Ravaged by sickle cell anaemia
...A tale of woes, anguish over misdiagnosis, negligence and 'blind love'
The facility’s reception room is artistically decorated. There are paintings on almost all the walls. Most of the artworks have children-related themes.
And although the motive for such interior décor is unknown, the ambience tends to bring hope for kids who walk in with their parents crying or tensed.
The most prominent art piece are the multiple-coloured cubes stacked on each other to form three pillars standing on old car wheels—these are the first things you see as you walk in with a suffering child.
But this ambience was not enough to ease Kola’s agony who laid on the soft black-leather sofa rolling side-ways and holding his back and leg at the same time, while his mother ran to get help.
Kola, now 11 years, has been suffering from sickle cell anaemia all his life. Since he was found to have the disease, his parents have been making trips to various hospitals seeking better management alternatives.
“We noticed when he was 6 months old. We just saw that his leg was swelling. So, when we got to the hospital, we were told it was a sign of SS, and that we should go and run a test. Since then, we’ve been managing it,” his mother, Yinka, said holding drugs contained in a black nylon.
Yinka, who is also a secondary school teacher, said managing the situation has not been easy since they started going to the clinic every three months, stating that there has not been any complication since then.
“There hasn’t been any problem because we follow the rules and instructions. We also get health talks at clinics. He’s 11 years old and he’s been admitted only twice since then. The last time he was admitted was in 2013, he’s never had to need blood since.
“It has been God, even today I have cried. But I know I will overcome it. What I know is that he will die. I have that faith. But I know it’s my cross. I have to carry it and bear it,” she said.
However, despite using the drugs, she told BDSUNDAY that during cold weather, he has crisis more than normal as a result, “he’s been complaining of pain in the back, in the leg, and everywhere.”
“There is a drug we give him. So, we’ve come for the drugs. But at times, the pain persists, but there is an injection they give to him for the pain to stop,” she further said.
While explaining the situation to this reporter, the teacher said she never imagined she would have a child affected with sickle cell, having gone into marriage with the hope of having an AA genotype. According to her, she had done a genotype test as a student of a higher institution, but it turned out she was given “a fake result.”
“They gave me AA. It was a school hospital and the thing (result) is in my documents. So, when we found out he was SS after 6 months, I said it couldn’t be because I know my husband is AS. So, I was asked to go and do it again that’s when I realised that it is AS,” she said.
“I sometimes feel tensed that he would have a crisis at any time,” said Juliana Williams. “To manage it, we have to work with what works for him. His main problem is malaria, so we have to make sure that there are no mosquitoes around.”
Juliana and her husband, Pius Williams had come to the sickle cell centre located at Idi Araba, Lagos, Nigeria, with their son (name withheld) who is under five years to get drugs. This would be the second time of the visit since they found that their boy has sickle cell disease when he was only six months old.
The couple were not expecting to have a baby with the disease as they had done a genotype test before marriage. “The test said my wife was AA, so we didn’t know we would have an SS child,” Pius Williams, a businessman, said.
“When we travelled out, he did not have a crisis. So, I think this is just in Nigeria because of the mosquitoes and everything….It is expensive to manage and we cannot keep record of what we’ve spent because it’s a continuous thing,” he further explained.
Somewhere in Surulere, Lagos State, a crowd that of the size of a mini-market, composed of children, teenagers, and adults (mainly women) covered the entrance of a sickle cell advocacy centre, all seeking for help in form of treatment.
Sitting in a corner and bending over holding his waist, was Tunde Fredals, a boy around 11years. He told BDSUNDAY that he could not describe the pains he goes through.
His sister, Opeyemi Fredals, who brought him to the centre, said he has problems in his waist and leg.
“He had an attack once in school, but because his teachers know he is SS, they called my mum because he was running temperature and was going through pains. Before, we didn’t know he has sickle cell. The genotype was wrong, maybe a mistake because they said he was AA. So, when he fell sick, they had to take him to the hospital and discovered he is SS when they did his genotype,” she explained.
