Sickle cell disease results from genetic malformations that cause red blood cells to take on an abnormal sickle shape and rigid texture. Some people with the condition inherit irregular haemoglobin S genes from each parent, while others only inherit one haemoglobin S gene and one haemoglobin C gene. This leads to a less serious form of the disease known as HbSC.
People with HbSC disease often experience intense periods of pain (known as pain crises) and anaemia. Doctors can address these health effects with medication, which can improve the function of red blood cells in the body. For instance, some drugs stop cells from sticking together and forming clumps, while others prevent cells from clinging to the walls of the blood vessels.
When it comes to pain crises, a few medications can help alleviate discomfort. Along with nonsteroidal anti-inflammatory drugs and opiates, patients may also receive L-Glutamine, which protects red blood cells.
Acute chest syndrome is a serious complication of sickle cell disease. It occurs when abnormal red blood cells block the airways in the lungs, which can significantly restrict breathing. Hydroxyurea is a chemotherapy drug that can prevent complications or decrease their severity.
Along with the above medications, management of sickle cell disease can also involve blood transfusions. These transfusions reduce the impact of abnormally shaped red blood cells by providing the body with healthy versions of these essential components.
Because HbSC disease is associated with less serious health effects, people with this form of sickle cell disease will require blood transfusions less frequently. However, they are still a crucial aspect of the treatment regimen when it comes to reducing the chances of serious complications.