Sickle cell disease - Myths and facts
You may have heard about the Sickle Cell Disease (SCD) or probably have a friend or family member who has the disease. But what do you know about it?
Let me explain in simple terms what the SCD is. Health experts say it is a blood disorder that a child is born with.
Advertisement
It is passed down through both parents’ genes. Children with the SCD make an abnormal type of haemoglobin.
The haemoglobin is the protein in red blood cells which carries oxygen to all parts of the body. With the SCD, the body organs and tissues do not get enough oxygen.
Healthy red blood cells with normal haemoglobin are round, flexible and move easily through blood vessels.
Sickle cell disease turns the red blood cells into the shape of a banana or the letter “C” (and like a farm tool called a sickle). They also become stiff and sticky.
These misshapen red blood cells (sickle cells) clump together under certain conditions. As a result, they are unable to move easily through the blood vessels.
They get stuck in small blood vessels and block blood flow. This blockage can cause pain. It can also damage major organs.
There are several complex types of the SCD. Some do not cause symptoms or severe problems but others do.
The World Sickle Cell Day was celebrated on June 19 to raise awareness of the disease. One major challenge with the SCD is the myth and misconceptions surrounding it.
Below, a Clinical Paediatric Haematology/Oncologist at the Presbyterian Hospital, Agogo, Dr Lawrence Osei-Tutu, clears the air about some misconceptions surrounding the disease.
Persons with sickle cell are “sicklers”
Some people unpleasantly describe persons with sickle cell as sicklers. They are not and are able to live healthy lives so long as they are well supported by family, friends and health experts.
It is important that you do not discriminate by tagging people with their disease. Dr Osei-Tutu advises that we support people with sickle cell to live a normal life. “He or she has sickle cell. He or she is not a sickler”.
Sickle cell is caused by curse or witchcraft
Sickle cell is not caused by curses or witchcraft. Dr Osei-Tutu explains that it is a well-known fact that sickle cell is a genetic disorder due to the inheritance of abnormal genes from both parents, resulting in rigid, sickle-shaped red blood cells.
You will die by age 18 if you have sickle cell
No. Sickle cell is not a condemnation to death at an early age. Currently, several interventions exist to support persons with sickle cell to live a long, fulfilled life.
Dr Osei-Tutu advises that parents with children with sickle cell disease should find a comprehensive care centre to learn more and take advantage of these interventions.
Once born with sickle cell, you will have eternal pain
Sickle cell does not mean you will have to live with pain, anaemia and frequent hospitalisations. Dr Osei-Tutu explains that hydroxyurea therapy for sickle cell is now accessible in Ghana.
The therapy reduces bone pain episodes and life-threatening chest pain episodes (called acute chest syndrome); need for blood transfusions and frequent hospitalisations.
Excessive oil consumption is responsible for sickle cell
Sickle cell is not a result of excessive oil consumption by mothers during pregnancy. Sickle cell is NOT an acquired disease; it is genetic.
Each parent contributes one copy each (one from the father and the other from the mother) of the pair of abnormal genes that cause sickle cell.
Persons with sickle cell should avoid certain foods
There are no food taboos for persons with sickle cell. They can eat all foods just as those without sickle cell. Both children and adults with sickle cell should eat at least three-square meals.
The meals should be balanced and must include fruits and vegetables. And, if you have sickle cell, remember to drink lots of water to stay well hydrated.
Persons with sickle cell cannot have malaria
We know from scientists that carriers of one “S” gene (those with “AS”) do not usually experience the severe form of malaria when they suffer from it. Children with sickle cell disease (“SS” type) can get malaria.
Malaria can cause them to be anaemic and experience pain. Children with sickle cell must sleep under long lasting insecticide treated mosquito nets. Your medical team may also give you some malaria medicine weekly or monthly.
Normal red blood cell Sickle Cell