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Management of Sickle Cell Disease
Date Posted: 10/Oct/2019
The Anambra State House of Assembly recently outlawed marriages involving people living with sickle cell anaemia. The bill, cited as Sickle Cell Disease Control and Eradication Repeal, sought to eradicate sickle cell anaemia in Anambra State and set those living with it free from all kinds of oppression, abuse and exploitation. The law barred parents, guardians or group of persons from giving out their children/wards in marriage without verification of a qualified sickle cell disease prevention certificate. It also provided that no religious body or marriage registry “shall perform solemnisation of Holy Matrimony” without first demanding and obtaining a sickle cell disease prevention certificate from intending couples.
 
Nigeria has the highest burden of sickle cell disease with over 150,000 children born annually with sickle cell anaemia.
sickle cell disease
I will start this piece with the account of a Nigerian, Pastor Ibekwe, the father of  Angela Dickson-Ibekwe, who died as an undergraduate in one of the foremost  private universities in Ogun State as a result of complications from sickle cell disease.  He later founded Dabma Sickle Cell Foundation. He has set up this foundation to support indigent sicklers with treatment, medical supports and educational support for higher institutions.
 
According to him, the vision is to see that all persons with sickle cell disorder have access to basic medical treatment free of charge. Campaign warn strongly against ignorance and educating the populace of the dreadful consequence as they intend to give scholarship to brilliant but indigent sicklers who secure admissions into institutions of higher learning, adopting sickle cell orphans.
 
 He also wrote a book titled, ‘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 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.
 
Mrs Ezebuike and her husband had six children, but their two sons and one of their daughters eventually died of sickle cell anaemia.
 
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 others had SS genotype. We were devastated.”
 
Couples who are planning to have a baby can get carrier testing (genotype) at medical centres, laboratories and sickle cell treatment facilities. A genetic counsellor can refer a couple for testing and discuss the risks their union poses 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 don’t 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 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 if these complications are not well managed

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