Sickle Cell Disease is basically an outcome of genetically passed down disorders, sickle cell anemia being the most common of all. The normal Red Blood Cells which contain Hemoglobin, a protein that transports oxygen in the blood, are round and flexible which enables them to pass through narrow blood vessels and carry oxygen to all parts of the body. This disease deforms them into a crescent shape, more like a sickle. Such shaped RBCs easily break apart causing anemia.
Where healthy RBCs live for about 120 days, there on the contrary sickle RBCs live for mere 10-20 days. Not only this, these sickle cells form a cluster, stick to the walls of the blood vessels, thereby hindering normal blood flow.
What Causes Sickle Cell Anemia
It is caused due to mutation in the gene that directs our body to form hemoglobin. A child suffers from this disease if and only if both his parents pass on this defective gene i.e when he inherits two abnormal genes. In case only one parent passes on the mutated gene, the child is said to have a sickle cell trait where the child becomes only a carrier of this disease and not a sufferer. However, they do have sickle RBCs in their blood but not enough to show any symptoms or cause any problem.
How To Know
The symptoms of Sickle Cell Disease do not show up in an infant until the age of 4 months. It may result in both acute and chronic complications with a high mortality rate. The signs include:
- Chest pain due to restricted blood flow.
- Frequent infections due to spleen damage.
- Swelling in hands and feet.
- Difficulty in breathing.
- Anemia leading to pale appearance, fast heart rate and fatigue.
- Accelerated drops in hemoglobin level.
How To Fight Sickle Cell Disease
The infections can be taken care of through vaccination and antibiotics, high fluid intake, folic acid supplementation, and pain medication. Other measures may include blood transfusion and medication hydroxy-carbamide which is used to reduce the rate of painful attacks. It can also be cured by bone marrow cells’ transplantation in some cases. Progress is being made in the development of new medications to prevent the formation of sickle cells. Scientists still need to go a long way to produce better results.