with SCD. Design, Setting, and Participants: This is a cross-sectional analysis of patient registry data collected at baseline (2017-2018) from patients treated at 8 sites of the US Sickle Cell Disease Implementation Consortium. Data analysis was performed from September 2020 to March 2022. Main Outcomes and Measures: Electronic medical record abstraction and a participant survey provided demographic data, mental health diagnosis, and Adult Sickle Cell Quality of Life Measurement Information System pain scores. Multivariable regression was used to examine the associations of education, employment, and mental health with the main outcomes (pain frequency and pain severity). Results: The study enrolled a total of 2264 participants aged 15 to 45 years (mean [SD] age, 27.9 [7.9] years; 1272 female participants [56.2%]) with SCD. Nearly one-half of the participant sample reported taking daily pain medication (1057 participants [47.0%]) and/or hydroxyurea use (1091 participants [49.2%]), 627 participants (28.0%) received regular blood transfusion, 457 (20.0%) had a depression diagnosis confirmed by medical record abstraction, 1789 (79.8%) reported severe pain (rated most recent pain crises as ≥7 out of 10), and 1078 (47.8%) reported more than 4 pain episodes in the prior 12 months. The mean (SD) pain frequency and severity t scores for the sample were 48.6 (11.4) and 50.3 (10.1), respectively. Educational attainment and income were not associated with increased pain frequency or severity. Unemployment (ß, 2.13; 95% CI, 0.99 to 3.23; P < .001) and female sex (ß, 1.78; 95% CI, 0.80 to 2.76; P < .001) were associated with increased pain frequency. Age younger than 18 years was inversely associated with pain frequency (ß, -5.72; 95% CI, -7.72 to -3.72; P < .001) and pain severity (ß, 5.10; 95% CI, -6.70 to -3.51; P < .001). Depression was associated with increased pain frequency (ß, 2.18; 95% CI, 1.04 to 3.31; P < .001) but not pain severity. Hydroxyurea use was associated with increased pain severity (ß, 1.36; 95% CI, 0.47 to 2.24; P = .003), and daily use of pain medication was associated with both increased pain frequency (ß, 6.29; 95% CI, 5.28 to 7.31; P < .001) and pain severity (ß, 2.87; 95% CI, 1.95 to 3.80; P < .001). Conclusions and Relevance: These findings suggest that employment status, sex, age, and depression are associated with pain frequency among patients with SCD. Depression screening for these patients is warranted, especially among those experiencing higher pain frequency and severity. Comprehensive treatment and pain reduction must consider the full experiences of patients with SCD, including impacts on mental health.

Evaluation of point-of-care International Normalized Ratio in sickle cell disease.

BACKGROUND: Point-of-care (POC) International Normalized Ratio (INR) measurement provides efficient monitoring of warfarin therapy; however, its reliability may be affected in patients with anemia, such as those with sickle cell disease (SCD). OBJECTIVES: To evaluate the correlation of POC-INR to clinical laboratory INR (CL-INR) in SCD and use of a correction factor. PATIENT/METHODS: In this retrospective study, the accuracy of POC-INR compared to CL-INR was evaluated in a cohort of patients with SCD and in a non-SCD Black cohort. RESULTS: Despite the difference in anemia, the SCD cohort showed a similar percentage of in-range POC-INR values as observed in the non-SCD cohort (37% vs 42%). The SCD cohort was randomly divided to form discovery and validation cohorts. In the discovery cohort, 86% of POC-INRs were in range when the POC-INRs were ˂4.0, but only 24% were in range if POC-INRs were ≥4.0. A linear regression of CL-INR versus POC-INR for POC-INR values ≥4.0 yielded a coefficient of 0.72 (95% confidence interval, 0.69-0.75); Multiplying POC-INR by this correction factor, rounded to 0.7 for ease of use in clinical practice, improved the proportion of in-range POC-INR values ≥4.0 from 24% to 100% in the SCD discovery cohort and from 19% to 95% in the SCD validation cohort. Similar findings applied to analyses of the non-SCD cohort. CONCLUSIONS: POC-INR and CL-INR in patients with SCD are similar when POC-INR is <4.0, and the accuracy of POC-INR values ≥4.0 can be improved by applying an institution-specific correction factor.