Treatment

If you develop 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.

A red blood cell transfusion can temporarily provide your body with healthy red blood cells to combat severe anemia. It may also help with other life-threatening effects of sickle cell, like acute chest syndrome or splenic sequestration. 

So far, a bone marrow transplant is the only treatment with the potential to cure sickle cell disease. It is an important option for some adults, but it does have risks and side effects. 

Doctors at the Fred Hutch Bone Marrow Transplant Program have performed more than 16,000 bone marrow transplants — more than any other institution in the world — including for people with sickle cell. 

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

It may help reduce the frequency of vaso-occlusive crises (VOCs), which happen when sickled red blood cells block the flow of blood. VOCs can lead to pain and other health problems, like damage to your organs from lack of oxygen. 

This medicine is given by infusion. For an infusion, liquid medicine is put into a vein through an intravenous (IV) line. Nurses who are experts in infusions give you these treatments. Usually, the first two infusions are given two weeks apart. Then you will get another infusion every four weeks. 

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 blood counts (level of blood cells) from time to time and adjust your dose as needed.

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. 

This oral medicine 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.

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 ensure you get the care you need for pain. 

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 instance, research shows that overall African Americans tend to receive less treatment for pain than whites do, despite reporting more pain. Also, doctors 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 combat health disparities and improve care for everyone with sickle cell disease by educating other health care providers about these issues. In partnership with colleagues at UW School of Medicine, our sickle cell experts and some patient-advocates have teamed up to educate first-year medical students about the racial and socioeconomic disparities some people with sickle cell disease face. Together, they challenge young medical students to care for people with sickle cell disease with compassion and respect.

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

When children with sickle cell disease are nearing adulthood, it’s time for them to transition from care with pediatric experts to care with doctors who specialize in treating adults. 

At Fred Hutch, we work closely with Seattle Children’s to help young adults and their families make this transition. Our focus is on providing young adults with the information, services, skills and support they need to live their best, healthiest and longest life.

Pain medicine and increased fluids are the main treatments for hand-foot syndrome, in which your hands and feet swell because your blood vessels are blocked. You might also hear this called “dactylitis,” which refers to inflamed fingers or toes.

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.