Personal involvements with the sickle cell unit of the Child Health Department of the Korle – Bu Teaching Hospital (KBTH), Ghana, has led to unbelievable revelations about the large number of children who come to seek medical attention as a result of crisis of Sickle Cell Disease (SCD). Some of them started coming to the clinic as far as 4 years ago. For them, it has been appointments upon appointments. One could see the worries and frustrations in their parents (mostly mothers). These mothers have to bring their children to join long queues after which they are given numbers in ascending order, first come first served! Woe unto any parent who tries to dodge the queue. They sit in the queue long before the doctor attends to their children. These mothers, some of whom are workers virtually have to miss working hours and if not lucky to be attended to early, miss the whole day of work. The question is why are parents putting themselves through such ordeals of life? Did these parents actually have prior knowledge of the implications of genotype incompatibility? Did they receive adequate counseling on this issue prior to marriage? Why should these children suffer even in the abundance of knowledge?
Sickle cell disease sometimes referred to as sickle cell anemia is a significant public health burden in Ghana. It is a group of disorders affecting the red blood cells in the body. A research conducted in Ghana recently revealed that 2% of Ghanaian newborns are affected by SCD and one in every three Ghanaian has the hemoglobin S and/or C gene. SCD affects the blood and due to this, symptoms can appear almost anywhere.
In a research published by PubMed on “Mortality in sickle cell disease. Life expectancy and risk factors for early death”, where 3764 patients were enrolled, the findings were as follows: among children and adults with sickle cell anemia (SS), the median age at death was 42 years for males and 48 years for females. Among those with sickle cell-hemoglobin C disease (SC), the median age at death was 60 years for males and 68 years for females. Among adults with sickle cell disease, 18% of the deaths occurred in patients with organ failure (predominantly kidney). Thirty-three percent (33%) were clinically free of organ failure but died during a severe sickle crisis (majority with pain, the chest syndrome, or both; whilst some had stroke).
Some of the presentations of SCD include vaso-occlusive crisis, chronic pain, anemia, aplastic crisis, splenic sequestration, susceptibility to infection, growth, retardation, delayed sexual maturation, being underweight, exquisite pain and soft tissue swelling of the dorsum of the hands and feet (hand-foot syndrome), acute chest syndrome, central nervous system -, cardiac -, renal (kidney) -, and eye problems, leg ulcers, priapism (unwanted erection which may lead to impotence when prolonged), vascular necrosis and pulmonary hypertension among others.
Some few discussions held with some SCD patients made it known that people with SCD have a greater risk of problem with thinking especially during their crisis. It is therefore important for people with SCD to be regularly seen by a doctor.
“The pain I go through is so unbearable. This doesn’t make me concentrate fully in class, making me perform poorly. Just this is enough for me to make a decision not to go along with a similar union my parents put themselves into.” Says Edwin.
“I spend most of my time in the hospital. I get saddened when I see my friends getting involved in all sorts of activities whilst I cannot. Sometimes I blame my parents for causing me so much pain”. Says Jake.
Journaling, an act of keeping personal records of occurrences on daily basis helps SCD patients to understand what type of activities triggers episodes of illness or pain. With these strategies, persons with SCD are capable of achieving a near-normal life span with a reasonable quality of life. Here is the experience of a young lady whose parents applied journaling during her younger days:
“My mum and dad told me about the journal they kept for me whilst I was growing up. They only handed it over to me when I turned 12 because they believed I was old enough to handle a journal. Now I’m in my mid-twenties and just smile anytime I view the journal. A close eye was kept on me to monitor my everyday moves. I never liked that because I needed some privacy. It just felt like there was a camera on me every time. I remember every time I came back from school mum would always inspect what I had jotted down. My parents are very caring and loving and they still are. I love them for that. Since they were both carriers they were very hopeful none of their children would be sickle cell. Out of three siblings I’m the only sickle cell one. I know it really hurts them I have to go through this. From this experience there’s no way I will even get married to a carrier.”
An on-line survey in Accra involving 45 respondents was conducted to assess the level of knowledge among young people. The result for the survey had shown that all (100%) of the participants have heard of sickle cell disease and agreed that it’s a genetic disorder of the red blood cell. All of the respondents were educated as 71.1% had tertiary education. About twenty-six percent (26.7%) and the remaining were educated to the post-graduate and secondary school levels, respectively. From the 11 respondents who had indicated they were married, 72.7 % had shown that they were tested as well as received premarital counseling before getting married whiles the remaining 27.3% did not have any form of counseling or screening before getting married.
Most studies and data from urban areas in Nigeria have indicated that there is a fair knowledge among the population. Nonetheless, the rural communities and people living in less endowed communities in sub-Saharan Africa like Ghana seems to face greater risks, hence less survival of persons of SCD compared to those in developed countries.
The UN Sustainable Development Goals emphasized non-communicable diseases as public health concerns, and sickle cell disease deserves recognition as a widespread disorder that can lead to serious morbidity, poor quality of life, and early mortality.
It is very significant for elders in churches and communities to educate their members on SCD and its psychosocial issues so that carriers especially are given enough information to make strategic reproductive health choices. Two carriers may decide to go ahead into a relationship but their choice should be based on the full knowledge of the issues at stake so they would not venture.
Discussions with a certain staunch Muslim about whether premarital health counseling is done by their marriage counselors for members who intend to marry revealed this: “We don’t have marriage committees or counselors devoted to counsel intending couples but sometimes the elders of the mosque do request that their HIV and Hepatitis B status be checked but not that of sickle cell or hemoglobin genotype. Sometimes the wife only gets to know her sickling status during prenatal care”– AY.
The question is, if an expectant mother only gets to know that she is sickling positive (especially if her husband is also same) during prenatal care, what hope lies in there for her baby?
Mr. Boateng, a marriage committee member/counselor of a church has this to say on the issue: “We require compulsorily that intending couples who come to the committee do a sickling or hemoglobin genotype testing and based on the results we advise them. Those of them who are not compatible are discouraged from going ahead with the union and as a way of ensuring adherence; the church does not conduct such marriages.”
Traditional marriages across the country are also less concerned with scientific checks of SCD status before marriages are conducted.
Pre-marital genetic testing for the hemoglobin S or C gene equips individuals with knowledge of their carrier status before entering into a relationship with the view to having children. For this to be effective, it should be done before emotional bonding is advanced between two people in a relationship. Currently SCD only has management options through the use of several drugs. However, there is an effective treatment options which seems to be the only effective means that is the Bone Marrow Transplant (BMT). Nonetheless, BMT is not available in Ghana which means that patient have to travel to a developed country like U. S. A or South Africa for such an option. Therefore, currently in Ghana whiles BMT is not available, SCD must be tackled from the root cause.
Authors: Mary Nafaye, Promise E. Nukunu, Sharon Antah, Prince O. Sarpong, Prof. Major (Rtd.) George Asare,