Mr Nick Watkin is a consultant urological surgeon at St George’s Hospital having trained in Cambridge and London. He leads the genito-urethral surgery team which provides a tertiary service for South Thames and Wessex for both benign and malignant genital disorders. He supports the regional haematology units in management of sickle cell priapism and erectile dysfunction. The team were “National Oncology Team of the Year” in 2009, and Mr Watkin was Urologist of the Year in 2010. He has trained urologists in developing countries and is on the committee of Urolink, an organisation dedicated to train and support urology units in developing countries.
Abstract: Sexual dysfunction, fertility issues and priapism in sickle cell disease
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. Case studies will be described which illustrate the key areas of importance
Management of recurrent Stuttering priapism (SP)
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. The commonly held simplistic 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. 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
Pathway for management of acute SD
Our experience of the care of patients with acute SD is that the optimum pathway is rarely followed. The length of time of the episode is crucial for the integrity of the corporeal sinusoids. Co-operation between haematologists, urologists, emergency physicians and the patient are vital for keeping the treatment time as short as possible.
Diagnosis and management of erectile dysfunction (ED)
ED is the main concern of patients who have experienced recurrent/stuttering SP or acute SP. It is challenging to discuss this with patients and the cause is often multi-factorial. Furthermore, the existing pharmacological treatments have the potential to trigger SP.
The commonest cause is ischaemia induced fibrosis of the corporeal sinusoids, but our experience with dynamic penile Doppler studies have revealed other causes including high flow priapism, arterio-venous shunts and arteriogenic ED. In the most severe cases, corporeal fibrosis renders the penis unresponsive to pharmacological therapy and surgical treatments with implantable penile prostheses are an option. Different types will be discussed.