The many facets of sickle cell priapism – SCiF 2013

Mr Nick Watkin, Reconstructive Urology Unit, St George’s Hospital, London

Sickle cell priapism (SP) and erectile dysfunction are potentially devastating complications of sickle cell disease. Patients may conceal their condition from family and health care workers due to cultural factors and embarrassment. The talk will focus on current concepts of pathophysiology of priapism, new techniques for evaluation, and pharmacotherapy. A critical review of 5 years of a dedicated sickle cell pripism clinic will also be presented.

1. Recurrent Stuttering priapism (SP):

Pathophysiology: The commonly held view on the cause of SP assumes that there is sludging of sickle cells in the anoxic enviroment of the corpora cavernosa. However, recent evidence form animal models and clinical effects of phosphodiesterase inhibitors (eg sidenafil) suggests that SP is influenced by a change in the regulation of the cGMP/NO synthase pathway, critical for sinusoidal smooth muscle relaxation within the corpora.The potential of sildenafil will be discussed in relation to the current theories of pathogenesis.

Evaulation: Recent studies with dynamic penile Doppler Ultrasound have given insights into the potential for predicting risk of acute ischaemic priapism.

Pharmacotherapy: Literature reviews highlight the lack of large-scale studies evaluating the efficacy of existing treatments for the prevention of SP. Alpha-agonists and anti-androgens are the mainstay of treatment. Male factor infertility may be a significant problem both with treatment of priapism with anti-androgens and potentially with hydroxycarbamide used for treatment of sickle disease.

2. The challenge of long term care: the priapism clinic.

Our experience of the care of patients with priapism is that the optimum pathway is rarely followed. There are issues surrounding non-attendance and non–compliance with medication which make delivery of care challenging.

Erectile dysfunction is the main concern of patients who have experienced stuttering SP or acute SP and we need to improve how we discuss this with patients for whom cultural fcators are a significant impediment to good communication.



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