In normal form, red blood cells are flexible and round which can easily pass through the blood vessels. But in a sickle cell disease, the RBCs become sticky or rigid just like a sickle. This irregularity inshore causes the cells to get stuck in the vessels causing pain and obstructing blood flow and causing deoxygenation in several organs of the body.
There is no firm cure to delay with sickle cell disease. Although, treatment can help to minimize pain and relieve symptoms.
What are the major signs to look for a sickle cell disease in a person?
Following are a few signs and symptoms of the disease:
• Pain episodes: Sickle cell crises causes pain in legs, joints, chest etc. Swelling might appear because of blood flow obstruction.
• Anemia: Sickle cell has a short life cycle of 10 to 20 days in comparison to the normal cells which have 120 days of the cycle. So this leads to lesser blood cells and hence makes a person anemic.
• Infections: SCD makes a person very prone to liver and spleen infections. Children are vaccinated to protect against pneumonia etc.
• Vision troubles: Since the blood supply gets affected due to sickle cell obstructing the vessels. This may cause retina damage and vision issues.
What are the recent treatment options for sickle cell disease?
1). Bone marrow transplant:
This is the best procedure to cure SCD but it has its own risks and it is not suitable for every patient. BMT is mainly advisable for young patients below 18 years of age. But finding a matching donor is a hurdle in this process. Your doctor can suggest you best if your child or relative is suitable for BMT or not. This treatment procedure might not be affordable for every patient although India provides BMT at economic prices.
2). Gene therapy:
Several types of research are still ongoing to see if the defected gene can be replaced with a normal gene. This can help the body to make normal red blood cells.
3). New medications:
New drugs are being designed and synthesized as per the requirement. The main goal of these drugs is to prevent the sickle cell from sticking with the walls of vessels or interfere in sickle cell formation or increase hemoglobin levels etc. Each drug has its own specific task and accordingly as per the patient’s need it can be given. It might take some time for these drugs to be readily available.
1) Q: What are the complications expected along with sickle cell disease?
A: Several complications might arise along with sickle cell disease. Some of them are listed below:
• Diseases like gallstones, stroke, eye damage, leg ulcers which do not heal, lung crises, and pulmonary hypertension etc.
• Regular blood transfusions might lead to iron accumulation or overload in the body. Most of the patients receive occasional transfusions but stroke and acute chest syndrome patients might require monthly transfusions and hence chances of iron accumulation increases. But Hydroxyurea treatment can further help in reducing transfusion need.
2) Q: When should one expect to observe symptoms of sickle cell disease?
A: Sickle cell disease symptoms usually do not appear until 4 months of age after the baby is born. But symptoms once appear includes painful crises which cause pain in legs, joints, lower back, and chest.
3) Q: What happens to the hemoglobin in sickle cell disease?
A: Hemoglobin is present in the red blood cells. But in sickle cell disease the presence of hemoglobin S makes it C-shape or crescent-shaped. Sickle cell disease is normally has a link with a shorter span of life than normal. However, currently with the available treatment options for SCD makes the survival rate for the patients better than before.