E20: See Why Couple Must Have HB Electrophoresis Test Before Settling For Kids.
The journey to marriage is a series of steps with vital and delicate decision making. Of all those, I am impressed when the to-be- couples final decide to walk into the hospital laboratory for some tests. Love can a crazy and blinding thing but has not stopped some sober minded love birds from thinking about who they will settle with health wise. HIV is a common test run even of self-level and some even do pregnancy test before they settle off. Other tests include Hepatitis and other STIs. My keen interest is sickle cell disease because it is inherited and you can be actually a carrier without knowing. You have a probability of passing that to your child. I will put this clear that it’s not marginalizing the affected but creating a factual awareness as we honor this day.
Most level 3 and 4 hospitals run the 2% sodium metabisulphite test for sickle cell screening. It’s good in a primary setting but may be subjected to pre analytical and analytical errors associated. Hemoglobin electrophoresis a laboratory hematological test that’s the most preferred for such. This technique works on principle that different forms of hemoglobin have different electrical charge which appear as different color bands when subjected to electrical charge of electrophoresis chamber. This is able to be analyzed and shows a wide spectrum of your hemoglobin abnormalities or disorders. This includes sickle cell disease, thalassemia and HbC disease. The first two are genetic, giving a keen interest of possible inheritance.
What Is Hemoglobin and Its Electrophoresis
Hemoglobin is an erythrocyte hemeprotein consisting of chains which are associated with heme prosthetic group, a tetrapyrride ring having central ferrous atom. This atom binds reversibly to oxygen molecule. Hemoglobin playing a big role in oxygen transportation in our bodies.
Sickle cell disease is the first molecular disease to be identified (Pauling et al 1949). Frank B. Livingstone and Antony C. Allison studied sickle cell and its distribution in period 1950-1980. Up to now, Africa has the highest burden of sickle cell disease while Arabian Peninsula has the highest variety in terms of sickle cell disease genotype and phenotype. Sickle cell anemia topics has featured into my AfyaStoryline Weekly Edition blogs for health, lifestyle and mental awareness and sensitization.
Sickle cell anemia is a primary feature of a sickle cell disease. This disease etiology arises when there is a change of a single beta-globulin gene of hemoglobin causing amino acid substitution in normal hemoglobulin (HbA) protein from glutamic acid to valine at chromosome 11. This results to production of abnormal hemoglobin (HbS). This leads to sickled shape red blood cells. This means that the cells will carry less oxygen and the body tissues and organs will receive less oxygen. This explains the reason why most patients battle jaundice, paleness, fatigue, and sickle cell crisis.
Having that in mind, it’s basic for me to put it straight that the decision is yours. We can actually pass the disease to our children, or probably our parents have already done that. Understand this that a sickle cell carrier person looks normal, though he/she is carrying those genes. In this case, his/her genotype is heterozygous where the normal gene is dominant while sickle cell is recessive. For those with sickle cell disease, they have homozygous genotype consisting of abnormal gene dominating.
Probability Of Having Sickle Cell Disease
This is the simplified probability I have been working on to illustrate the genetics of sickle cell anemia disease. If all the parents are normal i.e. lack any trace of the disease, they will inarguably sire normal children. If the father is a carrier while the mother is normal, they will sire 50% normal children and 50% sickle cell carrier children. This is the same when the mother is the carrier while the father is normal. If the father is a sickle cell diseased while the mother is normal, they will sire 50% carrier daughters and 50% carrier sons. This is same outcome if the mother is sickled while the father is normal. If sickled husband marries a carrier wife, they will sire 25% sickled daughters, 25% sicked sons, 25% carrier sons and 25% carrier daughters. Lastly if both parents are sickled, they sire all sickled children.
How Lucky Are Sickle Cell Patients In relation To Malaria
Since 2020, I have been promoting, creating awareness and reaching out to people through health, lifestyle and mental wellness. This is through my health care and initiative called AfyaFitness20. Finally let me put across that having a sickled child should not be an odd. They are mysteriously blessed much than the normal one when it comes to malaria attack especially those in prone areas. These are some of the features that make the phenomenal: reduced cytoadherence, decreased rossete formation, impaired growth of Plasmodium falciparum and enhanced removal of parasitized red cells. However much the excruciating pains, financial-medical demands and those emergencies that face the affected, lets embrace them as we celebrate this day.
I am Isaac Ogutu – a 3rd year Medical Student at Kenyatta University. I am the founder of AfyaFitness20 – a healthcare initiative for health, lifestyle and mental wellness.