The Curbsiders Internal Medicine Podcast

#136 Sickle Cell Disease, Management & Complications

Jan 21, 2019

Guest

Sophie Lanzkron MD

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ADVICE

Managing Chronic Transfusions

Chronic transfusions reduce stroke risk but require iron overload monitoring.
Use exchange transfusion to limit iron accumulation; regularly check ferritin and liver/heart MRI.

INSIGHT

Cognitive Impact of Silent Infarcts

Silent cerebral infarcts are common and cause mild cognitive impairment in sickle cell patients.
This often leads to executive dysfunction, complicating disease management and communication.

ADVICE

Pain Crisis Management Philosophy

Assume all sickle cell pain crises are real; focus on appropriate pain treatment, not pain verification.
Every patient should have a hematologist with an individualized treatment plan to guide care.

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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!

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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