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What You Need to Know About Sickle Cell Disease

According to the World Health Organization (WHO), over 500,000 babies are born with sickle cell disorders worldwide, making it the most common inherited disorder in the world, with majority of the cases occurring in underdeveloped and developing countries, especially in African countries like Nigeria.

A recent report by the Centers of Disease Control and Prevention stated that Nigeria accounts for about 75 per cent of infant sickle cell cases in the continent with over 100,000 children dying from the scourge every year in Nigeria while 40 million Nigerians are carriers and an estimated one million persons are living with sickle cell disorder. This prevalence rate has resulted in Nigeria commonly referred to as the country with the highest level of sickle cell disorder in Africa.

Despite this high prevalence rate and the innumerable suffering and hardship this disease has caused families who have children or siblings living with Sickle Cell Disease, many people do not know their genotype and do not see the need to.

 

WHAT IS SICKLE CELL DISEASE?

Sickle-Cell Disease (SCD) is a hereditary blood disorder, characterized by an abnormality in the oxygen-carrying haemoglobin molecule in red blood cells leading to a tendency for the cells to assume an abnormal, sickle-like shape under certain circumstances. SCD is associated with a number of acute and chronic health problems, such as severe infections, attacks of severe pain which are referred to as ‘sickle-cell crises’ and stroke.

HOW COME?

Sickle-cell disease occurs when a person inherits two abnormal copies of the haemoglobin gene, one from each parent. A person with a single abnormal copy does not experience symptoms and is said to have sickle-cell trait.

 

MANAGEMENT

1. Regular Checkups

Children born with Sickle-Cell Disease should be closely monitored by a Paediatrician and a Haematologist to ensure they remain as healthy as possible.

a. Babies from birth to 1 year of age should see a doctor every 2 to 3 months.

b. Children from 1 to 2 years of age should see a doctor at least every 3 months.

c. Adults and children from 3 years of age or older should see a doctor at least once every year.

 

2. Daily intake of Folic Acid

Patients born with Sickle Cell Disease will be required to take a 1 mg dose of Folic Acid daily for life.

 

3. Analgesics

Most people with Sickle-Cell Disease have intensely painful episodes called vaso-occlusive crises. Painful crises can be treated symptomatically with analgesics.

 

HOW CAN WE PREVENT THIS?

People who are known carriers of the disease should undergo genetic counselling before marriage and before they have a child.  There should be increased awareness to ensure more people are aware of the importance of knowing their genotype and requiring their partners to go for testing as well.

 

WAY FORWARD?

In 2001, it was reported that Sickle-Cell Disease had been successfully treated in mice using Gene Therapy. The mice – which had the same defect that causes sickle cell disease in humans – through the use of a viral vector, were made to express the production of foetal haemoglobin (HbF), which normally ceases to be produced by an individual shortly after birth. This could be the way forward because studies have shown that in humans, the use of hydroxyurea to stimulate the production of HbF temporarily alleviates the symptoms of sickle cell disease.

Phase 1 clinical trials of Gene Therapy for Sickle Cell Disease in humans were started in 2014. Medical experts at University of Benin, Nigeria, successfully drained infected red blood cells from a 27-year-old patient and replaced them with uninfected ones in 2014. However, both methods are capital intensive and require highly skilled medical practitioners to execute successfully.

Extensive research is still ongoing for the cure of Sickle Cell Disease but till a permanent and affordable cure is found for this debilitating disease, all we can do is educate as many people as possible to go for genetic counselling and care for friends and family members suffering from Sickle Cell Disease.

 

For more information on Sickle Cell Disease please visit: http://www.cdc.gov/ncbddd/sicklecell/index.html

 

 

About the Writer: Ejura Salihu is a freelance Writer and Copyeditor. She tweets @Icyquin_msc

 

 

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Ejura Salihu is an Anatomist, Writer and Editor. She is currently the Editor-in-chief for Connect Nigeria. You can contact her via email: ejurasalihu24@gmail.com or follow her on twitter @icyquin_msc

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