Also narrating her story, a woman who gave her name as Linda said she discovered that her 12-year old daughter, Lola, is sickle cell anaemic when she was six months old as well.
She added that taking care of her since then has not been a jolly ride as she has since been moving from hospital to hospital.
“Most times she complains of pains all over her body. At times, stomach and chest pain. It’s not all the time she has crisis I am able to take her to the hospital. So when it starts, I give her the first aid to calm her down,” she said.
Like some other women who spoke to the reporter, Linda was also a victim of misdiagnosis. According to her, the result of the first genotype test she did was AA, but was proven otherwise when Lola was born.
“Before I got married, I and my husband did the genotype, but I was given a fake result, this was years before the wedding. I did not check my genotype again after then even after I gave birth to my first child. It was when I gave birth to her, (Lola), that I checked and I was told am AS.”
In her pains, little Lola confirmed to BDSUNDAY that her condition is affecting her studies as attendance in school is affected. “It’s not every time I go to school,” she said, almost whispering.
For Awosope Michael, he visits the clinic every three months for checkups, except when he has crisis, then he goes for treatment. The student and entrepreneur said when he falls sick; he gets back up within two weeks.
According to him, although his family has been a strong support, helping out in times of crisis, his condition affects him as a person.
“The crisis comes in different forms; today, it might be the leg, the hand, the head and the back- serious pains. This really affects me as a person. Most times, it keeps me out of school; I won’t be able to do anything.
“I will just be lying down receiving series of injections, and that could go on for a month. I also face peer pressures here and there, but thank God for the kind of family I have, they have been very supportive,” the 25-years-old said.
Michael told BDSUNDAY that his condition prevents him from socialising much due to stigmatisation. Though he plays when he can “but there is a limit; so I don’t over-stress myself,” he said.
Sickle cell disease (SCD) is a common haematological disorder, affecting millions of people worldwide.
It is most prevalent in malaria-endemic areas in the tropics where outcomes are often poor due to resource constraints, resulting in most children dying before reaching adulthood, said a study published in the Journal of Global Health.
As a global burden, it accounted for over 30,500 births in 2010, with millions of people currently affected across the globe. From their findings, “it is clear that both the highest prevalence and highest mortality of SCD is in Africa,” the researchers said.
A report by United Nations, said it is present on four continents: in sub-Saharan Africa and in the Maghreb, in Asia (Middle-East, Arabic peninsula, India), in the Americas, on the North (USA), centre (Guatemala, Caribbean islands), and on the South (Brazil, Surinam, Guiana), in Southern Europe (Southern Italy and Sicily, Greece, Turkey).
A more recent study by the World Health Organisation (WHO) said five percent of the world’s population carries trait genes for haemoglobin disorders, stating that over 300, 000 babies with severe haemoglobin disorders are born each year.
“The majority of children with the most severe form of the disease die before the age of five, usually from an infection or severe blood loss. In countries such as Cameroon, Republic of Congo, Gabon, Ghana and Nigeria, the prevalence is between 20 to 30 percent,” WHO reported.
Last year, the Coalition of Sickle Cell non-governmental organisations in Nigeria decried the prevalent rate, stating that at least 150,000 children are born with sickle cell disease in Nigeria every year, while about 40 million Nigerians are healthy carriers of the sickle cell gene.
Speaking to BDSUNDAY, Maureen Nwachi, head of administration, Sickle Cell Advocacy Management Initiative (SAMI), said the situation had improved from what people used to know it to be and think it was due to ignorance and lots of myths that surround it.
“Now, we have lots of advocacy and awareness going around, people are beginning to understand it better,” she said, adding that the level of awareness and management has contributed to increasing life expectancy as compared to the past.
“It’s not every doctor somebody living with sickle cell should see. There are people who specialised in that area; they are called haematologists. They are the ones who can identify some things a general practitioner would normally misdiagnose as another health problem, and then it would cause a complication which can lead to death. But with time, doctors have been able to bridge the gap where there is no longer ignorance in the management system. So, I think that has helped over the years,” she said.
