World Sickle Cell Day: Why couples should consider Genotype tests before marriage

As Nigeria joins the rest of the world on Wednesday to celebrate World Sickle Cell Awareness Day, thousands of the country’s citizens are still under the grasp of the deadly disease.

According to experts, the disease prevails in the country because of the wrong matching of conflicting genotypes in marriages and procreation.

Known for distorting the red blood cells which mostly result in severely painful blood clots, sickle cell anaemia is among the top genetic haemoglobin disorders.

The genetically inherited chronic condition which can also lead to death troubles a little less than five per cent of the global population. But for people whose origins can be traced to Africa, the risk is disproportionately higher.

While over 300,000 babies globally are born with severe sickle cell disease, 75 per cent of that number, 225, 000, are born in Sub-Saharan Africa and Nigeria carries 66 per cent of the burden in the region, the American Centre for Disease Control and Prevention (CDC) states.

This means that in Nigeria alone, nearly 150,000 children are born annually with sickle-cell anaemia. The country bears 50 per cent of the global burden.

According to a report from the World Health Organisation (WHO), 24 per cent of the Nigerian population are carriers of the mutant gene and the prevalence of sickle-cell anemia is about 20 per 1,000 births.

Wrong matching

Experts have linked the prevalence of the deadly disease to wrong matching of couples with conflicting genotypes, especially in rural areas.

They described as critical, the need for couples to check their genotypes before marriage.

People with sickle cell disease mostly have it because they inherited two sickle cell genes, one from each parent.

For instance, if a man with AS genotype marries an AS woman, they are bound to beget at least one SS (Sickle Cell) child.

To check this, a bill seeking to make haemoglobin-genotye testing compulsory for intending couples in Nigeria was passed by the National Assembly in 2017, according to Punch Newspaper. It is however yet to be assented to. There is no such report yet.

The bill is aimed at reducing the chances of marriage between people with the wrong match of genotypes.

“The major cause (of sickle cell disease menace) is marriage or procreation of the same genotype”, said a sickle Cell survivor, Shirley Hills, in an interview with Leadership Newspaper.

According to the report, Mrs Hills said a way to free the nation from the clutches of the deadly disease is by assenting to the genotype test bill and other relevant bills to accommodate people living with such conditions.

“What people can do to avoid sickle cell is to check their genotypes because it is a genetic blood disease from two people that bear the same traits of AS, that is the only way it can be prevented.”

Experts also say the disease remains endemic in Nigeria because of lack of proper funding and government policies.

World Sickle Cell Day

Since 2008, World Sickle Cell Awareness Day has been held annually, in order to help increase public knowledge and raise awareness of Sickle Cell Disease (SCD) and the struggles sufferers and their families go through.

The date June 19 was chosen to commemorate the day on which a resolution was officially adopted by the General Assembly of the United Nations, recognising SCD as a public health concern.

At least 100,000 babies die from the disorder in Nigeria every year, according to 2014 statistics by the WHO, making it the number one sickle-cell endemic country in Africa.

Hope?

Hope beckoned for sickle cell patients in Nigeria following an agreement to start commercial production of a home grown drug for the disease.

PREMIUM TIMES reported last June that the National Institute for Pharmaceutical Research and Development (NIPRD) signed the agreement with a Nigerian pharmaceutical company, May and Baker, to produce the drug called Niprisan.

The agreement is coming after the Federal Executive Council gave approval to the agency and the pharmaceutical company to scale up production and marketing of the anti-sickle cell formula discovered by NIPRD researchers several years ago.

The move is aimed at reducing the burden of the disease in the country and the continent as a whole.

Quick Facts About Sickle Cell Disease in Nigeria

Nigeria is the country with the highest burden of sickle cell disorder in the world.

Over 150,000 babies are born each year with sickle cell anemia in Nigeria.

Over 40 million Nigerians are carriers of the sickle cell gene.

People with sickle cell usually suffer severe pain.

Children with sickle cell can get stroke – which could be fatal and which could leave them paralysed for life.

People with sickle cell sometimes suffer a breakdown or degeneration of the hip joint/bones and end up unable to walk.

Persons with sickle cell tend to suffer extensive, chronic leg ulcers or sores that cause them much debilitation and social isolation.

Socially, they suffer stigma and discrimination.

Bone Marrow Transplantation (BMT) is a cure for sickle cell, but it is not widely accessible and not everyone with sickle cell is a suitable candidate.