Sickle cell disease, also known as sickle cell anemia, is a group of disorders that lead to deformation and break down of red blood cells. A new study by researchers at the Massachusetts Institute of Technology (MIT) analysed blood from patients with sickle-cell disease and revealed the process of cell clumping. It showed how the disease occurs when deformed red blood cells clump together, blocking tiny blood vessels and causing severe pain and swelling in the affected body parts.
“These painful crises are very much unpredictable. In a sense, we understand why they happen, but we don’t have a good way to predict them yet,” said Ming Dao, a principal researcher and one of the senior authors of the study. The findings of this study are a major step towards being able to predict when such a crisis might occur.
The study showed that these painful events are most likely to be produced by immature red blood cells, called reticulocytes, which are more prone to stick to blood vessel walls.
To prove how red blood cells interact with blood vessels to set off a vaso-occlusive crisis, a specialised microfluidic system was built by the researchers that mimics the post-capillary vessels, which carried deoxygenated blood away from the capillaries. These vessels, about 10-20 microns in diameter, are where vaso-occlusions are most likely to occur.
What is sickle cell anemia?
Red blood cells or RBCs are normally shaped like discs providing them the flexibility to travel through even the smallest blood vessels. However, in a person suffering from sickle cell disease, the RBCs have an abnormal crescent shape resembling a sickle. This makes them sticky, rigid and prone to getting trapped in small vessels, which blocks blood from reaching different parts of the body. It causes pain and tissue damage.
Sickle cell anemia symptoms
The symptoms usually start to appear at a very young age. While there are multiple types of SCD, they all have similar symptoms, which vary in severity. These include –
1. Excessive fatigue or irritability, from anemia
2. Fussiness, in babies
3. Bedwetting, from associated kidney problems
4. Jaundice, which is yellowing of the eyes and skin
5. Swelling and pain in hands and feet
6. Pain in the chest, back, arms, or legs
Sickle cell anemia prevention and treatment
There are a number of ways to treating this disease including bone marrow transplant. These include –
1. Rehydration with intravenous fluids. This helps the RBCs to return to a normal state.
2. Blood transfusions improve transport of oxygen and nutrients as needed. Packed red cells are removed from the donated blood and given to patients.
3. The patients are given supplemental oxygen through masks which makes breathing easier and improves oxygen levels in the blood.
4. The patients are also given pain medication to relieve the pain during a sickle crisis.
The methods of prevention include immunizations as patients tend to have lower immunity. Eating an adequate amount of fruits, vegetables, and whole-wheat grains help your body make more RBCs. Drinking more water reduces the chances of sickle cell crises. Regular exercise helps to reduce stress which reduces crises.