What is meant by sickle cell disease or SCD?
Sickle cell disease is a group of inherited and lifelong health conditions that affect the red blood cells. The most serious condition is called sickle cell anemia. It is common among African and Caribbean family background population. In the United States, it affects between 70,000 to 100,000 Americans.
Patients that suffer from sickle cell disease produce abnormal red blood cells that have a sickled shape, that’s why it is called sickle cell disease, the abnormal shape of the red blood cells make them unhealthy leading to different complications as a result of blood vessels blockage.
Symptoms of sickle cell disease range from mild to severe depending on different factors and based on the haplotype. The main symptoms of SCD include:
- Sickle cell crises painful know of Sickle cell crisis
- Increased risk of serious infections
- Anemia leads to shortness of breath and fatigue
Additionally, some patients may suffer from brain strokes, lung problems, and delayed growth. Some patients may need to start blood transfusion to correct the anemia and reduce the risk of brain strokes, especially if blood velocity in certain blood vessels exceeds a certain threshold. A non-invasive technique known as TCD is used to check the blood velocity, this technique is effective for patients below 18 years old and needs a skilled and well-trained radiologist.
SCD patients are always advised to
1. Drinking plenty of fluids and staying warm to prevent painful episodes
2. To take painkillers, such as paracetamol or ibuprofen
3. To take daily antibiotics like penicillin
4. To reduce symptoms through taking hydroxyurea which is a prescription medication
5. To correct hemoglobin levels through regular blood transfusions.
Some patients may get rid of SCD and achieve cured with bone marrow transplantation, but not all of them. Today there are a lot of researches that are investigating the role of gene therapy in managing SCD.
Recently, the FDA approved new medications for SCD that aims to reduce the frequency of vaso-occlusive crisis such as crizanlizumab, amino acid L-glutamine and voxelotor.
Despite being a complicated disease the future is very bright in our battle against SCD with the newly approved medication and ongoing trials.
