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Printable Handouts
Navigable Slide Index
- Introduction
- Objectives
- Case JP
- Sickle cell anemia
- Hemoglobin structure
- Sickle cell structure
- Vaso-occlusion
- Free hemoglobin
- Nitric oxide and vascular homeostasis
- Epidemiology of SCD pain
- Case presentation
- Diagnosing painful crises
- Evidence base for the treatment of VOC
- What's the best way to treat pain?
- Treating acute SCD pain
- Case
- Acute chest syndrome (1)
- Acute chest syndrome (2)
- Acute chest syndrome (3)
- Transfusion
- Simple transfusion in SCD
- Blood viscosity and sickle cell
- Exchange transfusion
- NHLBI guideline recommendations for ACS
- Transfusion pitfalls
- Patient case
- Current medicine cabinet
- Hemoglobin production
- Effects of HbF in SS disease
- Multicenter study of hydroxyurea
- Additional benefits of hydroxyurea
- Probability of 10-year survival in SCD patients
- Treat with hydroxyurea
- Common mistakes with hydroxyurea
- L-glutamine
- RCT of L-glutamine
- L-glutamine side effects
- How I use L-glutamine
- JP
- The difficult patient
- High utilizers' or 'frequent flyers'
- Coping skills
- Summary
Topics Covered
- The pathophysiology of vaso-occlusive crises (VOC) in sickle cell disease (SCD)
- Vaso-occlusion is a complex process involving increased adhesion molecule expression
- Intravascular hemolysis and the resultant scavenging of nitric oxide
- Apply guideline based care to adults with SCD
- Exchange transfusion can be used when a simple transfusion may lead to hyperviscosity
- Treatment using Hydroxyurea or L-glutamine
- Neurocognitive dysfunction as a complication of SCD
Talk Citation
Lanzkron, S. (2019, May 16). Management of sickle cell disease: what to do when there is no evidence base [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved October 12, 2024, from https://doi.org/10.69645/EAFF3532.Export Citation (RIS)
Publication History
Financial Disclosures
- Prof. Sophie Lanzkron, Site PI for several industry sponsored studies. The studies are sponsored by Pfizer, Global blood therapeutics, Prolong Pharmaceuticals and Ironwood
A selection of talks on Clinical Practice
Transcript
Please wait while the transcript is being prepared...
0:00
I'm Sophie Lanzkron, I am the Director of
the Sickle Cell Center for Adults at Johns Hopkins,
and I'm going to talk about the "Management of Sickle Cell Disease" today.
0:10
I'm going to start by describing
the pathophysiology of vaso-occlusive crises in sickle cell disease,
talk a little bit about recognizing the clinical presentation of vaso-occlusive crisis,
and then talk about guideline-based care for adults with sickle cell disease.
0:25
I'm going to do that in the context of a patient.
So let me introduce you to JP.
He's a 23-year-old male with hemoglobin SS disease.
He presents reporting typical vaso-occlusive crisis type pain in his arms and legs.
He was last hospitalized two weeks ago.
His medications include hydromorphone,
4 mg every 4-6 hours as needed,
and folic acid 1 mg a day.
He's talking to his mom when you walk in the room,
when he sees you he crawls into a ball and starts to complain of 8/10 pain.
0:54
Sickle cell anemia is due to a single base substitution of a valine for
a glutamine at the sixth amino acid of the gene encoding for the hemoglobin Beta- chain.
It affects approximately 100,000 Americans,
but millions are affected worldwide.
In the United States compared to the general population,
it decreases life expectancy by 25-30 years.
Interestingly, a recent study shows that
the sickle mutation occurred once 7,300 years ago,
and the reason that the gene mutations has survived is because having
sickle trait provides a significant survival advantage against Falciparum malaria.
So if you are born with sickle trait,
you're far more likely to survive having malaria than if you don't have sickle trait.
1:36
Here is the hemoglobin molecule.
It is made up of two Alpha-chains and two Beta-chains.
The mutation we're talking about occurs in the Beta-globin chain.
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