Sickle cell is the leading genetic disease in the world. Every year, 50 to 70 per cent of children born die before they are 5 while those with it suffer from social exclusion
By Boris Esono Nwenfor
BUEA, July 16, 2023 – The World Health Organization estimates that two (2) million people currently live with sickle cell anemia in Cameroon. The Ministry of Public Health estimates that 6000 children are born yearly with sickle cell anemia.
According to health experts, the disease is inheritable, with deformity at the level of the genotype. They recommend blood tests and genetic counselling before marriage as a means to fight against sickle cell anemia.
Dr Owono Gilbert, General Practitioner at the Nkoabang Baptist Health Centre in Yaounde said: “The management of the disease starts with education. The main thing about this condition is to diagnose it early and prevent complications. When we receive an intending couple, we have systematic tests that we perform to make sure they are biologically compatible. One of the most important tests is the Hemoglobin Electrophoresis which tells us the genotype.”
“From the genotype, we will know if the couple is compatible or has a high or low risk of giving birth to a person with the disease. The results of the blood tests will help us know how to advise this couple, and either allow them to marry or we discourage the marriage.”
This year, world sickle cell anemia day was observed on June 19 under the theme: Building and Strengthening Global Sickle Cell Communities, Formalizing Newborn Screening and Knowing your Sickle Cell Disease Status.”
Despite the high figures for prevalence, sickle cell disease remains a misunderstood malady and is surrounded by many misconceptions. These can lead to the stigmatization of those with major sickle cell syndrome and their relatives, which poses an obstacle to proper patient treatment.
Thus, integrating sickle cell disease into primary healthcare and more generally, developing strategies to combat the disease on a national scale is imperative in treating it. Sickle cell is the leading genetic disease in the world. Every year, 50 to 70 per cent of children born die before they are 5, while those with it suffer from social exclusion.
Sickle cell disease or sickle cell anemia affects most countries in the African region. In sickle cell disease, the normal round shape of red blood cells becomes like crescent moons. Round red blood cells can easily move through the blood vessels but sickle-shaped cells interconnect and can result in blood clots. Environmental factors often play a role in the occurrence of painful attacks, according to the World Health Organization. Common triggers include cold temperatures, dehydration, excessive amount of exercise and tobacco and more.
To Dr Owono Gilbert, genotype awareness is crucial in choosing a partner. He said AA-AA has a zero probability of transmission. AS-AS have a 50 per cent chance of having a baby with sickle; 25 per cent of having a carrier and a 25 per cent chance of having a normal child. If any of the partners is already SS, Dr Owono recommends cancellation of the marriage as there is a 100 per cent chance of having children with sickle cell.
“AA is a normal person with no risk of transmitting the disease to the next generation. AS are carriers. Most of them don’t develop the sickness, but they can transmit the disease if they meet another carrier like them. SS are the ones who are sick and will transmit the disease,” said Dr Owono Gilbert.
“The underlying problem is poverty. Patients accept what we tell them about the importance of blood tests but when they step out, the reality comes back to them. It’s not just the disease, but if we can improve people’s lifestyles, they will be ready to welcome the knowledge.”
