Sickle Cell Disease

SickleGenAfrica

Sickle Cell Disease Genomics Network of Africa

Sickle Cell Disease


Sickle cell disease affects one in fifty births in Ghana. This means that about

14,000 babies are born each year with sickle cell disease in Ghana. Of the

record number of 2,400 babies born on New Year’s day in 2018 in Ghana; 48

would have been born with sickle cell disease without their parents knowing

about it because we don’t have a national program for screening for the disease.


Sickle cell disease is an inherited blood disorder caused by a mutation in

haemoglobin; the red coloured protein inside our red blood cells. The sickle

haemoglobin causes red blood cells to change their normal shape into banana-

like shapes, which are unstable resulting in the cells breaking up easily, and

blocking the blood vessels.

The common symptoms are:

Anemia. 

Sickle cells break apart easily and become damaged leaving patients without enough intact red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually become irreversibly damaged in 10 to 20 days, leaving a shortage of red blood cells (anemia). Without enough red blood cells, your body can't get the oxygen it needs to feel energized, causing fatigue.

Painful swelling of hands and feet. 

The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.

Episodes of pain.

Periodic episodes of pain, called crises, are a major symptom of sickle cell disease. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to many parts of

the body including the chest, abdomen and joints. Pain can also occur in bones. The pain varies in intensity and can last for a few hours to a few weeks. Some people have only a few pain episodes. Others have a dozen or more crises a year. Patients may need to be hospitalized if a crisis is severe enough. Some adolescents and adults with sickle cell disease also have chronic pain, which can result from bone and joint damage, ulcers and other causes.

Frequent infections. 

Sickle cells damage the spleen, which is an organ that fights infection, leaving patients more vulnerable to infections. Doctors commonly give infants and children with sickle cell disease vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.

Testing:

A simple blood test called hemoglobin electrophoresis can determine whether you have sickle cell disease or not.

You can get if from only one parent:

Not true, both parents must transmit their sickle haemoglobin (or another haemoglobin such as haemoglobin C, which when combined with one sickle haemoglobin can cause sickle cell disease) to the child, for the child to get sickle cell disease.

Sickle cell patients are bound to die before the age of 21, and not suitable for marriage:

Not true, with proper management sickle cell patients can live a productive adult life. It is important to find them at the time of birth to put them on therapy to stop infections. If managed well sickle cell patients grow to become healthy adults with active employment in the medical profession, as actors.

Myths about sickle cell disease:

The disease occurs only occurs in blacks;

That is not true. It is found among people in India, the Mediterranean, and African origins.

Sickle-trait people get sickle cell disease as they get older;

Not true. Once you have sickle cell trait, that is what will remain with you till you die. The genes you inherent do not change over time.

There is no cure for sickle cell disease:

Not true, there is actually a cure for sickle cell disease called bone marrow transplantation (BMT). However, this cure is challenging, and expensive. There is no BMT in many African countries including Ghana.

SickleGenAfrica is funded by NIH Common Fund Award / NHLBI Grant Number U54HL141011

The SickleGenAfrica website content is solely the responsiblity of the authors and does not necessarily represent the official views of the National Institutes of Health.

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Sickle Cell Disease Genomics Network of Africa (SickleGenAfrica).


SickleGenAfrica: A research network of the H3Africa consortium

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