How your genotype affects your marriage

 

How your genotype affects your marriage is a very important topic .Over 40 million Nigerians are healthy carriers of the sickle cell gene (Hb AS), while over 150,000 babies are born each year with sickle cell anaemia (Hb SS). This is by far the largest burden of sickle cell disorder (SCD) anywhere in the world. Most of these children may die in childhood or later from ignorance or lack of access to proper diagnosis and care. However, with proper education about genotypes by experts, SCD will be better understood by couples while other viable options will be revealed to families already battling with this condition.

 

Mariam was distraught; tears flowed ceaselessly down her eyes. The salty droplets turned black from the hue of her mascara as they fell on the paper she loosely held onto…the piece of paper that was potent enough to transform her sweet dreams into horrific nightmares. She loved Baba, she had loved him since she was eight and he eleven. As children, they co-starred in the neighbourhood plays as ‘mother and father’ and the whole community has for decades anticipated their wedding. “How come we never checked our genotypes till now, it is too late?”Mariam screamed as an unruffled looking Baba dashed in, placating her. He reassured her of his love and told her he was willing to take the risk if she was too. “I am ready to accept the consequences, what is the worst that can happen?” Baba said. She wasn’t ready to accept that. The marriage couldn’t hold. They broke up, otherwise they would have ended up having sickle cell anaemic children.

 

Implication of Sickle Cell Disorder (SCD) for about-to-wed or married carrier couples

Professor Olu Akinyanju (OON), a former president of the Association of Physicians of Nigeria and current trustee of the Nigerian Society for Haematology and Blood Transfusion who in 1994 founded the Sickle Cell Foundation Nigeria and established a National Sickle Cell Centre in Lagos, in a bid to explain the implication of Sickle Cell Disorder for about-to-wed or already married couples who are carriers of the sickle cell trait stated that “Healthy carriers of the sickle cell gene usually have Haemoglobin (Hb) AS, having inherited HbS from only one parent. Consequently, they must seek and obtain expert genetic counseling.”

According to the expert, “each offspring of the couple would have a 25 per cent chance of inheriting Hb SS and a 75 per cent chance of not doing so. That is a 50 per cent chance of Hb AS and 25 per cent chance of Hb AA. Thus, they may have six children and none would have sickle cell anaemia or on the other hand, their first two or three children, though rarely, may each choose the minority 25 per cent chance of having Hb SS and end up with SCD.”

However, Akinyanju explained that there could be other options for couples at risk of reproducing offspring with SCD. “The couple could work through the options of having prenatal diagnosis or pre implantation genetic diagnosis (PGD), they could choose to have no children of their own if they have not begun child bearing or they may dedicate themselves to caring for the children with SCD they may have already had.”

He noted the fact that with better care, which is now available, many affected individuals with SCD are living to old age as useful members of society. He stressed that with increasing research, better care may convert sickle cell anaemia into a treatable condition that is compatible with a normal quality and duration of life, just like hypertension has become.

 

Misconceptions and myths about SCD

  • SCD can be eradicated by selective mating

“This appears so logical and straightforward that many individuals, religious bodies and charitable organizations have tried to implement it themselves without careful consideration. They simply screen some young people and instruct those shown to be carriers to avoid mating with others who have the S gene. Some churches even refuse to marry them, which they deem as their own contribution to SCD control.”

“This strategy is based on the false assumption that SCD can be easily eradicated by mass population screening of genotype as a basis for coercive selection of spouses. The military Administration of Oyo State in Nigeria had in 1995, proposed a punitive edict aimed at prohibiting marriages between carriers of the sickle cell gene, but the conferences of Solicitors – General in Nigeria thwarted its introduction by declaring that it was unconstitutional and offended the human rights charter to which Nigeria is a signatory.

“The reality however is that enforced selective mating of couples has never been shown anywhere in the world to have reduced the incidence of any inherited disorder.”

  • Marriages between carriers are contracted in ignorance

The expert explained thus: “Another popular but false assumption is that all marriages between carriers of the sickle cell gene are contracted in ignorance. As specialists in this area, we in fact see many marriages contracted between carriers who are not only well educated but also well informed about their Hb status.

“A research study of the effect on choice of marriage partner of informing young people and their families of their carrier status was carried out in the Arta area of Greece, where 20 per cent of the population carries either thalassaemia or sickle cell gene. All young people of marriageable age were screened and counseled and counseling contact was maintained for a two-year period. When the pattern of marriage was assessed at the end of the period, screening had no measurable effect on choice of partner.

“Also of note is that if all people with Hb SS are forced to marry partners with Hb AA, all children will be carriers with Hb AS. If all HB AS children married partners with Hb AA, on average, 50 per cent of their children will be carriers with Hb AS. The prevalence of carriers will increase and given our present state of knowledge, eradication of SCD can only be feasible by the unthinkable genocide of all healthy carriers of the gene or the enforced banishment from the country or continent.

“In short, we cannot eradicate sickle cell anaemia and the sooner we accept this reality, the better. We need to increase our capacity to treat them effectively and invest in research to discover even better treatment.”

 

Is there any permanent cure for Sickle Cell Disease?

“There is a procedure known as the bone marrow (stem cell) transplantation. The transplantation of stem cells derived from the bone marrow of Hb A compatible siblings (with Hb AA or Hb AS) has been used for the cellular and symptomatic cure of sickle cell anaemia. This is not a genetic cure and the genes for Hb S remain in the transplanted (ie the recipient) individual. However, the recipient would, after a successful transplant, produce only Hb AA or Hb AS – depending on whether the donor is AA or AS.

“Although this is a landmark achievement in the treatment of sickle cell disorder, its rigorous pre-conditions and great expense make it, for now, inaccessible to all but the fortunate minority. World-wide, from all transplant centres, only 400 patients have received this treatment with 90-95 per cent success rate as at 2013,” the expert said. He bemoaned the fact that what in Nigeria, there is a lack of the political will to invest in research as dreams and wishes cannot bring success but research will.”

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