patient care perspectives

Acute Management of Ischemic Priapism in Sickle Cell Disease

by Michael Rutledge DeBaun, MD, MPH

Overview

Individuals with sickle cell disease (SCD) who experience priapism may not bring the issue to medical attention. As part of an overall approach to acute management, strategies are needed to improve the recognition and awareness of ischemic priapism in SCD so that patients know to seek prompt medical attention.

Expert Commentary

Michael Rutledge DeBaun, MD, MPH

Professor of Pediatrics and Medicine
Vice-Chair for Clinical and Translational Research
J.C. Peterson Endowed Chair in Pediatrics
Director, Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease
Vanderbilt University Medical Center
Nashville, TN

“Bidirectional communication between individuals with SCD and health care providers is required for the education, detection, and timely management of priapism.”

Michael Rutledge DeBaun, MD, MPH

Priapism is a neglected but significant cause of morbidity in boys, adolescents, and men with SCD. The first challenge in addressing priapism is acknowledging that the condition exists. Just having that conversation with the mother or the child, the adolescent, or the adult is often awkward, perhaps even more so if the dialogue is happening across genders. In many cases, families and clinicians may avoid having this discussion about the onset and treatment of priapism.

Bidirectional communication between individuals with SCD and health care providers is required for the education, detection, and timely management of priapism. We recently published the results of a survey of 500 men with SCD in Nigeria, which revealed that almost 50% of those who experienced SCD-related priapism had never sought medical attention for their priapism. Many thought that their priapism was unrelated to their SCD.

An awareness campaign to improve communication between health care providers and nurses is necessary to enhance priapism management. For a priapism episode that lasts for 2 to 3 hours, individuals with SCD should know that prompt medical attention is needed in the Emergency Department. An episode of priapism that lasts for 4 hours is a urological emergency. The patient should be seen by a urologist who can aspirate blood from the penis to resolve the episode.

At present, there are limited strategies for preventing priapism episodes. In individuals with SCD, the basic biology of priapism is related to nitric oxide depletion. Hydroxyurea is a nitric oxide donor and may therefore be part of a reasonable secondary prevention strategy, although most of our patients are already on this disease-modifying agent. Antiandrogen therapy may be considered, but this class of treatment has significant adverse effects. Oral phosphodiesterase type 5 inhibitors have been proposed for the secondary prevention of priapism, which may seem counterintuitive because these drugs have been approved by the US Food and Drug Administration for erectile dysfunction. However, oral phosphodiesterase type 5 inhibitors such as tadalafil and sildenafil also increase nitric oxide and may be considered as an option to prevent priapism on a case-by-case basis.

Until a disease-modifying therapy has been demonstrated to prevent priapism in a randomized controlled trial setting, limited evidence-based options are available for avoiding priapism and its severe mental health sequelae.   

References

Anele UA, Burnett AL. Erectile dysfunction after sickle cell disease-associated recurrent ischemic priapism: profile and risk factors. J Sex Med. 2015;12(3):713-719. doi:10.1111/jsm.12816

Anele UA, Le BV, Resar LMS, Burnett AL. How I treat priapism. Blood. 2015;125(23):3551-3558. doi:10.1182/blood-2014-09-551887

Arduini GAO, Trovó de Marqui AB. Prevalence and characteristics of priapism in sickle cell disease. Hemoglobin. 2018;42(2):73-77. doi:10.1080/03630269.2018.1452760

Broderick GA. Priapism and sickle-cell anemia: diagnosis and nonsurgical therapy. J Sex Med. 2012;9(1):88-103. doi:10.1111/j.1743-6109.2011.02317.x

Idris IM, Abba A, Galadanci JA, et al. Men with sickle cell disease experience greater sexual dysfunction when compared with men without sickle cell disease. Blood Adv. 2020;4(14):3277-3283. doi:10.1182/bloodadvances.2020002062

Idris IM, Bonnet K, Schlundt D, et al. Psychometric impact of priapism on lives of adolescents and adults with sickle cell anemia: a sequential independent mixed-methods design. J Pediatr Hematol Oncol. 2021 Feb 3. doi:10.1097/MPH.0000000000002056

Joice GA, Liu JL, Burnett AL. Medical treatment of recurrent ischaemic priapism: a review of current molecular therapeutics and a new clinical management paradigm. BJU Int. 2021;127(5):498-506. doi:10.1111/bju.15370

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