Sickle Cell Disease (SCD), also known as sickle cell anaemia, is a hereditary blood disease, which is transmitted by genes.
SCD is a genetic disease that is suffered if two abnormal haemoglobin genes are inherited, one from each parent.People with SCD have a problem with the gene that produces hemoglobin.
Hemoglobin is a protein present in red blood cells, the blood cells responsible for transporting oxygen from the lungs to every part of the body.
In healthy red blood cells, hemoglobin causes these cells to be flat, round and flexible. In contrast, in a person with SCD, abnormal hemoglobin, also called sickled haemoglobin, causes red blood cells to have a curved and rigid shape (sickle-shaped).
Sickle red blood cells can appear in the blood vessels, slow down or block blood flow to different parts of the body, possibly causing pain and other health conditions.
A person inherits the sickle cell trait or SCD in the same way as he or she inherits the colour of his or her hair or eyes, through the genes they receive from their father and mother.
A person is considered to have the sickle cell trait when they inherit a gene that produces normal haemoglobin and another gene that produces sickle haemoglobin. People with the sickle cell trait generally have no symptoms and, in most cases, they are healthy. However, they can transmit the altered gene to their children.
A person with SCD inherits an abnormal haemoglobin gene from each of their parents.
A blood test may show whether SCD or the sickle cell trait has been inherited. Diagnostic confirmation is performed using various methods.
The detection of SCD in new-borns helps to best manage the disease from its early diagnosis.
In some countries such as Spain, families are also informed if it is detected that the baby is a healthy carrier of the sickle cell trait so that they can receive general recommendations and genetic counselling, which is very important when planning a pregnancy.
If a neonatal screening test has not been performed, people with SCD are generally diagnosed when they have a symptom or disease-related complication.
Each person has two genes for haemoglobin, one received from the father and another from the mother.
Figure 1 (above) – When one of the parents has a sickle cell trait, i.e., they have the gene but do not have the disease, and the other has he disease, the following scenario plays out (as illustrated in the diagram):
In every pregnancy, there is a:
This is likely the same regardless of whether it is the father or the mother who has the disease.
Figure 2 (above) illustrates the following: In every pregnancy, there is a:
Genetic compatibility checking is very important. If the father and mother suffer from the disease, all their children will inherit the disease. If you are planning on having children, take advantage of the LifeHealth Registry
The signs of SCD appear during the first year of a child’s life, at approximately 5 months of age.
Symptoms of SCD are different for each person. Most are related to the complications of the disease.
The sickle red blood cells are destroyed faster than normal. The body may have problems creating enough new cells and replacing those that are lost, causing what is known as anaemia. Anaemia results in a state of fatigue, irritability, extreme tiredness and difficulty breathing.
As they are sickle-shaped, sickle red blood cells cannot flow freely through the blood vessels. As a result, oxygen does not reach all body tissues and organs, which can cause different types of problems such as:
Blockage of blood flow may cause severe pain, called pain episodes or vaso-occlusive crises, one of the most common complications associated with SCD.
The severity, duration and frequency of vaso-occlusive crises varies depending on the person.
You may feel pain in any part of the body and possibly in more than one place at the same time. It often occurs in the:
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