What is sickle cell disease?
Sickle cell disease is an inherited blood disorder, meaning it is passed down through the parents’ genes. A person will be born with sickle cell disease only if two genes are inherited– one from the mother and another from the father. Hemoglobin, marked as the flawed protein in the red blood cells, carries oxygen to the tissues of the body. So, sickle cell disease interferes with the delivery of oxygen to the tissues.
Normal hemoglobin cells are smooth, round, and flexible, like the letter “O”. Sickle cell hemoglobin cells are stiff, sticky, and shaped like a sickle or the letter “C.” This change in shape can make sickle cells cluster together and block small arteries or capillaries, which prevent the movement of healthy, normal oxygen-carrying blood. This blockage can cause acute or chronic pain and can also lead to infection or organ damage.
Normal red blood cells can live up to 120 days, but sickle cells only live for about 10 to 20 days– causing a shortage of red blood cells. With less healthy red blood cells circulating in the body, you can become chronically anemic. Also, the sickle cells can get stuck in the spleen; once stuck in the spleen filter, these cells damage the spleen and then die, putting you at a greater risk for infection.
Sickle cell disease can cause anemia. Sickle cell anemia is the most common symptom of all the sickle cell diseases. Jaundice, or yellowing of the skin, is a common symptom of sickle cell disease that is caused by the breakdown of the red blood cells. Acute chest syndrome occurs when cells block the flow of oxygen in the lungs. This can be life threatening and often occurs suddenly, when the body is under stress from infection, fever, or dehydration. It looks like pneumonia and can include fever, pain, and violent cough. The symptoms of sickle cell disease may look like other disorders or medical problems. Always check with a doctor for diagnosis.
Sickle cell mainly affects those of African descent and Hispanics of Caribbean ancestry. The trait has also been found in those with Middle Eastern, Indian, Latin American, Indian American, and Mediterranean heritage. Having a family history of sickle cell disease increases your risk for the disease. A person who inherits just one gene is healthy and said to be a “carrier” of the disease. For parents who are each carriers of the sickle cell gene, there is a 1 in 4, or 25% chance, of having a child with sickle cell disease. Many states routinely screen newborns for sickle cell so that treatment can begin as soon as possible. Early diagnosis and treatment can reduce the risk of complications.
Any and all major organs are affected by sickle cell. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can suffer damage from the abnormal functioning of the sickle cells and their inability to flow through the small blood vessels correctly. Complications of sickle cell disease may lead to increased infections, leg ulcers, bone damage, early gallstones, kidney damage, loss of water in the urine, eye damage, and multiple organ failure. Sickle cell disease is a lifelong condition. Although the complications of sickle cell disease may not be able to be prevented entirely, living a healthy lifestyle can reduce some of the complications.
If you or your child suffers complications from sickle cell disease, visit your nearest CHI St. Luke’s Health emergency department.