A systematic review to assess the burden of ischemic priapism in patients with sickle cell disease

Introduction: Ischemic priapism is a serious condition characterized by a persistent and painful involuntary erection. Patients with sickle cell disease (SCD) are at an elevated risk of experiencing priapism, with approximately 40% experiencing priapism during their lifetime. Objectives: This systematic literature review (SLR) sought to identify studies reporting the clinical, humanistic, and economic burden of SCD-related priapism. Methods: MEDLINE, Embase, Cochrane CENTRAL/CDSR, and 14 congresses were searched. Results were screened against predefined criteria and studies assessing relevant aspects of burden for human patients with SCD-related priapism were included. Outcomes of interest included rates of erectile dysfunction (ED), other disease-related complications, health-related quality of life (HRQoL), and economic burden. Results: Of 1,236 publications identified, 34 were included. Most studies were retrospective observational (21). Sample size varied widely (range, 15-10,788). Study publication dates ranged from November 1980 through April 2018. Twenty-one studies reported clinical burden, with findings falling into 4 categories-priapism duration, recurrence frequency, incidence/severity of ED, and other complications. Nineteen studies reported humanistic burden, finding limited use of validated HRQoL measures such as the International Index of Erectile Function and the Priapism Impact Profile. Other patient-reported humanistic outcomes included worse scores on sleep-related indices and low rates of satisfactory intercourse. Eight studies reported economic burden. Direct treatment costs ranged from $4,996 for patients without penile operations to $22,906 (2019 USD) for patients receiving transfusions and urologic procedures. Conclusions: This SLR identified 34 studies providing evidence of the burden of priapism for patients with SCD, including painful complications and diminished physical, mental and sexual function.