AN ARTICLE ON SICKLE CELL DISEASE AND MARRIAGE

            Jackie Houston was in her final year at Imo State University when her boyfriend, John Edward, proposed.  They began making plans to get married, but there was an unexpected pitfall.  The pastor of their church asked them if they were aware of each other’s genotypes.  That was a prerequisite for their wedding. Jackie and John, who had already been dating for over a year, then went to get tested.

John waited until after Jackie’s final exams before breaking the news to her.  Laboratory results showed that they were both carriers of the sickle cell gene.  They called off the wedding and broke up. “At first, I wanted to go ahead,” Jackie said, “but John told me to imagine how much more painful it would be if we lost a child to sickle cell disease in future.”

What then is Sickle Cell?

            Sickle Cell is an inherited gene blood disorder which occurs in eight out of every hundred thousand Africans. This Sickle Cell disease is said to be found in majority of the people from African descent most especially and other countries like; Sub-Saharan, South –America, Cuba, Central America, Saudi Arabia, Turkey, Greece and Italy.

What is Sickle Cell Disease about?

            Sickle Cell is a situation whereby the Red Blood Cells (RBC) which are normally disc-shaped, soft and flexible become crescent or sickle-shaped and hard causing the Red Blood Cells to be abnormally stiff and fragile and also the hemoglobin (which is the building block of Red Blood Cells) becomes abnormal.

            These normal Red Blood Cells that are soft and round move easily through the blood vessels but a patient with the elongated, stiff Red Blood Cells called Sickle Cell have a tendency of experiencing clump together and clogged up blood cells in the small blood vessels throughout the body which can break apart causing blockages in the vessels. This can lead to decreased blood flow resulting in oxygen deprivation which causes severe pain and damage to organs and tissues that need the oxygen to function properly.

            These abnormal Red Blood Cells are usually recognized by the body and are destroyed at an unusually high rate than they can be replaced, causing a shortage of Red Blood Cells called Anaemia especially under these conditions which also triggers the “Pain Crises” ;

-Fever      

-Dehydration  

-Infection

 -Drop in oxygen or change in air pressure (mostly in occurs in the airplane).

The only way to get infected with Sickle Cell disease is through the:

Genetic Factor: When one inherits two defective genes, which is ‘SS’ that causes Sickle Cell disease, from one’s parent. But, in a situation where one receives only one defective gene from a parent and one normal gene from another parent giving a child a genotype of ‘AS’, he/she is said to have a Sickle Cell trait and not the Sickle Cell Anaemia disease. Although the ‘AS’ patient won’t usually have the symptoms, he/she can pass the gene onto his/her children.

SYMPTOMS

The symptoms of Sickle Cell Anaemia may be noticed as early as four months of age or may go undetected until later at the age of one year. Also, when the abnormal Red Blood Cells block blood vessels, oxygen deprivation occurs causing severe pain episodes called “Pain Crises”. The location of this pain depends on the tissue or organ deprived of oxygen. Therefore, the symptoms Sickle Cell disease is;

-Fever 

-Pain in: chest, arms, joints, abdomen, legs and bones

-Enlarged organs like the heart, liver, spleen

-Increased risk of infection especially pneumonia

-Symptoms of Anaemia: severe fatigue, headache, dizziness, shortness of breath and heart   failure

-Yellowish tone to the whiteness of the eyes and skin (Jaundice).

-Episodes of Sickle Cell Crises like severe chest pain, in males, painful prolonged erection (called “Priapism”) which may result in impotence and severe abdominal pain.

-Medical Conditions like leg sores, gum disease, enlargement of the heart attack, bone infection and abnormal bone growth.

TREATMENT

There is no permanent treatment or cure to Sickle Cell disease but that the “Pain Crises can be reduced to minimal through the consumption of much fluid, that is, “WATER”.

           

Sickle Cell therefore, will always be a torment to the bearer and pain to his/her loved ones if not prevented. In our society therefore, it should be a thing one would avoid at all cost and also try to stop by all means possible starting from educating his/her family, friends and loved ones and thereafter, other means possible. Then and only then will it become classified as a thing of the past.  

                                                                                                                       

                                                                                              OBIDOA CHIAMAKA IFEOMA

                                                                                                            BY/13A/1038