Sickle cell anemia is a form of the inherited blood disorder, sickle cell disease. Sickle cell anemia changes your red blood cell shape, turning round flexible discs into stiff and sticky sickle cells that block blood flow. Thanks to early detection and new treatments, about half of all people who have sickle cell anemia live into their 50s.
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 do not live as long as normal red blood cells. As a result, you do not 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 do not have access to effective medical treatment for sickle cell anemia.)
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 do not receive enough oxygen. People with sickle cell anemia are at increased risk for stroke and lung, kidney, spleen and liver damage.
What is it like to live with sickle cell anemia?
New treatments are helping people with sickle cell anemia live longer and with better quality of life. The U.S. Centers for Disease Control and Prevention (CDC) has the following suggestions for living well with sickle cell anemia:
- Find good medical care. Sickle cell anemia is complicated. Many times, managing sickle cell anemia requires a multidisciplinary team of healthcare providers who specialize in blood disorders.
- Get regular checkups. Regular health checkups with your primary provider care may head off serious medical complications. Having a relationship with your healthcare provider who understands your situation may make it easier to get help if you have an acute pain crisis.
- Pay attention to your emotional health. Unfortunately, there are stigmas associated with sickle cell anemia. Sometimes, these stigmas cause people to have depression or anxiety. Talk to your healthcare provider if you are struggling with emotions related to feeling stigmatized. They can recommend resources to help you. More than that, they may be able to advocate for you by educating peers about sickle cell anemia complications and symptoms.
- Prevent infections. Talk to your healthcare provider about vaccinations and take steps to protect yourself from infection.
- Eat a healthy diet. Eat a balanced diet and drink eight to 10 glasses of water every day.
- Manage your environment. People who have sickle cell anemia need to maintain a balanced body temperature to avoid VOC/acute pain crises, so it is important that you avoid getting too hot or too cold.
- Get some exercise. Regular physical activity boosts your mood and your health. But be sure to take it easy, rest when you need to and stay hydrated.
- Get support. Because sickle cell anemia is a rare disease, most people do not understand what it is like to live with it. Ask your healthcare provider for support group suggestions.
- Look for clinical trials. Healthcare providers and researchers are constantly testing new treatments. Joining a clinical trial may be a way to obtain new medication and treatment. Ask your healthcare provider for help finding a clinical trial.
- Living with medical complications
- Sickle cell anemia is a chronic illness with symptoms that change over time. For example, children and adults have to cope with pain from acute chest syndrome or VOCs. They are at increased risk for stroke. As people grow older, they are likely to develop new complications such as chronic lung disease and painful leg ulcers.
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If you or your child has sickle cell anemia, ask your healthcare provider what you can do now and what you should expect. They will likely plan regular tests to monitor signs and symptoms so they can diagnose and treat complications as early as possible.
Managing pain
Studies show people with sickle cell anemia are constantly trying to manage pain that many times affects their quality of life. Sometimes, they have acute pain that happens when sickled cells block blood flow. This is VOC and is a medical emergency. Other people have chronic pain — pain that lasts for more than three to six months. If you have chronic pain, consider working with a pain management specialist. They can recommend different ways you can manage pain.
When should I go to the emergency room?
Sickle cell anemia may cause serious medical conditions. Go to the emergency room if you have the following symptoms:
- Extreme fatigue.
- Shortness of breath.
- Dizziness.
- Irregular heartbeat.
- Symptoms of acute chest syndrome
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- Fever of more than 101.3 degrees Fahrenheit (or 38.5 degrees Celsius).
- Chest pain.
- Cough.
- Symptoms of stroke
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- Sudden weakness.
- Numbness on one side of your or your childs body.
- Confusion.
- Trouble speaking, seeing or walking.
- Other symptoms
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Painful erections that last four hours or more. This is a symptom of priapism.
