Sickle cell: what is it and why should i be concerned?

If you are like our athletes (and their parents!), you may wonder why it is important to get tested for sickle cell. You are not alone in this as most people do not know or understand what “sickle cell” actually is.

Sickle cell is a catch all term that has become interchangeable to most people when referring to sickle cell trait and sickle cell disease.

Infants are typically tested at birth for this condition and parents should be contacted by their pediatrician if the results are positive. Sickle cell trait (SCT), also known as HbAs, is not a disease but the person has received one gene from their parent for sickle cell. There is a possibility that if a person has the trait, they can pass it on to their children. If the partner also has the trait, there is a 25% chance that their child will have the sickle cell disease (West Central Ohio Comprehensive Sickle Cell Center). A person who receives the gene from both parents has sickle cell disease (SCD). Sickle cell disease is the most common genetic blood disease in the United States (Ohio Department of Health). There are 3 common types of SCD which we will discuss below.

HbSS occurs when the person receives both genes of sickle cell from their parents, which can also be called sickle cell anemia. HbSS is the most severe form of sickle cell (cdc.gov). HbSC is a form of sickle cell disease when the person receives one gene from their parent for sickle cell and the other parent has abnormal hemoglobin called ‘C’. HbSC tends to be a milder version of sickle cell (cdc.org). HbS beta thalassemia occurs when a person receives the sickle cell trait from one parent and the other parent passes on a gene of beta thalassemia, which is another type of anemia. This form of SCD tends to be mild (cdc.org). The only cure for sickle cell disease is bone marrow transplant or stem cell transplant.

In order to understand what sickle cell is, one must first understand the role of hemoglobin and of red blood cells. Hemoglobin is found in red blood cells, which helps carry oxygen through the body (West Central Ohio Comprehensive Sickle Cell Center). In sickle cell, the red blood cells change from soft to hard and sticky. Their shape also changes from being round and flat to being quarter-mooned or ‘sickled’. These sickled cells loose oxygen and may clump together causing intense pain. The sickled cells die early which can lead to anemia. You should now be able to see what it is imperative for the sports medicine team to know if an athlete has sickling of their red blood cells!

Sickle cell can affect anyone. But, sickle cell is more common in those whose ancestors are from Africa, India, Central and South American, the Middle East, the Caribbean, Italy, Greece, France and Turkey (Ohio Health Department). About 8% of African-American population has sickle cell and about 1 in 2,000 Caucasians have sickle cell (ncaa.org). Athletes who have sickle cell disease are allowed to participate in sports but they must understand the risks they may face and learn how to handle sickle cell. Sickle cell can be life threatening since it can lead to anemia which causes fatigue and damage to blood vessels and vital organs (Ohio Health Department). Athletes with sickle cell need a longer acclimation period during conditioning and will need more water breaks. An athlete with sickle cell trait should slowly build up their intensity training and not jump right into hard conditioning. Their fitness tests should be scheduled later in the training program. Athletes should be allowed to rest when they start to have a hard time breathing, feel light headed or their chest feels heavy. Pushing too hard through conditioning may cause a fatal collapse. In 2000-2009 7 football players with sickle cell trait died during football conditioning (ncaa.org). It is important for coaches and athletic trainers to know if any of their athletes have sickle cell and to understand how to deal with the condition.

Resources

Get the Facts. (2014). Dayton: West Central Ohio Comprehensive Sickle Cell Center.

Face Sickle Cell: What everyone should know about sickle cell disease. (2014). Ohio Department of Health.

Facts About Sickle Cell Disease. (2014, January 16). Centers for Disease Control and Prevention. Retrieved August 30, 2014, from http://www.cdc.gov/ncbddd/sicklecell/facts

NCAA. (n.d.). Sickle Cell Trait. Retrieved August 29, 2014, from http://www.ncaa.org/health-and-safety/medical-conditions/sickle-cell-trait

Sickle Cell Trait. (n.d.). A Fact Sheet For Coaches. Retrieved August 30, 2014, from

http://www.ncaa.org/sites/default/files/NCAASickleCellTraitforCoaches

Sickle Cell Trait. (n.d.). A Fact Sheet For Students. Retrieved August 30, 2014, from

http://www.ncaa.org/sites/default/files/NCAASickleCellTraitforSA

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