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To understand what sickle cell disease is, first we have to understand a bit about our blood cells composition.
There are different types of blood cells. Some of these are red blood cells.
These are the cells that help with the delivery of oxygen throughout the body. In healthy individuals, these cells maintain a round shape that is flexible enough to move easily throughout all the blood vessels’ intricate network.
In sickle cell disease, the red blood cells cannot maintain their round and flexible shape. Instead, they have a tendency to lose this shape under certain conditions. They collapse and assume the shape of a sickle or a crescent moon. When this happens regularly, these cells become inflexible and cannot move easily through blood vessels.
Sickle red blood cells usually stagnate in the blood capillaries. When this happens, blood supply to certain areas of the body (or parts of an organ) is greatly impaired. This leads to some tissues being starved of oxygen. This can lead to small parts of the starved areas dying due to lack of oxygen.
This state can lead to increased physiological stress, which can further lead to more cells
sickling thereby setting a vicious cycle of events in motion.
Signs and symptoms of sickle cell disease can vary in presentation and severity in different people. Some people will remain with no symptoms for many years.
Even in the same individual, these symptoms can vary drastically from one episode to another.
Sickle cell disease is inherited. This means that the disorder is transmitted from parent to child. This occurs when an abnormal change happens in the gene which regulates the making of haemoglobin part of the red blood cells.
In sickle cell, the haemoglobin is abnormal and does not support the integrity of the shape of a normal cell. This makes the cell to collapse when under certain stressful situations. This includes the normal physiological stresses of the body. For a person to get sickle cell disease, he must inherit two defective haemoglobin genes from both parents.
If both parents have sickle cell disease, then all their children will inherit the condition.
Both parents may be what is referred to as sickle cell trait individuals. Here, they have both normal and abnormal haemoglobin genes. They are usually without symptoms but they can pass the defective genes to their children who will develop sickle cell disease. If the child receives one normal and one abnormal haemoglobin gene, then they become sickle cell trait victims. They will remain without signs and symptoms but with the risk of passing the defective gene to their children.
The above medical genotype diagram illustrates what can happen where both parents have sickle cell traits. That is, they have a pair of both normal and abnormal haemoglobin genotype. when they have children, chances are:
Sickle cell disease is a serious condition. In our follow up article we will be discussing how to live with the sickle cell disease. There has been a lot of medical advances which have made sickle cell a manageable condition.
If you have any concerns we always advise to contact your healthcare medical service provider.
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