World Sickle Cell Day: Symptoms and treatment options for Sickle Cell Disease – Times of India

Sickle cell disease (SCD) is an inherited health condition and one of the most common blood disorders globally. In India, over 1 million people are affected by sickle cell disease every year; geographically, there is a sickle cell belt that comprises of some tribal communities in Madhya Pradesh, Chhattisgarh, Maharashtra, Kerala, Gujarat, Tamilnadu, and Odisha.World Sickle Cell day is observed every year on June 19thto raise awareness about sickle cell disease, itschallenges and treatment options.Sickle cell disease (SCD) is a group of blood disorders inherited by a child from its parents.The most common type of sickle cell disease is known as Sickle Cell Anaemia,an abnormality in the oxygen-carrying protein (hemoglobin) in red blood cells. Due to this abnormality, the body does not have sufficient healthy red blood cells to carry oxygen.Normally, the flexible, round red blood cells move easily through blood vessels. In sickle cell anemia, the red blood cells are shaped like sickles or crescent moons. Thus, the name Sickle Cell Disease was derived. These rigid, sticky cells get stuck in small blood vessels, slowing down or blocking the blood flow and oxygen to parts of the body.Symptoms to watch out forSymptoms of sickle cell anemia usually appear when the child is around 5 months old. Major symptoms include: Episodes of pain in joints, abdomen or chest Swelling of hands and feet Frequent infections Delayed growth or puberty Vision problems

Sickle cell anemia is caused by a mutation in the gene found on the short arm of chromosome 11.In this, hemoglobin S replaces both -globin subunits in the hemoglobin (HbS). During the last 50 years, several epidemiological surveys conducted in various ethnic groups have found the prevalence of sickle gene to be 0-18% in north eastern India, 0-33.5% in western India, 22.5-44.4% in central India, and 1-40% in southern India.

Treatment options to considerTreatment of sickle cell focuses on preventing and treating complications associated with the disease. People with Sickle Cell Anaemia need regular life-long blood transfusions. However, a stem cell transplant (Peripheral Blood Stem Cell) offers the best chance of survival and cure. This transplant can come from a sibling or a family member. However, there is only a 30% chance of finding a matched sibling donor in the same family. The remaining 70% patients look for a matching donor through a stem cell registry or donor Centre a database of voluntary donors between the age group of 18 to 50 years.

Ethnicity a key factor while looking for a matching donorA stem cell transplant is successful only when the donors HLA type closely matches that of the patient. The bodys immune system has proteins known as Human Leukocyte Antigen (HLA) to distinguish cells that belong to the body from those that do not.

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World Sickle Cell Day: Symptoms and treatment options for Sickle Cell Disease - Times of India