Sickle cell disease
What is it ?
Sickle cell disease
Sickle cell disease is a genetic disorder of hemoglobin, a substance in red blood cells that gives blood its red color and carries oxygen. The defective gene is inherited from both the mother and father. 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 become abnormally deformed, taking on the shape of sickles, thus blocking the small vessels. This leads to a lack of oxygenation of the organs.
Jean-Benoît Arlet, hospital practitioner in internal medicine, Georges Pompidou European Hospital.
National Reference Centre for Sickle Cell Disease.
The disease is manifested by anaemia (which can lead to fatigue, dizziness, shortness of breath, etc.), susceptibility to infections, and above all sudden, unpredictable and excruciating painful attacks. unpredictable and excruciating attacks of pain. The latter are called vaso-occlusive attacks. They are caused by poor blood circulation and mainly affect the bones (back, ribs, pelvis, legs, arms, etc.) and the stomach (in children). They can be very temporary or, on the contrary, last for a long time (from a few hours to several weeks). The violence, brutality and intensity of the pain felt by the patients characterise the disease. A high infant mortality rate in Africa is due to a lack of resources to treat these children.
A universal challenge
Since 2009, the fight against sickle cell disease has been one of the WHO's priorities for Africa. priorities for the African region and ranks fourth in global public health public health priorities, after cancer, HIV and malaria. and malaria. It is the most common genetic disease in the world.
In Africa, a child is born with sickle cell disease every
every 2 minutes. The estimated total number of patients in this continent is between 10 and 15 million.
continent is between 10 and 15 million patients. 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 insufficient access to drugs and lack of training of professionals.
lack of training of professionals. Carriers of the disease, who are not sick,
who are often unaware that they have this genetic anomaly,
of the population in sub-Saharan African countries.
countries. There are therefore hundreds of
of millions of healthy carriers of this disease in Africa. But a couple
each of whose members is a carrier, has a one in four chance of giving birth to a child
of giving birth to a sick child.
In France, there are more than 27,000 patients (children and adults) living with
with this disease. It regularly requires emergency hospitalisation to manage
to manage painful attacks, requiring morphine injections.
morphine injections. Infant mortality is very low in industrialised countries. However, the disease remains unknown
of the general public and, more seriously, of healthcare workers. The
affected populations, often in social difficulty without political or media
or media, have great difficulty in making their voices heard.
their voices heard.
Strong training actions for professionals are necessary. It is urgent to get out of this oblivion which costs the life of young patients, energetic and optimistic despite their disability.
Doctor, professor of medicine,
Coordinator of the Scientific Council of Drep.Afrique
For more information...
SICKLE CELL DISEASE IN AFRICA
To learn more about the issue of sickle cell disease in Africa, here is Dr Honsel's medical thesis, under the direction of Dr Jean-Benoît Arlet, October 2018.
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