Good day my dear readers, it’s been a while! How’s life treating you all? I hope this New Year brings a change in the way we handle our health, and other aspects of our lives, amen!
Another opportunity has come for us to learn about and discuss an important issue, one so pertinent in our society now, and I know some of us would have had personal experiences regarding this issue.
Time and time again, we have heard through various sources e.g., the media, family and friends, health workers, etc., about the importance of genotype compatibility before commencing on an intimate relationship with the opposite sex. While some of us heed this advice and abide, to an extent that at first meeting with a person you like and would envisage yourself in a relationship with, you ask the rather pushy and weird question ‘Please what’s your Genotype?’ Such a question might put the other person off, but trust me, especially for those who have the AS (like me) and SS genotypes; it pays off in the long run.
You love the person so much you’re willing to gamble the health of your kids? Even though no one knows the future, it may not be wise to be the potter of a cracked clay jar. You think you can’t live without the person and you would die? Come off it! I have had three ‘love-lost’ experiences due to genotype incompatibility and here I am, alive and kicking. That it would hurt leaving such a person is certain, but the love you once proclaimed might be put into question when handling kids with the sickle-cell disease.
My personal experiences were not funny. The first one happened when I was just a teenager. The dude didn’t even know what his genotype was when I asked him innocently, and he promised to go do a test. True to his word, by the next time we met, he had a result, AS. I started withdrawing from then, very difficult I must tell you, although we remain friends till date. The next two happened when I was much older, and one would think it would be less difficult to break away. In fact I ‘loved’ so much that I started thinking of in-vitro fertilization (test tube babies) and all those complexities. But finally, as a ‘sure girl’, I had to think with my head before my heart. Consequently, it became one of the first questions I ask a guy I like.
…cool story sis.
I shared my personal experiences with you because I have, severally, witnessed the pain and trauma people living with the sickle-cell disease (SCD) go through, especially children. It is TOTALLY heart-wrenching. Some would say, ‘Adesuwa there is nothing God cannot do’, but also we know we are not meant to put our God to the test. Let me educate you a bit on what SCD is about.
Sickle Cell disease, otherwise known as Sickle cell Anaemia, or Drepanocytosis, is a genetic blood disorder in which, simply put, the red blood cells of a person is ‘sickle-shaped’, named after the shape of the farm tool, sickle. This ‘sickling‘, which occurs due to a mutation in the Hemoglobin (Hb) gene, affects the flexibility of the cells, resulting in the symptoms and complications associated with this condition. Normal red blood cells have the hemoglobin gene designated as HbAA, carriers of the sickle cell trait carry the HbAS
gene, while sufferers of SCD carry the HbSS
gene. Under conditions such as low Oxygen, these red blood cells (containing sickle cell hemoglobin) aggregate, causing a distortion of these cells and resulting in loss of their elasticity. This loss of elasticity of the red blood cells is central in understanding this disease condition. In patients with HbAS (carriers of sickle cells), the distortion problems are minor, because the normal allele, that is, the ‘A‘ portion of the gene is able to produce over 50% of hemoglobin. They only have symptoms when deprived of oxygen, or when dehydrated.
Microscopic view of blood sample from a SCD patient. See the cells with the sickle shape?
Red blood cells are transporters of oxygen throughout the body, the Hemoglobin content being the oxygen carrier. Normal red blood cells are elastic and flexible and are able to pass through blood vessels, their flexibility allowing them to change shape when necessary, carrying oxygen to all parts of the body where blood is supplied. So imagine a sickle shaped red blood cell attempting to pass through blood vessels. Because of their lack of flexibility, they get stuck, therefore preventing the transport of oxygen in the body (Ischemia). Assuming they get stuck in the blood vessels supplying the brain, and the brain cannot work without oxygen for more than 5 minutes…death ultimately results.
Red blood cells typically have a life-span of 90-120 days after which they are filtered from the blood by the spleen and new ones are produced from the bone marrow. Sickle cells, however, have a life-span of just 10 to 20 days after which they are destroyed, causing the anemia. The bone marrow in patients with SCD constantly tries to produce new cells to compensate for the lost ones, but it does not match the rate of destruction.
