Respiratory Exam 1 - CYSTIC FIBROSIS Flashcards
Aerosolized Antibiotics
Activity against Pseudomonas aeuroginsoa
Tobramycin Inhaled Solution (TOBI)
Aerosolized Antibiotic
Tobramycin Inhaled Solution (TOBI) - SPECTRUM
Gm(-) spectrum
Tobramycin Inhaled Solution (TOBI) - DOSING
> 6yo: 300mg Q12H
(QD in renal impairment)
Takes 15-20min to administer
28 days on, 28 days off
Tobramycin Inhaled Solution (TOBI) - AEs
Bronchospasm
Hoarseness
Pregnancy Category D
Aztreonam Inhaled Solution (Cayston)
Aerosolized Antibiotic
Aztreonam Inhaled Solution (Cayston) - DOSING
> 7yo: mix 1 vial of medication with 1 ampule of saline and add to ALTERA nebulizer handset
75mg TID (at least 4h between doses)
2-3min to administer
28 days on, 28 days off
Aztreonam Inhaled Solution (Cayston) - AEs
Bronchospasm Sore throat Nasal congestion Fever Pregnancy Category B
Colistimethate (Colistin)
Aerosolized Antibiotic
Colistimethate (Colistin) - DOSING
75-150mg BID
Colistimethate (Colistin) - AEs
Bronchospasm:
Use Albuterol after reconstitution to decrease
Mucolytics
Decrease viscosity of pulmonary secretions
Increase clearance of sputum
Dornase alfa (Pulmozyme)
Mucolytic
Dornase alfa (Pulmozyme) - MOA
Presence of DNA produces viscous mucus
-Selectively cleaves DNA & decreases mucus viscosity
Increase airflow and decrease risk of infection
Dornase alfa (Pulmozyme) - DOSING
2.5mg QD via nebulizer
Not approved in children <40)
Dornase alfa (Pulmozyme) - CONSIDERATIONS
Improvement in lung function in 8 days
-Increases adherence
Preserves lung function
Hypertonic Saline
Mucolytic
Hypertonic Saline - MOA
7% saline solution
Increases volume of fluid on epithelial lining of the airway to maintain normal ciliary flow and increase sputum expectoration
Hypertonic Saline - AEs
Bronchospasm
-Pretreatment with albuterol
Inhaled Corticosteroids
Anti-Inflammatory Agent
Inhaled Corticosteroids - INDICATIONS
Reserved for patients with reactive airways (asthma)
Oral Corticosteroids
Anti-Inflammatory Agent
Oral Corticosteroids - INDICATIONS
Long term:
Benefit lost shortly following DC
Short term:
Used in pts with aspergillus colonization
Helpful in acute exacerbations
Oral NSAIDS (Ibuprofen)
Anti-Inflammatory Agent
Oral NSAIDS (Ibuprofen) - INDICATIONS
Benefit not seen immediately
Shows slowed rate of pulmonary decline
Does not improve lung function
Oral NSAIDS (Ibuprofen) - DOSING
20-30mg/kg BID
Azithromycin
Anti-Inflammatory Agent
Azithromycin - INDICATIONS
Decreases sputum production
Preserves lung function & decreases pulmonary exacerbations
B2-Adrenergic Receptor Agonists
Bronchodilators
SABA (Albuterol) - INDICATIONS
Used in combination with airway clearance to stimulate mucociliary clearance
Used in combination with other inhaled therapies to prevent bronchospasm
LABA (Salmeterol)
Benefit for pts with recurrent wheeze or dyspnea
Inhaled Anticholinergic Agents (Impratropium)
Bronchodilators
Inhaled Anticholinergic Agents (Impratropium) - INDICATIONS
Act to decrease bronchospasm
Ivacaftor (Kalydeco)
CF Transmembrane Conductance Regulator Potentiator
Ivacaftor (Kalydeco) - MOA
Potentiates epithelial cell Cl- ion transport of defective G551D mutant cell-surface CFTR protein
Improves regulation of salt and water absorption and secretion in various tissues
Improves lung function
Chest Physical Therapy (CPT)
The traditional form
Percussion & Postural drainage
Takes about 30min
Chest Physical Therapy (CPT)
The traditional form
Percussion & Postural drainage
Takes about 30min
CPT - Percussion
Chest is clapped to dislodge mucus
Done in various positions
CPT - Postural drainage
Pt gets into different positions
Allows gravity to assist with mucus drainage
Oscillating Positive Expiratory Pressure (PEP)
Vibrates small and large airways
Pt blows into device multiple times
Pt coughs or huffs
Repeat multiple times
High Frequency Chest Compression (HFCC)
Most commonly used technique
Inflatable vest that uses external oscillation
Every 5min pt stops machine and coughs
Sweat Cl- concentration <39mmol/L
CF unlikely
Sweat Cl- concentration 40-59mmol/L
CF possible
Sweat Cl- concentration >60mmol/L
CF diagnosis
Mutation of CF gene
Dysfunctional CFTR protein
Dysfunctional CFTR protein leads to
Decreased Cl- secretion
Increased sodium reabsorption
Altered viscosity of fluid secreted by exocrine gland
Early pathogens of CF
***Pseudomonas
Staph. Aureus
Non-typical H. Flu
CFF Guideline Recommendations - tobramycin for mild disease
FEV1 70-89%
Recommend the chronic use of inhaled tobramycin to reduce exacerbations - >/=6yo
CFF Guideline Recommendations - tobramycin for moderate to severe disease
FEV1 <69%
Strongly recommend the chronic use of inhaled tobramycin to improve lung function & reduce exacerbations - >/=6yo
CFF Guideline Recommendations - Other antibiotics
Not recommended
CFF Guideline Recommendations - Dornase Alfa for mild disease
FEV1 70-89%
Recommend chronic use to improve lung function & reduce exacerbations - >/=6yo
CFF Guideline Recommendations - Dornase Alfa for moderate-severe disease
FEV1 <69%
Strongly recommend chronic use to improve lung function & reduce exacerbations - >/=6yo
CFF Guideline Recommendations - hypertonic saline
Recommend chronic use to improve lung function & reduce exacerbations - >/=6yo
CFF Guideline Recommendations - inhaled corticosteroids
Not recommended
CFF Guideline Recommendations - oral corticosteroids
Not recommended in children
Insufficient evidence in adults
CFF Guideline Recommendations - NSAIDS
> /=6yo with FEV1 >60% recommends chronic use of ibuprofen to slow the loss of lung function
CFF Guideline Recommendations - azithromycin
Recommend chronic use to improve lung function & reduce exacerbations - >/=6yo
CFF Guideline Recommendations - leukotriene modifiers
Insufficient evidence
CFF Guideline Recommendations - B2-agonists
Recommend chronic use to improve lung function - >/=6yo
CFF Guideline Recommendations - anticholinergic agents
Insufficient evidence