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INTERVIEW: DOCTOR ALEXIS ELIRA DOKEKIAS. Full Professor in Hematology and Internal Physician, c

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Sickle cell disease is the first hereditary genetic disease in the world. It affects 1 in 4 Congolese in its partial form and 3 in 100 babies are born with the total form of the disease. In an interview, the Director General of the Centre national de référence de la dépanocytose (CNRDr) "Antoinette Sassou N'Guesso", Prof. Alexis Elira Dokekias noted the progress made thanks to the advocacy of the First Lady of the Congo and the construction of the CNRDr which bears her name. He invited the public to be screened early. Interview by Yvette Reine Boro Nzaba

THE HEALTH METROPOLIS +: What are the signs of panocytosis? ALEXIS ELIRA DOKEKIAS : Panocytosis is a very serious genetic disease that manifests itself in various signs: acute anaemia; or chronic, increased awareness; infections, painful seizures caused by poor blood circulation, especially in bones, etc. The manifestations of panocytosis are very variable for a person who is an agrovector. the other. Serious complications may occur, and diagnostics may not require close monitoring; Throughout life. In Congo, the abnormal gene S that characterizes this disease affects 1 in 4 Congolese in its partial form and 2 in 100 children are born in its total form. Scratch the Congolese population is sufficiently educated.
What is a vaso-occlusive crisis? How to calm her down? Are there complications?

A.E.D.nbsp: The vaso-occlusive crisis (VOC) is a complication of the panocytosis characteristic of local obstruction of blood circulation. It is the most common manifestation and the leading cause of hospitalization in adults with half-felicited disease. Therapeutic management of vaso-occlusive crisis in emergencies begins with rapid pain relief. It does not require an administration of opioiuml to the point of entry; the crisis is resolved. There are no complications if follow-up is sure to be successful.
How rural people know if they have this disease? Which part of the Congolese population is most affected by panocytosis?
A.E.D.nbsp.: the testing activity is conducted birth. All new data in the Congo should be screened for better follow-up if there were financial means. Screening can be done late from à people Risk, i.e., partial family members. There is also a need to identify all children who are not. of a couple with the germ of the panocytosis, or late when the person is experiencing symptoms such as the age, pain, disease or disease. From this moment on, it is possible to identify the panocytosis. But the best thing to do is to screen for the effects of certain forms of this disease. The test does not have any fault. If there are financial means, the awareness team can be trained. When people are sensitized, testing can be done in all villages, women in rural areas and children in rural areas. 15 years. It is also possible to track all cases of the past. Serious forms must be taken care of. The screening must be accompanied; a regular management. In Congo, we had à take 300 samples of all new products and are reported. 2.8 of the population, i.e. 3 out of 100, are born with the total form of disease and 1 out of 4 are born with the partial form of disease. The most important cases are identified. Brazzaville, in the departments of Bouenza, Kouilou, Niari and Cuvette-Ouest. In these departments, 3 in 100 children are born with the total form of illness. The other departments are also affected, but there is a frequency between 1 and 1.5. Based on the studies that were conducted 2 in 100 children are born with the total form of the disease. This difference is not so important because panocytosis is found throughout the country. However, with respect to Aboriginal populations (Pygmology), the experience has been demonstrated; they do not wear panocytosis. It is the Bantu populations who carry this disease for genetic reasons. But with mixed marriages, later or later, the pygmies will give birth to the pygmies. children carrying the tares of this disease, and still later, the total forms. Panocytosis is found in all categories of the population. How do you look at panocytosis? Why is it considered to be a pathology of shame or oblivion? A.E.D.nbsp: In the Congo, panocytosis changed facets. In previous years, penalty 1 & agrave; Two out of five Congolese children with total panocytosis passed the age of five. Girls in the family died from complications of illness during pregnancy. But today, thanks to CNRDR and Agrav; the support of Ms. Antoinette Sassou N'Guesso, we recorded; à In the case of children, the penalty is 14 years of age. Girls also give birth in good conditions. My highest total physician patient à 83 years. Another à 81 years of age with total family members. So today, the face of panocytosis has completely changed. However, there is a work to raise awareness of screening. do upstream and real online advice to reduce the significant burden of disease; and, most importantly, death; attributable à this disease. Efforts have been made. technology and treatment of patients with new treatments. When the patient is disabled, he or she will live like any human person, even if there are important precautions. take from à a normal subject. With the care of panocytosis in children and adolescents, can we say that today the prevalence of panocytosis tends to increase? Getting started? A.E.D : Support is a matter of organization. We have written standards that have trained nurses and doctors in all health facilities in the Congo. In principle, every child who is a health care provider must be cared for in a health centre. in a general hospital or hospital. Care is a serious problem of motivation and commitment of health personnel; for people who are ill because not everyone can make the move to CNRD for care. At the CNRDR, the management is carried out by the 5,000 Fcfa both à Three months. For those in need, the Centre pays their place. The donations we receive serve à help children with health problems because follow-up is very important, and life. Panocytosis is a genetic mutation, i.e., a genetic change. It will never disappear, and the S is perpetual. It is simply necessary to make the effort to ensure that the disease remains partial and eliminates the total form of the disease. In addition, stem cell transplantation is used to treat panocytosis. It is done in France, Italy, Nigeria, Tanzania, Morocco, etc. In Congo, the project is on the government table. We have a proposal. the project because we are able to make transplants here in Congo. The project is supported by the Congo Assistance Foundation. If Ms. Antoinette Sassou N'Guesso, President of the Congo Assistance Foundation, with her kindergarten aura and the 39; This will save many lives. This disease is not very severe or very serious in the world, does it not complicate the work of the researchers or doctors you have? What are the current treatments for this disease? Is research progressing to treat her?
A.E.D.nbsp: A lot of research has been done. This is because the mechanisms for understanding this disease have been successful. In addition, it is currently possible to develop panocytosis. It is possible to identify and 28 weeks of pregnancy and birth. The research is progressing and there have been some medicines that can make the life of a patient healthy, without complication. In the Congo, the CNRDr is a dowry; Since 2023, from a transfusion exchange automaton. In Central Africa, it is the first country in Africa. have this device which co-occurs excessively expensive. The practicality of the transfusion exchange is done, for the first time, on a young woman with a 7-month pregnancy. It consists of à replace a given volume; red blood cells sick of the patient with healthy red blood cells. The exchange will allow him to reach the point of 39; 37 weeks of uncomplicated pregnancy and can give birth normally. It must be continuously followed.
We have done much in the fight against panocytosis. His efforts have produced this centre that is perfecting. However, continue à organize information sessions for the benefit of physicians, equip hospitals for screening, treatment of patients, and build a transplant centre. Prevention, pre-screening, awareness-raising must be done upstream, as well as real genetic advice must be provided to reduce the significant burden of disease; and, most importantly, death

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