Sickle Cell Program
- Providers, call 206-987-7263 to refer a patient.
What is sickle cell disease?
Sickle cell disease is a group of blood conditions that affect hemoglobin (HEE-moh-GLOH-bin), the part of red blood cells that delivers oxygen to cells in the body. The genes that cause the disease are passed from parent to child.
Healthy red blood cells are round, soft disks. In sickle cell disease, red blood cells start out normal but become curved, shaped like a sickle. They are hard and sticky and can clog blood vessels. This causes pain and blocks blood flow.
Sickle cells do not last as long as healthy red blood cells. The body cannot make new red blood cells fast enough to replace them. This can cause low levels of red blood cells (anemia). One of the most serious forms of the disease is called sickle cell anemia.
In the United States, most people with sickle cell disease are of African or Hispanic heritage. But the disease can affect anyone, especially people of southern European, Middle Eastern or Asian Indian heritage.
Sickle cell disease is caused by a change (mutation) in genes for hemoglobin.
- Having sickle cell disease means a person received the sickle cell gene or other abnormal hemoglobin genes from both parents.
- Having sickle cell trait means a person received the sickle cell gene from only 1 parent. People with sickle cell trait are not sick. But they do carry the sickle cell gene and can pass it on to their children.
Sickle Cell Disease at Seattle Children's
Our multidisciplinary team works to help assure children with sickle cell have full, active lives. Seattle Children’s Sickle Cell Program brings together experts from many fields to care for all aspects of your child’s health. We work to support your child at home, at school and in the hospital. We teach about the disease, give preventative screenings and provide the most up-to-date treatments, including cure with stem cell transplant.
We work with you, your child, your family and your child's primary provider to get your child the right care and services. We provide most of our care and teaching at Seattle Children’s Odessa Brown Children's Clinic (OBCC) in Seattle's Central District.
Dr. M.A. Bender, our program director, is board certified in pediatric hematology-oncology. This means he is approved to give the specialized care your child needs and constantly expands his knowledge about blood disorders.
Our team includes a social worker and psychologist to help your child and your family through the challenges of sickle cell.
Genetic counseling and testing can help parents and other family members understand the chance of having the sickle cell gene or passing it on. Seattle Children’s genetic counselors can advise you about genetic testing and explain test results.
We offer a full range of treatments to keep your child as healthy as possible and help them control pain. Most often, we see patients at OBCC. But if your child has complications, we will care for them at Seattle Children’s hospital campus.
To help avoid complications and reduce pain, we focus on education on how to prevent problems. We also treat children with medicines and blood transfusions. Our treatment options include stem cell transplants, which can cure this disease.
Dr. M.A. Bender leads the Northwest Sickle Cell Collaborative (NWSCC). The group works to improve education about the disease, streamline screening and make sure that people with sickle cell across the state get the best possible care. He plays a lead role in the Pacific Sickle Cell Research Collaborative, a federally funded project to define quality care for people with sickle cell disease and overcome barriers to it.
Bender is part of a team working to assess the clinical and economic benefits of cures for sickle cell disease over the lifetime of patients. The project is funded by the National Institutes of Health and aims to clarify the long-term benefits of gene therapies for sickle cell.
In the lab, Bender studies the molecular biology of the abnormal genes that cause sickle cell. Other lab research may lead to better understanding of small strokes that affect many children with sickle cell disease. Dr. Andy Shih uses a mouse model of sickle cell disease to study tiny strokes called microinfarcts. Although the strokes are too small to cause obvious symptoms, Shih’s team has shown that they damage the brain. His team is learning about the effect of tiny strokes on brain tissue and nerve signals. Read more.
Having sickle cell disease can be stressful for the entire family. If your child has an urgent need, we will see them right away. If needs are not urgent, new patients can be seen in a month or sooner.
During visits, we take time to explain your child’s condition. We help you fully understand your treatment options and make the choices that are right for your family.
Our team cares for your whole child. We don’t just treat their disease. We connect you to community resources and support groups.
We can support you and your child with financial counseling, schooling, housing, transportation, legal aid, interpreter services and spiritual care. Read about our services for patients and families.
Our sickle cell team provides services throughout Washington, Alaska, Montana and Idaho. Even if you live too far away to visit us in person, we can consult with your family and child’s doctors so your child gets the best care possible. We want to help families with sickle cell get whatever they need, wherever they are.
Children with sickle cell disease do best when they, their families and their schools know about their disease and how to take care of it. We work with schools and community groups to increase understanding of sickle cell disease.
Symptoms of Sickle Cell Disease
Some children with sickle cell disease have only mild symptoms. For others, symptoms can be severe.
Symptoms of sickle cell disease include:
- Pain, which can be extreme.
- Feeling tired.
- Breathing problems.
- Having severe infections.
