A contemporary review of the management strategies for sickle cell disease related ischaemic and stuttering priapism

Sickle cell disease is one of the most common autosomal recessive genetic disorders with 23% and over 70% of men with this condition, experiencing episodes of ischaemic priapism and stuttering priapism, respectively, with potentially severe consequences. The effective prevention of sickle cell disease induced ischaemic priapism and stuttering priapism requires a multidisciplinary and multimodal approach. A search of the English literature was performed utilising Pubmed® and Google Scholar to identify publications on contemporary and novel treatment options, with their associated treatment outcomes if available, that are utilised to prevent stuttering priapism episodes and hence a fulminant ischaemic priapism. This narrative review focuses on three main aspects which include firstly, patient education and lifestyle modifications. Secondly, strategies aimed at preventing stuttering priapism episodes with traditional treatments such as alpha-adrenergic agonists and hormone manipulation strategies among others. Finally, we review treatments utilised to treat the underlying sickle cell disease with contemporary options such as hydroxyurea to more novel therapies such as crizanlizumab and voxelotor. The role of potentially curative techniques such as gene therapy and stem cell transplantation are also reviewed and summarised.