“To do things in a better way one has to be free from the disease and have a better life.”
Sickle (hasya in hindi), ¼ moon-shape
19th June is celebrated as World Sickle Cell Day all around the globe to spread the awareness about the Sickle Cell Disease which is a genetic, inheritable, and fatal disease causing red blood cell disorder which can lead to death also.
In Sickle cell disease the red blood cells are not properly shaped as they are required by the body. The red blood cells gets stuck into the small blood vessels and creates a blockage for the flow of blood in the body, because of which the reach of oxygen in the body is not proper which results in the Headache, Heart Disease, Severe Infections, Stroke, Damage of the Organ / Failure and many more.
It is an abnormality of haemoglobin (the oxygen carrying substance) structure in red blood cells (RBC’s). The RBC’s in patient are rigid as against malleable RBC’s in normal person. Hemoglobin is protein, which is present in the blood, gets affected due to Sickle Cell Disorder. This protein helps to supply oxygen throughout the body. The normal hemoglobin is called HEMOGLOBIN A (HbA) while the hemoglobin which is affected by sickle cell disorder is known as HEMOGLOBIN S (HbS).
In India Sickle Cell diases is commonly found in tribal population of central India, Northern Kerala, Tamil Nadu and North-Eastern India.
People suffering from Sickle Cell Disorder are more prone to other diseases as well like getting infection, more chances of getting stroke or acute chest syndrome because the flow of the blood in the body is not proper as it supposed to be.
The disease starts at the age of 5-6 months. This abnormal Haemoglobin of Sickle Cell disease gets distorted in Oxygen deficiency. The symptoms depend upon the extent of Oxygen Deficiency. The distorted RBC take the shape of Sickle and are not able to flow in blood freely and get stick to each other and thus blocking the blood flow in the particular area.
Oxygen deficiency can be precipitated by dehydration, infection, exercise, high temperature and high altitude. Sickle cell disorder is not contagious like cold and flu. A person who is suffering from sickle cell disorder has inherited two sickle cell genes, one from each parent. People who are suffering from this disorder aren’t aware that they carry the genes as they don’t have any sign of diseases, but they can pass the sickle cell disorder gene to their children.
People especially teens who have this sickle cell disease have some degree of anemia as they get tired easily, look pale or have fast pulse rating. People suffering from the disorder of sickle cell have tough time in fighting with other diseases / infections.
If we look at the database of Sickle Cell Disease in India, fewer than 1 million cases are there in a year. It is possible for old age people to be ill enough to die from this disease. Taking proper balanced and healthy diet, drinking plenty of fluid to prevent dehydration, exercising regularly, avoid alcohol, avoid extreme temperature & high altitudes,
avoiding smoking and taking plenty of rest are the basic health steps which a person can follow to stay healthy if he/she is suffering from sickle cell disorder, proper timely management of infections specially that of chest infection, apart from this medication is the basic cure for this disease prescribed by doctors in the early stages of the disease but when the severity increases Stem Cell Transplant is the only cure, but it’s a complex and risky procedure to be followed.
Gene Therapy is going to be one of the best treatment for the Sickle cell Anemia for which the research and study is still going on by our scientists.The patient should get properly evaluated by Clinical Psychologist.