- Losing balance or control of parts of the body (stroke).
- Yellow color in the whites of the eyes. Children with lighter skin tones may have yellowish skin. This is called jaundice. It happens if the liver cannot filter blood fast enough.
Diagnosing Sickle Cell Disease
All newborns in the United States get a blood test right after birth that checks for sickle cell disease. In Washington, newborns diagnosed with sickle cell are referred to specialty care right away. Bender works closely with the state’s Newborn Screening Program to assure all babies get the care they need.
If your child was not screened as a newborn, they can get a blood test at any time. This is very important for children who have a higher risk of the disease because they are of African or Hispanic descent or have family members with sickle cell disease or trait.
Knowing your baby has sickle cell helps get support and care before problems begin.
Pregnant women can have a test that checks for sickle cell disease before their baby is born. The test looks for the genes that cause sickle cell disease. The test checks a sample of amniotic fluid or cells from the placenta.
If your child has not been tested for sickle cell disease but has symptoms like pain or extreme tiredness, the doctor may check your child’s blood for:
- The type and amount of hemoglobin in the red blood cells
- The number of red blood cells in the blood and their size and shape
- The abnormal genes that can cause sickle cell disease
If you have a family member with sickle cell trait or sickle cell disease, there is a chance that other family members may have the trait too. People with sickle cell trait have 1 abnormal hemoglobin gene. People with sickle cell disease have 2 copies of the gene that causes the disease.
If you are concerned about passing the sickle cell gene on in your family, you can get a blood test to see if you carry it.
Whether or not you want this test, you can talk with a doctor to learn more about your risk of having the trait or passing it to your children. This is called genetic counseling. It can help you make informed decisions about family planning and your child’s treatment.
Treating Sickle Cell Disease
We watch your child’s health closely and recommend the best treatments for them. Keeping careful track of their health ensures your child gets the care they need before more serious problems start. That helps them feel better and gives them the best chance of successful treatment.
“Even if we need to call for help at 3 a.m., we know our medical team is there for us. They’re like our second family.”
Watch Saleea’s story (Video 3:40)
A loving father describes the journey since Saleea was diagnosed with sickle cell disease at 2 weeks old.
Regular check-ups are an important way to keep your child healthy. Most children with sickle cell disease visit the doctor every 3 to 12 months. The timing depends on their age, health and the treatments they are using. Some children need to see a doctor more often.
At clinic visits, we may check your child’s:
- Height and weight: to make sure your child is growing at a healthy rate.
- Blood: to count red blood cells and to look for signs of organ damage.
- Pee: for signs of kidney damage.
- Blood flow: to see if your child is at risk for stroke. We use sound waves (ultrasound screening) to check how blood is flowing.
- Lungs: to see how well they are working.
For children who live outside our area, we consult with families and doctors about check-ups and preventive care.
We help our patients, families and their schools know about their disease and how to take care of it. We teach about:
- Preventing infection
- Preventing and coping with pain
- Avoiding hospital stays
- Warning signs to watch for
- What do to when problems arise
- How to live a full and active life
Pain is a common problem, caused by sickle cells blocking the blood flow to an organ or bones.
We work with you and your child to plan how to deal with pain. The plan may include:
- What to do when new pain starts.
- How to deal with ongoing pain, if this affects your child.
- Ways to manage pain without medicine. These include drinking fluids, massage, deep breathing, relaxation exercises, heating pads, physical therapy and acupuncture.
- Medicines to manage pain. These include acetaminophen (Tylenol), nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen (Motrin or Advil) and stronger medicine like morphine. Newer medicines that cut down the number of pain episodes include hydroxyurea (hy-drahk-see-yoo-REE-uh) and L-glutamine (Endari).
- How to know when to contact us about your child’s pain. We may be able to help you manage pain at home or tell you to go to the hospital.
If pain cannot be controlled at home, your child may need stronger medicine and fluids through a vein, called intravenous (IV). They may need to stay overnight at the hospital to bring pain under control.
In children with sickle cell disease, the spleen does not work right or does not work at all. That makes it harder to fight off some germs. Because of this, infections that would be minor in other children can be very severe.
Most young children will need to take the antibiotic penicillin daily to help prevent infection. Your child also will need to:
- See a doctor right away to get antibiotics any time they have a fever (over 38.5° C or 101.3° F). Fever is a medical emergency for children with sickle cell disease. Sometimes children need to stay in the hospital for care.
- Stay up-to-date on all recommended vaccines.
- Get extra vaccines to protect against flu, lung infection (pneumonia), infection of the brain and spinal cord (meningitis) and other serious infections.
Some children with sickle cell disease have very low levels of red blood cells (severe anemia). This can be life-threatening. It can happen if red blood cells get stuck in the spleen or if the bone marrow stops making red blood cells because of an infection.
