Treatment for Sickle Cell Disease

Acute chest syndrome is a lot like pneumonia. It involves chest pain, coughing, breathing trouble and fever.

If you get acute chest syndrome, your team will choose treatments based on the cause. Common treatments include getting oxygen, taking medicine to fight an infection or open your airways and having a blood transfusion. 

Sickle cell disease increases your risk of infections, like flu, hepatitis, meningitis and pneumonia.

Special attention to steps like washing your hands, handling food safely and getting recommended vaccinations can help prevent infections. If you do get an infection, it’s important to see your care team right away for treatment, like antibiotics.

Hydroxyurea is a medicine taken every day to reduce pain episodes, recurrence of acute chest syndrome and the need for blood transfusions or hospital stays.

To make sure you are getting the right amount of hydroxyurea, your care team will check your level of blood cells (blood counts) from time to time. They will adjust your dose as needed.

This oral medicine (taken by mouth) may help reduce the number of sickle cell crises you have.

It may make your red blood cells more flexible so they can flow through your bloodstream more easily.

Hand-foot syndrome is swelling of the hands and feet from blocked blood vessels.

You might also hear this syndrome called “dactylitis,” which refers to inflamed fingers or toes. You might have a fever as well. Pain medicine and getting more fluids are the main treatments. 

This is the first medicine made specifically for sickle cell disease that can change the underlying problem of red blood cells taking on a sickle shape.

Voxelotor helps your hemoglobin hold on to oxygen, and it helps keep your red blood cells from sickling. It may help prevent symptoms, like pain, and complications, like organ damage. Voxelotor is a tablet that you take by mouth.

Getting a red blood cell transfusion or red blood cell exchange can help with anemia or other effects of sickle cell disease.

Anemia can make you tired, irritable, dizzy and lightheaded. It can lead to a fast heart rate, breathing trouble and jaundice, and it can make your skin paler. In children, anemia can slow growth and delay puberty.

A red blood cell transfusion gives you healthy red blood cells from a donor. It can temporarily provide what your body needs to fight severe anemia. It may also help with other life-threatening effects of sickle cell, like acute chest syndrome or buildup of blood in the spleen (splenic sequestration). 

We offer red blood cell transfusions at our outpatient clinic on Lake Union. If you need a red blood cell exchange (to remove your red blood cells and replace them with healthy donor cells), our patients get this type of care at UW Medical Center – Montlake. 

If you often have pain episodes, your doctor may recommend the medicine crizanlizumab.

The most common problem from sickle cell disease is pain from decreased oxygen to tissues in the body. It tends to flare up in what physicians call “pain episodes” or “crises.” Episodes can come on suddenly, be mild to severe and last for any length of time.

Crizanlizumab may help you have fewer pain episodes because of sickled red blood cells blocking the flow of blood. These are called vaso-occlusive crises, or VOCs. They can lead to other health problems, like damage to your organs. 

This medicine is given by infusion. For an infusion, liquid medicine is put into a vein through an intravenous (IV) line. You will get infusions in a special area of the clinic. Nurses who are experts in infusions give you these treatments. They also watch over you during the treatment. They will help with any medical issues that come up and will keep you comfortable.

Usually, the first two infusions are given two weeks apart. Then, you will get an infusion every four weeks. 

A blood or marrow transplant resets your body’s ability to make healthy blood cells. Researchers at Fred Hutch pioneered this form of treatment.

So far, a blood or marrow transplant is the only treatment that may cure sickle cell disease. It is an important option for some adults, but it does have risks and side effects. 

Physicians at the Fred Hutch Blood and Marrow Transplant Program have done more than 17,000 transplants, including for people with sickle cell. 

We can talk with you and your family about the benefits and risks, if we recommend a transplant for you and why, and what to expect. Our transplant specialists will answer your questions and help you decide what’s right for you. 

If you have a transplant, a team of Fred Hutch transplant experts will care for you. Your team will include a transplant oncologist, transplant nurse, radiation oncologist, advanced practice providers, pharmacist, registered dietitian, patient care coordinator and social worker.

Doctors and researchers at Fred Hutch pioneered blood and marrow transplants decades ago. Today, we continue to improve transplant techniques and to develop new options.

Learn more about Blood and Marrow Tranplants

Over-the-counter medicines, like ibuprofen or acetaminophen, may be enough to control pain from sickle cell disease. But sometimes you may need stronger opioid medicines to take at home or even during treatment at the hospital.

Your Fred Hutch team works to make sure you get the care you need for pain. We have an expert Pain Clinic team, including a dedicated nurse to help people with blood disorders. We can also help you learn to reduce your risk for painful episodes by avoiding triggers, like low fluid intake and extreme changes in temperature.

In the U.S., people with sickle cell disease are usually African Americans, who may have experienced unequal treatment for their health needs, especially their pain. For example, research shows that African Americans tend to receive less treatment for pain overall than white people do, even if they report more pain. Also, physicians may be more likely to see African Americans who say they are in pain as drug-seeking, even though white people are more likely to misuse opioids.¹

At Fred Hutch, our sickle cell team is working to end health disparities and improve care for everyone with sickle cell disease by teaching other health care providers about these issues. With colleagues at UW School of Medicine, our sickle cell experts and some patient-advocates talk with medical students about the racial and socioeconomic inequities faced by some people with sickle cell disease. Together, they teach young medical students to care for all people who have sickle cell disease with compassion and respect.

1. Pain and Ethnicity, Virtual Mentor/AMA Journal Ethics.

With any treatment, we talk with you about what to expect, based on your treatment plan, and what can help if you do have side effects. We will work closely with you to make sure that side effects are managed.

At your appointments, we want you to tell us about any side effects you are having. If you have questions or concerns between appointments, you can call or email us. We will make sure you know how to reach care providers at Fred Hutch after hours, if that is when you need us.

We have many tools to help you feel better, such as:

• Nutrition care and medicines to help with digestive problems
• Antibiotics and antiviral drugs to prevent or treat infections
• Medicines to remove excess iron (iron chelation therapy)
• Conventional and integrative therapies for pain

Side effects are different depending on which treatment you get. These are some of the common side effects of sickle cell disease treatment:

• Problems in your digestive tract, like nausea or constipation
• Low levels of blood cells if your hydroxyurea dose is too high
• Iron overload from having many blood transfusions
• Developing antibodies to donor red blood cells (alloimmunization) 

If your loved one is having treatment for sickle cell disease, there are many ways you can help. Caregiving often means doing tasks like these:

• Keeping track of their appointments and driving them to and from treatment
• Watching for changes in their condition and telling their care team about any symptoms
• Providing physical care, like helping them take medicines
• Spending time with them and encouraging them
• Taking care of things at home that they may not be able to do, like grocery shopping and cleaning

Caregivers have a special role in blood and marrow transplants. This intense treatment involves chemotherapy (and sometimes radiation) with serious side effects. During the initial recovery period, which often takes at least a month, your loved one will need daily help. We have classes to help transplant caregivers get ready. During recovery, a transplant registered nurse is available by phone 24 hours a day, 7 days a week, to help you.