According to her, some people who know that they have the disease have gone on to live their lives and are doing well. “Understanding themselves, and knowing what to do par time, what to eat. So I think that is what has helped over the years,” she said.
“Technological advancement has helped a lot,” said Olayemi Onijala, Scientist at Sickle Cell Foundation Nigeria.” Life expectancy is high now because so many things we were not aware of, now we can do them.”
“In the past, some of them, between the ages of two to 16 years usually have a stroke, and then, there was nothing for us to use in diagnosing the stroke. So now we can diagnose the stroke easily, and now we have many other drugs that are helping them to build their immune system,” the scientist said.
Despite improvements in awareness and advancement in technology, Nwachi, who is also a genetic counsellor, said the state of sickle cell in Nigeria may have improved but that it is still not enough, being the country with the highest-burden of with sickle cell.
Why the problem remains endemic despite improved state
From BDSUNDAY’s investigations, there is evidence that the problem persists, and the reasons include misdiagnosis, negligence of parents and love. This also shows why Nigeria’s situation has been described as endemic.
Most of the women who spoke to the journalist said they were victims of misdiagnosis. But a few others like Bilikis did not carry out a genotype test before marriage. Bilikis, a hawker, once had six children.
Three out of those children had SCD and one died two years at six years. She now roams with two affected children (both 15 and 12 years) from one clinic to another in search of relief.
Asked why she did not do a genotype test before marriage, she replied: “If person do am, anything wey go happen go happen. If you no do am, wetin go happen go happen. So, I no do. As you see me so, I no dey sick, but I don’t know my genotype,” she said in Pidgin English.
Simply put, what Bilikis meant by that statement is that, whether or not she had done the test from inception, whatever would have happened, would have eventually happened- an attitude that blames it on fate.
Despite her sufferings, Bilikis who is nearly 40 years old and has been married for 21 years, told BDSUNDAY that she does not know her genotype till date, and is determined not to know.
“Wetin I wan use am do again? Even if I do am now, e no change anything,” she added, arguing that it would make no difference if she checks her genotype.
Micheal, whose mum is a retired nurse, earlier said that his parents knew they were both carriers but went ahead with marriage with the hope of a miracle; thereby subjecting him to life-long crisis, unless that ‘miracle’ happens.
“By the time my husband and I were aware that we were both AS, we had gone far in our relationship; so we couldn’t break up. So, we started praying to God, that our lives are in your hands. We knew of people who do not have an SS and they are both AS. So, we had the faith that ours would be like that. But she came out SS as the firstborn.
“We took everything by faith; we believe that it was God who gave her to us, and so, we’ve been managing it,” Titilayo said while waiting for her 15-year-old daughter to receive care.
Nwachi, head of administration, Sickle Cell Advocacy Management Initiative, (earlier mentioned), further explained that ignorance was the first cause because people in the rural areas do not understand what it means to have a genotype, and the options they have to avoid having a child with sickle cell if they go into a relationship.
“Then two, we have problems with our diagnostics labs; misdiagnosis is a problem. I don’t know how we are going to curb it. So, sometimes, when we can’t come in during that genotype testing, we refer people to places we know are credible and they will get the result. These two are the major problems,” she said.
While blaming half-baked scientists in Nigeria, and obsolete equipment as factors the disease has remained in the country, Onijala, a scientist (earlier quoted), said the fact that nobody gets to sue anybody for wrong result which ends up causing families a lot of stress, also contribute to the problem
“Again, very few of us are married because of love. Some are saying that ‘because I love her, I don’t want to leave her. Even though we are going to have SS I don’t mind.’ But they don’t really know the challenges of having an SS baby because they’ve not seen one before. So, we have a lot of that in Nigeria,” he said.
Finding a cure
Experts confirm that a stem cell transplants (found in the bone marrow) through a bone marrow transplant is presently the only potential complete cure for SCD. They, however, say that “it is risky”.
