Sickle Cell Disease

What is sickle cell disease?

Sickle cell disease is a group of blood conditions passed from parents to children (inherited). In most cases the parents also have the condition.

Healthy red blood cells are round, soft disks — like doughnuts without holes. In sickle cell disease, red blood cells start out normal. But if they are stressed, they become curved, shaped like a sickle. They are hard and sticky and can clog blood vessels. This can cause pain and block blood flow.

Sickle cells don’t last as long as normal red blood cells. The body can’t make new red blood cells fast enough to replace them. This can cause low levels of red blood cells (anemia). That makes it harder to deliver oxygen around the body. 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 African-American. But the disease can affect anyone, especially people of Hispanic, southern European, Middle Eastern or Asian Indian backgrounds.

  • Sickle cell disease is caused by a change (mutation) in a gene that carries directions for making the oxygen-carrying part of red blood cells (hemoglobin). The sickle cell gene is passed down in families. 

    • 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

Experts at our Sickle Cell Program care for children with sickle cell disease, help them control pain and avoid serious complications from the disease. We also help family members understand their chance of passing on the sickle cell gene to their children.

Our goals are to achieve the best possible outcome for your child and to lessen how much the illness affects them. We work with you, your child, your family and your child’s primary doctor to get your child the right care and services.

We provide most care at Odessa Brown Children’s Clinic (OBCC) in Seattle’s Central District. If your child needs a hospital stay, they will get care at Seattle Children’s main campus in Seattle’s Laurelhurst neighborhood. We also consult with patients, families, doctors and school staff who are too far away to visit us in person.

You can call 206-987-7263 for more information from the sickle cell advanced practice provider at OBCC. Call 206-987-7402 for an appointment.

  • Our team includes a doctor and nurse practitioner with special training in the needs of children with blood disorders. Dr. M. A. Bender, the clinic director who guides your child’s care, is board certified in pediatric hematology. This means he is approved to give the specialized care your child needs and constantly adds to his knowledge about blood disorders.

    We offer a full range of treatments for children with sickle cell disease – including medicines such as hydroxyurea (hy-drahk-see-yoo-REE-uh), blood transfusions and stem cell transplants. We help your child control pain and avoid serious complications.

    We care for your whole child. We don’t just treat their disease. Your child’s team includes a nurse and social worker. Seattle Children’s genetic counselors help parents and other family members learn about their risk of having the sickle cell gene or passing it on.

    Our specialty is treating children's conditions while helping them grow up to be healthy and productive. For the 25th year in a row, Seattle Children’s ranks among the nation's best children's hospitals, according to U.S. News & World Report.

  • Having sickle cell disease can be stressful for the entire family. 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 social workers help your child and your family through the challenges of this condition. We connect you to community resources and support groups.

    We can support you and your child with financial counseling, schooling, housing, transportation, interpreter services and spiritual care. Read about our services for patients and families.

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 (a 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 can’t filter blood fast enough.

Diagnosing Sickle Cell Disease

Blood tests can show whether your child has sickle cell disease.

  • Newborns in the United States are tested for sickle cell disease right after birth. This helps parents find out if their newborn has sickle cell disease even before they show any symptoms.

    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.

    Knowing your baby has sickle cell disease means they can 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. It checks either: 

    • A sample of amniotic fluid that surrounds the baby in the womb
    • Cells from the placenta, which grows in the womb and connects the baby to the mother
  • If your child has not been diagnosed with sickle cell disease but has symptoms like pain or extreme tiredness, the doctor may do blood tests to check: 

    • The type and amount of hemoglobin (HEE-moh-GLOH-bin) in the red blood cells
    • The number of red blood cells in the blood and their size and shape
    • If there are 2 abnormal genes that can cause sickle cell disease
  • If you have a parent, sibling or child with sickle cell trait or sickle cell disease, there’s a chance that you or other family members may have the trait, too. Sickle cell trait means having 1 copy 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 simple blood test for sickle cell trait.

    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.

