Treatments and Therapies for Sickle Cell Disease

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If you are a person living with sickle cell disease, your needs and the effects of your disease will change over time. Your care should change, too. The most important step you can take is to partner with health care providers who will plan treatment that is right for you right now. Individualized care helps you live a full, active life.

At Fred Hutch Cancer Center, we believe sickle cell disease does not have to define who you are or what you can do. Our sickle cell specialists work closely with you, your family and each other to understand and meet your needs. We provide all standard therapies and offer access to promising new treatments through clinical trials.

Sickle Cell Disease Care Tailored to You

You and your family are our top priority. At Fred Hutch Cancer Center, we offer comprehensive and compassionate care — personalized to you. You'll have access to the latest treatment options, clinical trials and supportive care services.

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Why Choose Fred Hutch for Sickle Cell Disease Treatment

Because sickle cell disease can range from mild to severe and change over time, there is no single treatment approach that is right for everyone.

To help determine what is best for you, your Fred Hutch hematologist works closely with other physicians who specialize in blood disorders. Together, they regularly review treatment plans, discuss complex cases and determine whether new therapies or clinical trials may be appropriate. If a blood and marrow transplant is being considered, additional specialists join these discussions.

This collaborative approach allows you to benefit from the expertise of an entire multidisciplinary team.

Your treatment plan is based on many factors, including:

  • Your age
  • Your lifestyle
  • Your symptoms and how severe they are
  • How your disease has changed over time
  • Whether you have developed complications
  • Your overall health
  • Your personal preferences, including your treatment goals and interest in clinical trials

Your care team will review all appropriate options with you so that together you can decide on the treatment plan that best meets your needs.

Hear from Dr. Kleber Yotsumoto Fertrin, director of the Fred Hutch Sickle Cell Disease and Iron Overload Program, as he shares why it is important to get individualized care.

Dr. Kleber Yotsumoto Fertrin, director of the Fred Hutch Sickle Cell Disease and Iron Overload Program, shares the services offered at the Sickle Cell Disease Program.

Dr. Kleber Yotsumoto Fertrin, director of the Fred Hutch Sickle Cell Disease and Iron Overload Program, shares how the Sickle Cell Disease Program partners with local providers.

How We Treat Sickle Cell Disease at Fred Hutch

Our physicians provide the full range of standard therapies for sickle cell disease.

Medicines

Depending on your individual needs, treatment may include medicines such as hydroxyurea, L-glutamine and crizanlizumab.

Hydroxyurea

Hydroxyurea is taken daily to reduce pain episodes, decrease recurrence of acute chest syndrome and reduce the need for blood transfusions or hospital stays.

Your care team will monitor your blood counts regularly and adjust your dose as needed to help you receive the greatest benefit while maintaining a safe dose.

L-glutamine

L-glutamine is an oral medicine that may reduce the number of sickle cell crises you experience. It may help make red blood cells more flexible so they move more easily through the bloodstream.

Crizanlizumab

Crizanlizumab may be recommended for people who experience frequent pain episodes. It helps reduce vaso-occlusive crises caused by sickled red blood cells blocking blood flow.

The medication is given by intravenous (IV) infusion. Infusions are provided by nurses with expertise in infusion therapy who monitor you throughout treatment. The first two infusions are generally given two weeks apart, followed by infusions every four weeks.

Blood Transfusions

Red blood cell transfusions and red blood cell exchange are important treatment options for some people with sickle cell disease.

Transfusions may be used to treat severe anemia, acute chest syndrome and other complications. They may also help manage life-threatening conditions such as splenic sequestration (a drop in hemoglobin accompanied by a large spleen, primarily affecting young children).

Blood and Marrow Transplant

For some people with sickle cell disease, blood and marrow transplant may offer the possibility of long-term disease control or cure.

If your physicians believe transplantation may be appropriate, they will discuss the benefits, risks and what to expect so you can decide whether it is the right option for you.

Learn more about Blood and Marrow Transplant.

Gene Therapy

Gene therapy is an emerging treatment option for some people with sickle cell disease.

Fred Hutch physicians and researchers continue to advance gene therapy through research and clinical trials.

Learn more about Gene Therapy.

Kleber Yotsumoto Fertrin, MD, PhD

“With the array of medications that we have available, no sickle cell patient should be stuck with the same treatment for their whole life. We can always do better. We can always adjust over time to meet their changing needs.”

— Kleber Yotsumoto Fertrin, MD, PhD, hematologist

Complications and How They Are Managed

Treatment of sickle cell disease also includes preventing and managing complications.

Acute Chest Syndrome

Acute chest syndrome is a serious complication that can cause chest pain, cough, fever and difficulty breathing.

Treatment depends on the cause and may include oxygen therapy, medicines to treat infection or open the airways and blood transfusion.

Infection

People with sickle cell disease are at increased risk for infections such as influenza, hepatitis, meningitis and pneumonia.

Preventive measures—including recommended vaccinations, careful hand hygiene and safe food handling—help reduce this risk. If an infection develops, prompt treatment, including antibiotics when appropriate, is important.

Hand-Foot Syndrome (Dactylitis)

Hand-foot syndrome, also called dactylitis, is swelling of the hands and feet caused by blocked blood vessels.

Treatment generally includes pain medicine and increased fluids to relieve symptoms.

Pain Prevention and Management

Pain is the most common complication of sickle cell disease.

Depending on your specific pain symptoms, pain may be managed with over-the-counter medicines, prescription medicines or hospital-based treatment.

Fred Hutch has a dedicated Pain Clinic, including specialists experienced in caring for people with blood disorders. Your care team also helps you reduce the risk of painful episodes by identifying triggers, such as dehydration and extreme temperature changes.

