Facts about Sickle Cell Disorder and how to prevent it!

Sickle cell disorder is an inherited disorder from the parents and the fundamental problem is in the blood, specifically in the red blood cell. There is a substance in the red blood cells called Hemoglobin which carries oxygen. People who do have sickle cell disorder diseases have a tendency for their red

blood cell to take a different shape from the normal round shape and becomes 'sickled'.

The cell has the tendency to change shape when there is a stress to the system like an infection and what happens is that it becomes rigid and they don't live long. Normal red blood cell would last about 130 days in the blood, whereas if a person has sickle cell, this sickle red blood cells don't last more than 10 to 20 days and that is why there is a shortage of blood.

The fundamental problem with the condition is in that cell and that's why there is anemia sometimes referred to as sickle cell anemia and it refers to those who have the sickle cell homozygous (when both genes are present in that child).

In a nutshell, sickle cell disorder is inherited, it is not infectious, it is not umbilical, although people still believe sickle cell is umbilical and that speaks to the pain that they tend to have because pains tends to be the whole mark of this condition. So that set the stage to say that sickle cell condition is inherited, it is not an infection- you don't catch it.

Gone are the days when people say sickle cell was a death sentence. So much has happened in the past in the few decades where they live longer with good treatment. However, with early diagnosis and good treatment and proper management, they can live longer healthy life.

Complications of sickle cell disorder

The condition has to do with the blood, the whole system in the body would get infected, in many ways they tend to have damaged organs because that cell that has that abnormal doesn't have the capability to move smoothly and freely through narrow blood vessel, so there is a tendency to clock to block blood vessel, to gather and block blood vessels so that blood does not get to any organs and what happens is lack of oxygen and when that happens their body begins to have some problem.

The complications arising from sickle cell condition can be acute, they can be chronic. The individual presents with pain in the bones, in the joints and acute chest syndrome - severely devastating which causes a serious problem and it accounts for 20% deaths recorded.

Acute syndrome, a situation where the patient will present to you with fever, chest pain, cough, difficulty in breathing which is a very serious case, of course they have what we call stroke, some of them have weakness of the limbs and before you know what is going on, they collapse and die.

In acute chest syndrome, they can have fever, infection, especially when they are still young. Infection on its own is a complication of sickle cell anemia and is a problem. If its not managed well from when the patient is given birth to, probably five years and you don't put them on vaccination, penicillin,  prophylaxis, from time to time they will be having all sorts of infection like pneumonia and so on.

Recommendations for sickle cell disorder

In countries like UK where they don't have one tenth as much as sickle cell condition in Nigeria as Nigeria has been counted the highest country with sickle cell disorder. They have what we call new born screening, so at birth, parents know at once that their child has sickle cell and so they institute a treatment measure that prevent those condition that kill them.

The National Advise Committee of the Sickle cell Foundation in Nigeria has advised and recommended a new born screening. Because when babies are born, you can't tell the difference, you can't tell which child has sickle cell anemia and the child from childhood who does not have sickle cell anemia, they only begin to manifest symptoms and signs when they get to about 6 to 9 months.

When the child is in the mother's womb, there is another kind of hemoglobin that is just as efficient as the usual hemoglobin that everybody has and for them, it last for a period of time, it begins to wane though as they begin to grow older, so by the time they get to 6 to 9 months, it has waned enough for the symptoms to begin to show up.