Sickle Cell Disease

Hi friends! Howdy! Today, I would like to discuss a very important disease which cuts across all generations especially in Africa. It is a medical as well as a social and economic problem which is better addressed so that we will not make the same mistakes our parents made. Now, sit back, relax and flow with me as we try to shed a little light on this dreaded disease, Sickle Cell Disease. I promise to write in plain English as much as possible. Thanks.

Mrs B looked at her helpless son groaning in pains. Nothing seems to be working. All the analgesics seem to be like ordinary water. Even this new drug which the doctors say is still under study with great potentials did nothing to help her boy. Tears rolled down her cheeks and as she fumbled for her already tear soaked handkerchief, she wondered if her decisions were worth it now.

   She was a very beautiful lady in her early 30s when she met the gorgeous Mr B. he had a good set of white teeth and English rolled out of his tongue as though he was an English prince. She must have fallen for him there and then because when he asked her for a date moments later, she could not say no. And that was how it all started.

   Finally all her prayers were answered in this man except for just one thing. As she was already advanced in age with no possible suitors looking her way again, she had better "borrow herself some brain". This man's genotype was AS. Hers too was AS. There was a good chance she could have a child with SS. But she rejected it in Jesus name. There was only one chance in four that she would have a sickle celled child or so she was told. She would fast very well and pray that such would not be her portion. Amen.

  How mistaken she was. She had three (3 ) children. She had already lost one child due to sickle cell. He was very sick and died suddenly. Now she was losing the second son. Her husband had already left her after the death of the first son. Now she was alone to deal with all these emotionally, physically, financially and mentally.

  The sudden calmness of her surroundings juggled her back to the present only for her to discover that her son was lying still in bed, quiet. 'Doctoooorrrr!!!!!'

SICKLE CELL DISEASE

This is a hereditary blood disorder characterized by red blood cells that assume an abnormal, rigid,sickle shape unlike the normal red blood cells which are discoid in shape. Sickling decreases the cells' flexibility and results in a risk of various complications. In other words, the sickled cells are prone to destruction. The sickling occurs because of a mutation in the hemoglobin gene. This leads to a shortened life expectancy. In 1994, in the US, the average life expectancy of persons with this condition was estimated to be 42 years in males and 48 years in females, but today, thanks to better management of the disease, patients can live into their 70s or beyond.

Sickle-cell disease occurs more commonly among the African population where malaria is common. In malaria endemic areas, carrying a single sickle-cell gene confers a form of immunity i.e a person with genotype HbAS has a level of immunity against malaria.

The term disease is applied because the inherited abnormality causes a pathological condition that can lead to death and severe complications. 

Inheritance of the sickle cell gene is as a result of both parents being carriers of the sickle cell genes i.e both parents have HbAS. These two combine together to give HbSS. A lot of myths surround the sickle cell inheritance, one of which is that if two people with HbAS decide to marry, they have a chance of having only one HbSS child out of four children. This is a blatant lie. The probability of having HbSS child is 25% in every pregnancy.

Note

SCT- sickle cell trait ( carrier)

So every time the mother carries a pregnancy she has a quarter chance of the child being sickle celled and only a quarter chance of having a normal child.

I will seize this opportunity to implore you all to know your genotype and inform many more about it. I would like to let you know that if you are HbAS and you are in a relationship with someone who is also HbAS , it may be better for you to call it quits.

 The story above may have been like a worst case scenario. Maybe. Maybe not. There are cases not as bad and there are some much worse than that. So it will be better to save yourself from all that may be.

I will continue with this topic later to discuss the different types of sickle cell traits, the manifestations, complications and management.

Thank you very much.

Love you lots.