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Enduring ordeals of sickle cell couples

blood cells

SHARON OSAJI writes about experiences of loss and pain that couples, who married as carriers of sickle cell, endure in raising children they know can die at anytime

When Bukola sighted Kunle from across the room at a friend’s birthday party 37 years ago, it was love at first sight.

Kunle’s neat appearance, well-combed afro hair and towering physique made her heart flutter.

Actually, they attended the same secondary school together though there was no attraction as of then. However, it was known that once in a while, Kunle would fall ill but he always got better.

They met a few years after secondary school at their mutual friend’s birthday party; Bukola had turned 25 years by then.

Kunle had also recently come out of a relationship because his girlfriend at the time refused to marry him due to his frequent illness.

His path crossed with Bukola a year afterwards and in no time, they fell in love.

Although Kunle was from a devoted Muslim family, he didn’t mind getting married to Bukola, who was from an Anglican family.

The union was consummated in the church despite Kunle’s family’s opposition to the marriage and refusal to attend the ceremony.

A close family friend, who knew details of the relationship and spoke on condition of anonymity, said the lovebirds could not get enough of each other and everybody around them could testify to it.

“What they shared was special,” he said.

According to him, the couple did not conduct a laboratory test to ascertain their genotypes before the marriage or sought medical advice.

In the first year of their marriage, Bukola conceived and after nine months, she gave birth to a baby boy, Abidemi.

The source stated that as the boy grew, he started having health crises as his parents wondered about the root cause of the problem.

As a result, Bukola got a laboratory screening to ascertain her genotype and discovered she was AS, while her husband was SS.

The couple then agreed to have another child, hoping the baby would be AS.

Bukola conceived again and in the third year of the marriage, she gave birth to a baby girl.

The family friend said Kunle immediately requested a genotype test on the newborn, christened Abisoye.

A blood sample was taken as the couple prayed the result would be as they hoped. Unfortunately, she was SS.

Then both Kunle and Bukola agreed that they would not have children any more and decided to care for the two they had been blessed with.

Kunle, at the time, was said to be working with an oil company in Lagos and ensured that his home was always filled with medication that he and the children took daily.

“The situation drew the family closer to God and Kunle, along with his family, started attending a Pentecostal church. Their prayer was always for God to sustain, heal and take their pain away,” the family friend told Saturday PUNCH.

But when Abisoye was in Senior Secondary School 1, she had a crisis and did not survive it.

The incident threw the family into mourning.

“The incident drew them closer and they poured all their love on Abidemi, who had finished secondary school at the time,” the source added.

However, in 2005, three years after Abisoye died, Kunle himself had a crisis and died.

After being referred from one family member and friend to the other, Saturday PUNCH got the contact of one of Kunle’s former colleagues at his workplace, who agreed to speak on the tragedy on condition of anonymity.

He said, “Kunle was truly a fine man, both in person and in attitude. He was meticulous and religious with his medication and whenever he felt a little bit weak, he knew what to do. He would take time off work and go home to rest.

“If he needed to be on admission, he would know and would go to a hospital. He understood his body and took good care of himself. He personally battled death till the end because he was careful to take all the precautions.”

Kunle died at 51 after 22 years of marriage.

He was also an ordained pastor at a Pentecostal church where he worshipped with his family.

“Everyone knew how much he loved his wife and son. He was very fond of them,” the former colleague added.

“One thing with sickle cell patients is that one way or the other, the disease starts weakening their immunity and at age 51, Kunle could no longer battle. He was the second person to die from sickle cell in his family within the space of three years,” he stated.

After Kunle’s death, his wife, Bukola, had only Abidemi left.

The young lad, after some years of study, graduated from a university, and, according to Kunle’s former colleague, on compassionate grounds, the boy was employed in the oil company because of his father’s good records.

But then, tragedy struck again.

Abidemi, while driving home after a vigil, dropped off fellow worshippers, whom he took in his car because it was raining heavily.

He was driving back home alone when another vehicle rammed into his car.

In the process, Abidemi, who was in his late 20s, started bleeding.

“And you know someone with sickle cell disease shouldn’t be losing blood.

“At that point, his immunity was almost at zero level and after a few days in the hospital, he could not make it. He died. His mother (Bukola) was by his side through it all.

“That was a heavy blow to the mother. Friends and brethren from the church had to rally around her during the period, so she would not be overwhelmed with the thought of taking her life,” the family friend added.

