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Management of sickle cell disease

I welcome the esteemed readers of this column to the month of June; June 19 is world Sickle Cell Day. It is a day for the public to reflect on the effect of the disease on the lives and education of children, teenagers and adults.

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, Pastor Ibekwe, the father of Angela Chidima Dickson-Ibekwe, an Economics student at one of the private universities in Ogun State, who died as a result of complications from sickle-cell disease.

He founded Dabma Sickle Cell Foundation. He set up this foundation to support indigent sicklers with medical treatment and educational support.

According to him, the vision is to see that all persons with sickle cell disorder have access to basic medical treatment free of charge. It also educates the populace of the consequence of the disorder. People living with the disorder are encouraged to register on to benefit maximally from the initiative.

He also wrote a book, Cruel Wounds of Sickle Cell Anaemia, a family experience that will help to encourage families with the disorder and open the hearts of couples who are carriers and are still contemplating marriage.

Another parent, a mother, Mrs. Amaechi Ezebuike, also in a book titled Coping with Sickle Cell — A Mother’s Own Experience, narrates her ordeal. She concludes that a man and woman who are sickle cell carriers simply should not marry. She agrees that compulsory genotype testing could help eradicate the disease.

Ezebuike and her husband had six children, but their two sons and one of their daughters eventually died of sickle cell anaemia. She writes, “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.”

In the book, she also advises couples, who are planning to have a baby to get carrier testing (genotype) at medical centres, laboratories and sickle cell treatment facilities.

According to her, a genetic counsellor can refer a couple for testing and discuss the risks their union pose to their offspring.

Sickle cell disease is an inherited 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 do not form properly, causing red blood cells to be rigid and have a concave shape (sickle shape), hence the name ‘sickle cell disease.’

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 do not 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 these complication.

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