Priapism, Corticosteroids, Opisthotonus and LMP (which for all intents and purposes should be Last Menstrual Period, but is apparently not) are some names of teams that make up the (medliga) football league of the University of Ghana Medical School. It left me wondering how team names are chosen and what, if I ever ventured into that business of football club ownership, I would call mine.
Now, Priapism FC. They go by the mantra, STAND STRONG, HARD and FIRM. Interesting isn’t it? Team priapism, new entrants into the medliga last year, actually ended up league champions. In the words of their coach “It is proof that with hard work and determination you can turn the most dire situation into success” The guys actually stood long, hard and firm. Taking a cue from them, I guess WE ALL NEED SOME PRIAPISM IN OUR LIVES. Eeermm actually, you really do not want to have the medical condition though. U can take my word for it.
I think at this point, a disclaimer is in order. MEDICAL INFORMATION. BRACE YOURSELF!
WHAT IS PRIAPISM?
Priapism is a medical condition in which there is an abnormal, persistent, involuntary, prolonged erection unrelated to sexual stimulation and unrelieved by ejaculation. Simply, the erect penis does not return to the flaccid state despite the absence of both physical and psychological stimulation within 4 hours. Priapism is a MEDICAL EMERGENCY and should be treated as such,
It is categorized as either high-flow or low-flow (which accounts for about 80% of cases). Management is based on this categorization, as both are managed differently. There is a myriad of causes of this condition such as spinal cord trauma ( neurological), enzyme deficiency (G6PD), drugs, malignancies and vascular (haematological-Sickle cell disease and trait, thalassaemia and leukaemia).
Priapism in sickle cell disease is categorised as low-flow priapism. Sickle cell disease accounts for 89% of adult cases of priapism with studies indicating that 42% of adults with sickle disease will develop priapism at some point in their life. In the paediatric age group, 2/3 of cases of priapism are in children with sickle cell disease. Evidently, priapism affects people of all age groups with its peak between the ages of 19 and 21 in patients with sickle cell disease. It obviously affects only men, usually African or African America (even though my reading yielded mention of a female equivalent, clitorism, which is not widely accepted as a true equivalent).
Without going into much detail, what basically happens is that blood in the penis becomes trapped and unable to drain, leading to, ischaemia, scarring and permanent erectile dysfunction (if not promptly managed). In a person having a sickle cell crisis, just as occurs anywhere else in the body, there is occlusion of vessels, increased blood viscosity, stickiness of sickle cells to the blood vessel walls, depletion of nitric oxide ( a potent vasodilator), hypercoagulabilty of the blood, chronic inflammation and a lot more mechanisms by which vaso-occlusive crises cause damage. It is thus no wonder that sickle cell disease accounts for a large proportion of cases of priapism and that so many patients suffer this complication.
The key feature of priapism in sickle cell disease is pain, apart from of course an erection lasting more than 4 hours. Immediate medical attention should be sought to prevent occurrence of complications. On reporting to the hospital, labs will be done to determine the levels of all blood cells. This is important in determining the actual cause of the crisis. Blood samples will also have to go through checks to ensure that you get the right blood group because management actually involves a blood transfusion. The doctor will also perform other tests, including angiography if necessary.
General management of priapism involves both medical and surgical interventions. Every patient who suffers priapism will need to see a genitourinary system specialist. In sickle cell disease however specific protocols need to be observed. For one, priapism is a complication of a vaso-occlusive crisis. Thus, the crisis needs to be managed effectively. The doctor will take steps you are probably already familiar with such as adequate hydration, analgesia, alkanization and oxygenation as appropriate.
Next step is an exchange blood transfusion. This would seek to increase your red blood cell level and exchange the sickle cells in your blood for normal cells from the donor blood. After which oral medication can be given. Then the blood needs to come out. Under local anaesthesia the blood will be aspirated from the penis. This is crucial in reducing the swelling and built up pressure. If these medical options fail to bring relief, medication will have to be injected directly into the penis and if it still proves unsuccessful, a surgical shunt will have to be inserted.
As you have read above, this is complex stuff. Staying at home with priapism, even for persons without sickle cell disease, who may have it as a side effect of medication or due to recreational drug use, really is not a good idea. The complications as I already mentioned are ischaemia, due to the blood in the penis which can’t drain, thus oxygen cannot be supplied to the tissues as well as impairment to the removal of metabolic waste. Damage to blood vessels, resulting in long term reduction in erectile function (impotence) and in severe cases, gangrene which will result in the penis being surgically removed.
What can we do?
- Don’t have sickle cell disease. Radical as this may sound it has a simple explanation. Since you are more likely to have priapism as a complication of the disease, primary prevention, which I am very passionate about, is simply that you get tested and know your genotype and that of your partner and ensure that your child does not have sickle cell disease. Alternatively, IVF with genetic selection and bone marrow transplants and all the financial and heath implications that come with them need to be considered realistically and appropriate measures taken
2. For the rest of us who happen to already be born with sickle cell disease:
*stay healthy ie. anything to prevent a vaso-occlusive crises
*take your daily meds
*go for routine medical checks
*avoid use of recreational drugs
*pay attention to danger signs from your body and take appropriate measures…
Live each day to the fullest, make it count.
(I hope this has been educational. Please like this post, comment and share. Thank you.)