Sickle cell disease (SCD) is an inherited blood disorder that prevents red blood cells from being shaped normally.
When these misshapen cells travel through the body, they can clog the blood vessels and deprive organs of the oxygen they need to function properly.
This can lead to painful episodes, called sickle cell crises, as well as long-term complications such as organ damage and stroke.
Read on to learn more about the causes, symptoms, and treatments of sickle cell disease.
What is Sickle Cell Disease:
Sickle cell disease is an inherited blood disorder that causes the red blood cells to become rigid and shaped like a crescent moon (sickle shape) instead of the usual circular disc shape.
These sickle-shaped cells can cause blockages in small blood vessels throughout the body. This means that not enough oxygen gets to organs and tissues that need it.
The blockages can also cause complications such as stroke, kidney damage, pain episodes, bone fractures or infection.
The Different Types of SCD:
There are four types of SCD, which are differentiated by the severity of their symptoms. The four types are:
- SCD type I (the most severe);
- SCD type II (a milder form that affects children);
- SCD type III (known as sickle cell-Cooley's anemia); and
- SCD type IV (an even less severe form).
Causes of Sickle Cell Disease
SCD is an inherited blood disorder where red blood cells become sickle-shaped.
These cells cannot carry oxygen efficiently to various parts of the body, resulting in increased levels of pain and fatigue, organ damage, infections, strokes or heart attacks.
People with SCD can be treated for these complications with medications or surgery.
Early Signs of SCD:
Early signs of sickle cell disease are not always easy to notice. The most common symptom is pain in the bones.
The pain often starts gradually, but it can worsen over time. Other symptoms may include:
- Fatigue or lack of energy;
- High temperature (fever) with no infection present;
- Painful swelling in a joint or limb without an injury being present;
- Anemia (low levels of red blood cells);
- Dry cough that lasts for more than one week;
- Recurring bouts of acute chest illness with sudden fever, fatigue, shortness of breath, rapid breathing rate and sweating.
Doctors will diagnose SCD when there is at least two out of five symptoms. If you suspect you have SCD, talk to your doctor about getting tested as early detection and treatment improves quality of life and reduces complications.
Common Risks and Complications of SCD:
Sickle cell disease is a hereditary condition that causes red blood cells to take on an unusual crescent shape, known as a sickle shape.
This change in the shape of the cells makes them more vulnerable to damage and rupturing. The damaged cells are then removed from circulation, which can lead to anemia and other complications.
Patients with SCD often experience pain crises, periods of acute symptoms such as pain and fever, usually caused by infections or dehydration.
Pain may be felt over various parts of the body including bone (osteonecrosis), chest (pleurisy), abdomen (splenic sequestration), joints (arthritis), or skin (cutaneous vasculitis).
Diagnosing SCD:
Sickle cell disease is a serious genetic disorder in which the red blood cells are abnormal. Sickle-shaped red blood cells are rigid and sticky instead of flexible. These rigid cells clog up small blood vessels and slow or stop the flow of oxygen to organs.
Red blood cells with hemoglobin S (HbS) can't carry as much oxygen as normal red blood cells with hemoglobin A (HbA).
HbA can be made into HbA2 by a process called sickling crisis but this process doesn't work for HbS.
People who have SCD need help from doctors, nurses, and other healthcare professionals to avoid infections, maintain their general health, manage pain and protect their airways.
They also need regular lab tests to monitor the condition of their red blood cells. If needed, patients may receive transfusions of red blood cells that contain more HbA than HbS.
Hydroxyurea is an FDA approved drug for treating people with SCD. It helps reduce how often people get sickle cell crises and reduces how severe these crises are when they do occur.
Patients should take hydroxyurea exactly as prescribed by their doctor to help prevent side effects such as easy bruising or trouble breathing.
What Are the Treatment Options for SCD?
The most important treatment for sickle cell disease is regular blood transfusions. The red blood cells in the transfusion can carry oxygen to the body's tissues and organs.
Another type of treatment is hydroxyurea which helps slow down the destruction of red blood cells.
A doctor may also prescribe a pain reliever such as acetaminophen or ibuprofen to help with any aches and pains associated with SCD.
Lastly, physical therapy may be prescribed to improve mobility in patients with SCD.
It's important to talk to your doctor about how you should live your life if you have SCD. They'll give you some advice based on how severe your symptoms are.
Conclusion
It's important to be aware of sickle cell disease in order to identify it in yourself or others. Fortunately, there are many treatments available that can help make the condition more manageable.
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