Cystic Fibrosis Flashcards
True or false: Cystic fibrosis affects all races, but mostly affects caucasians
True
Which gender is affected more by cystic fibrosis?
Males, but not by very much
CF is an autosomal (dominant/recessive) disease
Recessive
What phenotype does a person with one mutated CFTR gene have?
Carrier of CF, but not affected with the condition themselves
What is the most common mutation in CF?
delta 508
What is the median age at death of patients with CF?
34.1 years old
Where are cystic fibrosis transmembrane conductance regulators (CFTRs) located?
Sweat glands
What are the functional and physical presentations of mutations in CFTRs?
Functional: inability to move chloride out to the cell surface
Physical: thick and sticky mucus
Where are CFTR proteins located?
. Lungs
. Pancreas
. Intestines
. Liver
. Sweat glands
. Reproductive
How is screening for CF done in newborns?
Blood sample from heel is analyzed
What is considered the gold standard test for CF screening?
Sweat chloride test
What components are required for diagnosis?
Positive sweat test and clinical symptoms
Clinical presentation of CF in pancreas
. Deficiency of pancreatic enzymes, insulin
. Malabsorption, malnutrition, CF diabetes
Which of the following is not a goal of therapy when treating CF?
a. Airway clearance
b. Prevent and treat sinusitis
c. Prevent and treat pulmonary exacerbations
d. Adequately digest fats, proteins and complex carbs
d. Adequately digest fats, proteins and complex carbs
What are the four step that should be implemented during airway clearance?
. Bronchodilator
. Mucolytic
. Enzyme mucolytic
. Inhaled antibiotic
The four steps of airway clearance should be performed in conjunction with ________ therapy
Percussion
Which of the following is a mucolytic? An enzyme mucolytic?
a. Inhaled hypertonic saline
b. Inhaled sodium bicarbonate (2%)
c. Inhaled N-Acetylcysteines
d. Inhaled dornase alfa
Mucolytic: a. Inhaled hypertonic saline (HyperSal)
Enzyme mucolytic: d. Inhaled dornase alfa (Pulmozyme)
Recommended age for use of pulmozyme?
Dornase alfa is recommended for patients 6 years old and up
CF Foundation recommends inhaled _______ or ______ to age ≥ 6 years old with mild-to-severe lung disease with persistent __________ in sputum cultures
Tobramycin or aztreonam, Pseudomonas
Which inhaled antibiotic is recommended for ≥ 6 years old with persistent P. aeruginosa infection?
Azithromycin
