Living with Sickle Cell Disease

Sickle Cell Disease

People with sickle cell disease can live a normal life and achieve their dreams and aspirations if the condition is well managed. Martins Ifijeh writes

It took Yemi and her husband almost a year before they discovered that their child Banwo had sickle cell disease. Though they knew all along that he was different from their previous two children, because his hands and feet were always warmer than usual, his feet unusually swollen, lack of sleep and appetite, and most significant was his constant cry. They didn’t realise the sickle cell pain was the reason for the cry and other unusual features until they took him to their doctor.

With the doctor’s check, Yemi and her husband realised that their son had sickle cell disease, a condition that affects about 150,000 newborns in Nigeria yearly. They knew it wasn’t a little illness they could treat before leaving the hospital that day. Banwo will live with the disease the rest of his life, except the family decides to do a stem cell or bone marrow transplant, which is a tall order because of the financial implication and significant risk involved.

Yemi and her husband both have the sickle cell trait, AS, but their first two children were lucky not to inherit the SS genotype from them even though they still have the traits in them, as they are both AS. But Banwo wasn’t so lucky at it. The chances of parents with the sickle cell traits to give birth to an SS is 25 per cent.

“Despite the fact that we left the hospital depressed that day, I and my husband resolved to give Banwo all the care and support he needs to be able to live a normal life like his brother and sister. We made sure he takes his drugs as at when due, make sure he doesn’t run around the house excessively and made sure he is kept in check even at school,” she said.

Yemi, whose husband is a senior lecturer at the University of Lagos, said since eight years ago that Banwo was born, she has devoted her time to learning new and better ways to manage his health issue, which she said was paying off.

“While growing up in Abeokuta, we often have the belief that a child with sickle cell disease doesn’t last more than few years,” adding that it was often believed that their lifespan was very short because of the resultant crisis due to the disease. “Now I know an SS can live up to a 100 years if properly managed. My son is eight years old now and he is doing fine. People need to understand it is not a death sentence,” she said.

She, however lamented that even with the high prevalence rate of the disease in the country, there was still no dedicated sickle cell centre in any of the 36 states of the federation, including the Federal Capital Territory, as she has made inquiries on it.

She called on the government and stakeholders to intensify awareness on the disease, as well as its management, so that there will not be needless deaths of children in the country due to the scourge.

Yemi’s family is not the only one going through the management of sickle cell disease. Millions of families across the country are grappling with the scourge, with majority knowing just little aboutthe disease.

According to the World Health Organisation (WHO), 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 the country, while 40 million Nigerians are carriers and an estimated one million persons living with sickle cell disorder, making it a prevalence rate that has resulted in Nigeria commonly referred to as the country with the highest level of sickle cell disorder in the world.

Despite the high incidence of the disease little is still known by Nigerians, especially those in rural and sub-urban areas, on the cause, management, prevention and a possible stem cell and bone marrow treatment.

Experts are of the opinion that the high death rate of the disease was highly preventable if there were proper management culture. They specifically advocate for increased awareness by stakeholders, Ministry of Health, health institutions, non governmental organisations and public spirited individuals.

Speaking with THISDAY, the Research Director, Fair Needs Africa, Lagos, Dr. Reuben Ogala said there exist proper management plans, such that persons living with it can lead normal lives and grow into old age. “Gone are the days when sickle cell disease is a death sentence. A sickle cell person should work closely with his or her doctor, eat good food and reduce unnecessary stress that could predispose them to crisis.

“In the United States, the life expectancy rate of a sickle cell person is 63 years, whereas the life expectancy of a person even without the disease in Nigeria is less about 50 years. This, therefore tells you that with proper management of the disease, a sufferer can live even longer and healthier than someone without the disease,” he added.

He also explained that the scourge can be prevented through genetic counselling, adding that when intending couples are educated on the disease, genotypes, risks, among others, it will help them in making informed decisions on whether or not to marry someone with sickle cell disease, the trait or someone with AA genotype.

Ogala, said that when both parents have the AS genotype, there was possibility of one in every four of their children having the SS genotype which is the sickle cell disorder; a diseases such a person will suffer throughout his or her entire life. He explained that a child born of the AS genotype has the sickle cell trait and can as well give birth to someone with the trait or someone who has the sickle cell disorder. He believed that someone with AS genotype poses no threat until he or she mates with someone who also has the AS genotype or worse still, someone with SS genotype.

According to him, there are other situations where the sickle cell trait is combined with other hemoglobinopathies; abnormalities of the hemoglobin in the blood, which in itself poses a threat. He said that the major issues emanating from sickle cell disorder affects children more, as their tolerance level was low compared to that of an adult, making them more at risk of the disease.

According to experts, sickle cell anemia is a disorder of the blood that causes the red blood cells to blow into a sickle shape. These cells however do not carry oxygen efficiently to areas of the body where it is needed.

Normal red blood cells have a 120-day life span, but people born with sickle cell disease have sickle-shaped blood cells that usually live not more than 20 days. These sickle cells can get stuck in blood vessels, blocking blood flow and less blood flow can damage the body’s organs, muscles, and bones, sometimes leading to life-threatening conditions.

Also lending her voice, the National Director of the Sickle Cell Foundation of Nigeria, Dr. Annette Akinsetein, in an interview with THISDAY noted that in Nigeria, not many children with the disease grow into adulthood because of low awareness and management culture in the country

She said gone were the days when SCD was a death sentence. “With proper information, the disease can be managed successfully, while the victim lead a normal life.”

She said contrary to beliefs in some areas that the disease was caused by witchcraft or ogbanje, that it was of genetic origin, which through counselling may be prevented, as the genotype of both parents play the role on whether their child will have the disease or not.

She called on the government to come up with newborn screening policy where SCD can be detected early in children. “This is done in the United Kingdom and the United States, and it has greatly helped in reducing number of crisis or deaths arising from it.

Shedding more light on how to tackle the scourge, the former permanent secretary, Ministry of Health, Lagos State, and pioneer Chief Medical Director, Lagos State University Teaching Hospital (LASUTH), Dr. Olufemi Olugbile, explained that due to the medical care available now, people hardly die young as it used to be because majority are living into full adults and even growing old.

According to him, people should be aware of their own genotype and that of their prospective partners. “Knowing the risks involved hopefully reduces the prevalence of it. Inasmuch as counseling has a major role to play, one cannot make a law that, if the partners’ genotype is not compatible, then they cannot get married. It should only be advisory. People should either take it or reject it. But generally, awareness is the key to reducing the disease,” he added.