World Sickle Cell Day: Know the facts
In Nigeria, over 150,000 children are born annually with symptomatic sickle cell anaemia, 1-in-4 people are healthy carriers of the disease.
Sickle Cell Disease (SCD) is an inherited disease in which the red blood cells in your body are shaped in sickles with jagged edges as opposed to smooth ovals or discs.
People with SCD have red blood cells that contain mostly haemoglobin S, an abnormal type of haemoglobin. The most common form of SCD is sickle cell anaemia.
Red blood cells carry oxygen throughout the body. Now if you suffer from sickle cell, your red blood cells are deformed and weak. So not only do they deliver less oxygen to the body’s tissues, but they also can get stuck and break off into little pieces in your vessels.
Symptoms begin to emanate around four to six months. This may result in a number of health problems with the baby such as attacks of pain (otherwise known as “sickle-cell crisis”), anaemia, swelling in the hands and feet, bacterial infections, and stroke. It can also cause lung issues, bone deterioration, asthma, hypertension, gallstones, stroke and even death. In general, symptoms can be mild for some and severe for others.
As with any disease, myths and misperceptions are inevitable and these often make it more difficult for those affected to obtain the care and support they need.
Patients Usually Die In Childhood
In the past, babies that were born with SCD had very little chances of making it to their teenage years, talk more of adulthood, but this doesn’t hold water anymore as medical advancements have brought blood therapies to the fore, which has helped to increase life expectancy of sufferers by decades. In fact, Alhaja Laguda, a sickle cell disease survivor will be 93 by November 1st, 2018.
Sickle cell disorders affect patient’s intelligence
Myth! Children with sickle cell disorders can always attend school and participate fully in normal activities. Only that the class teachers should know of the diagnosis and understand the limitations sickle can impose on a child.
Sickle cell disease is infectious
Myth! Sickle Cell disease is a genetically inherited condition (passed down from parent to child). For a child to inherit SCD, both parents must carry the sickle cell trait (or have the disease), and each must pass the sickle cell gene to the child.
This was true in the past but not anymore SCD patients can go for a transplant. According to a report in 2012, around 25 people woman in Chicago, have received a blood marrow transplant in hopes of being cured of SCD. And so far around 85 percent of those people have been successful in ridding themselves of the disease.
Sickle Cell and Genetics / Prevention
To have sickle cell, both of your parents must have the gene. If they do, with each pregnancy there is a 25 percent chance of having a baby with SCD. There is also a 25 percent chance of their child not having sickle cell and not being a carrier. But there is a 50 percent chance of having a baby who is a carrier with sickle cell trait but doesn’t have SCD.
If you carry the sickle cell trait, seeing a genetic counsellor before trying to conceive can help you understand your risk of having a child with sickle cell anaemia. He or she can also explain possible treatments, preventive measures and reproductive options.
How Do You Treat It?
As earlier explained, symptoms can be mild for some people and severe for others, so also will the treatment be prescribed. Treatment can be a combination of antibiotics, pain management, intravenous fluids, blood transfusion and surgery. Droxia — a cancer drug — has been approved to treat sickle cell in adults. It helps reduce severe pain, acute chest syndrome and the need for blood transfusions in adult patients.
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