    We offer genetic counseling and testing for sickle cell trait at Seattle Children’s main campus.

Treating Sickle Cell Disease

There are many steps to treating children with sickle cell disease.

  • Regular check-ups are an important part of keeping healthy. Most children with sickle cell disease visit the doctor every 3 to 12 months, depending on their age and health. Babies and children with certain conditions 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 there’s a higher risk of stroke. This is done using sound waves (ultrasound screening). 

    For children who live outside our area, we consult with families and doctors about check-ups and preventive care.

    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 teach about: 

    • Preventing infection
    • Coping with pain
    • Avoiding hospital stays
    • Warning signs to watch for
    • What do to when problems arise
  • Pain is a common problem for people with sickle cell disease. It happens when sickle cells block the blood flow to an organ or bones. 

    • Pain that comes on quickly and lasts for hours to days is called acute pain.
    • Episodes of sharp pain that repeat are called recurrent pain.
    • Ongoing pain that lasts for weeks or months is called chronic pain. 

    We work with you to plan how to deal with pain. The plan may include: 

    • What to do when new pain starts, such as drink lots of fluids, take a hot bath, use warm packs and take medicine your doctor recommends. Medicine options include acetaminophen (Tylenol), non-steroidal anti-inflammatory drugs (NSAIDs) like ibuprofen (Motrin or Advil), or stronger medicine like morphine.
    • How to deal with ongoing pain if your child suffers from chronic pain. We focus on nondrug ways to control pain, but some children may need to take medicine daily.
    • Ways to manage pain without medicine. These include massage, deep breathing, relaxation exercises, heating pads, physical therapy and acupuncture.
    • 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 can’t 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 correctly 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 prevent infection. Your child also will need to: 

    • See a doctor right away to get antibiotics any time they have a fever (over 38.30 C or 101.00 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 happen if red blood cells get stuck in the spleen or if the bone marrow stops making red blood cells because of an infection.

    Symptoms of severe anemia include: 

    • Shortness of breath
    • Being very tired
    • Feeling dizzy
    • Having paler-than-normal skin 

    See a doctor right away. This can be life threatening. 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, your child won’t get enough oxygen. This can cause lung damage.

    Your child may have these symptoms: 

    • Chest pain
    • Fever
    • Shortness of breath
    • Fast breathing
    • Coughing 

    See a doctor right away. Your child will need to be carefully watched and treated in the hospital with: 

    • Antibiotics
    • Oxygen
    • 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 for problems. If the test is abnormal, your doctor may recommend regular blood transfusions to improve blood flow and reduce the chance of having a stroke.

    Call 911 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.
  • 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 on the job.

    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.

  • Your doctor may recommend a medicine called hydroxyurea (hy-drahk-see-yoo-REE-uh) to reduce or prevent some of the complications of sickle cell disease. Your child may need this medicine if they have had: 

    • Acute pain episodes
    • Severe anemia
    • Lung problems
    • Stroke 

    Your child takes the medicine daily by mouth.

  • 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 

    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 related to sickle cell disease
    • To treat complications that do not improve with the medicine hydroxyurea 

    Our outpatient infusion unit at Seattle Children’s main hospital 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 (hematopoietic) stem cells from a healthy donor. Hematopoietic (him-at-oh-poy-EH-tik) stem cells are immature cells that grow into blood cells. This treatment is called a stem cell transplant or bone marrow transplant.

    A transplant helps your 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 and other noncancer conditions. Children need powerful drugs or radiation (called conditioning) to prepare their body for the transplant. Our team has created better ways to prepare patients and help them recover. This preparation is called reduced-intensity conditioning.

    We perform the transplants here at Seattle Children’s, working closely with Fred Hutch, our partner in the Seattle Cancer Care Alliance.

Contact Us

For an appointment, call 206-987-7402. For more information about our services, call 206-987-7263 to reach the OBCC sickle cell advanced practice provider.

We encourage you to coordinate with your pediatrician or family doctor when coming to Seattle Children’s.

Providers, call 206-987-7402 to refer a patient.