1) What is sickle cell disease?
Sickle cell disease is an inherited disorder that affects red blood cells. People with sickle cell disease have red blood cells that become stiff and crescent shaped instead of soft and round. Sickle cells cause anaemia, pain and many other problems.
2) What is sickle cell trait?
A person who inherits the sickle cell gene from one parent and the normal type of that gene from the other parent is said to have sickle cell trait. One in 12 Nigerians in this country has sickle cell trait. Carriers of the sickle cell gene generally are as healthy as non-carriers. Sickle cell trait cannot change to become sickle cell disease. However, when two people with sickle cell trait have a child, their child may inherit two sickle cell genes and have the disorder.
3) If sickle cell trait is not an illness, why are people tested?
Babies are tested to see if they have sickle cell disease. Teens and adults are tested mainly to see if they can have a baby with the disease.
4) What 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 haemoglobin 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.
5) What medical problems are caused by sickle cell disease?
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.
6) How is sickle cell disease treated?
Most individuals with sickle cell disease are generally well from day-to-day. However, babies and young children with sickle cell disease may take oral penicillin begining as soon as sickle cell anemia is diagnosed and continuing until at least 5 years of age. This penicillin can prevent fatal pneumococcal infection. If complications of the sickle cell disease occur, patients are treated and supported until they get better. For example, painful episodes are treated with pain medications and fluids. Blood transfusions are used to reduce some of the complications of sickle cell disease. Transfusions correct anaemia by increasing the number of normal red blood cells in circulation.
Problems from sickle cell disease can also be prevented. For example, regular (monthly) blood transfusions can prevent strokes in children as well as prevent pain and other complications of the disease. The medicine hydroxyurea can also lower the frequency of painful episodes and of acute chest syndrome. Patients taking this drug also needed fewer blood transfusions.
Stem cell (bone marrow) transplantation can cure children with sickle cell disease. However, this procedure is not without risk, and the transplant is safest when, the bone marrow comes from a healthy sibling donor (a brother or sister with the same mother and father) with the same tissue or bone marrow type. Only about 18 percent of children with sickle cell anemia are likely to have a matched sibling donor.
7) How can I be tested?
A simple, painless blood test called the haemoglobin electrophoresis can be done by your doctor or local sickle cell foundation.
8 ) When should I be tested?
Get tested now for sickle cell! If you are of child bearing age, you and your partner should be tested immediately so that you can make informed family planning decision.
9) How can I help?
If you do not have this disease you can help those who battle this disease by:
By donating blood to your local red cross, general hospitals or sickle cell foundations, because SCD patients often need blood transfusion to alleviate the painful crisis episodes.
Support local and National efforts to increase awareness about SCD Being an advocate.
For more information about sickle cell, visit the sickle cell Disease of America website on www.sicklecelldisease.org