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. 2022 Oct 25;6(20):5676-5683.
doi: 10.1182/bloodadvances.2022007285.

Incidence and predictors of priapism events in sickle cell anemia: a diary-based analysis

Ibrahim M Idris  1 , Akib Abba  1 , Jamil A Galadanci  2 , Sani A Aji  3 , Atiku U Jibrilla  3 , Mark Rodeghier  4 , Adetola Kassim  5 , Arthur L Burnett  6 , Michael R DeBaun  7
Affiliations

Incidence and predictors of priapism events in sickle cell anemia: a diary-based analysis

Ibrahim M Idris et al. Blood Adv. .

Abstract

We conducted one of the first prospective studies to test the hypothesis that the clinical history of priapism underestimates priapism incidence compared with a priapism pain diary. Eligibility criteria were men with sickle cell anemia (SCA) between 18 and 40 years of age who have had at least 3 episodes of priapism in the past 12 months. Seventy-one men with SCA completed the diary for at least 3 months. The first 3 months of the priapism diary were included in the analysis. A total of 298 priapism episodes were recorded, and 80% (57 of 71) of the participants had at least 1 priapism event. Priapism severity was reported in the range of moderate to the worst imaginable pain in 81.5% (263 of 298), and a total 57 participants (80%) had a median pain rating of 6 (interquartile range: 5-8) on a scale from 1 to 10. The monthly incidence rate of priapism per participant based on history versus self-report pain diary was 2.0 (95% confidence interval, 1.9-2.1) and 1.4 (95% confidence interval, 1.2-1.6), respectively (P < .001). For participants that had a prior priapism episode, 80% had another episode during the 3-month interval follow-up. The median time to that second episode was 27.5 days. Major priapism occurred in 9.9% of episodes and was associated with the sum of all prospective priapism events. Men with SCA and at least 3 priapism episodes in the past 12 months are at significant risk for recurrent priapism in the following 3 months.

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Conflict of interest statement

Conflict-of-interest disclosure: M.R.D. and his institution are the sponsors of 2 externally funded research investigator-initiated projects. Global Blood Therapeutics (GBT) provides funding for the cost of the clinical studies but is not a co-sponsor of either study. M.R.D. is not receiving any compensation for the conduct of these 2-investigator-initiated observational studies. Also, M.R.D. is a member of the Global Blood Therapeutics advisory board for a proposed randomized controlled trial for which he receives compensation, the chair of the steering committee for NOVARTIS-supported phase 2 (SPARTAN) trial to prevent priapism in men, and a medical advisor for the development of the CTX001 Early Economic Model. M.R.D. provided medical input on the economic model as part of an expert reference group for Vertex/CRISPR CTX001 Early Economic Model and provided general information to Forma Pharmaceuticals in 2021 and 2022. The remaining authors declare no competing financial interests.

Figures

None
Graphical abstract
Figure 1.
Figure 1.
Consort diagram for participant screening, enrollment, and completion of prospective diary.
Figure 2.
Figure 2.
Histogram of months to priapism events for all diary months. There is a substantial decline in events over time, so only the first 3 months of data are used for prospective analyses.
See this image and copyright information in PMC

References

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