The Curbsiders Internal Medicine Podcast

#136 Sickle Cell Disease, Management & Complications

Jan 21, 2019

Guest

Sophie Lanzkron MD

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INSIGHT

Sickle Cell Disease Pathophysiology

Sickle cell disease stems from abnormal hemoglobin polymerization causing stiff, sticky red cells and hemolysis.
This leads to microvascular blockage and reduced nitric oxide, causing vasoconstriction and complications.

ADVICE

Preventive Care Essentials

Vaccinate sickle cell patients against encapsulated bacteria and flu due to functional asplenia.
Annual labs should check kidney function via proteinuria and perform eye exams for retinal disease.

ADVICE

History Taking in Sickle Cell

In initial visits, ask patients about typical crises, pain management, complications, and transfusion history.
Know their antibody status and treatment preferences to guide acute and chronic 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