Day 6- Cystic Fibrosis Flashcards
Do both parents need to carry the gene for a child to develop cystic fibrosis and what gene causes it and who does it normally affect?
What is the gold standard for diagnosing CF?
How does airway clearance treatment with a vest or percussion work?
Yes, CRTR gene(codes for a chloride channel). Caucasian.
Sweat test, measure chloride in sweat.
Postural drainage and percussion(3x daily).
What to know about albuterol treatment?
What are your big airway clearance treatment drugs?
How do you treat chronic infection with pseudomonas in CF?
Normally PRN.
Dornase alfa( thins mucous, 2.5 daily). Hypertonic saline(increases hydration of airway clearance). Recommended in patients 6 years and above.
Aerosolized antibiotics–> Aztreonam(TID) and tobramycin(BID). Will cycle them 28-days every other month.
How do you treat nutrition issues in CF and are they interchangeable?
When are enteric coated preparations of pancreatic lipase not ideal?
What are your big counseling points with pancreatic enzymes?
Pancreatic enzymes. No not really.
Not ideal in patients on antisecretory therapy. Viokace is best for PPI’s or H2’s.
GI effects, need to take with a meal or right before(cannot take it after),Do not take with Ca and Mg,No crushing but can be sprinkled, Do not start antisecretory without contacting MD.
What are the vitamin supplementation CF patients need?
What can treat bile flow problems with CF patients?
What is the best CF inflammation treatment and what special things to know about Ibuprofen?
Fat soluble(ADEK), Iron.
Ursodiol.
Ibuprofen(only recommended patients between 6-17, need serum to be between 50-100 mcg/mL) and Azithromycin(use in pseudomonas patients). Can NOT give oral steroids.
What is a new G551D target medicine to treat CF?
How does Orkanmbi work?
How does Symdeko work?
Ivacaftor(G551D target), take with high fat foods and helps improve regulation of salt and water absorption/secretion in tissues.
f508Del mutation( moves flawed CFTR to new location), high fat foods.
f508Del mutation.
How do you treat acute exacerbations?
How do pharmacokinetics work in CF population?
What organisms cause pneumonia in CF?
Short course of systemic coricosteroids may be beneficial, hospitalization required for IV antibiotics(2 antipseudomonal drugs recommended), aminoglycosides used frequently.
Larger volumes of distribution and faster rate of clearance so you need to give a lot more of a drug(30-35% larger dose of aminoglycosides).
Pseudo, Staph, Haemophilius, Burkholderia cepacia.
