Sickle cell disease is a genetic condition caused by unhealthy and abnormal red blood cells. Instead of being soft and round, these red blood cells are hard and sticky, and shaped like a “sickle.” These unhealthy cells clog up blood vessels and reduce good oxygen flow, creating serious problems of intense pain, infections and more.
How do you get SCD?
It’s an inherited disease – meaning the genes were passed down to you from your parents, at birth. To have SCD, you have either inherited two sickle cell genes, or one sickle cell gene and a different type of “abnormal hemoglobin” gene from your parents. Often, parents aren’t aware that they carry the sickle cell gene or sickle cell trait, and sickle cell disease comes as a bit of a surprise after the baby’s born. Today in the US, screening automatically happens at birth to check for SCD.
SCD runs in families, but SCD is also more common in certain ethnic groups.
1 to 3 million
Americans have SCT
is not a disease (but it can still bring complications)
If you have
you inherited the gene from only one of your parents
8 to 10 %
of African Americans have SCT but it also affects other groups — including South Asians, Hispanics, Middle Easterners and Caucasians from southern Europe.
Types of SCD
There are several types of sickle cell disease, although some are more commonly known:
People who have this form of SCD inherit two sickle cell genes (“S”), one from each parent. This is commonly called sickle cell anemia and is usually the most severe form of the disease.
People who have this form of SCD inherit a sickle cell gene (“S”) from one parent and from the other parent a gene for an abnormal hemoglobin called “C”. Hemoglobin is a protein that allows red blood cells to carry oxygen to all parts of the body. This is usually a milder form of SCD.
Hemoglobin S beta thalassemia
People who have this form of SCD inherit one sickle cell gene (“S”) from one parent and one gene for beta thalassemia, another type of anemia, from the other parent. There are two types of beta thalassemia: “0” and “+”. Those with HbS beta 0-thalassemia usually have a severe form of SCD. People with HbS beta +-thalassemia tend to have a milder form of SCD.
and Hemoglobin SO
People who have these forms of SCD inherit one sickle cell gene (“S”) and one gene from an abnormal type of hemoglobin (“D”, “E”, or “O”). Hemoglobin is a protein that allows red blood cells to carry oxygen to all parts of the body. The severity of these rarer types of SCD varies.
Health problems and complications of SCD
Depending on the person and their specific type of SCD, there can be a wide range of health issues that stem from managing the disease. For example, you’ll often hear many warriors say that no two pain crises are alike and it’s hard to even describe how terrible they feel. Keeping in mind that not everyone will experience the exact same issue is key, but some of the biggest issues are:
or pain episodes
(lacking enough healthy red blood cells in the body)
Treatments for SCD have come a long way, and we’re grateful for all of the breakthroughs in recent times. Even with these advancements, however, not every treatment is right for every person with sickle cell disease. As every person is different, understanding your body and having the right treatment plan in place is key.
Some current treatments for SCD include:
Folic acid supplements
Stem Cell Transplant
Treating SCD can be highly complex and evolves over time as a person ages. To find the right treatment plan, it’s important that patients and care teams work together to address those needs.
Today, stem cell transplants (or bone marrow transplants) are the only recognized cure for sickle cell disease. Only a limited number of patients are good candidates to try these transplants, and it has to be cleared by your medical team. If you’d like more information about this, please speak with your doctor.
We are hopeful that one day a more universal cure will exist for the community. Until then, Sick Cells will continue to advocate for new treatments that are affordable and accessible to all.