2023 RxPrep Ch. 55 Cystic Fibrosis

Cystic Fibrosis

Incurable hereditary disease caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR)

The mutation in CFTR causes abnormal transport of which ions, leading to thick mucus.

- chloride

- bicarbonate

- sodium

What test is given to newborns to test for cystic fibrosis

sweat chloride test (chloride is high in sweat of people with CF)

CF

Signs & Symptoms

- salty skin

- poor growth and weight gain

- thick & sticky mucus

- frequent lung infections, coughing, SOB

- fatty stools

Order of inhaled medications in CF

- Bronchodilator (opens airways)

- Hypertonic saline (mobilizes mucus)

- Dornase alfa (thins mucus)

- Chest PT (mobilizes mucus)

- Inhaled Abx (controls airway infection)

Inhaled abx treatment is cycled with _____ days on therapy, followed by ____ days off therapy.

28 & 28

Which inhaled abx are used in CF?

- Tobramycin (TOBI)

- Aztreonam

Why is azithromycin given in CF?

to decrease biofilm formation, inflammation, & exacerbations (not given for antimicrobial purposes)

The dose of Pancreatic enzyme products is dosed based off which component?

lipase

How do CFTR modulators work?

increase the amount of time the CFTR channels remain open, increasing chloride transport

What are the CFTR modulators?

- Kalydeco (ivacaftor)

- Orkambi (lumacaftor/ivacaftor)

- Symdeko (tezacaftor/ivacaftor)

- Trikafta (elexacaftor/tezacaftor/ivacaftor)

Which CFTR modulator is NOT approved for homozygous F508del mutation?

Kalydeco (ivacaftor)