Sickle cell anaemia is an inherited disorder that causes the body’s red blood cells to lose shape and break down. As a result, the body does not have enough healthy red blood cells to transport oxygen to your organs.
Healthy red blood cells are round, flexible, and flow through your blood vessels with ease. Sickle cell anaemia causes red blood cells to become rigid, sticky, and crescent in shape. As a result, they can block blood vessels in the body, restricting blood and oxygen flow, and causing intense pain in the process.
According to the American Society of Hematology, sickle cell traits are more common in certain ethnic groups than others. Individuals of African descent, Hispanic-Americans from Central and South America, and individuals of Middle Eastern, Asian, Indian, and Mediterranean descent are known to be at higher risk of developing sickle cell anaemia.
According to WHO, between 20 and 30 per cent of the population in African countries such as Cameroon, Gabon, Congo, Ghana and Nigeria, carry the sickle cell trait.
Sickle cell anaemia causes painful swelling of the hands and feet, vision problems, and fatigue. The most prominent of these symptoms, however, is severe pain, that takes place in episodes and lasts from up to a few hours to a few weeks. The ailment can also make individuals more prone to infections. This is because the ailment causes red blood cells to attack organs that fight off infections.
Sickle cell anaemia can also cause complications such as stroke, acute chest syndrome, pulmonary hypertension (high blood pressure in the lungs), blindness, and organ damage.
Doctors generally prescribe painkillers to deal with the many painful symptoms that sickle cell Anaemia brings. Sickle cell anaemia is generally incurable. Treatment is aimed at avoiding episodes of extreme pain, complications, and relieving other symptoms that may arise.
If complications such as strokes do occur, a bone marrow transplant is recommended as a long term solution.
Masha Atola, a 9-year-old girl from Kenya, suffered from Sickle Cell Anaemia all her life. As a result, her daily life has been fraught with numerous challenges. The ailment caused her to develop acute chest syndrome and has kept her out of school for close to two years.
In 2015, Masha’s family approached Medanta after the little girl suffered a severe stroke that left the right side of her body paralysed. Her treatment was handled by Dr. Satya Prakash Yadav, Director of Paediatric Haematology, at Medanta’s Cancer Institute.
Dr. Yadav immediately recommended a bone marrow transplant - a medical procedure that replaces the sick cells responsible for the production of sickle-shaped red blood cells with new healthy ones from a healthy and compatible donor marrow. Fortunately, finding such a donor was easy in Masha’s case as her brother was identified as a strong match through an HLA ( Human leukocyte antigen) typing test. HLA typing is a blood test that is used to match donors with compatible recipients.
The procedure to replace Masha’s bone marrow was a flying success thanks to Dr Yadav and his team at Medanta’s team of Paediatric care professionals who went the extra mile to ensure that Masha’s quality of life was significantly improved for the better as she walked out of the doors of Medanta with her family.