Sickle cell disease turns normal, round red blood cells into abnormally curved cells that look like sickles or crescent moons. These cells can get stuck in blood vessels, blocking blood flow and causing severe pain. The sickle-shaped cells also can harm organs, muscles, and bones. It is a lifelong condition that puts you at risk for infections. Sickle cell disease is passed down in families. You can talk to your doctor about whether to have genetic tests to find out the chance of having a child with the disease. Your doctor also may recommend that your family members get tested for sickle cell disease.

Your doctor may treat you with medicines. Some people get blood transfusions or a bone marrow transplant. Managing pain and preventing infection are important parts of your treatment.

Painful events (sickle cell crises) in the hands or feet, belly, back, or chest are the most common symptom of sickle cell disease. They are periods of pain that happen when sickled cells get stuck in blood vessels and block the blood flow. This pain may last from hours to days.

Most people with sickle cell disease are anemic. Anemia can make you feel weak and tired. People with sickle cell anemia may look pale or washed out. They may be short of breath. Their skin and the whites of their eyes may have a yellowish look (jaundice).

Sickle cell disease is diagnosed when initial blood tests show abnormal hemoglobin. A sickle cell test looks for sickle cell trait and sickle cell disease.

The disease can be diagnosed soon after birth. A sample of blood is taken from the infant's heel. It's sent to a lab. There it is screened for the presence of sickle cell hemoglobin (hemoglobin S).

The disease may also be diagnosed based on the problems it can cause. More testing may be done if needed.

Doctors can diagnose sickle cell disease before a child is born (prenatally). Prenatal tests include amniocentesis and chorionic villus sampling (CVS).

Couples who are at risk for passing on this disease to their children may want to talk with a genetic counselor about prenatal testing before getting pregnant.

In many cases, you can treat pain at home. You can use a combination of pain medicines, plenty of fluids, and comfort measures such as heating pads or hot water bottles. Do not use heating pads with children.

A painful event (crisis) can become severe and may last for days to weeks. If home treatment doesn't help enough, you'll need strong pain medicine that must be monitored in a hospital or treatment center.

Pain can be treated at home with:

• Pain management skills. These skills can help you or your child focus away from the pain. They can help even when you also take pain medicines. A pain specialist can teach you pain management skills. These skills include:
  • Guided imagery.
  • Relaxation.
  • Distraction.
  • Deep breathing.
  • Positive, encouraging self-talk.
• Medicine. Use the pain management plan you developed with your doctor to help decide what type of pain medicine to use. You are probably the best judge of when it's necessary to use a stronger prescription medicine.
  • For mild pain, you might use over-the-counter ibuprofen (such as Advil) or acetaminophen (such as Tylenol). Do not give aspirin to children and teens. It has been linked to Reye syndrome, a rare but serious illness. Be safe with medicines. Read and follow all instructions on the label.
  • For moderate or severe pain, you might take pain medicine prescribed by your doctor. These are usually opioid medicines. You and your doctor will talk about which medicines to use, how much to take, and how often to take them. The instructions may change depending on how bad your pain is.

If you go to the hospital, you will be treated with a strong pain medicine. This is usually an opioid medicine, such as morphine.

• The medicine is given directly into a vein (intravenously, or I.V.). You may be given a button to push to release a dose when needed.
• When you feel less pain, I.V. opioid medicine is slowly reduced. It's replaced with a less powerful medicine you take by mouth. These actions can prevent your body from going through sudden drug withdrawal symptoms.
• In some cases regional anesthesia such as a nerve block may be used for pain in certain areas, such as the belly or leg.