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It represents a \u201cmedical emergency\u201d with the key determinant of outcome being the duration of penile ischaemia and time to detumescence of &lt;4\u00a0h associated with a successful treatment outcome. Management can be outpatient\u2010based and consists of pre\u2010emptive strategies for early stuttering attacks based on prior health education of the association between the 2 disorders, non pharmacological management, outpatient penile aspiration and irrigation with or without instillation of alpha and beta adrenergic agonists for acute episodes and secondary prophylaxis to prevent the high rates of recurrences. The evidence to recommend medical prophylaxis is sparse but based on a consensus of experts and small phase 2 or <jats:styled-content style=\"fixed-case\">III<\/jats:styled-content> clinical trials. A clearer understanding of the molecular mechanism(s) involving normal and dysregulated erectile physiology, scavenger haemolysis and nitric oxide pathway paves way for the use of phosphodiesterase type 5 inhibitors in medical prophylaxis of stuttering attacks. These agents will need to be studied in multi\u2010centre randomized phase <jats:styled-content style=\"fixed-case\">III<\/jats:styled-content> trials before they become standard of care. A multidisciplinary team approach is required to enhance \u201csexual wellness\u201d and prevent erectile dysfunction in this sexually vulnerable group.<\/jats:p>","DOI":"10.1111\/bjh.12199","type":"journal-article","created":{"date-parts":[[2013,1,7]],"date-time":"2013-01-07T13:31:59Z","timestamp":1357565519000},"page":"754-765","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":35,"title":["How I manage priapism due to sickle cell disease"],"prefix":"10.1111","volume":"160","author":[{"given":"Ade","family":"Olujohungbe","sequence":"first","affiliation":[{"name":"Department of Hematology CancerCare Manitoba Winnipeg MB Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur L.","family":"Burnett","sequence":"additional","affiliation":[{"name":"The James Buchanan Brady Urological Institute The John's Hopkins Hospital Baltimore MD 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