In an exclusive interview with Jannah Amiel, founder of SuperNurse Educator based in Florida, United States, BDSUNAY learnt that there’s about a 5 percent chance of death after stem cell transplant.
Amiel said that the procedure is not likely to be an option for most adults “because risks become greater with age.” She added that finding a bone marrow donor that is a match to the recipient can be challenging.
“And even when there is a match, like with other types of transplant procedures, there’s a risk for some serious complications like severe infections or the recipient’s immune system rejecting the transplant—all of which can be life-threatening.
“The stem cell transplant (via bone marrow transplant) procedure is successful in curing about 85 percent of people with SCD. But remember, because this treatment option is not available for most adults, this percentage largely reflects the paediatric population,” she said.
But Ameil also said that organisations like the National Institute of Health (NIH) have launched initiatives to help find a cure for SCD that’s not related to bone marrow transplant from donors, but will concentrate on how to genetically modify the stem cells of a person living with SCD.
NIH is the primary agency of the United States government responsible for biomedical and public health research, founded in the late 1870s. The initiative, called ‘Cure the Sickle Cell Initiative’ was launched in September of 2018.
“They’re also working on the ‘Production Assistance for Cellular Therapies’ (PACT) programme to support research on other curative therapy options. These will offer alternative treatments for those who aren’t candidate’s bone marrow transplant or cannot be matched with a donor,” she said.
But before the initiative, which Amiel described as “good news”, would begin to yield results for US and the world, the present hope for a permanent cure is still the stem cell transplant.
“But they are very expensive and inaccessible to people, especially the kind of people we cater to—very low income, even the middle-class income people will think twice before they attempt to go through such procedure because it runs into millions,” Nwachi lamented.
She further speculated that it could cost as high as N7 million (about 19,337 USD) in Nigeria, and only one doctor had successfully done such a procedure. Besides high cost, which Onijala projected to be around N12 to N20 million abroad, there are other issues to think about.
“It takes a lot and the period of stay for the procedure takes about five to six months for a complete bone marrow transplant. So, that is the known permanent cure for now for sickle cell,” said Onijala.
It all comes down to Nigeria’s government
Therefore, with little or no hope in sight for sufferers, the government is now looked upon for effective management programmes; or at least, to fund non-governmental organisations working to rid Nigeria of the burden.
“Presently, we don’t really get government support when it has to do with sickle cell. The way they fund malaria, HIV, if that is the way they are funding sickle cell, it will reduce the burden of sickle cell in the country. But we don’t see government funding anything. I’m sure there is a bill in the Senate lying there for over seven years; nobody has passed the bill up till today
“Sickle cell foundations should be funded. There should be a budget from Nigeria, such that anybody coming here should be treated free; anybody coming to that centre should get drugs free, pay the staff money, and employ more doctors for sickle cell,” said Onijala.
According to Nwachi, the government needs to recognise that this is a public health problem. She said this would help put some policies in place.
Similarly, on financing NGOs, Nwachi said: “Funding is always a thing, we can give palliative care. They come here now and we give them routine drugs which will last them for one month and they come back the next month. But someone would need extra attention. Sickle cell can cause a lot of other complications. Some people may need to do x-rays for bones, or run a test to check their kidneys,” Nwachi said.
According the researchers cited earlier, in order to address this burden, there is a need for national comprehensive newborn screening to identify patients, and the development of holistic SCD care programmes to provide therapeutics and education for families and children with SCD.
While that is yet to be in place in Nigeria, there remains bleak hope for affected children like 14-years-old Daniels Williams who wants to be a civil engineer, as parents continue to run helter-skelter wanting to drink from the milk of human kindness at NGOs.
“I’ve heard about SS but I didn’t know the level of problems associated with it, until I had him. My second one is AS, and it has not been easy,” said Yinka.
PS: Apart from the experts, the majority of the names in this story are not real, this is for protection. But these people need help. Contact us if you would like to assist these families.