Therapeutics: Cystic Fibrosis

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kyleannkelsey
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269463
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Therapeutics: Cystic Fibrosis
Updated:
2014-04-06 16:14:14
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Therapeutics Cystic Fibrosis
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Therapeutics: Cystic Fibrosis
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Therapeutics: Cystic Fibrosis
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  1. What is Cystic fibrosi?
    • Mutated CFTR gene on Chromosome 7
    • Autosomal recessive
    • Most common lethal AR disease in whites
  2. What is the most common genetic variation leading to CF?
    ∆F508 deletion (70%)
  3. What is G55ID?
    Genotypr of 4% of CF patients that leads to incomplete opening of CFTR channels
  4. What is the normal action of CFTR?
    • CTFR moves Cl out of cell
    • Na + water follow = keeps lungs moist
    • Prevents bacterial infection b/c cilia can operate effectively
  5. What are the main effects of a mutant CTFR gene?
    • Thick viscous lung mucus that promotes bacteria colonization
    • Sweat = usually reabsorb Cl (<20 mEq/L)
    • w/ CF, more Cl remains in sweat (>50 mEq/L)
    • increased inflammatory response: PMNs/Cytokines recruited
  6. What characteristic of CF allows detection by skin test?
    • Sweat = usually reabsorb Cl (<20 mEq/L)
    • w/ CF, more Cl remains in sweat (>50 mEq/L) = more Cl in sweat
  7. What parts of the body does CF mainly effect?
    • Effects all parts of the body:
    • Majorly = Lungs, Pancreas, fertility (both male/female), GI
  8. How is CF detected?
    • Newborn: screen
    • Children: Chronic RIs, failure to thrive or GI issues
    • Adults: Chronic RI, Skin Cl
  9. How is CF monitored?
    • FEV1 (forced expiratory volume/second) = hard aggressive breath
    • <60% of personal best = exacerbation
  10. What will happen to FEV1 throughout a CF patient’s lifetime?
    Personal best will decline over time
  11. What organisms usually infect pediatric CF patients/
    H. flu and MRSA (on the rise)
  12. Over time what infection does 60% of DF population get?
    Colonization by Psedomonas
  13. What can you expect from treatment of pseudomonas infection in CF patients?
    • Reduced numbers
    • Can never eradicate b/c mucus too thick for antibiotics to be fully effective
  14. What are the various treatment options: for CF?
    • Physiotherapy (removes mucus)
    • Bronchodilators
    • Mucolytics
    • Inhaled antibiotics
    • Hydrator Tx
    • Anti-inflammatories
    • Systemic antibiotics
    • CFTR modulators
    • Postural percussion and drainage (positioning to drain mucus)
    • Positive expiratory pressure (breathing device that dissects mucus)
    • HF chest oscillator
  15. What is available as a mucolytic therapy for CF?
    Dornase Alfa (Pulmozyme) = recombinant human DNAase I
  16. What is the dosing/administration for Dornase Alfa (Pulmozyme)?
    Nebulize 2.5mg QD
  17. Would you give Dornase Alfa (Pulmozyme) QD or BID and why?
    BID has same results as QD
  18. What are the benefits of Dornase Alfa (Pulmozyme) treatment?
    increased FEV1 and decreased Exacerbations
  19. What is the MOA o Dornase Alfa (Pulmozyme)?
    • Recombinant human DNAase I
    • Breaks up DNA/RNA from WBCs brought to the lungs by inflammatory response
  20. What is available for Hydration Therapy in CF?
    Inhaled Hypertonic saline 7%
  21. How does Hydration therapy work in CF?
    Draws fluid to lung surface = decreased mucus viscosity
  22. What are the benefits of hydration therapy for CF?
    • decreased Exacerbations, Cheap
    • DOES NOT IMPROVE LUNG FUNCTION/FEV1
  23. At what point should you start antibiotic treatment for CF?
    Don’t start until infected
  24. What are the normal long-term antibiotic treatments for pseudomonas in CF?
    • TOBI (inhaled/nebulized tobramycin)
    • TOBI podhaler (Dry powder tobramycin inhaler)
    • Aztreonam lysine (Cayston) Nebulizer
  25. When should intitate TOBI/TOBI podhaler or Aztreonam lysine (Cayston) Nebulizer?
    Initiate w/ 1st Pseudomonas infection
  26. What is the dosage/administration for TOBI nebulizer therapy?
    • 300mg BID x28 days (28 on/28 off)
    • What is the dosage/administration for TOBI podhaler therapy?
    • 4 (112mg) caps BID (28 on/28 off)
  27. What are the benefits of TOBI nebulizer therapy?
    decreased exacerbations, increased FEV1 (4-8%), increased nutrition status, No resistance
  28. How long does it take to administer a dose of TOBI by the nebulizer route?
    15-20 min
  29. What are the benefits of TOBI Podhaler therapy?
    decreased exacerbations, decreased IV antibiotic use, increased FEV1, Takes 4-6 minutes
  30. How long does it take to administer a TOBI podhaler dose?
    4-6 minutes
  31. What is the route and dose for Aztreonam lysine (Cayston)?
    • 75 g TID (28 on/28 off)
