It is almost time for World Sickle Cell Day which is on the 19th June, 2013. A day dedicated to all the sickle cell warriors/survivors, their parents, friends, siblings, relatives, their doctors and nurses, offspring, friends of friends, sisters/brothers of friends of friends ...the circle of life to be brief. You may wonder how or why it affects such a wide range of people, the answer is simple; Sickle cell is one of the most common diseases of the world. According to the World Health Organization, in some regions of the world (Africa), up to 25% of people are healthy carriers of the sickle gene.
Before I get into a lot of confusing details, I want us to first know that Sickle Cell Disease(SCD) is an inherited blood disorder passed from BOTH parents to their offspring. This means that each parent has to be a carrier of the sickle gene. We also need to know that sickle cell disease comes in various forms which include:
-Sickle cell trait (HbAS)
-Sickle cell anemia (HbSS)
-Sickle cell with hemoglobin C disease (HbSC)
-Compound heterozygotes which include HbSO, HbSE and HbSD.
Lets not forget the normal genes; HbAA. It may come in handy much later. *bright smile*.
My write up is mostly going to be referring to the sickle cell trait (HbAS) and especially sickle cell anemia (HbSS). Generally and under normal circumstances, HbAS people do not exhibit symptoms of sickle cell disease and are therefore known or called carriers. That's right because they have one normal gene (A) and (carry) one sickle gene (S). On the other hand, HbSS or sickle cell anemia(SCA) being the most common form of SCD and also the most severe form has a wide variety of symptoms exhibited by the patients or sufferers from this disease.
Some of the symptoms include:
-severe chronic anemia (hence the name)
-pain crisis or sickle crisis (vaso occlusive crisis)
-priapism (painful erection of the penis)
-acute chest syndrome
-bacterial infection due to low immunity
-and leg ulcers among other things.
Some SCA patients have delayed growth, pronounced forehead (like mine) *covers face*, dactylitis (inflammation of the finger or toe) ..... to be honest naming all the symptoms and complications of SCA will make this write up boring to read. I will however in following write ups try as much as I can to break them down for my very eager readers and friends that have asked me on numerous occasions to explain SCA to them.
Then again, before all these things occur, I wonder if we remember the part I mentioned "SCD (including SCA) is an inherited blood disorder"? How does it occur? How does one get it from both parents?
The blood. I don't know if it should be heading or not but that is where the whole story lies, from the beginning to the end.
The blood is a bodily fluid which transports nutrients and oxygen to the entire body. the human blood is made of many components; erythrocytes or red blood cells (RBCs), leukocytes or white blood cells (WBCs), platelets, eosinophils, blah, blah, blah .... our main business are the erythrocytes (RBCs).