‘Nigeria has largest cases of Sickle Cell Disease in the world’

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Nigeria has the largest population of people with Sickle Cell Disease (SCD) in the world, with over 150,000 babies born with the serious condition every year, researches have shown.
Mr Lanre Alege, a Pharmacist with the University of Ilorin Teaching Hospital, said this in a lecture delivered during June edition of the keep fit exercise for staff of the hospital at the Kwara stadium on Saturday.

He said it was estimated that only five per cent of the children with Sickle Cell Disease live past the age of 10 in Nigeria, compared to over 96 per cent surviving into adulthood in the United Kingdom and the United States.

Alege, who is the Coordinator of the programme, also disclosed that the recurrent pain and complications of the disease can interfere with many aspects of patients life, including education, employment and psychological development.

He said that Sickle Cell Disease is a genetic disorder due to the presence of an abnormal form of haemoglobin in the red blood cells, called haemoglobin S (Hb S) instead of haemoglobin A (Hb A).

According to him, the red blood cells (Erythrocytes) may contain normal haemoglobin AA only, a mixture of A and S (Hb AS) or S only.

“Persons with AS haemoglobin have sickle cell traits, and are referred to as sickle cell carriers, while those with haemoglobin SS only (Hb SS) suffers from sickle cell disease.

“Those with AA are normal without sickle cell traits.

“Haemoglobin in the red blood cell is responsible for the transportation of oxygen in the body,” he said.

Alege said that the topic, ‘Sickle Cell Disease’ was carefully chosen because June 19 of every year is celebrated as world Sickle Cell Day, and Nigeria joined the rest of the world to celebrate it on Friday.

According to him, there is presently no cure for sickle cell disease, but the cost effective treatment exists for the pain and other aspects of the disease.

He listed the most important components of the treatment to include early interventions with analgesics, antibiotics, rest, good nutrition, folic acid supplementation and high fluid intake.

“At times, invasive procedures such as blood transfusion and surgery may be needed. Sickle Cell Disease is completely preventable,” he added.

Among the recommendations, he gave, for the prevention are that carriers should not marry AS or SS, education about inheritable nature of the disease.

He stressed the need for public education to determine genotype, so that incompatible people do not fall into love let alone marry each other.

Alege added that Churches and Mosques should refuse to join such people in marriage.
Meanwhile, medical experts have urged the Federal Government to support SCD patients.
Dr Femi Adebayo, Medical Consultant, Federal Medical Centre (FMC), Lokoja, Kogi State, who spoke on the sidelines of this year’s “World Sickle Cell Day 2015 (SCD).”
reiterated the urgent need for the government to give proper and adequate support to those who have the disease to enable them live longer.

The medial practitioner also urged the patients to ensure that they maintain regular check-up in the hospital.

He advised parents to go for early medical test for their children if they see any sign, to commence early treatment.

He said people wrongly believe that SCD patient does not live above 21 years, adding that they could live as long as 50 and up to 70 years, if properly managed from the early age.

The expert defined the disease as an inherited blood disorder that affects red blood cells.

According to him, people with the disease have abnormal haemoglobin, called haemoglobin ‘S’ or sickle haemoglobin in their red blood cells.

“Haemoglobin is a protein in red blood cells that carries oxygen throughout the body.

“SCD is inherited in the sense that the disease is passed by genes from parents to their children, and not contagious like cold or other infections.

“People who have SCD inherit two abnormal haemoglobin ‘S’ genes, one from each parent.

“When someone has two haemoglobin “S’’ genes (Haemoglobin SS), the disease is called Sickle Cell Anaemia (SCA), which is the most common and severe type of SCD,” he explained.

He said further that the SCD is most common in West and Central Africa.

Adebayo said that Sickle Cell Disease Association of America (SCDAA), estimated that about 80,000 to 100,000 people have sickle cell disease and three million people have sickle cell trait in U.S.
He mentioned different kinds of SCD, which include: Haemoglobin ( SS, SC, SD, and SE ), Thalassemia S0, Thalassemia S+ and among others.

He added that SCD could be caused by abnormal sickle-shaped red cells inform of rod, which were not flexible and could stick to vessels’ walls to hinder the flow of blood.

The Consultant said most children with SCD are pain free between painful crises, but adolescents and adults might suffer with chronic persistent pains.

He said that over a lifetime, SCD could damage patient’s brain, heart, liver, lungs, kidneys, spleen, skin, bones, joints or eyes.

“Normal red blood cells live for about 90 to 120 days, but sickle cells only last for 10 to 20 days.

“The body always makes new red blood cells to replace the old and dead cells.

“However, in SCD, they might have trouble in keeping up with how fast the cells are destroyed, and the number of red blood cells will always lower than normal,” Adebayo said.

He said the severity of SCD varied widely from one person to another, adding that the life expectancy of patients in developed countries with the disease would be about 40 to 60 years.

“At present, Hematopoietic Stem Cell Transplantation (HSCT) is the only cure for SCD.

“Unfortunately, most people with SCD are either too old for a transplant or don’t have a relative who have good enough genetic match as donor.

“A well match donor is needed to have the best and a successful transplant, he said.

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