Asif Muneer consultant urological surgeon and andrologist, Jas Kalsi consultant urological surgeon and andrologist, Irwin Nazareth professor of primary care and population science, Manit Arya senior lecturer and honorary consultant urological surgeon
Muneer A, Kalsi J, Nazareth I, Arya M.
BMJ 2014; 348 :g129
Re: Erectile dysfunction
In their CME Clinical Review (February 1) Asif Muneer et al wrote "Fibrosis within the corpus cavernosum as a result of priapism can also cause erectile dysfunction"  But in "What laboratory tests are needed?" they omitted tests to exclude sickle cell disease, a cause of post priapism erectile dysfunction . Diggs and Ching  in 1934 first related “priapism with residual fibrosis of the penis to capillary stasis and a tendency to thrombus formation and infarction” [2, p 226]. Subsequent authors were Campbell 1935 observing priapism associated with abdominal sickle cell crises , Dawson 1939 , Rosokoff and Brodie 1946 , Margolies 1951 , Campbell and Cummins 1951 , Kraus and Fitzpatrick 1961  Hasen and Raines 1962 , Diggs 1965 , Audu and Rao 1967 , Gillenwater et al 1968 , Serjeant 1970 . Impotence was described by Phillips 1944 , Rosokoff and Brodie 1946 , Harrison 1947 , Levant and Stept 1948 .
PRIAPISM AND SICKLE CELL DISEASE PHENOTYPE
I found a higher incidence of priapism in sickle cell anaemia ‘SS’ (5% of 387 consecutive males) than in sickle cell haemoglobin C disease ‘SC’ phenotype (1.3% of 385 consecutive). These figures are less than found elsewhere because we included all ages . Serjeant in the West Indies found 26 priapism cases (18.6%) of his ‘SS’ adult patients . Caution is needed in using mere presence of Hb ‘S’ as a criterion because in a country like Ghana where 1 in 5 of us has sickle cell trait ‘AS’, any pathology needs interpretation without implying association. Collect patients with diabetic neuropathy/vasculopathy-related erectile dysfunction and 20% will be sickle cell trait. Indeed, 20% of my own parents’ 11 children have Sickle Cell Trait ‘AS’. Priapism should not be attributed to sickle cell trait as I once naively did until the astute British Haematologist Dr E J Watson-Williams wrote from Manchester University to correct me [2, p 364]. Sickle Cell Disease (2 beta-globin gene variants at least one of which is the sickle cell gene) is different.
POST SICKLE CELL PRIAPISM IMPOTENCE IS A WORLDWIDE PROBLEM
As sickle cell disease patients are found in Mediterranean countries (18-20), Middle East (21-23), India (24-25), West, Central, and East Africa and in descendants around the globe - USA, Canada, South America [26 & 2, pp 76-82] complaints of erectile dysfunction anywhere must evoke the question "Does this man have sickle cell disease?".
RIDING BICYCLE EFFECT PROVES PUDENDAL ARTERY/NERVE ROLE
“Prolonged cycling” I once recorded [2, p 235] “has been known to interfere with penile erection mechanisms (Goodson 1981), Kerstein et al (982), Desai and Gingell (1989)” [27-29] - a phenomenon which Muneer et al now quote authors to imply compression of the pudendal artery and nerve. [30-32]. Proof was shown in the case of my “Ghanaian patient who aborted his morning priapism attacks by cycling before going to work” [2, p 235]. If erectile dysfunction is hypo-function, priapism is hyper-function. If the former can be caused by compression of the pudendal vessel/nerve bundle, the latter can be relieved by the same mechanism, leading me to recommend: “A static exercise bicycle should be available on which priapism patients should be tried.” [2, page 235].
TWO IRRECONCILABLE TREATMENT PHILOSOPHIES
Blacks on both sides of the Atlantic realize “that although there are two irreconcilable philosophies in sickle cell socio-politics only one (the respectable one) is openly acknowledged.” [2, p 452]. The other philosophy which has secret support is not admitted to. These philosophies can be stated as follows [2 p 452]:
Philosophy Number 1: “Patients with Haemoglobinopathies require regular and continuous medical care….Since many patients with sickle cell thalassaemia, sickle cell Hb C disease, and even sickle cell anaemia (SS) will marry and have children, it is important that the affected patients be given genetic advice”. WHO 1972. Many of them have been known to carry genes of brilliance surpassing their siblings who do not carry the sickle gene [My younger brother with sickle cell disease carried genes for Mathematics far sharper than mine]. If they have erectile disorder, help them, says the WHO. 
