CF

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  1. Epidemiology of CF
    • • Most common life limiting disorder in Caucasians
    • • Current life expectancy of ~36 years
  2. Pathophysiology of CF
    • • Autosomal recessive
    • • Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
    • → Most common mutation: ΔF508
    • • CFTR regulates Na+ and Cl- ion transport and salt homeostasis in sweat glands
    • • Mutated sweat now contains large amounts of salt and leads to a negatively charged lumen
  3. Organ
    • Abnormality
    • Result
  4. Sinus/Lung Pathophysiology of CF
    • • Sinus cavity polyps
    • • Shortness of breath
    • • Cough
    • • Sputum production
    • • Barrel chest (air trapping)
    • • Decreased FEV1
    • • Bacterial growth in the lungs
    • → Exacerbations
    • → Increased cough
    • → Reduction in pulmonary function
    • → Increased sputum production
  5. GI Pathophysiology of CF
    • • Steatorrhea (greasy stools)
    • • Failure to thrive
    • • Malnutrition
    • → Fat soluble vitamin deficiency, decreased pancreatic enzyme, fat malabsorption
    • • Infants/Small children
    • → Increase frequency of small stools
    • → Meconium ileus (newborns): 1st stool passed after birth (blockage)
    • • Older patients
    • → Constipation
    • → Abdominal cramping/flatulence
  6. Endocrine Pathophysiology
    • • Insulin deficiency
    • • Weight loss
    • • Increase in blood glucose levels
    • • Failed oral glucose tolerance test (OGTT)
    • • Cystic Fibrosis Related Diabetes (CFRD)
    • → Seen in 20% of adolescents and 50% of adults
    • → Shares features of Type 1 and Type 2
    • → Primarily caused by insulin insufficiency
    • → Fluctuating insulin resistance also seen
    • → Treated with insulin
  7. Reproductive Pathophysiology
    • • Azoospermia
    • → Blockage of vas deferens
    • → Absence of vas deferens
    • • Females may have lower fertility due to decreased water content of the cervical fluid
  8. Dx
    • • All infants receive newborn screening (NBS)
    • → Identifies high levels of immunoreactive trypsinogen (IRT)
    • → If abnormal, DNA test preformed for known mutations
    • • Sweat Chloride Test
    • → Pilocarpine administered transdermally to stimulate sweat gland secretion
    • → If sweat chloride ≥60 mmol/L diagnostic for CF
    • ¤ Positive: test for every gene mutation
  9. Respiratory Therapy/”Pulmonary Toilet”
    • 1) Albuterol
    • 2) Hypertonic Saline
    • 3) Dornase alfa
    • 4) Airway Clearance
    • 5) Inhaled ABx
  10. Step One
    • Albuterol ((ProAir®, Ventolin®, Proventil®)
    • • Drug Class: Beta2 Agonist
    • • Mechanism of Action
    • → Relaxes bronchial smooth muscle
    • • Opens up airways to allow for better delivery of respiratory medications
    • • Dose: 2 puffs prior to therapy (2 – 4 times daily)
    • • Adverse Effects: May increase blood pressure/heart rate
  11. Step Two
    • Hypertonic Saline (HyperSal®)
    • • Drug Class: Mucolytic
    • • Mechanism of Action:
    • → Water distribution
    • → Hydrates airway mucus secretions
    • → Increases overall lung function
    • • Dose: 4 mL nebulized 2 – 4 times daily
    • → 3%, 5% (not commercially available), 7%
    • • Adverse Effects:
    • → Bronchospasm
    • → Irritation
  12. Step Three
    • Dornase alfa (Pulmozyme®)
    • • Drug Class: Mucolytic
    • • Mechanism of Action
    • → Cleaves neutrophil DNA found in airway secretions
    • ¤ Makes up ~10% of the weight of mucous
    • → Reduces mucous viscosity
    • → Improved lung function
    • • Dose: 2.5 mg nebulized daily
    • → Can’t be mixed with other products
    • → Must be protected from light and refrigerated
    • → Must be used with specific nebulizers
    • • Adverse Effects: Chest pain, cough, fever, voice alteration
  13. Step Four
    • Percussion
    • • Loosens mucus so it can be cleared by coughing/huffing
    • • Past therapies
    • → Chest Physical Therapy (CPT) and Postural Drainage
    • • Current therapies
    • → Oscillating Positive Expiratory Pressure (Oscillating PEP)
    • ¤ Examples: Flutter™, Acapella™, Cornet™
    • → High – frequency Chest Wall Oscillation
    • ¤ Vest vibrates at high frequency
  14. Step Five: Inhaled ABx (I)
    • Tobramycin (Tobi®)
    • • Drug Class: Aminoglycoside Antibiotic
    • • Mechanism of Action:
