What is it?
Sickle cell disease is an illness that affects the red-blood cells of the body, specifically a protein that they carry called hemoglobin. Hemoglobin carries oxygen throughout the body. Usually, a human’s red blood cells are round and smooth, almost disk-shaped. This enables them to carry oxygen through the small and narrow blood vessels. In people with sickle cell disease, their red blood cells are shaped like a “C” which looks like a sickle (a farming tool), therefore carrying the name sickle cell disease.
Well, sickle cell anemia is one of the most common genetic disorders in the world, after disorders like down syndrome and cystic fibrosis. There is no proper cure for it, as there are many uncertainties in the methods they currently have. Those who carry the trait are born with it, however signs don’t appear until the baby is about 5 or 6 months old. This means that the effects are felt during childhood.
Now that you have a better understanding of the disease, let’s dive into the specs of the disease.
1. How does one inherit the disease?
It’s passed down when a child inherits two abnormal hemoglobin genes, one from each parent. These abnormal hemoglobin genes are also knows as the sickle cell trait (SCT). When both parents carry the SCT, there’s a 25% chance that the child will have SCD (sickle cell disease).
If one parent has the gene for sickle cell disease and the other parent has a normal gene, the child will have the SCT. For most people, having this trait does not provoke any symptoms of SCD.
2. Where did it originate?
As per the SCDAA, sickle cell disease originated in 4 different parts of Africa and has spread wherever the Africans have migrated. It entered the slave trade in the U.S., and is now found in most areas of the world, ranging from Europe to Portugal, going as far as India and Sri Lanka.
Because of the area where this disease originated, not much attention to come up with a cure was brought to it. Personally, this is what fuels my anger.
It’s due to the racial stigma associated with this disease, that makes it the culprit. A cure for this disease could have been found much earlier, if people gave this disease the importance it deserves, regardless of the colour of skin of the people who have it.
3. Is there a cure?
Yes, well…no. There is treatment available, as SCD worsens over time. Babies with SCD must take daily doses of penicillin until the age of 5, as this is the age at which the risk of life-threatening blood stream infections decreases. After this point, children who are at a low-risk can take part in physical activities, but they must take breaks so that oxygen can travel through the body.
Adults who are having chronic pain due to SCD are prescribed hydroxyurea. This is an oral medicine that increases the amount of hemoglobin in the blood, which lessens their pain. Patients at a low-risk are required to take frequent blood and urine tests to watch for anemia and kidney damage.
It’s a problem. This disease has been in our world for more than 40 years, and there still isn’t a proper cure for it. It primarily affects children, babies for that matter.
40 years ago, the average lifespan of a patient with SCD was 14 years. That’s nearly a fourth of some of your lives. Now, the average patient can live to be over 50.
We’ve certainly made progress, don’t get me wrong, but I believe that SCD has a much bigger possibility of being cured than we think.