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Sickle cell disease, also known as sickle cell disease, is a disease that affects red blood cells. Red blood cells are the part of the blood that carries oxygen to the body's organs. Normal red blood cells have a round shape and therefore move easily into the blood vessels.
When people suffer from sickle cell anemia, their red blood cells have an abnormal shape. These abnormal red blood cells get stuck in the blood vessels and do not provide enough oxygen to the body's organs.
Sickle cell disease is a life-long illness that people are born with. It is caused by an abnormal gene. To contract the disease, people have to get the abnormal gene from their father and mother. If the abnormal gene comes from a single parent, it is called "sickle cell trait". People with sickle cell trait usually have no symptoms of sickle cell anemia.
According to the World Health Organization (WHO), it is estimated that more than 300,000 babies with severe forms of these diseases are born each year in the world, the majority in low- and middle-income countries.
About 5% of the world's population are healthy carriers of a gene for sickle cell disease or thalbademia. Sickle cell disease is also predominant in the African region.
In the Africa region, the majority of children with the most severe form of the disease die before the age of five, usually as a result of an infection or a significant blood loss .
In countries such as Cameroon, the Republic of Congo, Gabon, Ghana and Nigeria, the prevalence varies between 20 and 30%, while in some parts of Uganda it reaches 45%.
The symptoms usually begin after a baby of a few months. Most often, people with sickle cell disease have episodes of pain (also called "sickle cell crisis"). The pain usually occurs in the bones of the back, chest, stomach, arms or legs. It can be mild or severe, and last from a few hours to several days.
People may have other symptoms related to their pain, including fever, swelling (eg, hands or feet), nausea or vomiting.
Some situations easily trigger these painful seizures, and usually include infections, dehydration (when the body loses too much water due to diarrheal diseases, vomiting, less fluid consumption), extremely cold temperatures, stress or high altitudes. where there is less oxygen in the air, like mountains).
Children with sickle cell disease are also at risk of developing certain conditions, such as anemia (a condition characterized by the reduced number of red blood cells, which always causes a feeling of tiredness and weakness, recurrent infections, strokes (obstruction of blood vessels in the brain) and painful prolonged erections that may sometimes require urgent surgery.
There are different ways to look for sickle cell anemia in these children by performing different blood tests. In some centers, newborns are routinely tested for this disease. Some parents may also choose to test the fetus in their unborn baby.
Once sickle cell anemia is diagnosed in children, there are many treatment options, most of which help prevent and prevent these episodes – some episodes can be really painful and fatal if they are not properly cared for. .
This usually involves routine antibiotics to prevent serious infections, pain medications for seizures, folic acid tablets and blood transfusions for anemia, as well as avoiding triggers. .
A procedure called "bone marrow transplant" can cure sickle cell disease. This procedure replaces the cells of the bone marrow (the inner part of the bones responsible for the production of red blood cells) with healthy cells, which can be done for some people with sickle cell disease who meet the criteria of the procedure.
Prevention of this condition involves genetic testing for couples prior to marriage or pregnancy planning. Fortunately, as these genetic testing services and counseling services are more available, couples, especially those with a family history of sickle cell disease, are strongly encouraged to get tested before planning their pregnancy.
Dr. Ian Shyaka, Resident in Plastic Surgery,Rwandan Military Hospital,
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