Categorizing Sickle Cell Disease (SCD)
- Sickle cell disease (SCD) is not one condition but a group of several inherited blood disorders.
- There are a few different types of SCD, distinguished by the gene mutation that the patient has.
- The three most common types include HbSS (most severe form), HbSC and HbS beta thalassemia.
- Patients are encouraged to talk to their doctor about their type of SCD and what symptoms they have, which can help determine proper treatment.
They each affect people in different ways and can cause a variety of symptoms, including severe pain, organ damage, blood clots, infections and anemia (low red blood cell count).
What Is Sickle Cell Disease?
Read Morebody. The abnormal hemoglobin sticks to the red blood cells, becoming stiff and sticky. This leads to a change in the shape of the red blood cells. Normal red blood cells are flexible and disc-shaped, with a thinner center and a thicker outer rim. However, cells with abnormal hemoglobin tend to become stiff
and "C" shaped, like the sickle tool used in farming.
When the cells change shape, they no longer move easily through blood vessels and may get stuck and block blood flow. These cells tend to die off quickly, and proper oxygen does not reach tissues.
And it is the lack of oxygen that leads to symptoms and complications.
Common Sickle Cell Disease Symptoms: Understanding the Pain
What Are the Types of Sickle Cell Disease?
Whether the hemoglobin inside someone's red blood cells is normal or abnormal is dictated by their genes and the genetic changes (mutations) they may carry.
The three most common types of SCD include:
- HbSS – This is also called sickle cell anemia and is the most severe form of the disease.
- HbSC – This type of SCD is typically more mild.
- HbS beta thalassemia – This type of SCD includes HbS beta zero and HbS beta plus. Those with “zero” tend to have a more severe form of SCD, while those with “plus” usually have more mild disease.
Less common forms of the disease include:
- HbSD
- HbSE
- HbSO.
“What mutation [a patient gets] speaks to the severity of how the disease presents,” Dr. Sophie Lanzkron, Director of the Sickle Cell Center for Adults at Johns Hopkins, tells SurvivorNet.
“But it’s important to note that we all live on a bell curve and there are some people who have SC disease who have severe complications with their disease. And there are some people with SS who have a more mild form of the disease,” Dr. Lanskron reminds patients.
The symptoms of SCD may vary depending on the type you have, which can also help doctors determine proper treatment.
Treating Sickle Cell Disease: Reducing Pain and Organ Damage
Questions to Ask Your Doctor
If you or your child have been diagnosed with SCD, knowing the type is important. Consider discussing the following topics with your healthcare provider.
- What type of SCD do I have?
- What complications are most common with this type of SCD?
- How can I monitor for complications?
- What treatments are available to alleviate symptoms and side effects associated with my type of SCD?
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