American Academy of Pediatrics Offers Guidance on Health Supervision of Children and Teens with Sickle Cell Disease

Sickle cell disease is a group of complex genetic disorders of hemoglobin and can cause painful episodes and damage to the organs
 
ITASCA, IL—The American Academy of Pediatrics offers practical guidance on the evaluation and management of children and adolescents with sickle cell disease, the most common inherited blood disorder in the United States, within a revised clinical report published in the August 2024 Pediatrics.
 
The clinical report, “Health Supervision for Children and Adolescents with Sickle Cell Disease,” published online July 22, discusses characteristics of the disease, its diagnosis and potential complications, as well as practical  management advice for pediatric and adolescent patients. The AAP Section on Hematology/Oncology and the American Society of Pediatric Hematology/Oncology, which authored the report, offer specific recommendations concerning comprehensive medical evaluation, immunizations, disease-specific treatments, psychosocial care, genetic education, counseling, and transition from the pediatric to adult health care system.
 
Clinical reports created by AAP are written by medical experts, reflect the latest evidence in the field, and go through multiple  rounds of peer review before being approved by the AAP Board of Directors and published in Pediatrics.  
 
“Sickle cell disease is a genetic condition, diagnosable at birth, which should be treated by knowledgeable providers as early as possible to minimize the risk of infections and other serious medical complications,” said Banu Aygun, M.D., FAAP, a co-author of the clinical report. “Infants and children with sickle cell disease typically have a compromised immune system, which means they are more likely to have certain infections which may be fatal. By working with your pediatrician and medical team, families can help to keep symptoms under control.”
 
Sickle cell disease results when a protein called hemoglobin that carries oxygen inside the red blood cells forms chains that clump together and cause the red blood cell to be shaped like a crescent moon―called a sickle cell. The sickled cells break up leaving the blood stream sooner than normal red blood cells. This results in blocking normal blood flow, preventing blood and the oxygen it carries to get to everywhere in the body.  The disease can cause unpredictable recurrent painful episodes and causes damage to organs.
 
An estimated 100,000 people live with sickle cell disease in the United States, more than 90% of whom are Black and between 3%–9% Hispanic or Latino, according to the Centers for Disease Control and Prevention. Although the disease occurs in all racial and ethnic groups, medical services for persons with sickle cell disease has been limited by access to care related to socioeconomic inequity, historic underinvestment in research for therapeutic development, and systemic racism.
 
The AAP recommends:

• To reduce racism in the medical care of children living with sickle cell disease, do not use the depersonalizing term “sickler.” Medical providers also should recognize their own biases and acknowledge that the difficulties faced by the patient and family may not be evident to them.
• Ideally, all newborns with sickle cell disease should be referred to a pediatric sickle cell center or hematologist by 3 months of age for co-management with a pediatric primary care home.
• All infants and children with sickle cell anemia (HbSS) and Sβ0-thalassemia should receive prophylaxis with penicillin V potassium until they are 5 years old and have completed the pneumococcal vaccine series.
• Iron supplementation should not  be given to persons with sickle cell disease for anemia unless deficiency is biochemically proven.
• Families should be educated on early recognition of acute illnesses, which can become life-threatening quickly, and the importance of identifying a medical facility with round-the-clock access to knowledgeable care for sickle cell disease.
• School performance should be monitored, and educational accommodations such as an individualized education program/504 plan should be provided if needed.

“People are living longer than ever with sickle cell disease because of strides in the medical field,” said Dr. Aygun. “This allows us to focus on improving the quality of life for patients. We are here to partner with families.”

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The American Academy of Pediatrics is an organization of 67,000 primary care pediatricians, pediatric medical subspecialists and pediatric surgical specialists dedicated to the health, safety and well-being of infants, children, adolescents and young adults.