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What is sickle cell anemia? Sickle cell anemia is a form of the inherited blood disorder, sickle cell disease. Sickle cell anemia affects your red blood cells, turning them from round flexible discs into stiff and sticky sickled cells. Sickled cells keep red blood cells from doing their job, which is carrying oxygen throughout your body. Sickled cells also don’t live as long as normal red blood cells. As a result, you don’t have enough healthy red blood cells and you develop anemia, the condition that gives sickle cell anemia its name. In the past, babies born with sickle cell anemia rarely lived to be adults. Now, thanks to early detection and new treatments, about half of all people who have sickle cell anemia live into their 50s. People who have sickle cell anemia still face potentially life-threatening medical complications. However, healthcare providers have treatments that reduce the risk of complications and ease symptoms when they happen. (Unfortunately, there are many places in the world where people still don’t have access to effective medical treatment for sickle cell anemia.) Who is affected by sickle cell anemia? Sickle cell anemia is rare in the United States, affecting about around 100,000 people. It mostly affects people whose ancestry links back to parts of the world where many people have malaria and carry a gene that provides partial protection against anemia. This gene also causes sickle cell anemia. In the United States, sickle cell anemia affects many people who are Black. It may also affect people from southern European, Middle Eastern or Asian Indian ancestry. How does sickle cell anemia affect people? Babies born with sickle cell anemia may not have symptoms for several months. When they do, symptoms include extreme tiredness or fussiness from anemia, painfully swollen hands and feet, and jaundice. Babies may also have spleen damage that affects their immune system and increases their risk for bacterial infections. As people with sickle cell anemia grow older, they may develop different and more serious medical problems that happen when organ tissues don’t receive enough oxygen. People with sickle cell anemia are at increased risk for stroke and lung, kidney, spleen and liver damage. SYMPTOMS AND CAUSES What causes sickle cell anemia? People with sickle cell anemia inherit the disease from their biological parents. In sickle cell anemia, the gene that helps make normal red blood cells mutates, or changes. People who inherit the mutated hemoglobin protein gene from both biological parents have sickle cell anemia. People who inherit the mutated gene from one biological parent have the sickle cell trait. How does this mutation affect normal red blood cells? Normal red blood cells contain hemoglobin. Hemoglobin is a protein and the main part of red blood cells. When the hemoglobin gene mutates, it creates sickled cells that can’t navigate the network of blood vessels that carry oxygen, nutrients and hormones throughout your body. Here’s why: Normal hemoglobin is soluble, meaning it dissolves in fluid. Abnormal hemoglobin isn’t as soluble and ends up forming solid clumps in your red blood cells. Red blood cells need to be flexible to squeeze and slide their way through narrow blood vessels. Red blood cells carrying abnormal solid hemoglobin can’t do that. Instead, blood cells with abnormal hemoglobin end up blocking blood vessels and blood flow. Normal red blood cells live about 120 days. Sickled cells self-destruct within 10 to 20 days. Normally, your bone marrow makes enough red blood cells to replace dying cells. When cells die earlier than usual, your bone marrow becomes like a factory struggling to match supply with demand. When the bone marrow factory can’t keep up, you don’t have enough red blood cells. What are the signs and symptoms of medical conditions linked to sickle cell anemia? Sickle cell anemia symptoms typically start when babies are 5 to 6 months old. As they grow older, most people with sickle cell anemia have increased risk for developing new medical conditions. Some of these conditions are life-threatening. But by learning about conditions and symptoms, people with sickle cell anemia can seek help at the first sign of trouble so healthcare providers can treat the condition. Vaso-inclusive crisis (VOC) Healthcare providers may call this condition acute pain crisis. VOC, or acute pain crises, is the most common reason why people with sickle cell anemia go to the emergency room or need to spend time in the hospital. Symptoms include: Sudden intense pain. Pain can be sharp or stabbing. VOC may affect any part of your body, but typically affects your abdomen, lower back, arms and legs. Living with VOC is one of the more difficult aspects of having sickle cell anemia. Among other symptoms and complications, people who have sickle cell anemia may feel depressed or anxious because they’re dealing with certain stigmas associated with the condition. #AnemiaDi
