Sickle cell disease is a genetic blood disorder that affects hemoglobin in red blood cells. Hemoglobin is a protein that carries oxygen to all parts of the body. Normal red blood cells are round and smooth; in a child with sickle cell disease, red blood cells are hard, sticky and shaped like a crescent, or sickle.
What are the three most common types of sickle cell disease?
- Hemoglobin SS disease (also called sickle cell anemia)
- Hemoglobin SC disease
- Sickle cell beta thalassemia
Pediatric Sickle Cell Disease Complications
Sickle-shaped red blood cells stick together and block blood vessels, which can cause complications like:
- Acute chest syndrome: Sudden damage to lung tissue is common in children with sickle cell disease.
- Aplastic crisis: The body stops making red blood cells for a short time, causing a drop in your child’s red blood count.
- Fever and infection: A fever is usually a sign of infection. Infections are a medical emergency for a child with sickle cell disease.
- Gallstones: A child with sickle cell disease has more bile (a liquid used to break down the fats we eat) than a child’s gallbladder can hold. The extra bile causes gallstones. Gallstones can cause jaundice, which is when the skin or eyes are yellow in color.
- Jaundice: Fragile sickle cells can break open, leaking bilirubin (a yellow pigment in bile) into the bloodstream and causing the skin and eyes to turn yellow.
- Nephropathy: Sickle red blood cells can get trapped inside the blood vessels of the kidneys and cause kidney damage.
- Pain: Sickle red blood cells can get stuck and block the flow of blood to parts of the body, such as the bones, lungs, spleen, brain, eyes and kidneys, causing pain.
- Retinopathy: Sickle cell disease can damage the retina in the eyes.
- Splenic sequestration crisis: The spleen suddenly gets swollen because sickle red blood cells block the blood vessels inside the spleen. This can lead to shock and is an emergency for children with sickle cell disease.
- Stroke: A blockage or bleeding of the blood vessels can cause a loss of blood supply to part of the brain, causing a stroke. Strokes occur more frequently in children ages 2 to 5 but can occur at any age.
At the Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta, our focus is on prevention, treatment and follow-up care for pediatric sickle cell disease and sickle cell disease complications in kids. We offer the following services to our patient families:
- 24-hour acute care.
- Health maintenance services.
- Comprehensive clinics at three sites in metro Atlanta.
- Specialty clinics.
- Sickle Cell Pain Treatment Program.
- Transfusion services.
- Newborn screening and follow-up.
- New patient evaluations.
- Genetic counseling (helping parents understand the sickle cell trait and how it can be passed along to their children).
- Patient counseling and education (teaching families how to prevent infections, pain and other health problems related to sickle cell disease).
- Teachers to help prevent school disruption from missed days.
- Child life services to help with anxiety during procedures or interactions.
- Social work services to provide outside resources.
Just as important, we offer innovative services, technology and research to manage—and even cure—sickle cell disease:
- Blood and marrow transplant (BMT): Also referred to as a bone marrow or stem cell transplant, this is the only known cure for sickle cell disease. We are a national leader in transplanting stem cells for children with sickle cell disease.
- Hydroxyurea: This medicine can help reduce sickle cell symptoms and improve quality of life. It helps red blood cells stay soft, round and slippery.
- Transcranial doppler (TCD) screening: TCD screening evaluates patients for stroke risk.
- Clinical trials: We are searching for better treatments, preventive measures and cures through our research efforts.
Read Stories of Hope and Inspiration From Our Patients at the Aflac Cancer and Blood Disorders Center.
Ariana Finds a Happy Distraction from Sickle Cell Disease
While recovering from a blood and marrow transplant (BMT), this sickle cell disease patient discovered a love of cooking.
Our Sickle Cell Disease Program is the largest pediatric program in the country, caring for more than 2,000 children and young adults. Combining the latest proven technology and research with a caring, child-friendly approach makes the Aflac Cancer and Blood Disorders Center a top choice for the treatment of sickle cell disease.
- We are a member of the Atlanta Sickle Cell Consortium, a citywide collaboration of physicians and researchers advancing the treatment of sickle cell disease and trying to develop a cure.
- We have been recognized as a national leader in transplanting patients with sickle cell disease through BMT, treating nearly 100 children.
- At each of our locations, a specialized multidisciplinary team of pediatric hematologists, nurse practitioners, nurses, social workers, psychologists, teachers and chaplains provides care to our patients.
At the Aflac Cancer and Blood Disorders Center, we are working vigorously to further our mission of making kids better today and healthier tomorrow. This rallying cry is the driving force behind our dedication to pediatric sickle cell disease research. Our team works with experts and treatment centers nationwide to conduct clinical research studies with a focus on improving quality of life and reducing complications for children and young adults living with sickle cell disease.
- Current annual funding for sickle cell disease research from federal agencies, pharma and private foundations totals $5.4 million in direct costs.
- Our annual research funding from federal agencies and private foundations exceeds $4 million and includes an Excellence in Hemoglobinopathies Research Award funded by the National Institutes of Health (NIH) to develop new treatments for sickle cell disease.
- We are currently evaluating two new medicines to reduce the severity and duration of episodes of severe pain. We are leading an international study on the long-term outcomes and late effects of BMT for patients with sickle cell disease.
- We have led more than 40 institutions nationwide in the Sickle Cell Transplant to Prevent Disease Exacerbation (STRIDE) study, investigating the role of BMT in providing safe and effective cures for young adults with sickle cell disease.
- We have status as one of a select group of centers offering gene therapy for sickle cell disease.
- Our team is leading a study, funded by the National Heart, Lung and Blood Institute (NHLBI), with institutions throughout the country. The aim of the study is to determine whether arginine infusions will shorten sickle cell disease pain.
FDA Approves Sickle Cell Disease Drug for Use in Young Children
A new drug targeting the underlying cause of sickle cell disease that was tested at Children’s was recently approved by the FDA for patients ages 4 and older. The treatment increases hemoglobin levels in red blood cells, which is associated with improved quality of life.read more
Pediatric Emergency Physician Receives $8.7 Million From NIH to Study Arginine Therapy for Sickle Cell Disease Pain
“If successful, arginine would be an important addition to the drugs available to decrease sickle cell disease pain, as it may reduce or eliminate pain that has already started,” said Carlton Dampier, MD, Co-Investigator and Professor of Pediatrics at Emory University School of Medicine.Read More
A sickle cell disease diagnosis for your child can be an emotional and overwhelming experience. At the Aflac Cancer and Blood Disorders Center, our number one priority is supporting you and your family. We make it our mission to provide the best care—and best experience—for every child we treat with sickle cell disease or complications of sickle cell disease. As a parent, you play an important role in your child’s well-being. Not only are you a vital member of your child’s healthcare team; you are a source of security and comfort.
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- Beatrice Gee, MD, Clinical Director
- Peter Lane, MD
- Marianne Yee, MD, MSc, Clinical Director
- David Archer, PhD
- Nitya Bakshi, MD, MS
- Carlton Dampier, MD
- Elaissa Hardy, MD
- Hyacinth Hyacinth, MD, PhD, MPH
- Angelica James-Herry, MD
- Clinton Joiner, MD, PhD
- Wilbur Lam, MD, PhD
- David Myers, PhD
- Tamara New, MD
- Elna Saah, MD
- Vivien Sheehan, MD
- Amy Tang, MD
Advanced practice providers:
- Andrea Nowlin, PNP
- Caylin Parker, PNP
- Enid Broyard, PNP
- Kelci Laporte, PNP
- Lindsey Jacobs, PNP
- Robin Pitts, FNP-BC
- Tina Taylor, PNP
- Tara Santee, PNP