Sickle cell anemia is an inherited blood disease that causes abnormal red blood cells. Red blood cells contain a protein called hemoglobin that carries oxygen from your lungs to the rest of your body. When you have sickle cell anemia, your hemoglobin is not normal and your red blood cells have an abnormal shape. Instead of being soft and round, the cells are a stiff crescent (sickle) shape and sticky. This makes it harder for the blood cells to pass through small blood vessels. The cells can get stuck and block the flow of blood and oxygen to parts of the body. The lack of oxygen can damage the body tissues and can affect all organs. When sickle cells block blood flow, it causes severe pain and fever. This is called sickle cell crisis.
Sickle cells are fragile and donâ€™t last as long as normal red blood cells. Your body may not be able to make new red blood cells fast enough. This can cause a shortage of red blood cells as well as low hemoglobin, which is called anemia. The fewer red blood cells and less hemoglobin there is, the less oxygen can be carried to your body.
Common symptoms of sickle cell anemia include tiredness or weakness, shortness of breath, pale skin, yellowing of the skin (jaundice), dizziness, and headaches.
Pain is also a common symptom. This happens when the body organs and tissues donâ€™t get oxygen because the abnormal cells block the blood flow. Pain can be chronic, but it most commonly comes on suddenly. It can be mild or severe. People also may have chronic bone pain, which can limit activities.
Almost all people with this disease will have painful crises. Risk increases if you donâ€™t drink enough fluids (dehydration), get an infection, are pregnant, have surgery, or are at high altitude.
Because sickle cell anemia and crises affect the whole body, there are many complications of this disease:
Hands and feet swelling and pain (plus fever) because the small blood vessels get blocked (this occurs most often in children)
Spleen enlargement. The spleen traps abnormal blood cells and filters them out of the blood. Sometimes it may trap good cells, causing anemia, and this can cause the spleen to get big.
Infections are common because the spleen is large and canâ€™t fight infections as well.
Acute chest syndrome, which is like pneumonia, and causes shortness of breath
High blood pressure in the lungs because the blood vessels there are damaged
Stroke because the blood vessels get blocked or are damaged and leak
Eye problems because blood flow is blocked and oxygen canâ€™t get to the eyes or the blood vessels leak
Men may have unwanted erections because of damage to blood vessels that doesnâ€™t allow blood to drain out of the penis
Gallbladder stones because dead red blood cells release their hemoglobin and that can cause stones
Skin sores, which are not well understood
Multiple organ failure, which involves two out of three major organs: the liver, lungs, or kidneys. This is rare, but very serious and happens with crisis.
How can I take care of myself when I go home?
How long it takes to get better depends on your treatment, how well you recover, your overall health, and any complications you may have.
Sickle cell anemia is a lifelong disease. It is important to have one primary care provider who knows you and manages your condition and who you see regularly. Your provider can:
Provide genetic counseling, if you plan to have children
Talk to you about treatment options, such as blood transfusions and bone marrow transplant
Monitor your treatment plan and make changes, as needed
Pain control is a primary focus of managing sickle cell disease. Your pain may be sudden and severe or chronic and hard to manage or a combination of the two. Talk with your provider about how to best manage your pain. Follow your providerâ€™s instructions and let him or her know when treatment is not helping.
Your provider will give you a list of your medicines when you leave the hospital.
Know your medicines. Know what they look like, how much you should take each time, how often you should take them, and why you take each one.
Take your medicines exactly as your provider tells you to.
Carry a list of your medicines in your wallet or purse. Include any nonprescription medicines and supplements on the list.
Your provider may prescribe medicine to:
Treat pain. Take your pain medicines as prescribed.
Treat or prevent an infection
Prevent your red blood cells from sickling
Help your body make red blood cells
Follow your provider’s instructions for follow-up appointments and routine tests.
Talk with your provider about any questions or fears you have.
Diet, Exercise, and Other Lifestyle Changes
Follow the treatment plan your healthcare provider prescribes.
Get plenty of rest. Try to get at least 7 to 9 hours of sleep each night.
Eat a healthy diet with lots of green vegetables.
Drink enough fluids to keep your urine light yellow in color, unless you are told to limit fluids.
Exercise as your provider recommends. Rest if you feel tired.
Don’t smoke. Smoking can worsen poor blood circulation.
Find ways to make your life less stressful.
Follow activity restrictions, such as not driving or operating machinery, as recommended by your healthcare provider or pharmacist, especially if you are taking pain medicines.
Get a flu shot each year and get a pneumonia vaccine if recommended by your provider.
Donâ€™t fly on airplanes without pressurized cabins.
Ask your provider if you can drink alcohol and what is a safe amount for you.
Call emergency medical services or 911 if you have new or worsening:
Shortness of breath
Weakness, numbness, or tingling in the face, arm, or leg, especially on one side of the body
Trouble with muscle movements, such as swallowing, moving arms and legs
Trouble speaking or understanding
Change in vision, such as double vision, blurred vision, or trouble seeing out of one or both eyes
Do not drive yourself if you have any of these symptoms.
Call your healthcare provider if you have new or worsening:
Pain that is not controlled with medicine
Fever above 101.5Â° F (38.6Â° C)
Dizziness or lightheadedness
Yellow color (jaundice) of your skin or the white part of your eye
Developed by RelayHealth.
Acute Care Advisor 2015.1 published by RelayHealth. Last modified: 2013-10-24 Last reviewed: 2013-10-12
This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.