■ Son born after 8-yr childlessness a sickler
■ How wrong lab test misled them into marriage
By Romanus Okoye
A family in Lagos is today experiencing the pain and bitterness of a wrong medical laboratory test result. The outcome of it has caused joy to take flight from the home of Mr Johnson Odogwu and his wife, Candice (real identities concealed), and cast a dark cloud over the couple’s marriage.
The foundation for the trauma that has beset the family was laid in 1997, when Johnson, as a young man, did a laboratory test that indicated his genotype to be AA. About nine years later, in 2006, he married Candice. After they announced their intention to wed, the parish of the Roman Catholic, where they both attended as members demanded to know their genotypes. Johnson presented the test result given to him by the laboratory while Candice also went for a test that showed her genotype to be AS. Based on these results, the church allowed them to marry as there was no medical ground to forbid them, given that they could not have children with sickle cell disease.
For eight years, the couple contended with delayed conception. However, the yoke of childlessness was broken in 2015 when their first child, a boy, was born. Then, he became sickly after the first year, to the point that he needed blood transfusion. As it is routine in medical practice, laboratory tests were conducted to determine his blood group and genotype and the results showed the little child to be sickler. Dumbfounded, the parents demanded a second test, which confirmed the first result. It was then decided that both parents be tested to find out their genotypes. When Candice was tested, the result also matched with the earlier one she had done prior to the wedding. But for Johnson, it was not so. His own test result showed that he has “AS” genotype contrary to what he was made to believe in 1997. Another test done in a different laboratory further confirmed his horror.
“I am finished,” Johnson had screamed in agony, when he was told the result of the confirmatory test.
With bleary eyes, he told Sunday Sun reporter: “What I feared most has happened to me. My marriage is threatened. I have heard about the pains of people living with sickle cell disorder (SCD); I never imagined it could happen to me; now my first child and only son is a sickler. My family members and in-laws are not yet aware of the development; I wonder how they will respond when they hear. We are really confused and devastated.
“Before our marriage, my wife and I discussed our genotypes. While my wife was AS, the genotype test I did in 1997 showed that I was AA. Even the church where we wedded was very serious about it. But we relied on the earlier tests, not knowing that there was error in mine. So after our marriage and the birth of our son, the boy fell sick in June 2015, warranting blood transfusion. When his blood group and genotype tests were done, the results showed him to be SS.”
He continued: “The result surprised us; so the doctors decided to do confirmatory tests for me and my wife, which surprisingly showed that we were both AS. We knew about the implications and would not have married since we were not under pressure. But now, if we separate and abandon our child in this condition, who will have the emotional attachment to take care of him,” Johnson wailed.
“If I marry another woman, would she be able to take care of the boy or if my wife marries another man, would he be patient to take care of another man’s sickler son. Only God can save us.”
While Johnson is ruminating over what has befallen him, a healthcare professional and medical laboratory technician at a popular facility in Surulere, Lagos, who spoke with Sunday Sun reporter under condition of anonymity confessed that he once committed such an error due to pressure of work. “When I collected the blood samples from the patients, I wrongly labeled the containers and that was how the results came out wrong. But I later realized the error because the patients use our hospital as their family clinic, so I had to repeat the genotype tests,” he explained.
A medical doctor who responded online sympathized with the couple but also said that the state of the Nigerian healthcare system has deteriorated so much that doctors now have too many patients to attend to, far beyond the accepted doctor-patient ratio. Sadly, he also acknowledged that some doctors may place priority on profit more than genuine healthcare delivery. This, he admitted, has seen some doctors in practice run quasi-units passed off as ‘medical laboratories’ in one room in their clinic, all in the bid to corner the laboratory fees charged to the patient and then turn around to make claims under the National Health Insurance Scheme. This unwholesome practice has caused a running battle with the appropriate regulatory body.
There now appears to be a silver lining in the dark sky for the couple as Sunday Sun reporter learnt from 47-year-old Mrs. Damilola Aseyege, who is living with Sickle Cell Disorder and is married with children. She emphatically said that there is hope for the couple as sickle cell disorder is no longer a death sentence. “I was preparing to die before I would reach 25 years. The general feeling those days was that people living with Sickle Cell Disorder do not live beyond 25 years,” she said.
“Now I do not feel sickly as before and I believe it is God’s doing. I take my drugs regularly and I also love to rest,” said Aseyege, a warrior, as people living with SCD are called. “Love yourself the way you are. Know your limits and understand what makes you feel better. Take care of yourself medically, nutritionally, mentally and physically.”
