Cystic Fibrosis Flashcards
(47 cards)
an incurable, hereditary disease caused by a mutation in the gene for the protein cystic fibrosis transmembrane conduct regulator (CFTR)
CF
What does the CFTR mutation cause…
abnormal transport of ____,____and____ ions across the epithelium.. which causes?
abnormal transport of chloride, bicarbonate, and sodium ions across the epithelium, leading to think, viscous secretions affecting the lungs, pancreas, liver and intestines
–> difficulty breathing, lung infections, digestive complications
When are CF patients normally diagnosed? what age?
by age 2
If the initial screening identifies a risk of CF, then what test is performed?
a sweat chloride test, “sweat test”
confirms diagnosis - HIGH sodium in CF sweat
What are the classic symptoms of CF?
salty tasting skin, poor growth, and poor weight gain, coughing, SOB.
Patients experience obstruction of pancreatic ducts causing…
steatorrhea (fatty stools).
Malnutrition can result if CF is not treated
In CF, what is the first intervention (opens the airways)
inhaled bronchodilators (albuterol)
In CF, what is the second intervention (mobilizes mucus to improve airway clearance)
hypertonic saline (HyperSal)
In CF, what is the third intervention (decreases viscosity of mucus to promote airway clearance)
Dornase alfa (pulmozyme)
In CF, what is the fourth intervention (mobilizes mucus to improve airway clearance)
chest physiotherapy
In CF, what is the fifth and final intervention (controls airway infection)
inhaled antibiotics (targets p. aeruginosa)
The correct order of inhaled medication for CF is critical for…
to maximize absorption and effect
Do inhaled therapies have large systemic absorption?
no! minimal, small risk of toxicity
Impaired mucus clearance causes bacterial colonization and lung infections.
The most common organisms seen EARLY in the disease are:
staphylococcus aureus
H. influenzae
Which organism is commonly found in adolescents and adults?
Pseudomonas aeruginosa
What is characterized as an increase in cough, sputum production with change in sputum color (green), SOB and a rapid decline in FEV1
acute pulmonary exacerbations
How are infections caused by p aeruginosa treated?
2 IV antibiotics to provide synergy and prevent resistance, doses tend to be LARGER than normal
options: AG, beta lactase, quinolone and others that covers pseudomonas
For chronic infections, what is the recommended treatment for patients with pseudomonas lung infections?
inhaled antibiotics to reduce the bacterial burden; treatment is cycled with 28 days on, 28 days off
For chronic infections when a patient is receiving inhaled antibiotics, when should their bronchodilator/mucolytic be administered?
BEFORE antibiotics
Does azithromycin have direct bactericidal activity against pseudomonas?
no! but it disrupts biofilm formation by the bacteria which can improve lung function and decrease exacerbations
List airway clearance therapies (3)
1) bronchodilator (albuterol)
2) Hypertonic saline (HyperSal, PulmoSal) - nebulizer
3) Dornase alfa (Pulmozyme)
This drug works by degrading extracellular DNA in the lungs to decrease viscosity of thin mucus
Dornase alfa/ Pulmozyme
Which drug should NOT be mixed with any other drug in the nebulizer?
dornase alfa/ Pulmozyme
Which drug has to have the ampules stored in the fridge and need to be protected from light?
dornase alfa/ pulmozyme
