Cystic Fibrosis Flashcards

Question

What is CF lung disease?

Answer 1

Cause of ~ 85% of CF deaths Due to CFTR dysfunction CF patients have thickened mucus This mucus is hard to clear and creates a good environment for bacteria to grow Respiratory exacerbations are common

Answer 2

Recommended for all CF patients No method proven better than another Method based on patient: Manual airway clearance techniques (P&PD); Therapy Vest; Flutter, acapella; Huff coughing; Meta neb

Answer 3

dornase alfa, hypertonic saline, inhaled mannitol

Answer 4

◦ MOA: Cleaves the extracellular DNA from expended neutrophils and other inflammatory cells in the CF mucus, thus reducing viscosity and promoting clearance (chops up mucus to help it get out easier) ◦ Nebulized solution ◦ Generally well tolerated but $$$ ◦ Recommended for daily use in CF patients ≥ 6 years old; Can use in select cases < 6 years old

Answer 5

◦ MOA: Exact MOA unclear Proposed MOA: NaCl in the airway creates an osmotic gradient, this osmotic gradient draws water into the airway, Increased water in the airway helps to reduce mucus thickness, Thinner mucus is easier for the patient to expectorate (helps draw water into airway, mucus can slide out easier) ◦ Can reduce the percent of saline if intolerance occurs (3% or 3.5%) ◦ Recommended in all CF patients ≥ 6 years; Can also use in select cases in patients < 6 years old bronchospasm issue - use bronchodilator (albuterol) prior

Answer 6

◦ MOA: Exact MOA unclear Proposed MOA: Draws water into the airways to hydrate the mucus ◦ Approved 18 years and older ◦ Dry powder inhaler ◦ Requires tolerance test before use ◦ Alternative to hypertonic saline ◦ Side effects: main one bronchospasm, hemoptysis - Administer albuterol 5-15 min before use

Answer 7

azithroymcin, ibuprofen

Answer 8

◦ MOA: Immunomodulating effects ◦ May not be tolerated due to GI side effects – dose can be reduced ◦ Recommended in patients with chronic pseudomonas ◦ Consider in patients without chronic pseudomonas

Answer 9

◦ MOA: anti-inflammatory NSAID ◦ Check levels: Goal peak > 50 mcg/mL and <100 mcg/mL ◦ Not well tolerated due to GI side effects; kidney injury risk

Answer 10

inhaled corticosteroids - flovent, asthmanex leukotriene modifiers - montelukast oral corticosteroids

Answer 11

Albuterol ◦ MOA: Short acting beta agonist ◦ Used to open up airways prior to airway clearance therapy and to decrease bronchoconstriction prior to inhaled hypertonic saline; Theoretically increases cilliary beating Ipratropium: Not recommended by current guidelines

Answer 12

FEVI, FVC, FEF25-75

Answer 13

Not well defined Typical clinical features ◦ Increased cough ◦ Increased sputum production ◦ Shortness of breath ◦ Chest pain ◦ Loss of appetite ◦ Loss of weight ◦ Decreased lung function Cover past and current bacteria – the big players

Answer 14

S. aureus (MSSA, MRSA) P. aeruginosa H. influenza, K. pneumonia E. Coli Stenotrophomonas maltophilia Burkholderia cepacia Aspergillus fumigatus Achromobacter

Answer 15

MRSA (only single coverage needed) example: bactrim, clinadmycin, vancomycin, tetracycline, linezolid MSSA (only single coverage needed) cefazolin, unasyn, coverage by anti- pseudomonal beta lactam

Answer 16

Double coverage Two different MOA (typically beta-lactam with aminoglycoside) Piperacillin-tazo, imipenem-cilast, ceftazidime, meropenem, cefepime with aminoglycoside (tobra, amikacin) Gentamicin no longer recommended If history of pseudomonas typically cover even if doesn’t grow in current culture

Answer 17

beta-lactams, aminoglycosides, and quinolones

Answer 18

increased renal and non-renal Cl - max dose at shortest interval or prolonged infusion at higher doses

Answer 19

icnreased Cl and Vd; higher doses

Answer 20

no significant change higher doses to penetrate thick, sticky mucus

Answer 21

tobramycin, aztreonam

Answer 22

◦ Recommended for initial pseudomonas eradication – One 28 day course ◦ Suppression therapy in 28 days cycle for patients with chronic pseudomonas

Answer 23

Administration time 15-20 minutes Premade solution

Answer 24

Used in patient with chronic pseudomonas Patients that can’t tolerate tobramycin Can be used in off months Treatment time: 5 minutes per treatment X 3 = 15 minutes per day Cleaning time: 15 minutes per day (depending on number of hand sets)

Answer 25

Administration time 2-3 minutes Bronchodilator pretreatment Extensive cleaning required

Answer 26

Delivery of the drug to the site of infection Reduced systemic exposure Decreased risk of systemic side effects Route allows for chronic administration of antipseudomonal antibiotics without IV Ability to get higher concentrations of the drug to the site

Answer 27

Exocrine insufficiency 85% ◦ Mucus obstructs exocrine ducts ◦ Decreased enzymes (amylase, lipase, protease) and HCO3 output

Answer 28

help pts absorb their food ◦ Do not exceed 10,000 units of lipase/kg/day Pancreatic enzymes are adjusted based on number of stools per day, fat content of stools, and growth/weight creon, pancrease, zenpep, pertzye, viokace

Answer 29

used in pts with tube feeds, secretes enzymes into tube feeds

Answer 30

Vitamin D: 25-OH – goal > 30; preferred supplement cholecalciferol (vitamin D3) Vitamin A Level Vitamin E Level Vitamin K: PT/INR (all fat soluble vitamins) Combination CF vitamins ◦ Aquadeks ◦ MVW complete ◦ DEKA essentials or plus

Answer 31

Diagnosis ◦ Fasting plasma glucose ≥ 126 mg/dL ◦ 2 hour plasma glucose ≥ 200 mg/dL Screening ◦ OGTT annually in CF patients > 10 years ◦ HgbA1c – not reliable for diagnosis Ketoacidosis rare (treat with insulin) Macrovascular effects rare

Cystic Fibrosis Flashcards

(55 cards)