By: Sharon Zoe Williams
Thursday, June 19th, is observed globally as World Sickle Cell Awareness Day. The observation aims at increasing public knowledge and awareness about the disease’s cause, available treatments, cautions, and challenges faced by patients, their families, and carers, amongst others.
This year’s theme, ‘Hope through Progress: Advancing Care Globally’, aims at sensitising the public to the progress in research about measures as well as the cure for sickle cell disease (SCD).
In an interview on Uniiq Breakfast Drive with Prof. Fifi Ofori Acquah, President of SICKLE CELL FOUNDATION-GHANA and Director of the West African Genetic Medicine Centre (WAGMC) at the University of Ghana, he described SICKLE CELL DISEASE (SCD) as an inherited alteration in the DNA that results in the blockage of oxygen flow in the body due to what he called a ‘mistake’ in the mutation of blood vessels.
It is said that one in ten Ghanaians may be a patient zero. That is, a carrier of sickle cell disease. Studies have also shown that people may respond to and react to the effects of SCD differently from each other. While some, depending on the type of SCD and/or their genes, may show no signs, others from 3-6 months of birth begin to show signs. It is therefore significant to know one’s status as soon as possible to prevent unforeseen circumstances.
As part of its preventive measures, children whose status is known at an early stage are given penicillin, which helps curb their chances of getting easily infected by other diseases while managing the effects of SCD. Some treatment methods have also been discovered. They include gene therapy, which corrects the mistake in the mutation of blood vessels to the way they ought to be, and bone marrow transplants. This corrects your genotype, but not your reproductive organ system. Implying that, while patient zero might be cured, he/she still has about 25% capability of reproducing offspring with the disease if their partner is or was a Sickle Cell Disease Patient.
Whereas some might be AS, that is an SCD trait carrying one copy, others may be SA, meaning the A trait produces less than 50%, thus having the S as a dominant factor making it a more severe type as compared to AS. Sickle Cell Disease is known to affect nearly all organ systems of the patient zero. Therefore, no matter who you are, seeing that it is strictly hereditary, it is important that you get tested today. Knowing your status is a step in the direction of preventing the passing on of those genes to your children, managing, curing, or curbing its effects on you, and your loved ones.