    • Nebulizer
  32. What is the major disadvantage to Aztreonam lysine (Cayston)?
    TID is a disadvantage to the BID administration schedule of TOBI variants
  33. What group was Aztreonam lysine (Cayston) studied in?
    Chronically colonized with Pseudomonas
  34. What are the benefits of Aztreonam lysine (Cayston) for Pseudomonas infections?
    decreased exacerbations, increased FEV1, increased Weight gain
  35. What are the Anti-inflammatory agents used for CF?
    • High dose IBU
    • Azithromycin (Macrolide)
  36. What is the MOA for High dose IBU in CF?
    • Anti-inflammatory
    • Decreases PMN migration to the lungs
  37. Why did High dose IBU treatments fall out of favor in CF?
    Causes hematochiza (bloody urine)
  38. What are the target plasma concentrations and normal doses for High dose IBU Anti-inflammatory treatment in CF?
    • 50-100 mg/L
    • 20-30g/kg
  39. Why is High dose IBU likely to make a comeback in CF treatment?
    Cochrane review found incereased survival (81% vs 70%)
  40. What is the dosing/administration for Azithromycin (Macrolide) anti-inflammatory treatment in CF?
    • 250-500 mg PO TID
    • Not used as antibiotic
  41. What are the cons of Anti-inflammatory treatment with Azithromycin (Macrolide) in CF?
    • increased Macrolide resistant H. flu and Staph
    • Not really a problem because Other treatment can be used
  42. What are the benefits of Anti-inflammatory treatment with Azithromycin (Macrolide) in CF?
    decreased exacerbations, increased FEV1 short term, increased Weight gain
  43. Should you treat Staph Aureus prophylacticaly , why or why not?
    • No
    • Prophylaxis increased risk of Pseudomonas infections
  44. How should you treat a staph aureus infection in CF?
    • Anitstaphylococcal penicillins or 1st gen cephalosporins:
    • Oxacillin, Cloxacillin, Dicloxacillin, Cephalexin, Cefazolin
  45. Why is MRSA becoming a concern in CF?
    • increased Mortality
    • Must isolate infected patients
    • Incidence increasing from 2-23% in last 13 years
  46. What are the Treatment options for MRSA in CF?
    • No guidelines, use normal drugs:
    • Clindamycin, SMZ-TMP, vancomycin, linezolid (minocycline if over 8 yrs)
    • Topical/ treatment for nasal infection = mupirocin and chlorhexadine
  47. How should you treat a Pseudomonas infection in CF?
    • AGGRESSIVE EARLY TREATMENTMENT
    • Penicillin or Cepahlosporin + Aminoglycoside x14 days
    • Pen/Cepahlosporin: Piperacillin, ceftazidime, cefepime
    • Aminoglycoside: Tobramycin (if resistant use Amikacin)
  48. How does treatment differ between parts of Europe and the US for Pseudomonas infections in CF?
    • Europe = Ciprofloxacin & Colistin (Poly E) = 80% uncolonized for 15 yrs
    • US = wait until colonization before we treat
  49. What are the s/s of a Pseudomonas exacerbation?
    increasedcough, increasedSOB , increased sputum, decreased FEV1
  50. How do you treat a Pseudomonas exacerbation in CF?
    • High doses tobramyin
    • Peak target for QD treatment: 25-35 mg/L
    • Peak target for BID/TID: 4-8 mg/L
  51. Why are we not concerned with nephrotoxicity with high doses of TOBI in CF?
    CF clears tobramycin quickly = no nephrotoxic concern
  52. Should you use Gentamycin for Pseudomonas infections in CF?
    • No
    • Usually resistant
  53. What treatment is only avaible for CF patients with G551D genetic dysfunction?
    Ivacaftor (Klydeco)
  54. What is Ivacaftor (Klydeco)?
    CFTR potentiator for G551D only (4% of CF pts)
  55. How is Ivacaftor (Klydeco) dosed/administered?
    150 mg PO BID
  56. What are the benefits of Ivacaftor (Klydeco) treatment?
    • decreased exacerbations (55%), increased FEV1, increased Weight gain, Normal sweat Cl
    • Opens the partially developed CFTR channels
  57. Why is Malnourishment a big issue w/ CF?
    • Leads to faster pulmonary decline
    • CFTR mutations also lead to GI and pancreatic problems that create malnutrition
  58. Why do CF patients need more calories?
    • increased E expenditure due to difficult breathing
    • Malabsorption
  59. How many more calories do CF patients need that normal individuals?
    20-50% more Calories than normal
  60. Other than more calories, what other supplemental/special dietary needs to CF patients have?
    • Need multivitamins - especially Fat soluble ones (difficulty absorbing Fats)
    • Decreased intestinal pH necessities PPIs
    • Pancreatic insufficiency necessitates Enzyme replacement
  61. Why do CF patients need PPIs?
    They have decresaed Bicarb production = decreased intestinal pH = malnutrition
  62. What percent of CF patients have pancreatic insufficiency?
    85%
  63. What are the guidelines for enzyme replacement in pancreatic insufficiency for infants with CF?
    • 2000-4000 lipase U/120 mL formula
    • titrate from guidelines as needed
  64. What are the guidelines for enzyme replacement in pancreatic insufficiency for 1-4 year olds with CF?
    • 1000 lipase U/kg/meal
    • Titrate from guidelines as needed
    • Max for 4years and up:
    • 2500 lipase U/kg/meal
    • 10,000 lipase U/kg/day
    • 4000 lipase U/g Fat/day
  65. What are the guidelines for enzyme replacement in pancreatic insufficiency for 4 year olds to adults with CF?
    • 500 lipase U/kg/meal
    • Titrate from guidelines as needed
    • Max for 4years and up:
    • 2500 lipase U/kg/meal
    • 10,000 lipase U/kg/day
    • 4000 lipase U/g Fat/day

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