How I manage priapism due to sickle cell disease

Ade Olujohungbe, Arthur L. Burnett

Research output: Contribution to journalReview article

Abstract

Priapism due to sickle cell disease is a common but less well characterized complication of the disorder. It represents a "medical emergency" with the key determinant of outcome being the duration of penile ischaemia and time to detumescence of <4 h associated with a successful treatment outcome. Management can be outpatient-based and consists of pre-emptive 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 III 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-centre randomized phase III trials before they become standard of care. A multidisciplinary team approach is required to enhance "sexual wellness" and prevent erectile dysfunction in this sexually vulnerable group.

Original languageEnglish (US)
Pages (from-to)754-765
Number of pages12
JournalBritish journal of haematology
Volume160
Issue number6
DOIs
StatePublished - Mar 1 2013

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Keywords

  • Haemoglobinopathy
  • Hemolysis
  • Nitric oxide
  • Priapism
  • Sickle cell disease

ASJC Scopus subject areas

  • Hematology

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