The young couple were referred to me by a friend; madly in love and giggling foolishly at each other, they sat opposite me with expectant faces. I was happy to see them as it is not everyday one sees young people being health conscious. They had come for pre-marital screening. I learnt that they had met during their university days abroad and that theirs had been a slow brewing friendship that turned into romance, spanning over seven years. Their families had met and it was finally time to tie the knot. They had this genuine sweetness about them that made it hard not to like them. I wished them well as I filled the forms for the tests: HIV, Hepatitis B&C, Syphilis and Haemoglobin genotype.
When they returned the next day, the bubble of excitement which had previously engulfed them was visibly absent. It was like someone had pricked it with a needle and replaced it with an invisible cloak of gloom. Without uttering a word, the lady handed over the results. Everything looked good: they were both negative for all the infections screened. Only that their genotypes did not match; they were both AS. Suddenly, I recognised the expression on their faces- Pain. Raw, unadulterated, heart wrenching pain. She started to cry softly while I tried to console her. The groom to be was equally devastated as he tried to look everywhere and anywhere else; everywhere but at me. I counselled them about the possibility of having a child with sickle cell disease and to think long and hard before making a decision.
In medical school, one of our paediatric lecturers used to explain the concept of sickle cell disease to parents using bananas and oranges. He described normal red blood cells as ‘Oranges’ because of their round shape and the abnormal or sickled red cells as ‘bananas’ because of their moon-like shape. When the bananas attempt to pass through a passage normally meant for the round orange shaped cells, they ultimately break leading to anaemia and pain in the form of what we call a ‘sickle cell crisis’. A sickle cell crisis is painful to watch and a lifetime of caring for a child with Sickle cell anaemia is guaranteed to break one-physically, emotionally and financially.
They returned a week later, armed with a barrage of questions. They had done their research and wanted to challenge all my years of training with their google search. The groom to be, an IT expert, talked about pre-implantation genetic screening (PGS) through IVF. He talked about embryo selection, gene therapy and bone marrow transplant. He raved on and on about how the world had progressed beyond banning AS-AS marriages and all the options available. I listened quietly, understanding that he wanted desperately to justify their reasons for going ahead with the marriage. When he was done, I asked them to take a walk with me.
Basically, there are three options available to couples who want to have children but posses the sickle cell trait; 1) Embryo selection through IVF 2) Aborting foetuses with Sickle cell Diseases until you get a foetus who is healthy and 3) Adoption. As we walked to the Emergency Paediatric unit (EPU), I explained the first option. Pre-Genetic Screening (PGS) alone costs about $2000-$4500 per test, excluding IVF. All the embryos cultured would be tested and those devoid of the sickle cell trait would be transferred to the mother for incubation. In the event that she does not miscarry, a healthy baby is born. The second option, although cheaper, is more disheartening. The woman is allowed to get pregnant naturally, however at about 10-11 weeks, a sample is taken and tested for the abnormal gene. If present, the baby is then aborted, otherwise a healthy baby is born. At present, I do not know if options 1 and 2 are carried out in Nigeria. They argued that they could relocate to the UK to have their babies and I just smiled. It was clear that they were both optimists but extremely naïve. They were both just starting out in their careers and so relied heavily on parental support for the wedding. Did they think their parents would continue to support them financially especially as they were cheaper options? Did they know the failure rate associated with IVF? Were they willing to keep aborting foetuses until they got a healthy baby? Did they know the mental and financial hardship they would undergo if they decided to follow this path? And that their love, rock solid as it seemed, might not withstand these hardships?
The third option, I explained was the safest and cheapest. They could adopt any child from the millions of orphans we have in this country. Adoption would give them an avenue to choose healthy children to nurture and earn them extra points in the hereafter without going through the anguish of pregnancy and its uncertainty. But I knew they would not. Nigerians generally associate adoption with failure; a grave misconception.
At any given time in the EPU, there is ALWAYS a child on admission undergoing a form of sickle cell crises. It is with that knowledge that I asked them to accompany me into the ward. I wanted them to see first-hand the painful situation of children with the illness. A child was crying and holding his leg in pain while his mother massaged it for him. Another one lay on the bed unconscious after suffering a stroke. Yet another child was seated, playing on the bed with his huge belly and yellow eyes. The couple remained quiet.
On our way back to the clinic I discussed the probability of having a child with SCD. There is a one in four (25%) chance of having a child with sickle cell in each pregnancy. They could be lucky, like a cousin of mine who went ahead and married her heart-throb and now has four healthy children without any form of intervention. Or they could be unlucky like my aunt who buried three children and currently has four others with the disease. She once called me in the middle of the night in tears and said to me ‘I wish they would just die’. One can only imagine the pain a mother would go through to utter those words.
Currently, the treatments available for patients with SCD are Bone marrow transplantation and gene therapy. However, not all individuals with SCD are eligible for bone marrow transplant as there are associated complications. The use of gene therapy in the United States is still under trial.
The couple looked lost and morose by the time I escorted them to the car. I felt like a monster for dashing their hopes, but the truth had to be told. Prevention is better than cure. Life is hard enough without throwing sickle cell disease into the mix.
Some days later, my friend who referred them to me, called. The wedding had been called off and the couple had gone their separate ways. The couple had wanted to go ahead with their plans but both families had refused. The felt the situation was too risky. Thank God for their collective common sense. I felt pained, but strangely at peace. People need to know that love alone is not enough and that we have a collective burden to reduce the burden of Sickle Cell Disease in Nigeria.
The argument of whether AS-AS couples should be allowed to marry or not is still very much alive, even in medical circles. While some of us believe in prevention as a tool in reducing the incidence of the disease, others rightly argue that people should have be left to make their choices in life and decide what is best for them.
Exactly a year later, I saw wedding pictures of the man on Instagram with another girl.
Life continues.
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