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What labs would be elevated in a Sickle cell patient?
- Reticulocyte count
- Unconjugated bilirubin
- LDH
- Fetal Hemoglobin
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What labs would be decreased in Sickle Cell?
Hemoglobin/Hematocrit
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What are the clinical hallmarks of Sickle Cells?
- Vaso-occlusion
- Hemolysis
- Multiple organ systems affected
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What organisms mainly infect Children with Sickle cell?
- Streptococcus Pneumoniae
- H. influenza
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What organisms mainly infect Adults with Sickle cell?
- E. coli
- S. aureus
- Salmonella
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Why do Sickle cell patients have such a high risk of infection?
Dysfunctional spleens are common
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What are the supportive treatments available for Sickle Cell?
- Immunizations
- Penicillin
- Folic acid
- Fetal hemoglobin inducers (Hydroxyurea)
- Chronic transfusion therapy
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What is the curative treatment for Sickle Cell?
Allogenic stem cell transplant
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What immunizations should sickle cell patients receive?
- PCV 13 (Prevnar if <24 mo) or 23 (Pneumovax if > 24 mo)
- Influenza
- Meniongococal > 2 years if splenic insufficient
- Hib
- Hep B
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How is Pneumococcal infections treated?
- Prophylaxis from 3 months to 5 years with 125-250 mg BID Pen VK
- If Allergic to Pen: Erythromycin 20 mg/kg day
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What nutritional supplements are required for Sickle cell patients?
- Folic acid (1 mg/day for adults)
- Multivitamin w/o Iron
- B12, B6 and D
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What tools are used to monitor Sickle Cell?
- Transcranial Dopplers (2-16 years)
- Routine Eye exams (Start at school age)
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What is the MOA of fetal Hemoglobininducers?
Increase fetal hemoglobin and decrease RBC sickling
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What are the fetal hemoglobin inducers?
- Hydroxyurea
- Butyrate
- Decitabine
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What is the MOA of Hydroxyurea in the treatment of Sickle cell?
- Stimulates production of HbF
- Unknown mechanism
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Hydroxyurea is FDA approved for what Sickle Cell symptom?
Prevention of painful crisis
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What are the benefits of Hydroxyurea in the treatment of Sickle cell?
- Prevents painful crisis
- Delays progression of organ dysfunction
- Decreases mortality, hospitilizations and transfusions
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What are the AE of Hydroxyurea?
- BM suppression
- Malignancy
- Infertility
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Why is Hydroxyurea controversial in adults?
Malignancy and infertility
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What are chronic transfusions indicated for in the treatment of Sickle cell?
Delay of organ damage by Stroke prevention
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What is the goal of chronic transfusions in the treatment of Sickle cell?
Reduce HbS to < 30% of total Hb
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How often are transfusions given in the treatment of Sickle cell?
Q 3-4 weeks
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What is the risk of chronic transfusions in the treatment of Sickle cell?
- Alloimmunization – Immune response to foreign antibodies
- Infections
- Iron overload = most common
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How is iron overload associated with chronic transfusions controlled in Sickle cell?
- Give Defuroxamine after 1 year of infusions to chelate the iron
- Deferasirox (Exjade oral)
- Deferiprone (Ferriprox oral)
- Monitor ferritin
- Monitor audio, ophthalm, nephrol, hematol and endocrinology
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What pain management would be recommended for mild to moderate pain in Sickle cell?
Outpatient treatment with NSAID, APAP and Codeine
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What pain management would be recommended for Severe pain in Sickle cell?
- Hospitilization
- Scheduled IV opioids
- PRN opioids for breakthrough pain
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What is opioid induced hyperalgesia and how is it treated?
- Increased pain with increased opioid dose
- Change opioids
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What is the supportive care regimen for scute Sickle cell complications?
- Analgesia (Morphine or Hydromorphone PCA)
- Fluid replacement (hydration)
- Adequate oxygen saturation
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What non-supportive/disease modifying care should be given in an acute Sickle cell crisis?
- Antibiotics
- Transfusions is hypoxic, has acute chest syndrome or stroke (maintain Hb at <11 g/dL)
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What antibiotics are usually used for infections in the treatment of sickle cell?
- Ceftriaxone
- Clindamycin
- Vancomycin
- Macrolides
- D/C PCN prophylaxis
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What is the only curative treatment for Sickle cell?
Allogenic stem cell transplant
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What are the requirements for an Allogenic stem cell transplant in Sickle cell?
- <16 yrs
- Severe complications of pain and stroke
- HLS-matched donor
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What are the risks of Allogenic stem cell transplant in the treatment of Sickle Cell?
- Graft versus host disease
- Secondary malignancies
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