We have already identified that Sickle Cell Disease is an inherited blood disorder that is present at birth, meaning that it is passed down through a parent's genes. Children are at a higher risk of getting SCD if both parents are carriers and having family history of sickle cell increases a child's risk for the disease. Children who have the disease start to display symptoms early on in life from between 5 months to 1 year; these may vary and could be either mild or severe. Symptoms include:
a) Pain Crisis, b) Jaundice, c) Anaemia, d) Acute Chest Syndrome, e) Pooling (Enlarged Spleen).
Because SCD causes complications and can affect major organs in both boys and girls, it is important to understand and identify the various issues they deal with so that we as parents and family are prepared and can learn how to care for them. Having said that, Sickle Cell Disease manifests itself in many ways that affect both boys and girls in the same way; for instance both sexes can suffer from leg ulcers or serious sores, gallstones, bone damage, kidney damage and frequent infections.
Men will more likely suffer from what is known as PRIAPISM. This is a situation where the sickle cells block the blood vessels in the penis, causing severe pain and if not treated immediately, can lead to impotence. In other words, priapism is a prolonged erection of the penis that is not caused by sexual stimulation and is most common in men in their 30s. There are 2 main types of priapism known as Ischemic and Nonischemic priapism. The former is a result of blood not being able to leave the penis while the latter occurs when penile blood flow is not regulated properly. Ischemic priapism is usually painful while Nonischemic priapism is usually painless with both typically lasting more than 4 hours. A doctor should be consulted immediately in order to avoid tissue damage that causes erectile dysfunction and/or impotence.
Sickle Cell Disease in women becomes a huge concern when they are of child bearing age because of the fetal risks associated with typical high risk pregnancies. Women also face serious risks of experiencing both medical and pregnancy related complications like preeclampsia, preterm labour etc. Women with SCD also have higher rates of cesarean deliveries and women with the sickle cell trait (SCT) are also known to be at higher risk for Urinary Tract Infections and other infections during pregnancy. Another area of concern for women with SCD is menstruation as this can often be very painful and can contribute to painful crises.
It is clear that both sexes really do go through a tremendous amount of pain and discomfort and have to deal with all the many complications of the disease pretty much all of their lives. It is an even scarier thought that the mortality rate for sickle cell sufferers is placed between 48 - 50, that is if they receive adequate care while they are still children and make it past the age of 5. There are people who obviously have lived longer due to medical breakthroughs from research and stronger medication.
Parents and siblings of sickle cell sufferers also go through a lot; maybe not with physical pain but certainly a lot of emotional, financial and psychological struggles and I will try to address this in next week's post because Sickle Cell is such a devastating disease that takes a toll on everyone in the family.
As always, I appreciate you stopping by and taking the time to learn a little more about Sickle Cell Disease.