Sickle cell HbSC is a form of sickle cell anaemia. Sickle cell anaemia is a disease that occurs when a child inherits abnormal haemoglobin genes. Haemoglobin (Hb) is a crucial component of the red blood cells, allowing oxygen to bind. Abnormally shaped haemoglobin changes the shape of the red blood cells to a “sickle” shape, causing them to become sticky and hard instead of flexible. As a result, sickle cells often live for less time than normal red blood cells would. This can cause a lack of red blood cells. Sickle cells also have a tendency to get stuck and stop proper blood flow.
HbSC is just one type of sickle cell disease (SCD). In HbSC, the gene from one parent encodes for haemoglobin “S”, the type of haemoglobin that causes the classic sickle cell to form. The haemoglobin gene from the other parent encodes for haemoglobin “C”. Haemoglobin C is still an abnormal form of haemoglobin, but it does not cause sickling of red blood cells.
Common symptoms of HbSC sickle cell include:
Those living with HbSC sickle cell typically experience mild to moderate forms of the above symptoms. Because people with HbSC have abnormal red blood cells – half of them being sickled- they still experience the symptoms and complications of SCD. However, since people with HbSC do not have two copies of haemoglobin “S”, they are likely to experience less severe symptoms than people with two copies of haemoglobin “S”.
HbSC sickle cell anaemia is caused by mutations in the genes coding for haemoglobin, an essential protein that helps oxygen bind to red blood cells. Specifically, it’s caused by inheriting a copy of a gene encoding for haemoglobin “S” from one parent and a copy of a gene encoding for haemoglobin “C” from the other parent. These are both abnormal forms of haemoglobin.
Having parents of African, Middle Eastern, or Mediterranean descent increases the chances that you will inherit the sickle cell trait or be born with sickle cell disease significantly.
A doctor will diagnose HbSC sickle cell based on a comprehensive medical history, current symptoms, and the results of a blood test. While newborns are often tested for sickle cell so that treatment can begin as early as possible, an older child or adult can be tested as soon as symptoms are suspected. A blood test for the sickle cell traits and other types of abnormal haemoglobin can confirm the diagnosis.
Complications of HbSC sickle cell can include stroke, chest pain, fever, trouble breathing, high blood pressure in the lungs, organ damage, blindness, leg ulcers, and clots in the lungs or veins.
It’s very important to note that people with HbSC sickle cell are prone to experience the complications listed above, but are much less likely to experience them than people with two copies of haemoglobin S. While it is possible for people with HbSC to experience these complications, it’s not common.
Treatment of HbSC sickle cell is focused on managing any symptoms and complications. There are a variety of preventative measures people with HbSC sickle cell can take to maintain a good quality of life.
These include lifestyle changes, like staying hydrated, maintaining a healthy body temperature, avoiding high altitudes, and avoiding places with low oxygen levels. Handwashing and proper cleaning of food can also help.
Medical interventions like childhood vaccinations and treatment with penicillin or another antibiotic can help prevent infections.
There are approved medications for the treatment of the symptoms and complications of SCD. Currently, stem cell transplant is the only way to potentially cure SCD.