He said Bukola, now 62, later adopted a child to “cope with loneliness.”

When contacted, Bukola declined to comment on the tragedy.

When Saturday PUNCH attempted to persuade her, she snapped, “Speak to a doctor, not me.”

Tricked into marriage

Adebayo, who lost his nephew to sickle cell anaemia, shared his family’s ordeals with Saturday PUNCH.

“The first child of my elder brother, Phillip, was born with sickle cell anaemia. They had four children; he was the first and the only one born with the disease; the others had AA and AS,” he added.

Adebayo said Phillip was cooperative as a child and never refused his medications, but noted that anytime he had a crisis, the whole family was in disarray.

He said the crises sometimes occurred during family gatherings, especially Christmas, adding that whenever they occurred, Phillip would be wriggling in pain as everyone would be in tears.

After Philip went through secondary school where he was in a boarding house, he was admitted to The Polytechnic, Ibadan, where he read surveying.

According to the family member, he went on to pursue his master’s degree in surveying at the University of Ibadan.

Phillip also became a senior lecturer at The Polytechnic, Ibadan, before he died a few days after celebrating his 41 years birthday after a crisis.

But Adebayo said there was more to Philip’s story.

“His parents, having known the grim reality of this sickle cell disease and that their son could die at any time, decided to arrange a wife for him. So, early in secondary school, his parents got him married.

“Some of us did not support it because they were getting somebody who was not enlightened enough to know that the person she was marrying could die anytime. They hid that part from the girl’s family, who were not educated; the girl was just a hairdresser, but she was AA,” he added.

Phillip, however, had three healthy children, with none carrying sickle cell anaemia.

“Although all of Phillip’s three children are graduates now, the wife became a widow at a very young age and that is how she has coped with her life even until now,” Adebayo added.


‘I buried four children’

A widower, Mr Akpan, said he had no knowledge of genotype screening before he married his wife in 1991.

“Honestly, I didn’t even know what genotype was at the time,” he confessed to this reporter, as he tried to laugh at his ignorance.

But there was nothing funny about what Akpan went through in his marriage.

It was until his first two children, a boy and a girl, began to fall sick regularly that he was educated by a nurse at a specialist hospital in Jos on the reality he had entered into.

“After she explained everything about genotype to me, she then advised that I and my wife do a test. When the results were out, we discovered that we were both AS.

“She told us that after our first two children, there was a probability that the third or fourth would be AA or AS,” he added.

  Unfortunately, Akpan’s first child died at five years, while the second died at eight.

“The most horrific experience any parent can ever go through is burying their child,” Akpan said as he fought back tears.

He did not bury just two children, but four.

“For my first child, I donated blood four times for him, each time was one pint of blood, but we still lost him,” he added.

Akpan, however, was lucky as his third child, Precious, a girl, came out as AA.

And soon afterwards, his wife got pregnant again and this time gave birth to twins, a boy and a girl.

He said they were elated and thought that God had answered their prayers by replacing the lost children.

However, shortly after their birth, Akpan discovered one of them was SS and the other AS.

“We entered another round of prayers because that was the only thing we could do. It was devastating, heartbreaking and tiring,” Akpan said, recalling the painful memory.

After a few months, the twins died.

“I still don’t understand why the AS child died; she died two months after her brother, who was SS,” he added.

For the sixth time, Akpan’s wife became pregnant and this time had another daughter, Glory, who was AS.

In their 16 years of marriage, Akpan and his wife buried four of their children and had two healthy ones.

Akpan lost his wife in 2007 after a brief illness.

He said, “Overall, it was a painful experience, especially when you see the children wriggling in pain and you can do nothing to help soothe that pain. It’s heartbreaking, to say the least.”

He said seeing his children dying one after the other, not knowing when the last day with them would be was miserable.

“Sometimes, when everything is going on fine, you feel like the children are winning over the disease, other times when the crisis

starts, it is like a glimpse of death,” he added.



Sickle cell disease

The American Centers for Disease Control and Prevention says sickle cell disease is a group of inherited red blood cell disorders.

Red blood cells contain haemoglobin, a protein that carries oxygen.

Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body.

However, in someone who has sickle cell anaemia, the haemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-shaped farm tool called a ‘sickle.’

These sickle cells die early, which results in a constant shortage of red blood cells.

Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious complications, such as infection, acute chest syndrome and stroke.

