Boys and Men with Sickle Cell Disease
Men and women have similar rates of sickle cell disease (SCD). This is because SCD is not sex-linked. This means that the mutated gene in SCD is not on the sex chromosome.
However, complications of SCD can affect men and women differently. In men, SCD can cause delayed puberty and issues having children. SCD can also block blood flow out of the penis and cause erectile dysfunction.
How does sickle cell disease affect growth and puberty?
A large study in 1984 found that boys and young men with sickle cell anemia are much shorter than their peers and weigh less across all ages. More recent studies have confirmed these trends.1,2
Boys with SCD also reach all stages of puberty later than expected. This includes the formation of body hair, facial hair, and voice changes. Some studies estimate that the delay is about 2 years.
Researchers do not yet know why SCD causes delays in puberty and growth for boys. There is limited evidence of treatments affecting growth or puberty. Researchers are also not yet sure if testicular function and hormone levels cause the delay.1
Is fertility affected?
Most young men with SCD eventually mature sexually. However, men with SCD are more likely to experience infertility because of hypogonadism (testosterone deficiency), sperm abnormalities, and erectile dysfunction.3
Up to 24 percent of adult men with SCD experience hypogonadism. This is a condition where the body does not produce enough testosterone, sperm, or both. There are some theories of why this occurs in men with SCD, but the exact cause is unknown.4
Over time, hypogonadism can cause:3
- Decreased muscle and bone mass
- Erectile dysfunction
- Low sex drive
Methods to treat hypogonadism are limited.3
Adult men with SCD are also more likely to have low sperm count and function. Some estimates say about 90 percent of men with SCD have sperm abnormalities. This is also a common cause of infertility. Low sperm count might be caused by hypogonadism or repeated pain crises. Some SCD treatments, like hydroxyurea, may also cause sperm abnormalities.1,4,5
What complications are specific to men?
Some studies show that men experience more frequent pain crises and more severe complications. This may explain why men with SCD have a slightly shorter life expectancy than women with SCD. More research to understand these trends is needed.6
About 20 to 35 percent of men with SCD experience erectile dysfunction. This is often caused by prolonged, painful erections, a condition called priapism. Experts think it is caused by sickle cells blocking blood flow from the penis.1,7
Most episodes of priapism are brief and can be resolved without medical intervention. Hydration, oral pain medications, exercise, and warm and cold compresses may provide relief. Erections lasting longer than 4 hours are medical emergencies and require immediate medical attention because permanent tissue damage can occur if left untreated.1,4
Prevention of priapism is typically more successful than treatment. Fever, dehydration, and sexual activity often trigger episodes. Certain medications may also increase the frequency of episodes of acute priapism. On the other hand, hydroxyurea and regular blood transfusions often prevent episodes of priapism.8