Fight against sickle cell disease: Interview Docteur Hèzouwè Magnang, Director of CNRSD

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Extract from the article: The whole world is celebrating World Sickle Cell Day this June 19, under the theme “Fighting sickle cell disease: training, an essential link”. Sickle cell anemia is a serious hereditary disease. Sickle cell disease is a public health problem in...

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“Anyone wishing to have children without sickle cell disease must choose to know their hemoglobin before marriage”.

The whole world is celebrating World Sickle Cell Day this June 19, under the theme “Fighting sickle cell disease: training, an essential link”. Sickle cell anemia is a serious hereditary disease. Sickle cell disease is a public health problem in Togo. Sufferers have to cope with multiple consequences: anemia, painful attacks that can affect various organs, and reduced resistance to certain infections.  However, there are simple measures and precautions that can be taken to live better with the disease. Doctor Hèzouwè Magnang, Director of the Centre National de Recherche et de Soins aux Drépanocytaires (CNRSD) (National Center for Research and Care of Sickle Cell Disease) discusses the mechanism of the disease and how to manage it.

Santé-Education: What is sickle cell disease?

Dr Hèzouwè Magnang: Sickle cell anemia is a hereditary genetic disease that affects hemoglobin, resulting in the appearance of hemoglobin S in place of the normal hemoglobin, hemoglobin A. Hemoglobin is a protein in the body that is responsible for the development of the disease. Hemoglobin is a protein that is the main component of the red blood cell, transporting oxygen in the blood and distributing it to all organs. Sickle cell disease results in a deformation of the red blood cells. In sickle cell disease, the red blood cell is deformed, looking like a sickle or crescent moon, and can no longer circulate properly in the blood vessels. This shape blocks the flow of blood and oxygen to the limbs and organs, creating the vaso-occlusion phenomenon that causes pain and anemia. It's a genetic and hereditary disease. Explain these terms.

It's a hereditary disease because it's transmitted from both parents to their children. When both parents carry the disease gene, there is a possibility that they will pass the disease on to their children, hence the genetic nature of sickle cell disease. A person can be a healthy carrier of the sickle-cell gene if the genetic anomaly in question is present in only one copy, i.e. inherited from only one of the two parents.

What is the prevalence in Togo?

In Togo, we don't have data from recent studies. What we do have is data from hospital studies which indicate that 4% of the Togolese population are carriers of symptomatic forms of sickle cell disease, and around 16% of the population are carriers of the gene responsible for the disease. The various complications associated with sickle cell disease are often due to a lack of medical follow-up.

What are the symptoms of the disease?

There are many clinical manifestations of sickle cell disease. The main sign the patient develops is bone and joint pain. Bone pain is much more common in adult sickle cell disease. But in infants and small children, abdominal pain is more common. Apart from bone pain, anemia is a permanent feature in some forms of the disease. This anemia may worsen at some point when the patient is experiencing a complication of the disease. Other signs include yellowing of the eyes, known as jaundice, stunted growth and increased susceptibility to bacterial infections.

How does pain occur in sickle cell disease?

Very often, there is a factor or circumstance that favors the onset of bone or abdominal pain in sickle cell patients. These include exposure to extreme temperatures, especially cold, intense and continuous physical effort, living in an oxygen-poor environment, dehydration, stress and hyperthermia. That's why we advise sickle cell sufferers to avoid these factors.

What are the complications of this disease?

The course of the disease varies greatly from one individual to another, with complications sometimes affecting every organ in the body. Chronic complications include osteoarticular, cardiac, pulmonary, renal, ocular and neurological damage, to name but a few. Diagnosis is often delayed, and the many complications of sickle cell disease justify the importance of screening at birth and early medical follow-up.

Is there an age at which the first symptoms appear?

The age of onset varies according to the type of sickle cell disease. In SS subjects, signs begin around 3 to 6 months. In SC forms, signs can begin so late. Generally, it starts around 3 years of age. But there are SC subjects who didn't start showing signs until after they were 15. Some start after the age of 20. It all depends on the type of organ damage, which may require specialized or multidisciplinary medical treatment.

Why is an electrophoresis test important?

Hemoglobin testing, or electrophoresis, is very important because it's one of the ways of knowing your hemoglobin phenotype, i.e. the type of hemoglobin you carry. From there, you can choose your spouse or partner with the aim of avoiding sickle-cell children. Any young person wishing to have healthy children, i.e. without sickle cell disease, should choose to know his or her hemoglobin before marriage, and seek genetic counseling if necessary, especially if you have an abnormal hemoglobin, such as hemoglobin S or C, for example. The doctor informs carriers of abnormal hemoglobins of the risks for their children, the treatment they will need if they have sickle cell disease, and the solutions available to the couple in gestation.

Where can I go for a hemoglobin electrophoresis test?

Electrophoresis tests can be carried out in all our country's intermediate and central hospitals, i.e. university hospitals (CHU), regional hospitals (CHR), prefectural hospitals (CHP) or district hospitals, and the National Institute of Hygiene (INH). There are also private clinics that perform this test.

How to live with the disease?

When you have sickle cell anemia, the main advice I would give is to opt for follow-up care. This follow-up is not necessarily provided by the Centre National de Recherche et de Soins aux Drépanocytaires (CNRSD), which currently only exists in Lomé. It's true that this is the ideal place for follow-up, but those who are far from Lomé can also be followed up by a general practitioner or specialist in their locality. As part of their basic training, GPs are trained to monitor sickle-cell patients. What's more, in the context of the Covid-19 pandemic, sickle-cell sufferers need to be even more vigilant, scrupulously observing barrier gestures to avoid contracting this infection, which becomes particularly dangerous in people already carrying another disease.

What is the right diet for sickle-cell sufferers?

When it comes to diet, sickle cell sufferers need to eat a balanced diet and take care with their meals. Sickle cell sufferers have a weak and fragile defense system. To this end, they need to eat a balanced diet to provide their body with nutrients that will boost its ability to defend itself against pathogens.

In addition to a balanced, well-cared-for diet, sickle-cell sufferers need to be vaccinated. This is because vaccination is the only way to increase a person's ability to defend themselves against microbes. Vaccines are an excellent way of preventing many illnesses. Children and adults with sickle cell disease must receive all recommended vaccinations.

"Stop the birth of sickle-cell children! How can we do this? By taking an electrophoresis test before choosing your partner".

Interview by Raymond DZAKPATA.