1What is sickle cell disorder?
Sickle cell disorder is an inherited genetic disorder that affects red blood cells. People with sickle cell disorder have red blood cells that become hard and pointed instead of soft and round. Sickle cells cause anemia, pain and many other problems.
2What is sickle cell trait?
If you have sickle cell trait, you have inherited the gene for sickle cell disease. Sickle cell trait does not turn into sickle cell disorder. If someone has sickle cell trait and his partner has sickle cell trait they may produce a child with sickle cell anaemia.
3What medical problems are caused by sickle cell disorder?
Lung tissue damage, pain episodes and stroke. The blockage of blood flow caused by sickled cells also causes damage to most organs including the spleen, kidneys and liver.
4Are people of Africa and African descent the only group affected?
No. It is also present in Portuguese, Spanish, French Corsicans, Sardinians, Sicilians, mainland Italians, Greeks, Turks and Cypriots. Sickle cell disease also appears in Middle Eastern countries and Asia.
5Is there a cure?
There is no universal cure for sickle cell disorder. Research in gene therapy, the ultimate universal cure, is currently underway. A relatively small number of patients with severe sickle cell disorder have been cured through stem cell transplant.
The stem cells (immature cells that develop into blood cells) come from bone marrow, or less frequently, from umbilical cord blood, usually donated by siblings who are a good genetic match. Most children with sickle cell disorder however do not have siblings who are good genetic matches. For this reason, researchers have recently begun performing stem cell transplants using umbilical cord blood from unrelated donors with apparent success.
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6What are some promising treatment developments?
The use of hydroxyurea has shown promising results on some adult sickle cell patients. It reduces the frequency of severe pain, acute chest syndrome and the need for blood transfusions.
7How many people have sickle cell disorder?
Sickle cell disease is a global health problem. In Nigeria one in every four persons are carriers of the sickle cell gene. It is estimated that over 150,000 babies are born with symptomatic sickle cell anaemia each year in the country.
8Can people with sickle cell disease live a productive life?
Yes. But like all patients with chronic disease, sickle cell patients are best managed in a comprehensive multi-disciplinary program of care and a strong extended support system.
9What are the chances that parents with sickle cell trait will pass it on to their children?
There is a 50 percent chance that a child born to parents who both carry a sickle cell gene will have the trait. There is a 25 percent chance that the child will have sickle cell disease. There also is a 25 percent chance that the child will have neither the trait nor the disease. These chances are the same in each pregnancy.
If only one parent has the trait and the other has no abnormal hemoglobin gene, there is no chance that their children will have sickle cell disease. However, there is a 50-50 chance of each child having the trait.
10Can a person catch sickle cell disorder from someone who has it?
No. The disease is inherited and is not contagious. To inherit the disease, a child must receive two sickle cell genes, one from each parent.
11Can a woman with sickle cell disorder have a safe pregnancy?
Yes. However, women with sickle cell disease are at increased risk of complications that can affect their health and that of their babies. During pregnancy, the disease may become more severe and pain episodes may occur more frequently. A pregnant woman with sickle cell disease is at increased risk of preterm and of having a low-birthweight baby. However, with early prenatal care and careful monitoring throughout pregnancy, women with sickle cell disease can have a healthy pregnancy. If the baby’s father has sickle cell trait, the baby has a 50 percent chance of having the disease. If he does not, the baby will have only the trait.
12Is there a test for sickle cell disorder or trait?
Yes. A person can have a blood test to find out if he has either sickle cell trait or a form of the disorder. There also are prenatal tests to find out if the baby will have the disorder or not. This procedure is available at our centre.
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13Is it possible for a child with sickle cell disorder to have stroke?
Yes, about 10% of children between the ages 2 to 16 years are at risk of stroke, but it is possible to determine the risk through a scan called Transcranial Doppler ultrasonography (TCD). This scan can be done at our centre.
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14Is it possible to test the genotype of a newborn?
Yes, it is possible and this is called Newborn Screening. It is hoped that the Federal Ministry of Health follows through with its plan for the establishment of Newborn Screening as a national programme.
