#136 Sickle Cell Disease, Management & Complications

Stuck on sickle cell disease? We hammer out the basics of diagnosis, common sickle cell variants and their manifestations, preventive medicine, acute and chronic pain management, opioid use, and how to recognize and treat common complications like anemia, fever and acute chest syndrome. Sickle cell expert, Sophie Lanzkron MD, Associate Professor of Medicine and Oncology and Director of the Sickle Cell Center for Adults at Johns Hopkins joins!

Full show notes available at http://thecurbsiders.com/podcast. Join our mailing list and receive a PDF copy of our show notes every Monday. Rate us on iTunes, recommend a guest or topic and give feedback at [email protected].

Sponsor

ACP's Internal Medicine Meeting 2019 April 11-13th in Philadelphia, PA. We'll see you there!

Credits

Written (including CME questions) and produced by: Justin Berk MD, Martha Brucato MD PhD, Beth Garbitelli MS1

Hosts: Paul Williams MD, Justin Berk MD, Matthew Watto MD

Edited by: Matthew Watto MD

Guest: Sophie Lanzkron MD MHS

Time Stamps

00:00 Announcements, intro 02:30 A quick refresher on hemoglobinopathy 03:45 Guest bio 05:03 Guest one-liner, movie recommendation, career advice, picks of the week 10:53 ACP Internal Medicine Meeting 2019 (ad read) 12:33 Clinical case; defining sickle cell; pathophysiology 16:23 Different types of hemoglobinopathy genotypes and phenotypes 20:20 Preventive care for sickle cell disease 22:56 Taking a history at the initial visit in patient with sickle disease 26:40 Life expectancy in SCD 28:30 Hydroxyurea 30:40 Chronic red blood cell transfusion therapy; complications; monitoring 37:07 Silent cerebral infarcts and mild cognitive impairment in SCD 

40:10 Chronic pain management; opioid use disorder in sickle cell disease 46:00 Acute pain crisis management; PCAs vs bolus therapy 54:08 Reticulocyte count in sickle cell disease 55:40 Itching, opioids and naloxone? 58:10 Red blood cell transfusion threshold in sickle cell disease (anemia) 60:03 Acute fever in SCD 61:48 Acute chest syndrome 66:45 Transition from pediatrics to adult medicine in sickle cell 68:00 Incentive spirometry to prevent acute chest syndrome 68:45 Take home points 70:00 Outro