Some years ago, Mr and Mrs Femi Adeboya watched in tears as life gradually slipped away from their 22-year-old daughter, Adetutu, at the Lagos University Teaching Hospital, Idi Araba, after three months of severe pains.Adetutu, a third-year student at the Bells University of Technology, Ota, died from complications arising from sickle cell anaemia and she was the second child in the family who succumbed to the disease.The loss of two children in a row was a very harrowing experience for Femi Adeboya and his wife, Opeyemi, but they have apparently been able to overcome their grief, as they now advise intending couples to check their genotypes before getting married. Mr. and Mrs. Adeboya, both with AS genotypes, placed love above other seeming considerations and never bothered to check if they were genetically compatible before they got married. Consequently, two of their four children were born with sickle cell disorder.Their trauma began with the death of their second child Oladapo, 24, a couple of years before the death of Adetutu, as the two children suffered from sickle cell anaemia.``We put love first; that's a warming for everyone to also take other things into consideration before getting married. We truly loved each other but we were ignorant of the fact that with our genotypes, we could produce sicklers,'' Opeyemi said.The Adeboyas' recalled that as they were always trying to contend with the financial, psychological and physical trauma of taking care of their two sick children, who at times fell ill at the same period, they were also struggling to keep their home together.``It was as if marriage was a curse, as the children were in and out of the hospital every fortnight,'' said Femi.His wife, Opeyemi, who urged intending couples to avoid making a similar mistake as theirs, estimated that the family spent about N5 million annually on the hospital expenses incurred on each of their two children suffering from sickle cell disorder.Medical experts say that there are different forms of sickle cell disease -- each determined by the type of genes inherited from the sick person's parents.A person, who inherits a sickle cell gene from each parent, suffers from Hemoglobin SS Disease, also referred to as sickle cell anaemia.Dr Tajudeen Salami of the General Hospital Marina, Lagos, said that a person could inherit a sickle cell gene from one of his or her parents and a different kind of abnormal gene from the other.He said that such people suffered from a different form of sickle cell disease referred to as Haemoglobin SC Disease or Haemoglobin S -- beta thalassemia.Salami said that someone who inherited only one sickle cell gene (AS) and a normal gene (AA) from the other parent would have the sickle cell trait but not the disease per se, adding that the person would be AS and not SS.He explained that people with sickle cell trait (AS) would not have the sickle cell disease or exhibit any signs of disorder but could pass the gene for the disease to their offspring, adding that many people did not even know that they had the sickle cell trait.Salami, however, said that when both parents had the sickle cell trait or if they were both AS, there was a 25-per-cent likelihood that a child would have sickle cell disease (SS).He added that if one parent was AS and the other SS, there was a 50-per-cent probability that their children would inherit the disease.``It is called a disease because the inherited abnormality causes pathological conditions which can lead to death or terrible consequences.``In sickle cell, life expectancy is shortened, with studies showing an average life expectancy of 42 years for men and 48 for women,'' he said.Salami said that in sickle cell anaemia, lower number of red blood cells occurred because sickle cells had a very short life span, as the cells usually died after 10 to 20 days.``The bone marrow cannot make new red blood cells quickly enough to replace the dying ones and therefore, the sufferers experience a major crisis,'' he said.Commenting on the sickle cell anaemia, Prof. Henry Okpala of the Department of Hematology, University of Nigeria, Nsukka, said that the medical condition was a genetic blood disorder characterised by red blood cells that assumed abnormal, rigid, sickle shapes.According to him, sickling decreases the cells' flexibility and results in a risk of various complications.Okpala noted that about 150,000 babies were born annually in Nigeria with sickle cell disorder, while 40 million Nigerians were carriers of the sickle cell gene.Mrs Ayo Otaigbe, a Genetic Counsellor at the Sickle cell Foundation Nigeria, said that discouraging sickle cell carriers from getting married would not stem the incidence of children being born with the disease.She said that part of her duties as a genetic counsellor was to provide intending couples with full and accurate information to guide them to make pragmatic decisions on issues regarding their marriage.She, nonetheless, advised pregnant women with AS, who were married to men with AS or SS genotypes, to go for a pre-natal diagnosis, where a sample of chorionic villus (placenta cell) would be tested when the pregnancy was a little after two months.``However, the test is quite expensive and not affordable for most of the people who require it,'' she said.Otaigbe said that the pre-natal diagnosis was a very sensitive test which would reveal if a foetus had the sickle cell anaemia, thereby giving the couple the ample chance to make informed decisions about the coming baby.She said that the test would enable the couple to have the necessary information that could help them in efforts to increase the life expectancy of the child.Otaigbe said that premature death of babies with sickle cell disorder could be avoided if their parents knew their genotypes at an early stage rather than waiting for a later stage when complications arose.She proposed that all babies brought to the hospital should be subjected to compulsory genotype tests, stressing that if the babies' genotypes were known on time, those with the sickle cell anaemia would be directed to appropriate quarters for assistance.Symptoms of sickle cell disease vary, ranging from mild to severe, and different in kids who have inherited a sickle cell gene from one parent and a different abnormal haemoglobin gene from the other (SC).Mr Emmanuel Ibekwe, the Chairman of Dabma Sickle Cell Foundation, bemoaned a situation where most of the families suffering from sickle cell anaemia refused to volunteer information about their individual experiences, which would be useful in efforts to create proper public awareness on the medical condition.Ibekwe, whose 20-year-old child died of complications from sickle cell disorder, said that most families refrained from speaking about the problems they encountered in managing sickle cell disease.He stressed the need to divulge such information because it would be useful in sensitising the public to ways of avoiding the risks of having children with sickle cell traits.Ibekwe emphasised that it was not advisable for persons with sickle cell genes to get married because of the harrowing experience of raising children suffering from sickle cell anaemia.He said that the management of sickle cell disorder was a costly venture, adding that it usually depleted the resources of parents of the children afflicted with the disease, eating up funds which could have been expended on profitable ventures.In spite of the rationale behind the policy, some critics argue that the measure can cause the stigmatisation of those affected or lead to the falsification of genotype status by people with sickle cell disease or healthy carriers.All the same, bone marrow transplant remains the only known therapy for sickle cell disease. To be eligible, the patient needs bone marrow or stem cells from a 'matched' donor with low risks of rejection.Even then, the therapeutic surgery has significant risks and there is a probability that the transplanted marrow could be rejected.On Sept. 29 this year, the University of Benin Teaching Hospital (UBTH) recorded the first successful bone marrow transplant in Nigeria by transmuting seven-year-old Matthew Ndik from SS genotype to AA genotype, using stem cells donated by his 14-year-old brother, Emmanuel.However, Mathew's parents conceded that the cost of the surgery and the post-transplant care was very expensive. Mr Ebenezer Olaleye, a Project Coordinator in the Sickle Cell Foundation Nigeria, said that even though the success of the first bone marrow transplant was laudable, it might be the only one in a long time because the infrastructure was not available in Nigeria to continue with the procedure.He, however, conceded that although it would be relatively cheaper to have the surgery performed locally rather than travelling abroad for it, the manpower and equipment were still lacking in Nigerian hospitals.``It costs around N17 million to carry out a bone marrow transplantation abroad; it would be quite cheaper to get it done in Nigeria but do we have the necessary equipment and manpower to carry out this intensive surgery''' he asked.
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