    • → Binds to 30S and 50S ribosomal subunits of bacterial cell membrane
    • → Improves pulmonary function
    • → Decreases density of P. aeruginosa in sputum
    • • Dose: 300 mg nebulization every 12 hours for 28 days followed by 28 days off
    • → Can’t be mixed, refrigerate, use with specific nebulizers, light protected
    • • Adverse Effects: Cough, voice alteration, sputum color change
  15. Step Five: Inhaled ABx (II)
    • Azetreonam (Cayston®)
    • • Drug Class: Monobactam Antibiotic
    • • Mechanism of Action
    • → Inhibits bacterial cell wall synthesis by binding to penicillin binding proteins (PBPs)
    • → Improves pulmonary function
    • → Decreases density of P. aeruginosa in sputum
    • • Dose: 75 mg nebulized three times daily for 28 days with 28 days off
    • → Must use with specific nebulizer, do not mix, store in fridge before reconstituted
    • • Adverse Effects: Cough, congestion, wheezing, sore throat, fever
  16. Airway Inflammation
    • • Azithromycin (Zithromax®)
    • • Drug Class: Macrolide Antibiotic
    • • Mechanism of Action
    • → Inhibits protein synthesis, binds to 50s subunit
    • → Inhibits neutrophil migration
    • → Decreases production of pro-inflammatory mediators
    • → Improves lung function
    • • Dose: 250 mg – 500 mg on Monday, Wednesday, and Friday
    • • Adverse Effects: Diarrhea, nausea, QT prolongation, increased risk for mycoplasma infections
  17. Fat Absorption
    • • Pancreatic Enzymes (Creon®, Pancreaze®, Zenpep®)
    • • Drug Class: Pancreatic enzyme (no product is interchangeable)
    • • Mechanism of Action:
    • → Contains lipase, amylase and protease
    • → Break down fats, proteins and starch
    • • Dose: 500 units of lipase/kg/meal (500 – 2500 units/kg/meal)
    • • Adverse Effects: Abdominal pain
  18. Fat Soluble Vitamins
    • • Vitamin A, D, E and K (AquaADEKs®)
    • • Drug Class: Vitamins
    • • Mechanism of Action
    • → Provides fat soluble vitamins to patients who have decreased absorption
    • → Vitamins enclosed in hydrophilic spheres
    • • Typical Dose: 1 softgel twice daily with food
    • • Adverse Effects: GI Upset
  19. Gene Modulation (I)
    • • Ivacaftor (Kalydeco™)
    • • Drug Class: Cystic Fibrosis Transmembrane Conductance Regulator Potentiator
    • • Mechanism of Action:
    • → Restores function of the defective CFTR protein
    • → Improves salt and water absorption
    • → Approved for 10 rare mutations: G551D, G551S, G178R, G1244E, G1349D, S549N, S549R, S1251N, S1255P and R117H
    • • Dose: 150 mg PO every 12 hours with high fat foods
    • • Adverse Effects: Headache, rash, URI, oropharyngeal pain, congestion, nasopharyngitis
    • → Heavy CYP 3A4, decrease dose if decreased liver function
  20. Gene Modulation (II)
    • • Lumacaftor and Ivacaftor (Orkambi®)
    • • Drug Class: Cystic Fibrosis Transmembrane Conductance Regulator Potentiator
    • • Mechanism of Action:
    • → Increased processing and trafficking of mature protein to the cell surface
    • → Ivacaftor MOA on previous slide
    • → Approved for homozygous ΔF508 mutation
    • • Dose: 400/250 mg every 12 hours with high fat foods
    • • Adverse effects: GI, changes in respiration, chest discomfort, dyspnea, nasopharyngitis
  21. IV Antibiotics
    • • Must be given
    • • Recommend to be given as an inpatient versus home health
    • • No recommendations on whether to continue inhaled antibiotics on admission
    • → Continue all other home treatments
    • • On every admission will treat for pseudomonas infections with double coverage
    • • Admissions typically last for multiple weeks
    • • Other antibiotics will be based on sputum growth
    • • Lung transplant is a possibility
  22. IV Antibiotics for Pseudomonas
    • • Piperacillin-Tazobactam (Zosyn®)
    • • Ceftazidime (Fortaz®)
    • • Cefepime (Maxipime®)
    • • Meropenem (Merrem®)
    • • Levofloxacin (Levaquin®)
    • • Gentamicin (once daily dosing)
    • • Tobramycin (once daily dosing)
    • • Amikacin (once daily dosing)
    • • Colistimethate (Coly-Mycin M®)
    • • Patients are rapid metabolizers and may need higher doses than average population
Author:
Anonymous
ID:
315852
Card Set:
CF
Updated:
2016-02-12 23:17:30
Tags:
IT CF
Folders:
IT2
Description:
IT2 (MT3): CF
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