Philosophy Number 2: A US Professor was asked in October 1976 “What are the factors in retaining or regaining sexual potency after an episode of priapism?” [2, page 453]. Answer: “The longer priapism remains unresolved with or without treatment, the more likely fibrosis of the corpora cavernosa will be a sequel and cause impotence …. If the priapism is unresolved within 24 hours after one or more aspirations a corpora cavernous-spongiosum shunt should be performed. This answer pertains only to the idiopathic, traumatic and neurologic varieties, since no treatment of priapism is indicated in most patients when the cause is metastatic cancer or sickle cell disease” [2, p 453].
This advice, I commented, is “incredible to adherents of Philosophy Number 1…It should be remembered that those who adhere to Philosophy Number 2 also find it incredible that physicians in West Africa should go to any extent possible to relieve the psychological trauma of post-priapism impotence by practical means” [2, p 453]. Why in a CME Clinical Review article on erectile dysfunction was sickle cell disease not mentioned once?
BRITISH SOCIETY FOR SEXUAL MEDICINE – ANNUAL CONFERENCE
On March 7 at The Royal Society of Medicine my fellow Ghanaian Dr Nick Ossei-Gerning, MD (Lond) FRCP (Lond), Consultant Interventional & General Cardiologist, University Hospital of Wales, Cardiff will 11.50 am – 12.30 pm give the “Master Lecture” on “The Role of Interventional Cardiology in Erectile Dysfunction”. I shall, God willing, be there if only to hear how he would now have managed the 70 year-old Gã fisherman whom I described in the BMJ on 8 February 1975: “I asked if he had any complaints. He eyed the attending staff nurse and asked her to move out of earshot, then leaning forward towards me he said in hushed tones ‘Tafracher, I am losing my manhood’” .
Competing Interest: I come from a Sickle Cell Disease family. My Abnormal Haemoglobin Trait parents had 11 children of whom 3 had sickle cell disease, 4 with Trait phenotype, and 4 with normal Haemoglobin phenotype.
Felix I D Konotey-Ahulu MD (Lond) FRCP (Lond) DTMH (L’pool) FGCP FWACP Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Sickle & Other Haemoglobinopathies, 9 Harley Street Ltd, Phoenix Hospital Group, London W1G 9AL [email@example.com
1 Muneer A. Kalsi J, Nazareth I, Arya M. Erectile dysfunction. BMJ 2014; 348: g129 doi: 10.1136/bmj.g129
2 Konotey-Ahulu FID. Sickle Cell Priapism. Chapter 19 In The Sickle Cell Disease Patient. The Macmillan Press Ltd, London and Basingstoke 1991, 1992 and T-A’D Co Ltd 1996 Watford – firstname.lastname@example.org
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32 Babaev A, Jhaveri RR. Angiography and endovascular revascularization of pudendal artery atherosclerotic disease in patients with medically refractory erectile dysfunction. J Invasive Cardiol 2012; 24: 236-40
33 WHO Technical Report Boyo AE, Cabannes R, Conley CL, Lehmann H, Luzzatto L, Milner PF, Ringelhann B, Weatherall DJ, Barrai I, Konotey-Ahulu FID, Motulsky AG. [Geneva, WHO Scientific Group on Treatment of Haemoglobinopathies and Allied Disorders, Technical Report Series No 509 1972, pages 31 & 36]
34 Konotey-Ahulu FID. Tafracher - Personal View (A Ghanaian de-vulgarizing prefix “not only used to prepare the listener for the unpleasant, but also to preserve the reputation of the speaker”. BMJ 1975; 1(5953): 329 doi: 10.1136/bmj.1.5935.329 www.bmj.com/cgi/reprint/1/5953.329.pdf & www.ucc.edu.gh/node/258
Competing interests: Competing Interest: I come from a Sickle Cell Disease family. My Abnormal Haemoglobin Trait parents had 11 children of whom 3 had sickle cell disease, 4 with Trait phenotype, and 4 with normal Haemoglobin phenotype.