Sickle Cell overview
Sickle cell anemia is a genetic disorder of the red blood cells, which significantly reduces their ability to carry oxygen throughout the body. Normal red blood cells are round-shaped and move easily around the blood vessels. However, those with sickle cell anemia instead have crescent moon shaped red blood cells, which leads to a host of complications.
These misshapen cells are liable to become lodged in smaller blood vessels, rapidly building up and blocking blood flow. This, in turn, reduces oxygen in the body a great deal. Even worse, sickle cells die much faster than healthy red blood cells do, which creates a shortage of red blood cells, also known as anemia. Anemia is well-known to cause severe fatigue, as your body simply isn’t getting the oxygen it needs.
The first signs of sickle cell will usually appear very early in life. While these complications can vary over time, for most people, they tend to appear as soon as five months of age. One of the most common symptoms of sickle cell anemia is periods of pain, known as pain crises, which cause intense pain as sickle cells try to move through the small blood vessels found in our joints and chest.
The pain can vary in both intensity and length quite a bit, as some only have one or two crises a year, while for others, it can be a monthly occurrence. Even worse, some will develop chronic pain as a result of joint damage or other complications.
Other known symptoms of sickle cell anemia include swelling of the feet or hands, vision problems, and chronic infections. Some research also shows that the lack of adequate oxygen can lead to delayed growth and even puberty.
Sickle cell anemia is caused by a specific gene mutation, which affects red blood cell’s ability to carry oxygen, leading to the misshapen cells. Research shows that both parents must pass on the defective gene for their child to develop the disorder. That said, if only one parent has the latent sickle gene, the child will also be a carrier of the gene. These carriers produce both normal hemoglobin and sickle cells, but generally don’t experience any symptoms.
As such, the only risk factor for developing sickle cell anemia is both parents carrying the sickle cell gene. While not true worldwide, in the US, people of African descent are more likely to be affected by the disorder.
Sickle cell anemia is the most common inherited blood disorder across the US. That said, it’s still relatively uncommon, with reports indicating between 70,000 and 80,000 Americans are currently affected. Estimates suggest around 1 in 500 African Americans, and 1 in 1200 Hispanic Americans are impacted.
Sickle cell anemia can impact the quality of life quite a lot, depending on the severity of symptoms. However, proper treatment can control pain and even reduce symptoms. If you’re worried you or a loved one may have sickle cell, be sure to talk to your parents and doctor as soon as possible. Getting the right diagnosis and on a path forward is paramount.