Since June 19th, 2008, World Sickle Cell Awareness Day has been held annually in order to help increase public knowledge and raise awareness of Sickle Cell Diseae (SCD) and the struggles sufferers and their families go through.
SCD is an inherited blood disorder that can be acquired from either one or both the parents. The irregular shaped red blood cells (RBCs) in this condition get stuck in the small blood vessels and slow down or block the blood flow and oxygen to various parts of the body. Imaginably, it is a painful disease and while there is no cure yet for sickle cell anaemia, certain treatments can help relieve the pain and avoid ailments associated with this disorder.
What are the symptoms?
* Patients can develop low haemoglobin, requiring transfusions.
* They can also develop periodic episodes of pain, called crises starting from the first year of life, which can be very disabling.
* Such patients are also prone to developing repeated pneumonia, painful swelling of hands and feet, sudden enlargement of the spleen, stroke, vision problems, delayed growth and other complications of the bone.
What are the precautions one can take?
* Dehydration and lack of oxygen must be avoided.
* Patients must remain hydrated at all times and should take extra care if they are travelling to higher altitudes.
* Patients affected by the disease should follow up with haematologists on regular basis.
What is the treatment available?
* Patients with this condition should have additional folic acid and protection from pneumonia in the form of a vaccine and antibiotic prophylaxis.
* Hydroxyurea can significantly benefit patients suffering from this disease in reducing or eliminating the requirement for blood transfusion, reduction in painful episodes and decrease in the risk of stroke and other complications associated with the disease.
* The only curative treatment of the disease is Allogeneic Bone Marrow Transplant, which has a very high success rate. If there is Human Leukocyte Antigen (HLA) matched donor in the family, then that donor is preferred the most.
* Currently, newborn screening programs have been initiated in certain parts of India, so that affected babies can be identified early and appropriate treatment can be started.