Sickle Cell Disease
Sickle Cell Disease
At the University of Michigan Health, we offer compassionate and expert care for adults and children with sickle cell disease (sometimes called sickle cell anemia).
What is sickle cell disease?
Sickle cell disease turns normal, round red blood cells into abnormally curved cells that look like sickles or crescent moons. These cells cause inflammation and can get stuck in blood vessels, causing blocked blood flow, severe pain, and organ damage throughout the body. Sickle cell disease also puts you at risk for infections.
Sickle cell disease is passed down in families. Please talk to your doctor about genetic testing to find out the chance of having a child with the disease if you or someone in your family has sickle cell disease or is a sickle cell carrier (called “sickle cell trait”). Your doctor also may recommend that your family members get tested for sickle cell disease or sickle cell trait.
Appointment Information
The U-M Health Rogel Cancer Center's Adult Hematology Oncology Program offers diagnosis and treatment for those with blood diseases, such as sickle cell disease.
If you are a patient who suspects they have sickle cell disease, please call 734-647-8902. If you are a healthcare professional looking to refer a patient, please contact our M-LINE service: 800-962-3555.
If you have more questions about sickle cell disease and possible treatment, please call our Cancer AnswerLine at 800-865-1125.
What are the symptoms of sickle cell disease?
Painful events (called “acute pain crises”) in the hands or feet, belly, back, or chest are the most common symptoms of sickle cell disease. These 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, tired, dizzy, and short of breath. Due to red blood cell breakdown in sickle cell disease, yellowing of the skin and the whites of the eyes may occur (called “jaundice”).
How is sickle cell disease diagnosed?
If there is concern for sickle cell disease, first a sickle cell screening test can be performed to show if someone is a sickle cell carrier (called “sickle cell trait”) or has sickle cell disease. This test cannot tell the difference between these two conditions. Another blood test, called the “hemoglobin electrophoresis,” can tell the difference between sickle cell trait and sickle cell disease and is often used to diagnose 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). If this finding is present, the patient will need to see a pediatric hematologist for more studies to confirm the diagnosis.
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) through several different tests. Obstetrician/gynecologists or maternal fetal medicine doctors can review testing options with you.
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. There may be options available to reduce the risk of passing on sickle cell disease using more advanced reproductive therapies.
How is sickle cell disease treated?
Everyone with sickle cell disease should have a hematologist and should receive medications or transfusions to treat the sickle cell disease.
The most common treatment for sickle cell disease is hydroxyurea, a safe medication which can reduce the number of acute pain crises per year, prevent organ damage, and reduce the risk of dying from sickle cell disease.
For people who cannot take hydroxyurea, two other medications are available. They are called crizanlizumab (or “Adakveo”) and L-glutamine (or “Endari”). Crizanlizumab is given via the vein, as an infusion, and L-glutamine is taken by mouth. Both may reduce the number of acute pain crises per year.
For people who cannot take any medication for sickle cell disease or who have severe complications from sickle cell disease, blood transfusions may be used to treat sickle cell disease.
Your hematologist can help to select the best medications or blood transfusions to treat your sickle cell disease.
For some patients with sickle cell anemia, bone marrow transplant or gene therapy may be excellent long-term treatment options to reduce many, if not all, symptoms of sickle cell disease. Your hematologist can help to determine if you qualify for either treatment option. Both treatments are available at the University of Michigan Health.
Pain Treatment
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 or hydromorphone.
- 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.
