It is often said that sickle cell disease does not kill. This series will focus on breaking down and understanding each of the complications that might develop from sickle cell, so that you can know how to prevent this, catch it early and deal with it. Knowledge is power!
Priapism refers to a prolonged penile erection (e.g., lasting longer than 4 hours) unrelated to sexual stimulation or desire. There are 2 types of priapism: Low flow priapism [LFP]) results from reduced venous outflow from the erectile tissue (usually due to sickle cells clumping up the penile artery), and this is the most common type that affects sickle cell patients. High flow is usually unrelated to sickle cell, so I won’t bother going into it.
Risk Factors: 38-42% of men with sickle cell (or any variance eg thalassemias) will have LFP at least once during their lifetime. LFP usually peaks between the ages of 5-10 and 20-50. Use of recreational drugs like Cocaine, Marijuana and alcohol further increase your risk.
How do I know if I have Priapism?
If you have an erect, rigid, painful penis and no feelings of arousal, then you probably have priapism.
Besides the fact that priapism is uncomfortable, if left untreated, LFP can result in hypoxia (lack of oxygen to cells) and acidosis (increase of lactate acid in cells), tissue damage, and permanent erectile dysfunction (Eek!) and is considered a urologic emergency. So please, if you do have priapism, visit your doctor or go to the ER to get it treated! If you have priapism for 24 hours, you will become permanently impotent!
The first treatment option is to place ice packs to the penis and perineum to reduce swelling. With SCD patients, blood transfusions or exchange transfusions are given to increase the hematocrit value to greater than 30%. Hydration through IV fluids is given to thin out the blood. If this does not resolve LFP, the next step is an injection of Terbutaline, and if this is unsuccessful, another drug called Phenylephrine could be administered. In some cases, the doctor can draw out the blood from the penis with a needle, followed by a saline irrigation (water follows salt which is then excreted by the kidneys reducing the pressure). If these treatment strategies fail, you might have to have surgery to create a shunt that diverts the blood or a bypass of the blockage.
There are adverse effects related to the medications that will be injected. Report to your doctor if you have a headache, hypertension, dizziness, slow heart beating, fast heart rate, palpitations in the chest, signs of infection, bleeding from the site, or any changes to your penis.
How do I prevent Priapism?
There is very little you can do to prevent LFP. The best tips are to avoid triggers of your LFP (certain medications like anti-anxiety pills, Viagra, Levitra, and Cialis, anticoagulants, antihypertensives, antidepressants, antipsychotics, tranquilizers, antihistamines, cocaine, marijuana and alcohol). Drink at least 2 liters of water a day. Hydration works both ways to prevent crises and prevent cells from clumping together. For some reason, Glutamine and Arginine have caused some people to have LFP, even though in some it has the opposite affect. You might have to learn how to give yourself injections in the penis if this becomes a permanent recurrent problem.
Okay warriors, that is a brief synopsis of priapism. It is not something to be scared of, and many males with sickle cell have had it and will have it. So just follow these tips, put some ice on it, and if it doesn’t go away in a couple of hours, head to the ER/doc to get it taken care of.
Remember, knowledge is power. Be well.