What is sickle cell anemia?

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 don’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 don’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 don’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 can’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 can’t get to the eyes or the blood vessels leak
• Men may have unwanted erections because of damage to blood vessels that doesn’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.

What can I expect in the hospital?

You may need to stay in the hospital because you have symptoms of sickle cell anemia that need immediate treatment or an adjustment to your treatment.

Several things may be done while you are in the hospital to monitor, test, and treat your condition. They include:

Monitoring

• You will be checked often by the hospital staff.
• Your heart rate, blood pressure, and temperature will be checked regularly.
• A cardiac (heart) monitor may be used to keep track of your heart rate and rhythm.
• Your blood oxygen level will be monitored by a sensor that is attached to your finger or earlobe.
• Your fluid intake may be monitored closely by keeping track of everything you eat and drink and any IV fluids you receive.
• Your fluid output may be monitored closely by keeping track of the amount of urine and bowel movements you produce.

Testing

Testing may include:

• Blood, urine, or other tests to monitor for infection, hemoglobin level, blood counts, and iron level. You will also be routinely checked for an enlarged spleen.

Treatment

The treatment for sickle cell anemia depends on your symptoms, how well you respond to treatment, your overall health, and any complications you may have. The goals of treatment are to relieve pain, to prevent or treat infection, stroke, organ damage, and any complications.

• Pain crisis is the most common problem requiring hospitalization, so pain control is usually the primary treatment for a crisis.
• You will have a small tube (IV catheter) inserted into a vein in your hand or arm. This will allow for fluids to be given directly into your blood and to give you medicine. IV fluids will prevent dehydration, which can worsen symptoms.
• You will receive oxygen through a small tube placed under your nose or through a mask placed over your face.
• You may need a blood transfusion to replace the abnormal blood cells with normal cells.
• Your provider may talk with you about the treatment option of a bone marrow or stem cell (young blood cell) transplant.
• Your provider may prescribe medicine to:
  • Treat pain
  • Treat or prevent an infection, especially in children
  • Help your body make normal hemoglobin that helps keep your red blood cells from sickling and helps prevent anemia

What can I do to help?

• You will need to tell your healthcare team if you have new or worsening:
  • Pain that is not controlled
  • Chest pain
  • 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
  • Dizziness or lightheadedness
  • Headache
  • Coldness in your feet or hands
  • Shortness of breath
  • Tiredness
• Ask questions about any medicine, treatment, or information that you do not understand.

How long will I be in the hospital?

How long you stay in the hospital depends on many factors. The average amount of time to stay in the hospital with sickle cell anemia is 4 to 6 days.