Nigeria has the highest burden of sickle-cell disease with over 150,000 children born annually with sickle-cell anaemia.
I will start this piece with the account of a Nigerian, Mrs. Amaechi Ezebuike, who wrote a book entitled Coping with sickle cell — A mother’s own experience, narrating her ordeal. She concludes that a man and woman who are both sickle cell carriers simply should not get married. She agrees that compulsory genotype testing could help eradicate the disease.
Her story: At the age of 22, she left Nigeria for Germany to be the wife of a medical student whom she had never met. The people of the young man’s home village, Amucha, had raised the funds to send him abroad to study. When the time was right, they felt it was also their responsibility to send him a wife, which they did.
Mrs. Ezebuike and her husband had six children, but their two sons and one of their daughters eventually died of sickle cell anaemia. The couple had no idea that they were sitting on the keg of gun powder until their children started falling ill. It was then they learnt that either of them were carriers of the sickle cell gene.
She wrote: “My third daughter, Nneka, was two years old when she started running a very high fever which my husband, a seasoned medical doctor, couldn’t control.
“After a laboratory genotype test result, it became clear to us. We then screened all our children and found out that two more were SS. We were devastated.”
She further said that couples should bear in mind that it is not all about them and that kids from such union will blame all the excruciating pain they’ll go through on their parents.
Couples who are planning to have a baby can get carrier testing (genotype) at medical centres , laboratories and sickle cell treatment facilities. A genetic counselor can refer a couple for testing and discuss the risks their union poses to their offspring.
Sickle cell disease is a disorder that affects the red blood cells, which use a protein called haemoglobin to transport oxygen from the lungs to the rest of the body. Normally, red blood cells are round and flexible so they can travel freely through the narrow blood vessels.
Patients with sickle cell disease have a mutation in a gene. As a result, haemoglobin molecules don’t form properly, causing red blood cells to be rigid and have a concave shape (sickle shape), hence the name ‘sickle cell disease.’
Sickle cell disease is inherited in an autosomal recessive pattern. This means that a child will not inherit the disease unless both parents pass down a defective copy of the gene. People who inherit one good copy of the gene and one defective copy are carriers.
To determine one’s genotype, a blood sample can be used in a test called haemoglobin electrophoresis. This test will determine whether a person has sickle cell disease — SS, SC, CC, or S/β. It will also determine whether a person is a carrier of the faulty haemoglobin gene AS, AC; or confirm those who are AA (normal haemoglobin gene). The AS and AC are carriers and are not in any danger.
To eradicate the incidence of sickle cell anaemia, intending couples who are carriers are counselled on the need to marry those who are of genotype AA because of the great risk to their offspring.
Symptoms
Sickle cell disease prevents oxygen from reaching the spleen, liver, kidneys, lungs, heart, or other organs, causing a lot of damage to these organs. The red blood cells of patients with sickle cell disease don’t live as long as healthy red blood cells. As a result, people with this disorder often have low red blood cell counts (anaemia).
Childhood symptoms
These include acute and chronic pain in any body part, bone pain crises, infection, delayed growth, strokes, jaundice because of liver damage, growth retardation, delayed sexual maturation, being underweight, hand and foot syndrome (bilateral painful and swollen hands and/or feet).
Others are acute chest syndrome, which include chest pain, fever, cough, stroke, enlargement of the heart, cholelithiasis (gall stone), priapism, and leg ulcers.
People with this disorder are likely to have their life span reduced by 30 years or more because of all these complications.
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