We also recognize that people living with sickle cell disease have historically experienced disparities in pain management. Fred Hutch physicians work to improve equitable care through education, advocacy and compassionate, evidence-based treatment.

Your First Appointment with the Sickle Cell Disease Program

Our Sickle Cell Disease Program is on the fourth floor of the Sloan Clinic. Your first appointment usually takes 1.5 to 2 hours. You will spend about one hour with your physician. Here’s what you can expect to happen.

Checking Your Health

When you arrive, a medical assistant will greet you, and you will get forms to fill out. The forms help us learn important details about your condition, symptoms and current treatments, like the medicines you take. The history you share will help us understand your exact needs. We also want to know any questions or concerns you would like to discuss.

Next, you will see a hematologist, who will talk with you about your health and how sickle cell disease is affecting your body and life. They will do a physical exam. If you need any tests, like blood work, to get more information about your current health, your physician will order the tests. You can have blood drawn for laboratory tests the same day on the first floor of Sloan Clinic 1.

Discussing Your Treatment

Your physician will want to know all the treatments you have been using and how well they have worked for you. You know your body best. Tell your physician what works for you and if any treatment isn’t working the way you need it to.

Based on your exam, your conversation and any test results, your physician will explain the treatment we recommend for you and why. We want to make sure you understand all your options, including the chance of a blood or marrow transplant.

These appointments are also a time for you to tell us about yourself. Each patient and family have their own needs and preferences. We want to get to know you so we understand the best way to care for you.

Connecting You with Other Specialists

Many types of care providers are here for you as a Fred Hutch patient. Your hematologist will talk with you about other Fred Hutch experts who can help you stay as healthy and active as possible, now and over the long term. For example, we have registered dietitians to help with food choices, acupuncturists to help with pain relief and a special Pain Clinic if you have complex pain.

Along with meeting your current needs, we are here to prevent and manage serious complications that can happen. We will help connect you with other specialists you may need to see, like physicians who specialize in the eyes (ophthalmologists), heart (cardiologists), kidneys (nephrologists) and digestive system (gastroenterologists).

Answering Your Questions

Starting with your first appointment (and after), we are here to answer your questions. We want to help you understand as much as you want to know about your disease, your treatment and how care happens at Fred Hutch. We invite you to bring a friend or family member with you to help keep track of your questions and the information that your team gives you.

We also encourage you to talk with your care team about your hopes and concerns. Knowing more about you helps your team recommend the right treatment for you. Before you leave, we will make sure you know what is going to happen next and how you can reach us if you have questions later. We will also schedule your next visit.

Many new patients have blood tests the same day as their first appointment. Then, they will return soon for another visit to talk about the results and how the results might affect their treatment choices.

Meeting With a Social Worker

Often, new patients meet with a social worker from our team the same day as their first appointment with their hematologist. Social workers are a resource for you and your family. They can help with many parts of living with sickle cell disease and getting the health care you need. Part of their role is to help patients get mental health support and manage the financial challenges that can come with a serious illness.

We understand that starting care with a new team at a new clinic can be complex. It sometimes feels overwhelming. Your social worker will help you get to know our system. They are by your side, making it easier to find the places you need to go and the providers you need to see.

Dr. Kleber Yotsumoto Fertrin, director of the Fred Hutch Sickle Cell Disease and Iron Overload Program,  shares what can be expected at a first appointment.

Monitoring Your Health

Throughout treatment, your care team will regularly evaluate:

  • How well your treatment is working
  • Whether changes to your treatment plan are needed
  • Whether complications are affecting your heart, lungs, kidneys, liver, spleen, eyes or other organs
  • Whether supportive care services may benefit you

As new therapies become available and your health changes over time, your treatment plan will continue to evolve.

Managing Side Effects

Your physicians, nurses and advanced practice providers work closely with you to prevent, monitor and manage any side effects you may have throughout treatment.

Supportive therapies may include:

Tell your care team about any side effects you experience. If questions arise between visits, you can contact your team for help.

Get Help with Side Effects

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

Common Side Effects

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)

Supportive Care

Treating sickle cell disease means caring for the whole person.

Fred Hutch offers supportive care services that address your physical, emotional and practical needs throughout treatment. Depending on your situation, your supportive care team may include registered dietitians, physical therapists, social workers, psychologists, pharmacists, Spiritual Health providers and other specialists.

Caregiving During Treatment

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

Caregiving for Transplant Patients

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.

Continuing Care

Sickle cell disease requires lifelong follow-up.

Your follow-up schedule is individualized based on your treatment plan and your health needs. At each visit, your care team will review your progress, monitor your health and discuss any adjustments to your treatment.

If you live outside the Seattle area, we will work closely with your local health care providers to coordinate your ongoing care.

Schedule For Follow-up Visits

Just like we personalize your treatment plan for you, we personalize your follow-up schedule, too. Usually, people taking hydroxyurea have visits at least four times a year. People on crizanlizumab are seen once a month before their infusion. If you need a high dose of pain medicine, your team may want to see you every two weeks to check how well your pain is being managed. Your hematologist will base your schedule on many factors, including how your disease and the treatments are affecting you.

What Happens at Follow-up Visits

Follow-up for sickle cell disease usually means seeing your hematologist for a physical exam and having blood tests. It’s also important to see specialists who can check other parts of your health, like your eyes, lungs and heart.

Together, you and your care team will go over the benefits and risks of any follow-up options and decide what is right for you.

Pediatric to Adult Care for Sickle Cell Disease

Young adults with sickle cell disease will transition from pediatric care to adult care with hematologists at Fred Hutch who specialize in treating adults with this disease. Learn more about how this works.