I’m sure you have heard of a SCD patient hospitalized for experiencing ‘crisis. Sickle cell crisis is used to describe several independent conditions which may occur simultaneously. Most episodes of crisis last between 5 to 7 days, although in some patients, may last for longer. Such conditions include:
- Vaso-occlusive crisis, which is as a result of the sickle cells being stuck within blood vessels, causing blockade of blood flow and oxygen to an organ which results in pain and consequently, organ damage. The pain is often said to be indescribable, requiring strong opioid analgesics such as morphine to bring temporary relief.
- Splenic sequestration crisis, which occurs as a result of blood flow blockade to the spleen, causing its painful inflammation. Because the spleen stores red blood cells and other blood components (which it releases into circulation in conditions of low blood volume or hypovolemia), an inflammation causes blood to suddenly move from circulation into the spleen, leading to sudden low blood volume. If not treated as an emergency, a patient may die within 1-2 hours.
- Acute chest syndrome, characterized by fever, chest pain, difficulty in breathing and infiltrates in the chest.
Complications of this disease are diverse and include decreased immunity leading to opportunistic infections, silent strokes, kidney failure, Priaprism (unwanted erections in males), chronic pain leading to patients being dependent on opioids, Pulmonary hypertension, fertility, and bone wasting, among others.
The prognosis of SCD is that about 90% of patients survive to age 20, and close to 50% survive beyond 50 years. The frequency of SCD is found in tropical regions, particularly Sub-Saharan Africa, India and the Middle east; Nigeria is in Sub-Saharan Africa. A recent WHO report estimated that around 2% of newborns in Nigeria are affected by SCD, giving a total of 150,000 affected children born yearly.
You might not fully understand the meaning of all these medical expletives I just typed, but I am sure you understand how pain feels. Not just the type of pain from headache or bruises that makes us often cry and gnash our teeth to God, but deep seated pain that only the hand of God can save a person from.
How deep is that love, which you decide to gamble on, using the health of your unborn children as bait? My dear, only Agape love is sure in this world of ours. I know of a couple, who are both medical doctors and possess the AS genotype. One would expect that, health professionals that they are, they wouldn’t be so non-chalant about such an issue. Or is there a special copulation style that allows one to predict the genotype of an unborn child? As fate would have it, all their 4 kids had the HbSS genotype, which means, they were all ‘sicklers‘. Every other week, I see them in the hospital due to one problem or the other. Imagine the physical, emotional and financial stress they go through. They both are yet to start their residency program because of the overwhelming nature of taking care of 4 kids living with SCD. There was also a very beautiful girl in my secondary school, one of the most beautiful I had ever seen. She died at 22 years of age, after she just completed her first degree at the Buckingham University, United Kingdom. Can you imagine the pain the parents would have gone through, paying health and academic bills, and the child dying at her peak?
Checking for genotypic compatibility is very easy. After confirming your genotype, if you are AA, you have no reason worrying about incompatibility. You can even have a child with a person having SCD. None of your kids will suffer from SCD, they would just be carriers. Likewise for those having SCD, they can only have kids with those with the AA genotype.
If you are AS, you are only compatible with the AA genotype. The probability of having a child with SCD from two people with the AS genotype is 1 in 4, that means, you have 0.25% chance of having a child with SCD with each pregnancy.
I sympathize with those who have lost family and friends due to sickle cell disease. Don’t let love, money or whatever passions lead you into making a wrong decision. Firsthand, I know several people living with SCD and it’s a struggle consoling them anytime I hear they are being hospitalized. Learn, educate and heed the warning today. In ancient times, patients with sickle cell disease in Nigeria were said to be ‘ogbanjes‘ among the easterners and ‘Abikus‘ among the westerners, due to the thought that the children die young and are re-incarnated in the next child born to that family. Now, we know better. Let us have a generation where the prevalence of sickle cell disease reduces drastically. It begins with us.
Any experience to share, or questions concerning SCD and how to manage it? Please drop your comments and questions. I’ll be here to answer them.
Be safe, be happy.