Unraveling Sickle Cell Disease; Know Your Genotype Before Marriage.

 

Sickle Cell Anemia which is commonly known as Sickle Cell Disease is an inherited form of anaemia whereby the red blood cells that carry adequate, sufficient, and enough oxygen through the human system are not healthy enough. 

Normally, red blood cells are shaped like discs which give them the flexibility to move and travel even in the smallest blood vessel. 

This disorder breaks down the red blood cell into a sickling shape or crescent moons. The World Health Organization states that over time, the red cells become hard, rigid, and sticky and look like a C-shaped farm tool called a “sickle”. When this happens, the red blood cells get trapped in small vessels, unlike the healthy red blood cells.

The difference between Normal red blood cells and sickle cells. 

There are 4 haemoglobin pairs or formations in humans. Children inherit this from both parents. They are AA, AS, SS, and AC. The healthiest genes among these four groups is “AA” closely followed by “AS”, and the abnormal genotypes (sickling cell) are “SS” and “AC”. 

Upon conducting a survey to determine how knowledgeable people are with regards to sickle cell disease, I noticed a common error amongst many individuals where oftentimes, the genotype is confused with the blood group. More staggering is the fact that many others even do not know what “genotype” is. It was indeed surprising to me that an issue of such great importance was little known or understood by many! This piece is to enlighten people and also debunk some myths surrounding this disorder. 

It is worth noting that the people I interviewed were between the ages of 15 years and early ’40s. A 16-year-old sickle cell male patient I interviewed, disclosed; 

“When my crisis comes it’s very painful and I constantly blame my parents for doing this to me, I don’t understand why my siblings do not experience that, there have been times I feel like committing suicide. And because of my situation, I constantly skip school due to the pains I experience often.”

One must consider these before any undaunted budge is taken; 

• An “AA” person can marry anybody. 
• An “AS” genotype person is better off settling with an “AA” person. 
• If your genotype is “AS” it is extremely risky to settle down with persons with the “AS” genotype. Also, settling with persons with the “AC” genotype is equally risky. 

Below is a chart of the various genotype matching, their compatibility ratings, and remarks.

It becomes menacing when two sickle cells marry as sickle cell disease is a very dangerous disorder. What’s more, it is not curable and is only manageable, requiring a lot of hospital stays. 

According to World Health Organization, the health burden of haemoglobin disorders can be effectively reduced through management and prevention programmes. 

Sickle cell disease comes about when particular groups from any of the pairs mentioned earlier sexually meet with the aim of bearing children. Many times, proclivity leads us on. This disorder occurs when sickle cell traits mate or when two sickling cell genes meet. 

Therefore, it is mostly advisable that people with sickle cell traits do not marry because the chances of genetically passing it on to their children are higher. 

Sickle cell disease has different types; “SS” which is the most common type occurs by taking an “S” gene from both parents which later forms “SS”, people with this type experience the worst symptoms mostly at a higher rate. Another type is “SC”, which is the second common type that occurs by inheriting a “C” and “S” gene from bothering parents. 

The disease comes with what is known as sickle cell “crisis” It may include; acute chest syndrome, chest pains, stroke, difficulty in breathing, anaemia, frequent infections, swelling of hands and feet, vision problems, delayed growth, or puberty among others.

The misconception and the notion associated with sickle cell disease as “witchcraft” must be curbed, it is just a disorder, and victims must not be stigmatized or shunned under any circumstance. 

In other to save yourself from going through any form of stress, halt on your love first and go to the nearest hospital, clinic, or certified laboratory center and check your genotype. 

Have knowledge of sickle cell disease? school a fellow and help demystify the disorder.

Writer: Christiana Aba Dadzie.