Sickle cell disease affects how your body makes red blood cells, which carry oxygen throughout your body.
Red blood cells are normally flat, flexible discs. But if you have sickle cell disease, your body makes red blood cells that are stiff and shaped like a crescent, or a sickle. That can cause major health problems, called complications. Most of the time, your doctor can help you treat or even prevent these issues.
It’s the most common complication of sickle cell disease. It happens when the sickle-shaped cells get stuck and block small blood vessels. The pain often flares in the lower back, legs, arms, belly, or chest. It can come in sudden, intense attacks, called a crisis or an episode. Older teens and adults can also have long-term pain from sickle cell disease.
Most of the time, over-the-counter drugs like aspirin or ibuprofen can ease pain during a crisis. (Anyone under age 19 should never take aspirin, though.) But sometimes, doctors need to prescribe more powerful medications like opioid drugs. Some people may need to go to the hospital for treatment.
Your doctor will suggest other ways to ease or prevent a pain crisis: Drink enough water, avoid extreme heat or cold, and don’t spend time at high altitudes. One drug, called hydroxyurea, can keep pain episodes from happening as often for children and adults.
It’s another common complication, which happens when sickled blood cells die faster than your body can replace them. That makes it harder for your cells to get oxygen, which can leave you feeling tired, weak, and dizzy. You can also have a fast heartbeat and trouble breathing. Anemia may also make it harder for children to grow.
If anemia is severe, blood transfusions can help. But if you get them often, you may end up with too much iron in your blood, which can cause problems with your heart, liver, or pancreas. Your doctor can help you manage your iron levels. For adults, the drug hydroxyurea may also help with anemia.
People with sickle cell disease, especially kids, are more likely to get infections. That’s because the disease damages the spleen, an organ that helps your body filter bacteria out of your blood.
Antibiotics can treat infections, but it’s important to get treatment ASAP to keep a minor infection from turning into a more serious one. If you or your child has a fever over 101, get to the doctor or emergency room right away.
Children are especially prone to this complication, which happens when sickle cells block blood flow through the spleen. That causes the organ to swell painfully. Some children won’t show many symptoms, but others will feel weak, have pale skin or lips, feel sleepy or sluggish, have pain in the left side of the belly, and have a fast heartbeat.
Splenic sequestration may be a life-threatening emergency. So if you or your child has symptoms, get to the hospital right away. Most people will need a blood transfusion to treat it.
Acute Chest Syndrome
This problem happens when sickle cells block blood flow in the lungs. It’s very serious, so if you or your child has any symptoms — fever, trouble breathing, chest pain, and cough — get to the hospital right away.
Doctors may give you oxygen, antibiotics, medicines to help you breathe better, and possibly a blood transfusion.
The drug hydroxyurea also can help prevent acute chest syndrome.
When sickle cells block blood flow to the bones of major joints, it can cause bone tissue to die. This is known as avascular necrosis, and it often happens in the hip joints of teenagers and adults with sickle cell disease. Pain medicine can help, but some people need surgery to repair the damage.
People with sickle cell disease are also more likely to have eye, heart, kidney, and liver problems, blood clots, and stroke. Your doctor can check you regularly for these problems.
Bone Marrow Transplant for Sickle Cell Disease
People who have a lot of severe complications from sickle cell disease may be able to get a bone marrow transplant. The procedure replaces the cells that create sickle-shaped red blood cells with healthy ones. It can cure the condition.
But the process may not work for everyone. Before you get the transplant, you’ll need to find someone whose tissue matches yours to see if they can donate their healthy cells to you. Problems with other organs, like your heart or kidneys, also may make it too risky. The procedure also requires a long hospital stay. And sometimes, bone marrow transplants don’t work. Families should ask their doctor whether a transplant is likely to help.
Can You Prevent Complications?
Many of the problems that come with sickle cell disease have to be managed as they happen. But some things can help you make them less likely.
Some medicines help some people prevent complications or make them less severe. The drug hydroxyurea can help adults and children have fewer pain crises and acute chest syndrome attacks. It might also keep the disease from damaging organs. The FDA has also approved another drug, L-glutamine, to prevent pain crises in adults and children with sickle cell disease. Talk to your doctor about whether these medicines might help.
Exercise and deep breathing help prevent complications and keep you healthy. Just make sure you don’t get overheated or dehydrated — that can lead to dangerous problems. Talk to your doctor about the safest ways to work out.
To prevent infections, wash your hands with soap and water several times a day. Learn about safe food prep to avoid bacteria in food. Children should get all regular vaccines on time, and they may need to take antibiotics like penicillin every day to prevent infections.
- National Heart, Lung, and Blood Institute: “Sickle Cell Disease.”
- CDC: “Sickle Cell Disease — Complications and Treatments.”
- New England Pediatric Sickle Cell Consortium: “Splenic Sequestration in Sickle Cell Disease.”
- New England Journal of Medicine: “Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease.”
- British Journal of Haematology: “Guideline on the management of acute chest syndrome in sickle cell disease.”
- Ortopedia Traumatologia Rehabilitacja (Poland): “Avascular necrosis of the femoral head in sickle cell disease patients.”
- Expert Review of Hematology: “Hydroxyurea Use in Sickle Cell Disease.”
- National Organization for Rare Disorders: “Sickle Cell Disease.”
- St. Jude Children’s Research Hospitals: “Bone Marrow (Stem Cell) Transplant for Sickle Cell Disease.”
- FDA: “FDA approved L-glutamine powder for the treatment of sickle cell disease.”