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A few highlights from the #ASH20 Blood Drop session on outpatient sickle cell disease management in adults. #thread
ASH guidelines recommend cognitive impairment screening with simplified questions, as well as at least a 1-time MRI to detect silent infarcts. #sicklecell #SCD
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ASH guidelines recommend cognitive impairment screening with simplified questions, as well as at least a 1-time MRI to detect silent infarcts. #sicklecell #SCD
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Hydroxyurea is recommended for adults with #SCD, with slightly different recommendations by FDA and NHLBI.
FDA: adults with 3 or more pain crises in 1 year
NHLBI: patients with pain that affects quality of life
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FDA: adults with 3 or more pain crises in 1 year
NHLBI: patients with pain that affects quality of life
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Remember: we have new therapies for #sicklecell #SCD!
Let's go over each of them:
- transplant
- crizanlizumab
- voxelotor
- L-glutamine
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Let's go over each of them:
- transplant
- crizanlizumab
- voxelotor
- L-glutamine
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Crizanlizumab is a P-selectin inhibitor that interferes with endothelial wall-RBC interactions to reduce vaso-occlusive events. It was superior to placebo in the randomized SUSTAIN trial ( https://www.nejm.org/doi/full/10.1056/NEJMoa1611770) in reducing the # of pain crises. #ASH20 #SCD
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Voxelotor is a HbS polymerization inhibitor, so it reduces hemolysis and increases hemoglobin. This was demonstrated in the placebo-controlled HOPE trial ( https://www.nejm.org/doi/full/10.1056/nejmoa1903212), and increased Hgb ~1-1.5 g/dl. #sicklecell #ASH20
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L-glutamine is super interesting. It reduces oxidative stress in RBCs and actually decreases the number of pain crises and the number of hospitalizations compared to placebo ( https://www.nejm.org/doi/full/10.1056/NEJMoa1715971). It does not affect anemia. It is a useful adjunct to hydroxyurea. #ASH20
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One of the most important points: hydroxyurea is the only agent we currently have in #SCD that actually decreases mortality!
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Other important guidelines for adults:
- no role for screening PFTs, echo in asymptomatic patients
- should screen for albuminuria!
- BP goal <130/80
- renal transplant should be considered for CKD as trend towards better survival
- treat anemia of CKD with ESAs (goal ~10)
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- no role for screening PFTs, echo in asymptomatic patients
- should screen for albuminuria!
- BP goal <130/80
- renal transplant should be considered for CKD as trend towards better survival
- treat anemia of CKD with ESAs (goal ~10)
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#SCD is a risk factor for thromboembolism, so quick summary slide on ASH recommendations for anticoagulation in #sicklecell disease. #ASH20 #hemetwitter
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Also, don't forget about:
- annual retinal exam
- ferritin screening for iron overload if patient receives transfusions (if ferritin >1000, liver MRI to quantify iron content, and consider iron chelation therapy)
#ASH #sicklecell #SCD
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- annual retinal exam
- ferritin screening for iron overload if patient receives transfusions (if ferritin >1000, liver MRI to quantify iron content, and consider iron chelation therapy)
#ASH #sicklecell #SCD
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Now, RBC transfusion recommendations in #sicklecell.
This slide is an amazing summary slide for all episodic and chronic transfusion indications. Save it to your phone! #ASH20 #SCD
Red cell exchange recommended for acute stroke and severe ACS in updated ASH guidelines.
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This slide is an amazing summary slide for all episodic and chronic transfusion indications. Save it to your phone! #ASH20 #SCD
Red cell exchange recommended for acute stroke and severe ACS in updated ASH guidelines.
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Red cell antigen matching (including extended antigen profiling) is recommended in patients with #sicklecell disease to avoid long-term alloimmunization. #ASH20
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Great clinical pearl: if a patient with #sicklecell disease has an asymptomatic acute exacerbation of anemia 7-10 days after recent transfusion, consider delayed hemolytic transfusion reaction and check hemolysis labs (don't just jump to another transfusion). #ASH20 #FOAMed
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From the Q&A session: try to get patients on maximally-tolerated doses of hydroxyurea (can take 6 months to work) prior to introducing the novel agents, as hydroxyurea has a mortality benefit and the most amount of evidence.
Thank you for tuning into this #ASH20 thread!
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Thank you for tuning into this #ASH20 thread!
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