A couple of days back i was in my consulting room when a promising 14 year old girl was rushed in, taking a look at her, she was having fever, Yellow eyes, in Painful distress and had swollen abdomen. The Father Said she was A Sickle cell diseased patient (Sickler) and has been having several hospital admissions and blood transfusions every year. Before coming to our hospital the girl was already taking to two hospitals and with no improvement thats what necessitated presentation to our facility, the young lady was in a SCD Crises known as Sequestration Crisis. I couldn’t focus or slept that day cause all my attention was on how to see that the young girl made it, But after all efforts by the health workers, parents and the girl herself, atlast we lost her; Another Brilliant Fellow, who may have been a great treasure to the her parents and her immediate society. So I asked, Is the Risk Worth Taking.
Sickle cell Disease (SCD) is a disease in which an individual inherit from both parents if they parents are both carriers of the sickle cell trait. It is inherited in an autosomal Recessive pattern (Meaning before an individual can have SCD, both of the parents must have the sickle cell traits, or, one or both must be a sickler).
The SCD gives the Red Blood Cell (RBC) an abnormal shape, so the RBC struggle to pass through blood vessels and they blood cells are easily destroyed when exposed to stress, so the Sickler will always be in need of blood transfusion, most of the times Bone pains, catches infection easily and kill by simple infections easily than other ‘normal’ individual.
Every Year about 300,000 Babies are born with SCD with the highest population been in India, Nigeria and DR Congo. 10-30% of Nigerians have the Sickle Cell Traits (AS) While about 3-4% have the Sickle Cell Disease (SS). Half of the Children with SCD do not make it before their fifth Birthday and about 50-90% of the people with SS die from the disease and its complications.
A child will SCD will usually present with Fever, Severe Bone Pain, Chest pain, Swollen Abdomen, not growing like other ‘normal’ Children and lack of blood always. Infestations like malaria can easily kills a SS Child.
When Both Parents have the Sickle cell Traits (Both AS) the possibility of having a SS Child is 25% in each pregnancy. But this is just a probability, some couple have been lucky not to have a SS child, some unlucky to have SS Children all through and some Partly lucky to have a combination of SS, AS and AA Children.
Looking at the Physical, Psychological, Financial and Social Implication of SCD, I think the ‘risk’s is not worth taking. How will yoi feel as a parent giving birth to a child who since at the age of 1 is always sick, necessitating hospital admissions, receiving blood transfusion, Medications and in a long-run after all the stress you went through the child couldn’t make it after a couple of years.
You as the parents would have spent a lot of money that’s if you dont even go into Debth (Financial), You would have been physically exhausted, days you out to have gone to work, business or certain trips you will spend them in the hospital ( Physical stress and economic loss) and the psychological risk you will be under; thats you as the parent ooh, now imagine what the child himself goes through, the stress, Pains, School days loss and then finally died after all efforts.
The only cure to SCD is Stem Cell transplant which is excellently expensive and not readily available in the country.
Now before you married that handsome dude or that pretty damsel please endeavor to know Your genotype with that of your spouse. A genotype test doesn’t really cost much in Nigeria, an average of 2000 Naira will have the test done for you.
AA ( Normal Genotype)
AS ( Sickle Cell Traits or Sickle Cell Carrier)
SS ( Sickle Cell Disease or Sickler) Sickler is an informal word.
Please If Both of You have the Sickle Cell Traits please reconsider and dont make a selfish decision cause of yourself at the risk of an innocent child who would possibly pass through a lot of stress there after.
If You have married without that knowledge and you know noticed you and your partner are both AS after conducting the test, have your children screened and if any have the disease, he/she should be fully immunized, promptly treated for any crisis or infection.
The place of Faith is Vital, I believe in Miracle But if the Genotype can change let it change before marriage Cause many couple have come up with Faith Theories.. please and please make a wise decision for the future of your children Today. Get to a standard health Facility and have your Genotype Done today.
Dr. Jethro Musa Wunukhen