Aseyege said that genotype should no longer be a condition for marriage. “It is difficult to give advice on this. Things are getting better with the availability of more and better information. The old myth of people with SCD not living beyond 25 years is gone. I am now 47 and there are many others older than me and living well. The feelings one person has for another person cannot be conditioned by genotype. There are cases of both parents being AS and having several children with none being SS and cases of one AS and one SS marrying and having only children that are AS. I think the most important thing is for the couple to know very early the status of the child and with adequate care, the child will live well.”
Another anonymous respondent who is living with SCD said: “I wonder why people just talk with careless abandon; it’s very unfair and insensitive to think that every sickler will automatically live a life of pain. Yes, there will be occasions when things will trigger a crisis and they will fall ill; some mild, some severe. But don’t other people fall ill? Do people who do not even have sickle cell not die from minor illnesses? Are millions of children not born with other congenital effects? There are a million and one things worse than sickle cell. A sickle cell carrier can live a crisis-free life if in the right environment and gets the right care. I have seen obese children with sickle cell! Who would have thought that possible? These days, you only get to know that somebody is a sickler, when the person says, “Hello, am a sickle cell warrior!” I think that says a lot. It does not matter whether someone has sickle cell disease or not, the key to general well being is maintaining a healthy lifestyle.”
“Developed countries all over the world are actively working on research to cure diseases that affect their race. Don’t ever believe that there is no cure, there will always be breakthrough in science and medicine as research is ongoing.
“I am not advocating for AS couples to make babies but when the deed is done you look on the brighter side, that child is human and you would not give him or her up because of sickle cell! Sickle cell pales in comparison to some other diseases.”
The Permanent Secretary, Ministry of Health, Lagos State, Dr. Omodele Osunkiyesi, concurred with Aseyege, explaining that with “advancements in early diagnosis and treatment as well as life-saving measures, most children born with SCD grow up to live relatively healthy and productive lives.
“Most people with SCD now live into middle age and even well beyond. A critical time is the first few years of life, which is why early diagnosis and treatment are so important. Medicaments are now available to help manage the pain, while immunizations and antibiotics can help prevent infection. The place of stem cell transfer in non-technologically constrained climes is also relevant.”
No doubt, experts agree that the last few decades have seen the evolution of SCD from a lethal condition, to a chronic disorder that is increasingly compatible with good quality lives and longer life expectancies. But they also agreed that all strata of government in Nigeria and individuals need to do more in terms of provision of facilities and financial support to alleviate the challenges associated with managing SCD.
Sunday Sun’s visit to the National Sickle Cell Centre, Idi Araba, Lagos, revealed that the centre offers genetic counseling, sickle cell clinics, automated red blood cell exchange transfusion, stroke prevention through Transcranial Doppler Ultrasound Screening for detection of children at risk of developing stroke which occurs in about 9 per cent of children with SCD; leg ulceration care services and prenatal diagnosis of HB genotypes in unborn babies from 11 weeks of pregnancy on pre-booked pregnant women who are at risk of bearing children with SCD, among other relevant services.
Meanwhile, Nigeria has the largest Sickle Cell Disorder burden in the world with about 150,000 cases annually compared to America’s 2000. But while the American Congress in 1972 passed the Sickle Cell Anaemia Act and made available the necessary annual budgetary allocations to SCD, thereby raising the life expectancy of Americans living with SCD from 14 years in 1973 to 48 years in 1994 and 53 years in 2003, the National Assembly in 2012 failed to pass the SCD Act owing to reasons suspected to be related to its financial implications.
Commenting on this, an Associate Professor of Medicine and Clinical Director, Adult Sickle Cell Disease/Hemoglobinopathy Program, Vanderbilt University Medical Centre, USA, Professor Adetola Kassim, in an interview with the foundation’s publication, said, “For now, bone marrow transplant (BMT) is the only cure for SCD which offers the victim a chance to live a normal life with longevity, but most patients in Nigeria who wish to undergo a BMT, will for now need to travel abroad, usually with family members, including the donor at a significant cost.”
To reduce the prevalence of SCD and make the conditions easier, the Nigerian Sickle Cell Expert Advisory Committee had suggested firstly, the urgent introduction of newborn or infant screening for the early diagnosis of sickle cell disorder, explaining that its introduction would significantly reduce the large number of deaths and suffering caused by SCD, especially in early childhood.
They also suggested the free provision of prophylactic anti-infective measures against pneumococcal, meningococcal and other prevalent bacteria to infants and children with SCD; adequate safe blood for transfusion provided in all the states to save and support lives. And introduction of the training and recognition of a cadre of sickle cell nurse specialists as is the case in England and America as well as a publication of guidelines for the diagnosis and treatment of SCD, primarily, for the benefit of health workers in all parts of Nigeria.