According to the American Red Cross, research shows that the mutation causing sickle cell disease originated in Africa thousands of years ago to help protect against malaria, which is a major cause of death on the continent.

June 19 is officially designated as the World Sickle Cell Awareness Day to increase public knowledge and an understanding of sickle cell disease, as well as the challenges experienced by patients and their families.

An American, Dr William Winther, who wrote a brief history of sickle cell disease, said the disease was discovered in the United States of America in 1910.

Reports suggest that Nigeria, India and the Democratic Republic of Congo account for the highest percentage of persons living with sickle cell disease in the world.

Meanwhile, the National Heart, Lung and Blood Institute of the United States notes that a blood and bone marrow transplant is currently the only cure for sickle cell disease.

It, however, adds that it is not for everyone as many patients who have sickle cell do not have a relative who is a close enough genetic match to be a donor. A well-matched donor is needed for a patient to have the best chance for a successful transplant.

According to the institute, most transplants to treat sickle cell disease are performed in children as blood and bone marrow transplants are riskier in adults.

It further notes that these transplants are successful in about 85 per cent of children when the donor is related and human leukocyte antigen (HLA)–matched.

However, even with this high success rate, transplants still have risks as about five per cent of people who have received such transplants have died.

Complications from the procedure can include serious infections, seizures, and other clinical problems.

Sometimes, transplanted cells attack the recipient’s organs, which is called graft-versus-host disease.

‘God loves me’

Madam Bose has been married for 54 years. She has eight children; three of whom have sickle cell anaemia.

The 76-year-old woman said the fact that all her children were alive was a miracle and a testament to how much she was loved by God.

She said, “It is not an easy thing. My knowledge as a nurse, God’s financial provision to take care of the children and the cooperation of the children themselves saw me through.

“As a parent, you must also try to bear the pain with them with plenty of reassurance that all will be good and okay.”

The grandmother said she discovered that she was AS when she was a student nurse, adding that a consultant advised her to endeavour her partner went for a genotype test as well before they got married.

“I told my husband, who was my boyfriend then, to go for a test. Despite pressuring him, he refused, saying he wasn’t a sickler. But because I loved him, I couldn’t say I was going to leave him and so, we got married,” she stated.

 According to her, after realising that her first child was SS alongside two others, she stopped bothering herself.

“If I can go back in time to make things right, with the experience I have had in life, I would make sure I find out his genotype before getting married to him,” she added.

The former nurse stressed that managing special children could be difficult, adding that it was always necessary to observe the weather and clothe them accordingly.

She said she bathed her children only once a day mostly in the evenings.

“In the mornings, I washed their faces and brushed their teeth and after they returned from school, I bathed them thoroughly before they go to bed.

“While they went to secondary school, I advised them to bathe only once a day when it’s cold. Then when it’s hot they should wear clothes that will help them have enough air. Most importantly, they must take their vitamins daily; vitamin C, vitamin B complex, multivitamins and folic acid. I made it mandatory for all eight of my children,” she added.

The 72-year-old said feeding was another important aspect that many neglect, noting that it was important to ensure that SS children had enough protein in their food, including beans and milk.

She also advised a reduction in fried foods and seasoning cubes.

“So, parents who have sickle cell warriors should not be downcast. I am a living testimony that it is possible to overcome against all odds.

“My first daughter, who is SS, is over 50 years old and her first son just graduated from a university in America at 24. I wish all mothers who are going through similar experiences God’s grace to have their testimonies,” she added.

Relationships ended

 A 35-year-old lady, Peace, said she ended her last relationship of over two years after finding out her then fiance, who had proposed marriage to her, was AS like herself.

She said her partner had assumed he was AA because he was always having malaria and it was believed that people with AA were prone to the sickness.

Peace said she did her test first, and when her result was out, she learnt that she was AS.

Scared, she hoped her fiance’s genotype would be AA.

But the results came out otherwise. Her partner, Joshua, was a carrier of the sickle cell.

“I believe in miracles but it is not wise going into something like this with your eyes open. I don’t think it will be fair on the children that such a marriage will produce,” she told our correspondent.

She explained that they could not end the relationship immediately, adding that the process was gradual and painful.

“We both knew each other’s families and they all knew we were engaged. Friends were already beginning to ask if we had fixed a date for our introduction and wedding, and it was difficult cutting that off. But it was something that had to be done,” she added.

Peace said it took one year after they found out their genotypes before they parted ways.

