Sickle Cell Disease, Part 1

We all have heard of Sickle cell disease, but have we discussed care across the lifespan for patients with this condition? Dr. Nnenna Badamosi, a Pediatric Hematologist, joins Dr. Zachary Hodges and Second Year Medical Student Kayla Cooper to discuss the important aspects that every pediatrician should know when treating patients with Sickle cell disease. Specifically, they will:

• Recognize newborn screening results that are consistent with sickle cell disease
• Recognize the need for additional routine vaccines for meningococcal and streptococcal pneumonia disease in children with sickle cell disease.
• Understand the indications and initiate appropriate antibiotic prophylaxis for infants and young children with sickle cell disease.
• Recognize the risk of early acute complications of sickle cell disease including dactylitis, splenic sequestration and invasive bacterial infections
• Recognize the benefits and risks of disease-modifying therapy with hydroxyurea early in childhood.
• Recognize the importance of primary stroke prevention with transcranial doppler screening and treatment with chronic transfusions.
• Recognize the importance of screening for end organ disease including cardiac dysfunction, retinopathy and kidney disease.

Peer Reviewed by MCG Pediatric Podcast Committee

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Thank you for listening to this episode from the Department of Pediatrics at the Medical College of Georgia. If you have any comments, suggestions, or feedback- you can email us at mcgpediatricpodcast@augusta.edu

Remember that all content during this episode is intended for informational and educational purposes only. It should not be used as medical advice to diagnose or treat any particular patient. Clinical vignette cases presented are based on hypothetical patient scenarios. Thanks again for your support!

 

References:

1. Minkovitz, Cynthia S et al. “Newborn Screening Programs and Sickle Cell Disease: A Public Health Services and Systems Approach.” American journal of preventive medicine 51,1 Suppl 1 (2016): S39-47. doi:10.1016/j.amepre.2016.02.019
2. https://www.nhlbi.nih.gov/health-topics/evidence-based-management-sickle-cell-disease
3. Cober MP, Phelps SJ. Penicillin prophylaxis in children with sickle cell disease. J Pediatr Pharmacol Ther. 2010;15(3):152-159.
4. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, Abboud M, Gallagher D, Kutlar A, Nichols FT, Bonds DR, Brambilla D. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. 1998 Jul 2;339(1):5-11. doi: 10.1056/NEJM199807023390102. PMID: 9647873.