See a doctor right away if your child has symptoms such as:
- Shortness of breath
- Being very tired
- Feeling dizzy
- Having paler-than-normal skin
Your child may need a transfusion of red blood cells from a healthy donor. Some children need hospital treatment.
If sickle cells clog blood vessels in the lungs, it can damage the lungs.
See a doctor right away if your child has these symptoms:
- Chest pain
- Shortness of breath
- Fast breathing
Your child will need to be carefully watched and treated in the hospital with:
- Blood transfusion
Sickle cell disease increases a child’s chance of having a stroke.
At yearly check-ups, your doctor will use sound waves (ultrasound) to check your child’s blood flow. If there are problems, your doctor may recommend regular blood transfusions to improve blood flow and reduce the chance of having a stroke.
Call 9-1-1 if your child has symptoms of stroke:
- Weakness on 1 side of the body
- Trouble speaking, walking, or understanding
- A lopsided smile
- Problems with balance
- Painful headache
If your child has had a stroke, their treatment may include:
- An “exchange transfusion.” Doctors slowly remove some of your child’s blood and replace it with blood from a healthy donor.
- Monthly blood transfusions.
- Medicine or other treatment to help prevent another stroke.
- Physical therapy or occupational therapy (PDF) if needed to help with your child’s movement, breathing and activities of daily life.
We regularly check your child’s thinking skills. This is called neurocognitive testing. It can show areas where your child may need extra help in school or at work.
There are many possible reasons for problems with learning or decision-making in children with sickle cell disease. One cause is small strokes, called “silent strokes,” that can be seen on an MRI (magnetic resonance imaging) scan of the brain.
For certain types of sickle cell, the doctor will recommend a medicine to reduce or prevent some of the complications of the disease. The options include hydroxyurea (hy-drahk-see-yoo-REE-uh) and voxelotor (Oxybryta).
Transfusing blood means giving red blood cells from a healthy donor. Your child receives the blood through a vein in their arm.
A transfusion helps to:
- Raise the number of healthy red blood cells
- Reduce blockage in blood vessels
- Deliver more oxygen to the tissues and organs
- Dilute the sickle cells
Doctors give blood transfusions:
- To treat complications that cause severe anemia
- If a person has had a stroke or is at higher risk for stroke
- After serious breathing problems
- Before surgery to prevent complications
- To treat complications that do not improve with the medicine hydroxyurea
Our outpatient infusion unit at Seattle Children’s hospital campus is staffed by expert nurses and has weekend hours. This helps your child get care without having to spend a night in the hospital.
Some children may be cured of sickle cell disease by a transplant of blood-forming stem cells from a healthy donor. This treatment is called a stem cell transplant or bone marrow transplant.
A transplant helps a child’s bone marrow make healthy red blood cells that do not become sickle-shaped. With healthy red blood cells, a child is no longer at risk for pain, severe anemia, infections or other complications.
If transplant may be an option for your child, we will talk with you about the risks and benefits. We also talk with you about whether transplant fits with your family’s values, goals and priorities.
Our Non-Malignant Transplant Program specializes in stem cell transplants for children with blood disorders like sickle cell disease. We do the transplants here at Seattle Children’s, working closely with Fred Hutch, one of the most experienced stem cell transplant centers in the world.
We help our patients keep getting the care they need as they become adults. We work to make sure there is a smooth transition to the adult sickle cell clinic at the Seattle Cancer Care Alliance.
Where and How We Help
In addition to in-person visits, we use video phone calls (telemedicine) to check on patients and families and provide education and advice.
We provide care to children with sickle cell disease at Odessa Brown Children's Clinic (OBCC), in Seattle’s Central District. You can get care at the clinic even if you get most of your healthcare elsewhere. We can work with your child’s other doctors to coordinate care.
If your child needs hospital treatment, they will get care at Seattle Children’s hospital campus.
For children who live in other parts of the state and get most of their care from doctors and hospitals close to home:
- You may visit us at OBCC
- Your families and doctors can consult with us
- We can help coordinate your care
Most summers we hold free camps for children with sickle cell disease. Camps take place on Vashon Island in Puget Sound.
We work with children, families and doctors from other places in the Northwest to learn about children's needs and care.
We get information from children’s families and doctors about:
- Their health
- Their care
- The effects of sickle cell disease on their life at home and school
Based on this information, we check how their bodies are working. We also check to see if:
- They are getting the exams and tests they need
- They are getting the treatments they need for fevers and pain
- They are getting care for any problems with their breathing, heart or nervous system
- Their families know signs to watch for and what to do if their child shows signs that they need other treatment
- Their schools and doctors at home have the information they need about the disease
- Their families are connected to a hospital near home where doctors know about sickle cell disease
Our team works with professionals from other fields to plan treatment and to care for your child.
- Providers, call 206-987-7263 to refer a patient.