World Sickle Cell Day: Symptoms And Treatment Options For Sickle Cell Disease

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. World Sickle Cell day is observed every year on June 19th to raise awareness about sickle cell disease, its challenges 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.

World Sickle Cell Day 2021: Symptoms to watch out for

Symptoms 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 beta-globin subunits in the hemoglobin (HbS).

Also read: Sickle Cell: Know The Types, Symptoms, Causes And Treatment

Treatment options to consider

Treatment 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.

Also read: Do You Know What Causes Sickle Cell Anaemia? Find Out Here

Ethnicity - a key factor while looking for a matching donor

A stem cell transplant is successful only when the donor's HLA type closely matches that of the patient. The body's immune system has proteins known as Human Leukocyte Antigen (HLA) to distinguish cells that belong to the body from those that do not.

India is a melting pot of various cultures, ethnic groups, languages and mixed marriages. This has resulted in a unique gene pool and HLA frequency pattern. Since Sickle Cell Disease is more prevalent in certain ethnic groups in India, there is an urgent need for people to register as potential blood stem cell donors with any of the stem cell registry in India. This will add to the database and increase the chances of finding a donor from various ethnic groups for a patient.

Also read: Sickle Cell: Causes, Symptoms And Survival Rate Of This Common Genetic Disease

(Dr. Sanjana Dontula, Quality Management and Medical Partnerships at DKMS BMST Foundation India)

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