Therapeutics - Sickle Cell
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What labs would be elevated in a Sickle cell patient?
- Reticulocyte count
- Unconjugated bilirubin
- Fetal Hemoglobin
What labs would be decreased in Sickle Cell?
What are the clinical hallmarks of Sickle Cells?
- Multiple organ systems affected
What organisms mainly infect Children with Sickle cell?
- Streptococcus Pneumoniae
- H. influenza
What organisms mainly infect Adults with Sickle cell?
- E. coli
- S. aureus
Why do Sickle cell patients have such a high risk of infection?
Dysfunctional spleens are common
What are the supportive treatments available for Sickle Cell?
- Folic acid
- Fetal hemoglobin inducers (Hydroxyurea)
- Chronic transfusion therapy
What is the curative treatment for Sickle Cell?
Allogenic stem cell transplant
What immunizations should sickle cell patients receive?
- PCV 13 (Prevnar if <24 mo) or 23 (Pneumovax if > 24 mo)
- Meniongococal > 2 years if splenic insufficient
- Hep B
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
What nutritional supplements are required for Sickle cell patients?
- Folic acid (1 mg/day for adults)
- Multivitamin w/o Iron
- B12, B6 and D
What tools are used to monitor Sickle Cell?
- Transcranial Dopplers (2-16 years)
- Routine Eye exams (Start at school age)
What is the MOA of fetal Hemoglobininducers?
Increase fetal hemoglobin and decrease RBC sickling
What are the fetal hemoglobin inducers?
What is the MOA of Hydroxyurea in the treatment of Sickle cell?
- Stimulates production of HbF
- Unknown mechanism
Hydroxyurea is FDA approved for what Sickle Cell symptom?
Prevention of painful crisis
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
What are the AE of Hydroxyurea?
- BM suppression
Why is Hydroxyurea controversial in adults?
Malignancy and infertility
What are chronic transfusions indicated for in the treatment of Sickle cell?
Delay of organ damage by Stroke prevention
What is the goal of chronic transfusions in the treatment of Sickle cell?
Reduce HbS to < 30% of total Hb
How often are transfusions given in the treatment of Sickle cell?
Q 3-4 weeks
What is the risk of chronic transfusions in the treatment of Sickle cell?
- Alloimmunization – Immune response to foreign antibodies
- Iron overload = most common
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
What pain management would be recommended for mild to moderate pain in Sickle cell?
Outpatient treatment with NSAID, APAP and Codeine
What pain management would be recommended for Severe pain in Sickle cell?
- Scheduled IV opioids
- PRN opioids for breakthrough pain
What is opioid induced hyperalgesia and how is it treated?
- Increased pain with increased opioid dose
- Change opioids
What is the supportive care regimen for scute Sickle cell complications?
- Analgesia (Morphine or Hydromorphone PCA)
- Fluid replacement (hydration)
- Adequate oxygen saturation
What non-supportive/disease modifying care should be given in an acute Sickle cell crisis?
- Transfusions is hypoxic, has acute chest syndrome or stroke (maintain Hb at <11 g/dL)
What antibiotics are usually used for infections in the treatment of sickle cell?
- D/C PCN prophylaxis
What is the only curative treatment for Sickle cell?
Allogenic stem cell transplant
What are the requirements for an Allogenic stem cell transplant in Sickle cell?
- <16 yrs
- Severe complications of pain and stroke
- HLS-matched donor
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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