2023-06-12 04:40:11
Little known to the general public, sickle cell disease is nevertheless the most common genetic disease in the world. Originally, thehe disease mainly affected the African continent considered to be one of the original foci of the pathology. But with migratory movements, all countries are now affected by the scourge.
With just over 400 births of infected babies each year, France no longer escapes the phenomenon. In ten years, the number of cases has increased by more than 50% in the territory. The French regions are very unequally concerned. The disease is more present in the territories where the populations originating fromAfricad’Indethe West Indies, the Brazil and D’Europe from West. Île-de-France and the overseas departments are the regions most affected by the pathology.
Multiple and varied symptoms
Sickle cell disease is a severe chronic blood disease. It affects hemoglobin, a protein present inside red blood cells, which is used to transport oxygen from the lungs to all the organs of the body. It causes the deformation of the red blood cells which lose their rounded shape and take the form of a scythe, hence the name given to the disease. This deformation leads to the obstruction of small blood vessels and causes anemia. Some factors such as cold, stress, dehydration or a sudden change in temperature also lead to acute pain. Finally, it promotes sensitivity to certain microbes, such as pneumococcus, which can be responsible for very serious infections.
“Every time I see my 19-year-old daughter plunged into excruciating pain in her joints, chest or hips, I can’t help but suffer with her,” says Pauline Mbaku with a sigh. president and founder of the French association Espoir de vivre Since she was born, we go to the emergency room as soon as her temperature reaches 38.5°C because the situation becomes too critical for her. of sickle cell disease is to spend a lot of time in hospitals to see him undergo very heavy treatments.
Increase screening tests for couples
Doctors have solutions to try to treat the symptoms of the disease, such as antibiotics, morphine or preventive medication. Blood transfusions can also be performed on an ad hoc basis when complications arise. But to date, the only curative treatment remains bone marrow transplantation. However, this intervention remains complex to implement, risky and expensive. “However, there are simpler and less expensive solutions for the health system, gets carried away the volunteer of Congolese origin. It would be enough that there is more communication and prevention around this disease with couples of age to have children.”
To date, the only way to prevent a couple from having a child with sickle cell disease is to test both parents to verify that they are not healthy carriers of the disease. “From there, the partners can take their responsibilities: either they decide that love is stronger than anything and take the risk of having a sick child, or they decide to separate. In my case, I would have preferred to do the screening test and break up immediately. Anyway, when the sick child is there, the vast majority of couples tested by the disease in their life end up separating.”
“We talk a lot less regarding this disease than regarding the coronavirus”
The president of Espoir de vivre has since campaigned for public health awareness campaigns to be carried out among young workers. She would also like pastors to encourage more future spouses to carry out tests before uniting. In summary, she would like “that we take this disease seriously”, as we did for the Like.
“We forget this disease because it is not contagious, explains in a BBC article Robert Hue, former French parliamentarian and president of the NGO Drep Afrique. It’s not a disease like… AIDS. And yet, it kills a lot. There are 15 million sickle cell patients in Africa, with a severe form of the disease and very high mortality. About 200,000 people die from sickle cell disease every year in Africa, more than the coronavirus kills. And we talk a lot less regarding this disease than regarding the coronavirus“.
In addition to the burden of the disease, affected individuals living in Africa experience different forms of discrimination. “Children who suffer from it are mocked because they carry the stigma of the disease, especially with anemia, continues Pauline Mbaku. And then, they are pointed at because they show delays in growth and development. Moreover, in France too, the look that some have on the disease hurts.
“It’s not just a black disease”
Pauline Mbaku intends to take advantage of World Sickle Cell Day on June 19 to raise awareness of the disease by bringing together doctors, patients, association leaders and elected politicians at the National Assembly. The objective of this day is twofold: to encourage decision-makers to give additional resources to research and to alert the public authorities to the spread of this disease by developing communication campaigns. “Far too many people do not know this disease. It is urgent that everyone be made aware of it because it can affect everyone, it is not just a black disease”.
The fight of the association is not in vain. Activists for the cause recently won a great victory: since January 2023, on the recommendations of the Haute Autorité de Santé, France has now been organizing systematic screening of newborns. They were previously reserved for families most at risk of transmitting the disease, such as people of African, Caribbean or Mediterranean origin. “This measure was both discriminating and showed flaws in the system,” said the president of the association, who recently received the Legion of Honor for her fight once morest sickle cell disease. Research on this disease allows scientists to affirm that early management of this pathology can avoid serious health consequences. “The better we are informed, the more we will fight the disease”, concludes the associative activist.
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