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
