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Sickle cell disease (SCD), is a genetic disorder, where the red blood cells become rigid and stick together and change from being disc-shape to crescent-shaped (like a sickle). The change in shape is cause by the presence of an abnormal form of haemoglobin, this is the protein in the blood cells that carries oxygen around the body.
In people living with SCD, the abnormal shaped red blood cells block the blood vessels, restricting the flow of blood to the organs and also limiting the amount of oxygen being carried into the body system. This can invariably dame the heart, lungs, spleen and other organs. The situation causes episodes of severe, or acute pain called vaso-occlusive crisis. These crescent-shaped red blood cells are destroyed at a faster rate than normal red blood cells.
There are currently a very limited range of treatments available for SC patients all over the world. The two most significant being the use of blood transfusions and these medicines. The oral medications available for SCD are limited to Oxbryta, Hydroxyurea and Crizanlizumab, (this is injected into the vein). These drugs do reduce the frequency of SC crises.
Another genetic disease that is called Cystic Fibrosis (CF) and it mainly affects white people, whereas Sickle cell affects ethnic minorities. Sickle cell was identified in 1956 and CF was discovered in 1988. To date, there are three tablets developed for SCD and 511 for CF. There has been medical transplantation available for SCD since 2019 but for CF, transplantation has been available since 1989.
The bias and prejudice can no longer be ignored. Every SCD crisis has the potential of being the last one because of how the blood and oxygen are constricted, when one has a crisis. A Sickle cell crisis blocks blood flow to organs and deprives the affected organs of blood and oxygen, as previously stated. Due to the crescent-shaped blood cells, blood in the body is chronologically low in oxygen. This lack of oxygen-rich blood can damage nerves and organs and this makes a crisis fatal.
We are grateful for the three tablets that can make a positive difference in the life of anyone living with SCD. However more funding is needed into SCD like it’s counterpart CF. The disparity in how people who live with SCD are suffering because of lack of research available is glaring for all to see.
It is clear that decades of underinvestment in SCD research has led to a dearth of treatment options for SC patients. Increasing the level of research and the availability of treatment option is key t improving SC care outcomes says the SC Society.
Can I ask that those who are interested in medicine, those who are already studying medicine and can specialise in haematology, could you please do so?. The future generation need more of our people studying and looking into our genetic disorder.
My book – How to Live with Sickle Cell is available on www.amazon.com for purchase.
