Sickle Cell Anemia is a disease that affects the red blood cells. Anemia means: a problem with the blood cells. And sickle cell is one disease that makes the blood cells look like sickles or crescent.
A normal blood cell would look a bit like a donut. It’s circular in shape and has three very important features. It has iron on it that binds temporarily to oxygen, until it reaches the tissues of the body and then releases the oxygen. Number 2 is that it lives up to ninety days, three months, and then it brakes down and the body remakes new cells. And then also it’s very flexible. So, if it goes into the small capillaries, the small blood vessels, it can bend itself to fit into the very tiny blood vessels.
On the other hand, a patient with sickle cell anemia, his cells look like this, like crescents. They have three main defects. The iron on it cannot bind so well to oxygen, so the oxygen will remain in the blood without being able to get into the tissues. That’s one thing that causes the tissues of the body to lack oxygen in the patient. Number 2 is that it only lives ten to twenty days. So, a long period of time when the patient’s body is missing red blood cells. Number 3, the third and very important problem, is that the red blood cells that are normal can go into the big blood vessels easily, and then bend themselves to go through the tiny blood vessels. They’re very flexible. No let’s see what happens with the sickle cells. The sickle cells go easily through the big ones, big blood vessels, and then get stuck at the edge of the small blood vessels. They cannot get inside. They’re not flexible enough. And then they cause clumping here, and then in the end this organ will lack the blood supply, whatever it is. It can be the brain or hand or the penis or any tissue that is unlucky to have this attack of shortage of blood supply.
And as a result you’ll have attacks of hypoxia, which means lack of oxygen arriving to the tissues, and this can cause severe pain, sometimes death of the tissue or at least an infection occurring there.
So, the sickle cell patient mainly has anemia problems, because he’s lacking always red blood cell functions, so he’s always tired, having short breath, headaches, is always pale, sometimes he’s yellow skinned, because the sickle cells, when they die, the breakdown material is yellow in color. And then another thing is that he’s suffering from pain all the time, because these attacks are happening, so the cells are getting stuck at the edge of the tiny blood vessels. So you’re always expecting the patient to end up having either an attack of pain or an attack of being very tired and not able to function normally. Let alone other diseases that can occur, several infections in the lung can occur. So, the patient is continuously in need of medical care and checkup and prevention of certain diseases.
Another problem is the delayed growth of young people, because of the lack of blood supply to their organs. So, a sickle cell patient unfortunately is in need of continuous medical care to help the growth and prevent complications.
Currently there’s no permanent treatment of sickle cell anemia, but we try to give medications to prevent medication and also vaccinations to help the patient overcome, or not catch, infections all the time. There’s new lines of gene therapy and bone marrow transplantations, but it’s very important to also screen before marriage, do premarital screening to expect the outcome of the marriage and explain that to the parents. So, health education is very important. We also need to diagnose early if the child will be having the disease, so we can take a sample from the amniotic fluid to find out whether the mother is carrying a child with the disease.
In the treatment of sickle cell anemia we are highly depending on the health education of the parents and the caregivers of the child, so that we would prevent complications. So, early diagnosis, proper treatment and prevention will help the child grow up in a healthy way and we will help them have as much normal life as possible. So, don’t forget health education is the most important thing you should have if you find out that your child is carrying the disease.
I wish you the best of health!