Cystic Fibrosis part one Flashcards
(39 cards)
CF is the most Most common life-limiting genetic disorder in which population?
caucasian
expected life span of someone with CF
about 40 years
CF Goals of treatment
Slow or stop the progression of the disease and Allow for normal growth and development
Approaches to care for CF
good nutrition, Pancreatic enzymes and vitamin supplementation, Airway clearance and anti-inflammatory therapies, Antipseudomonal agents, Recognize altered pharmacokinetics
examples of chest physiotherapy
postal drainage, chest percussion, vibration technique devices
clearance therapy is for
for individuals ≥6 years and are administered concurrently with percussion therapy:
what is the purpose of a bronchodilator?
Albuterol helps open up the airways and prevents bronchospasm
what is the purpose of Hypertonic saline (HyperSal®)?
how does it work?
hydrates the airway mucus secretions and facilitates mucociliary function
draws water from the airway to establish aqueous surface layer (ASL)
what is the purpose of Domase alfa (Pulmozyme®)?
this enzyme cleaves extracellular DNA which results in decreased viscosity of mucus therefore airflow in the lung is improved and risk of bacterial infection may be decreased
very $$$$$
when are aerosolized antibiotics used? when are they indicated
administered after the CF patient completes percussion therapy
indicated based on severity of lung disease and sputum cultures
Albuterol is a
Βeta-2 adrenergic agonists bronchodilator
Theophylline is an
Anti-Obstructive-Bronchodilators that is infrequently used due to Lack of proven efficacy, narrow therapeutic margin, GI symptoms and Tachycardia
what are examples of Anticholinergic anti obstructive bronchodilators?
Ipratropium – short acting
Tiotropium – long acting
Tiotropium shows no
significant improvement
In Cf there is a presence of abundant, purulent airway secretions composed primarily of what?
highly polymerized DNA which produces a viscous mucous
principal source of highly polymerized DNA is from
the nuclei of degenerating neutrophils which is present in large concentrations in infected lung secretions
in CF the decreased mucociliary transport and persistent infections is due to what?
large amounts of viscous mucous
what are the main ADE of Dornase alpha?
chest pain, fever, rash, pharyngitis, rhinitis, dyspepsia, conjunctivitis, laryngitis
is Chronic treatment with oral antibiotics to control infection encouraged?
no, Benefits have not be shown to outweighs the problems associated with antibiotic resistance
exceptions to the discouragement of chronic antibiotic treatment
Oral Azithromycin
Nebulized Tobramycin
Nebulized Aztreonam
Inhaled colistin (2nd line)
discuss the use of azithromycin in CF patients
Benefits may be due to its anti-inflammatory and/or antibacterial properties
Macrolides are unable to kill pseudomonas bacteria, but reduce the ability of pseudomonas to produce biofilms
this macrolide suppresses the excessive inflammatory response
who is given azithromycin?
children > 6yrs, with clinical evidence of airway inflammation
this may present as a chronic cough or any reduction in FEV1, whether or not they are infected with P. aeruginosa
discuss the use of Nebulized Tobramycin in CF patients
improves lung function and reduces acute pulmonary exacerbations in patients who are chronically infected with P. aeruginosa
Mechanism-Interferes with bacterial protein synthesis by binding to 30S and 50S ribosomal subunits, resulting in a defective bacterial cell membrane
how do patients take Nebulized Tobramycin
administered by nebulizer twice daily for 28 days, alternating with 28 days off treatment
