Sickle cell anemia, also called sickle cell disease, affects roughly 4,500 people in South Carolina. It’s a disease that doesn’t always get much attention — and that’s a serious problem. Sickle cell anemia symptoms often appear during infancy. Identifying the disease early allows children to manage the disease and grow into healthy adults.
Sickle Cell Disease, Explained
“Healthy blood cells are round and flat, like a disc,” says Annemarie Gault, NP-C, who provides acute and comprehensive, routine care to sickle cell patients at the Beaufort Memorial Sickle Cell Clinic. “With sickle cell disease, the cells are curved and shaped more like a banana or a sickle, which is how the condition got its name. Sickle cells are more rigid than healthy cells, and they don’t travel through blood vessels as easily. This can hinder blood flow to organs and tissues throughout the body, leading to fatigue and other health problems.”
Sickle cell disease is an inherited condition, which means that it’s present at birth. It cannot develop later in life.
“Most babies show symptoms at some point during the first year of life, usually around 5 or 6 months of age,” Gault says. “But we test for sickle cell disease as part of routine prenatal care screenings, so parents can be prepared.”
Common Early Signs of Sickle Cell Disease
Some of the most common symptoms children with sickle cell disease have include jaundice, fatigue and swollen hands and feet. Most symptoms, however, are actually those related to the disease’s complications. For example, anemia, a condition in which blood doesn’t carry enough oxygen throughout the body, is often a complication for babies and children, as well as adults.
“The average lifespan of a healthy red blood cell is 90 to 120 days,” Gault says. “Sickle cells live only 10 to 20 days. Patients develop anemia because their bodies can’t replace red blood cells as fast as they need to.”
Anemia may cause someone to feel tired, cold, dizzy and/or irritable. In severe cases, patients with anemia may need blood transfusions.
Pain is another common symptom of sickle cell disease — sickle cells stick to blood vessel walls, slowing or stopping blood flow and oxygen to the organs and tissues. This can cause significant pain.
“We call this a pain crisis, and it’s serious,” Gault says. “A pain crisis can happen anywhere on the body and last hours or even days. In some cases, patients require hospitalization.”
Other complications of sickle cell disease include:
- Acute chest syndrome, in which the lungs are deprived of oxygen, leading to chest pain, fever and trouble breathing. Acute chest syndrome is a medical emergency that requires immediate care.
- Chronic pain, which is different from pain crises
- Eye problems, as a result of damage to the eye’s blood vessels, particularly in the retina, which can lead to vision loss
- Heart problems, including pulmonary hypertension and coronary artery disease. If a patient needs frequent blood transfusions, they may also have heart damage from iron overload.
- Splenic sequestration crisis, or an enlarged spleen. Most common in newborns and children, the condition occurs when red blood cells get trapped in the spleen, increasing the size of the spleen and preventing enough red blood cells from circulating. As a result, people can develop severe anemia.
- Stroke
Why It’s Important to Manage Sickle Cell Disease
Sickle cell disease can be cured with stem cell transplants, but finding a matching donor can be difficult and there are risks involved with the procedure. There is hope on the horizon for another cure – CRISPR technology, which modifies the DNA of patients’ blood cells, is currently being researched.
Certain medications can help people with sickle cell disease manage their symptoms and live a healthy life. The FDA recently approved a new medication which helps reduce vasocclusive crisis in sickle cell patients. Our sickle cell clinic has several patients on the new medication and has seen positive results.
Beaufort Memorial established the sickle cell clinic in 2018 to provide ongoing health maintenance and pain management for area residents suffering from the disease. In addition to disease-modifying therapy, many sickle cell patients visit the clinic for regular blood transfusions. The U.S. Food and Drug Administration recently approved a new medication that helps reduce vasocclusive crisis in sickle cell patients. Our sickle cell clinic has several patients on the new medication and has seen positive results.
Read More: Danielle Jaimison’s Story of Living With Sickle Cell Disease
“One of the best things patients with sickle cell disease can do is maintain a relationship with their primary care provider,” Gault says. “Keeping in touch will help manage sickle cell anemia symptoms and prevent problems later in life.”
Help members of your community who have sickle cell disease by donating blood today at OneBlood, our local blood bank.
