What is Sickle cell disease?

Sickle cell disease is a genetic disorder of hemoglobin, a substance in the red blood cells that gives red colour to blood and is used to transport oxygen. The defective gene is passed on by both father and mother. In patients with sickle cell disease, these red blood cells have two problems:

-1- they are more fragile and destroyed much faster (10 days against 120 days normally) which leads to anemia.

-2- they deform abnormally, take the form of a sickle which thus clog small vessels. This results in a lack of oxygenation of the organs.

THE FIGURES :

- 30,000 patients in France, 7 to 10 million in Africa

- In 2021 worldwide, 515,000 people were born with sickle cell disease, of which 405,000 in Africa (79%)

- In Africa, 1 birth/minute is affected by the disease

- Unfortunately one person with sickle cell disease dies every 2 minutes (265,000 sickle cell deaths in Africa in 2021, mainly children)

The disease manifests itself in anemia (which can lead to fatigue, dizziness, shortness of breath, etc.), a sensitivity to infections, and especially brutal, unpredictable and excruciating painful attacks. The latter are called vaso-occlusive crises. They are caused by poor blood circulation and mainly affect the bones (back, ribs, pelvis, legs, arms, etc.) and belly (in children). They can be very transient or, on the contrary, settle down over time (from a few hours to several weeks). The violence, brutality and intensity of pain experienced by patients are characteristic of the disease. High child mortality is hitting Africa for lack of resources to care for these children.

Fact sheets

A Universal Challenge

The fight against sickle cell disease has been one of the WHO’s priorities for Africa since 2009 and ranks fourth in global public health after cancer, HIV and malaria. It is the most common genetic disease in the world.

In Africa

In Africa, a child is born with sickle cell disease every minute. The estimated total number of patients in this continent is between 7 and 10 million. This is close to the number of HIV patients in Africa (26 million). Unfortunately, many of these children will die before they become adults due to inadequate access to medicines and lack of training for professionals. The carriers of the disease, not sick, and who most often do not know that they have this genetic anomaly, represent up to a third of the population in sub-Saharan African countries. So there are hundreds of millions of healthy carriers in Africa. However, a couple with each of the members carrying it has, in every pregnancy, one risk out of four to give birth to a sick child.

In France

In France, more than 30,000 patients (children and adults) live with this disease. It regularly requires emergency hospitalizations to manage painful seizures, requiring morphine injections. Infant mortality is very low in industrialized countries. However, the disease remains unknown to the general public and, more seriously, to health care personnel. The affected populations, often in social difficulties without political or media outlets, have great difficulty making their voices heard.