The sickle cell program at Johns Hopkins All Children’s Hospital provides comprehensive pediatric care to patients from birth to age 21. As one of the largest pediatric sickle cell programs on Florida’s west coast, we help families deal with challenges when their child has sickle cell disease. Since sickle cell is a chronic disease that lasts a lifetime, our experts also specialize in transitioning pediatric patients to adult care. Children with sickle cell still can live a healthy and active life.
Sickle cell disease can have a variety of conditions, and we collaborate with medical specialists throughout our hospital, including hematologists, neurologists, neurosurgeons, cardiologists, nephrologists, pediatric surgeons, anesthesiologists and pharmacists. We also work with Child Life specialists, social workers, school teachers and psychologists to help our patients and families cope with the psychosocial impacts of sickle cell. Symptoms and their severity vary widely among children. We offer routine preventative care, disease education and emergency care for our patients in outpatient and inpatient settings. We also offer consultations with our physical and occupational therapy teams to help keep our patients as strong and active as possible. Knowing that stem cell transplant can provide a cure, we work closely with our bone marrow transplant team to refer patients for transplants when needed.
What is Sickle Cell Disease?
Sickle cell disease is the most common inherited blood disorder and is often found during a routine newborn blood test. It may also be diagnosed before birth with an amniotic fluid test or a sample of tissue from the placenta.
It exists in many forms, depending on what hemoglobin is affected. The most common forms:
- Sickle cell anemia (HbSS): The child has two copies of the Hbs gene, one inherited from each parent. This is marked by reduced oxygen flow through the body resulting in fatigue, skin pallor, shortness of breath, lightheadedness, dizziness or a fast heartbeat.
- Sickle cell with hemoglobin C disease (HbSC): The child has one HbS gene from one parent and one defective hemoglobin C gene from the other. This is generally a less severe form of anemia with similar but milder symptoms.
- Sickle beta thalassemia (HbS beta-thalassemia): The child has one HbS gene and one beta thalassemia gene. Sickle beta thalassemia zero is very similar to sickle cell anemia. A person can exhibit symptoms of varying severity.
- Other forms of sickle cell disease (HbSE, HbSO and HbSD) the child has one HbS gene and another abnormal hemoglobin gene. Symptoms vary depending on the specific gene defect.
Sickle cell disease affects families of a variety of ethnicities but primarily affects children of African descent, Hispanics and those of Caribbean ancestry. It also can affect children of Middle Eastern and Indian descent. It is rarely found in European descent. A child develops sickle cell disease when both parents have sickle cell trait—an abnormal gene—and both pass that gene to the child.
Sickle cell disease impacts the red blood cells that carry oxygen to the body’s tissues. It is caused by a change in hemoglobin genes that carry oxygen inside red blood cells. Normal red blood cells are smooth, flexible discs shaped like the letter O and move easily through a child’s blood vessels. Sickle cells, on the other hand, are stiff and sticky and are often shaped like the letter C. They tend to cluster together at the lining of blood vessels, making it difficult for them to move through small blood vessels. These clusters can create blockages and can stop the movement of healthy, oxygen-carrying blood. This blockage decreases oxygen delivery to the tissues and organs, which can cause pain and organ dysfunction. It is also what causes the majority of sickle cell complications such as anemia, pain, acute chest syndrome and stroke.
Sickle Cell Care at Johns Hopkins All Children’s Hospital
Treatment and Preventative Care
- Hydroxyurea therapy. This preventative oral medication causes the body to increase the production of fetal hemoglobin and therefore decrease the production of sickle cells. This medication is not a cure for sickle cell, but helps to significantly decrease the risk of life threatening complications such as stroke and acute chest syndrome. It can also decrease the frequency of vaso-occlusive pain crisis and decrease the risk for organ damage that can occur later in life. With hydroxyurea, there is a decrease of pain crises, hospitalizations and episodes such as acute chest syndrome or dactylitis.
- Penicillin. Sickle cell patients have decreased function of their spleen, an organ that filters the blood cells and helps remove bacteria from the blood. For many of our patients, taking penicillin twice daily until at least the age of five years old in addition to completing all routine childhood vaccinations and pneumonia and meningitis vaccinations can help a child with sickle cell disease stay healthier and decrease their risk of those illnesses.
- Blood transfusions. Blood transfusions may be given acutely in order to treat severe anemia, acute chest syndrome and other life-threatening complications of sickle cell disease. Some children with sickle cell disease may receive chronic blood transfusions to prevent or manage complications such as stroke.
- Iron chelators. Sickle cell anemia patients who require chronic red blood cell transfusion to prevent or manage stroke gradually become iron overloaded. Without removal of iron, it can build up and become toxic to several vital organs such as the heart and the liver. Iron can be removed from the body by medications called chelators. Children may take chelators orally or by injection in order to remove excess iron from the body. Specific treatments may be based on your child’s symptoms.
- Transcranial Doppler Ultrasound. Primary prevention of stroke is accomplished with transcranial doppler ultrasound done yearly starting at the age of two until the age of 16 in the most common types of sickle cell disease.
- Stem cell transplant. The only available cure for sickle cell disease at the current time is bone marrow transplant.. In sickle cell disease, the best transplant outcomes are almost always when the donor is a healthy sibling with compatible stem cells. The stem cells replace the sickle blood stem cells and restore normal blood production in children with sickle cell disease. Our team of transplant doctors and hematologists are working closely to participate in current nationally available transplant studies.
- L Glutamine (Endari). Endari is one of the latest drugs approved by the FDA to help in the management of sickle cell disease. Our center will be offering it to patient in addition to Hydroxyurea for management of the complications related to sickle cell disease.
Our dedicated Pain Clinic works with health care teams, patients and families to provide an individualized pain management plan for each patient. The plan may include pain medicine, physical therapy or complementary therapies such as massage therapy and hypnosis. Our goal is to treat the patient's pain and help him or her continue to participate in daily activities.
Our acute pain service team focuses on specialized sickle cell care that includes:
- Comprehensive pain management (pharmacological and non-pharmacological interventions)
- Inpatient consultation with the Acute Pain Service
- Outpatient chronic pain management
Sickle Cell Clinic
The sickle cell program offers outpatient care regularly through our Sickle Cell Clinic in St. Petersburg, Tampa and Lakeland. Services include routine surveillance care, collection of lab specimens, treatment of acute pain episodes (main campus and Tampa), treatment of fever (main campus and Tampa), echocardiogram (all locations with advance scheduling), transcranial doppler ultrasound (main campus with advance scheduling), MRI (main campus and Tampa with advance scheduling).
Sickle Cell Transition Program
The Johns Hopkins All Children’s sickle cell transition program, SCULPT, offers patients with sickle cell disease medical and psychosocial care in addition to emotional and educational support. Our team of experts consists of a physician, nurse, psychologist, transition coordinator and a social worker who work closely with patients to create an individualized transition plan.
For information or to schedule a new appointment, please call us at 727-767-4176.
Outpatient Care Center, Main
Outpatient Care Center, Tampa
Outpatient Care Center, Lakeland
Read inspiring stories about our Sickle Cell Program
A mom of two boys with sickle cell disease fights for a better life for her sons.
Joao is thriving after a bone marrow transplant to treat his sickle cell disease.
Desmond was born with sickle cell anemia. After suffering a stroke at 4 years old, he and his family found Johns Hopkins All Children’s Sickle Cell Program and began a near life-long relationship with the hospital.
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