Living with sickle cell anaemia? Facts to keep eyes on

Globally, over 300,000 children are born with sickle cell anemia every year. Over 80 percent are born in Sub-Saharan Africa. About 240,000 children in Africa are born each year with Sickle Cell Disease (SCD). In Kenya, it is estimated that 6,000 children are born with the disease every year. According to the Ministry of Health, between 50 to 80 percent of children who are born with Sickle Cell disease die before their fifth birthday. In Kenya, it is estimated that 3 out of 10 people who live in the Coastal and Western regions are likely to have this disorder.

What is it?

According to the Ministry of Health, Sickle Cell is a genetic disease characterised by a change in the shape of a red blood cell. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. With this disease, the red blood cells become hard and sticky and change from a smooth circular shape to a crescent shape. According to the Centre for Disease Control (CDC), sickle cells die early. This results in the patient having a constant shortage of red blood cells. “When they travel through small blood vessels, the sickle cells with abnormal haemoglobin (haemoglobin-HbS) get stuck and clog the blood flow. This can cause pain and other serious problems such as infections, acute chest syndrome, and stroke,” the CDC explains. In addition, clogging leaves a patient feeling tired and out of breath with sudden attacks of severe pain called sickle cell crises. Without adequate oxygenated blood, tissues and organs such as the spleen, liver, and kidneys suffer damage over the years, with potentially life-threatening results. In a normal system, red blood cells deliver oxygen throughout the body, with the haemoglobin bending and adjusting to fit into narrow blood vessels in order to deliver oxygenated blood to tissues and organs. The CDC also explains that out of the various forms of Sickle Cell Disease, Sickle Cell Anemia is the most common and severe form of disease. This form of Sickle Cell is known as HbSS.

Causes and diagnosis

According to the CDC, Sickle Cell is a genetic condition usually present at birth. This condition is inherited when a child receives two sickle cell genes, one from each parent. The World Health Organization estimates that approximately 5 percent of the world’s population carries trait genes for haemoglobin disorders, mainly, sickle-cell disease and thalassaemia. Sickle Cell is usually diagnosed at birth using a blood test, but it can also be diagnosed before a baby is born.

Mitigation and drugs

According to guidelines from the Ministry of Health titled 'Sickle Cell Disease Control and Management', Kenya is looking to reduce the number of children born with sickle cell disease by more than 12 times. The Ministry of Health has announced attempts aimed at providing basic treatment medication required by patients. These include folic acid, hydroxyurea, vaccines, and analgesics for pain management. The hydroxyurea drug works in patients with sickle cell anemia by increasing fetal haemoglobin and decreasing the number of attacks. Apart from treating sickle cell anemia, the hydroxyurea drug is used in the treatment and management of cervical cancer, myelogenous leukemia (popularly known as myeloid leukemia or cancer of the white blood cells), and essential thrombocythemia (a condition in which the body produces too much blood platelets). 

There are newer drugs that have been developed and approved by the Food and Drug Administration (FDA) to reduce the number of painful crises. These include Endari (also called L-glutamine oral powder) which is recommended for patients aged five years and above. There’s also Adakveo® (crizanlizumab-tmca) a once-a-month drug administered as an infusion through the veins. This drug reduces the frequency of vaso-occlusive crises in adults and pediatric patients who are aged 16 years and older.

The CDC states that bone marrow or stem cell transplant is the only way to cure this condition. However, this is an expensive procedure that will be out of reach for the affected people in Kenya and the larger Sub-Saharan Africa region. “This procedure is done in cases of severe Sickle Cell and for children without any major organ damages from this disease,” the CDC states. With this procedure, only very close relatives such as a brother or sister are best for donation. “The procedure takes healthy cells that form blood from the donor and puts them into the patient.”