“Even after that, he would still call me up every once in a while to find out if I had changed my mind about marrying him, saying I should believe in God and have faith because he loved me and it was difficult to move on. But my answer never changed.

“Since then, whenever any suitor comes, that is the first question I ask and we proceed to do a test. I’d rather wait to get it right than rush into a marriage I will regret because of my age,” she stated.

Another lady, who agreed to speak to Saturday PUNCH on condition of anonymity, said she ended three relationships after finding out her proposed suitors were AS.

She said the first relationship was close to marriage, as they had fixed a date for the wedding and even proceeded to do their introduction  before the discovery.

The girl noted that she was advised to go for the test by her aunt, who was a midwife and owned a clinic, following her frequent malaria trips to the hospital.

Although she had previously done the genotype test prior to the relationship and the result showed that she was AA, she did not really bother asking for her partner’s status.

Her partner, on the other hand, knew his genotype was AC (another AS variant).

As things progressed, the marriage counselling committee in their church asked them to present hospital tests to prove their genotypes, saying word of mouth was not sufficient evidence.

It was around the time she was sick and her aunt advised her to run a test, which showed she was AS.

“At first, I thought it was a mistake, so I proceeded to carry out the test in three other hospitals, including the General Hospital, Gbagada. It was at that point I gave up and told my partner that we could not proceed.

“It wasn’t an easy decision and we were both heartbroken, especially because we had already gone far in the marriage preparation and even paid for a hall and sent out invitations,” she added.

The lady said her partner’s family didn’t agree with her as they called a series of meetings with her family to see how they could manage things, but her parents stood with her and shut down the ceremony.

She added that their pastor also preached to them about having faith that their children would not be SC, but she said her faith could not carry her.

“I cannot start what I cannot finish,” she said.

According to her, the decision to end the relationship was informed by her experience as a teacher at a primary school, where she had dealings with children who suffered from sickle cell anaemia.

“I know what these mothers go through and I don’t think it will be fair to bring forth a child that will be SC or SS knowing full well our status before marriage. I won’t forgive myself, so the best option was to end it,” she added.

The woman said to avoid wastage of the resources already expended on her marriage preparation, the bookings were passed on to another family member, whose marriage was moved to her date.

“At least another wedding held on that day, but it wasn’t mine,” she said.

“Apart from miracles that happen, which is supernatural, I don’t think it is worth it, and one shouldn’t even tempt God. When you know, no matter how much the love is, it is necessary to do the right thing.

“It is better imagined than experienced. Once you know your status, don’t let any pastor or prophet brainwash you. Others might be lucky but you might not be,” she advised.

Avoid chance wedding

According to a consultant haematologist at the General Hospital, Isolo, Dr Chris Onalu, whenever a couple, who are both AS decide to marry, it is a chance union.

“Anything that has to do with chance is a probability and it depends on which gene is being transmitted at the point of conception from the male and female.

“However, no medical practitioner will advise a couple to take that kind of risk because of what sickle cell disease patients pass through,” he added.

Onalu said there are a lot of technologies outside Nigeria that had been developed to detect sickle cell disease at an early stage

“For instance, there is what we call intrauterine diagnosis. Here, the fetus is still in the womb at the early stage of formation. The test is carried out to discover if the child has sickle cell disease. If it comes out positive, they have the option to terminate it.

“Also, through IVF, they will select the genes from the man and woman and make sure there is no SS gene included,” he added.

The haematologist further stated that the management of patients with sickle cell was multi-dimensional because a lot of medical disciplines were involved.

“Some may have severe anaemia, where their blood will be low; sometimes, there is severe pain, stroke, renal or kidney problems and the like.

“We also have a supportive treatment that we encourage them to do, observe and imbibe in their everyday life, such as drinking a lot of water, ensuring they sleep under mosquito nets, not getting engaged in very critical and strenuous activities. So, it is not just about medication and giving blood; there are lifestyle changes that they must make on their own,” he said.

The National Manager of Lancet Laboratories, Nigeria, Dr Aliu Israel, said the risk of AS couples getting married was not worth it.

He said, “Mathematically and biologically, it is not worth it. About 25 per cent of children such a marriage will produce will be AA, while 50 per cent are likely going to be AS and the other 25 per cent, SS.

“In this information-driven age, there should be no excuse for ignorance, unlike in the days of our parents. We should make the best use of the knowledge. God has given us brains, so we can give Him rest.”

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