Cystic Fibrosis Flashcards
Which chromosome is the cystic fibrosis mutation on?
chromosome 7
Which class of mutation has defective protein synthesis?
Class I mutation
Which class of mutation has incorrect protein folding?
Class II mutation
F508 delta mutation is the most common mutation
Which class of mutation has reduced # of active proteins?
Class V mutation
Which class of mutation has limited function of Cl- proteins in the cell membrane?
Class IV mutation
Which class of mutation has defective regulation of Cl- protein makes it to membrane but doesn’t work?
Class III mutation
What are the 4 approaches to care for CF?
- Nutrition
- Pancreatic enzymes
- NSAIDS
- Anti-pseudomonal
Which CF drug selectively cleaves DNA which reduces mucus viscosity?
Anti-Obstructive - Dornase alpha (rhDNase) [Pulmozyme]
Effects of Azithromycin
- Doesn’t kill pseudomonas but reduces ability to cause biofilm
- Anti-inflammatory–>suppresses excessive immune response
Tobramycin (aminoglycoside) nebulized
- 28 days on treatment, 28 days off
- Interferes with 30s and 50s ribosomal subunits
- Decreases exacerbations in those chronically infected with Pseudomonas
Which two medications are alternated in CF?
Tobramycin (nebulized - aminoglycoside) and Aztreonam (inhaled - monobactam)
SE of Tobramycin
- Abnormal taste
- Eosinophilia
- Cough, wheezing, rales
- Voice alteration
- Tinnitus
For which CF patient is ibuprofen indicated for?
- Under 18
2. FEV >60%
Adverse drug events: IBU
- Gastrointestinal bleed
- Nephrotoxicity
- Tinnitus
- Headache
- Edema
- Fluid retention
Ivacaftor (Kalydeco) effect
“potentiator” of CFTR, meaning it increases the probability that the defective channel will be open and allow chloride ions to pass through the channel pore
Name 3 SE of Ivacaftor
- Increased LFTs
- Arthralgia
- Hyperglycemia
Orkambi (Lumacaftor + Ivacaftor) effect
Lumacaftor–> increases # of CFTR molecules that make it to the cell surface
Ivacaftor–>Improves Cl- conductance through CFTR
ADE of Orkambi (Lumacaftor + Ivacaftor)
- Nasopharyngitis
- INCREASED creatinine phosphokinase
- Menstrual disease
What is essential to remember about how to take Symdeko (Tezacaftor + Ivacaftor)?
This must be taken with fat containing food
Which liver enzyme metabolizes Symdeko (Tezacaftor + Ivacaftor)?
CYP3A
So Rifampin, St. John’s Wart will lower Symdeko level
Ketoconazole, Fluconazole, Grapefruit, Seville oranges will increase level
What are 2 important things to monitor with a patient taking Symdeko (Tezacaftor + Ivacaftor)?
- ALT and AST!!!
- check prior to starting medication
- check every 3 months for 1st year
- After 1st year, check yearly - Monitor for cataracts in children
When are O2 and BiPAP indicated for CF?
- Advanced CF
- Hypercapnea
What 4 things help us consider lung transplant in CF patient?
- FEV <30%
- Increased exacerbation frequency despite ABO
- Recurrent Pneumothorax
- Recurrent Hemoptysis
For giving pancreatic enzymes, how is the dose calculated?
Pancreatic enzymes are dosed based on LIPASE
ADE of pancreatic enzymes
*Oral ulcers
so, give with applesauce
Which vitamins need to be supplemented in Cystic Fibrosis?
Fat soluble
Vitamins A, D, E, K
What is the desired effect of Urodeoxycholic acid in CF?
Urodeoxycholic acid is a gallstone dissolution agent.
-decreases cholesterol content of bile
Name 4 side effects of urodeoxycholic acid
- Alopecia
- Thrombocytopenia
- Leukopenia
- INCREASED serum creatitine
Which CF patient is ibuprofen indicated for?
> 18 years old
and
FEV >60%
Ivacaftor is indicated for which gene mutation?
G551D mutation
Lumacaftor is indicated for which gene mutation?
F508 delta
Tezacaftor is indicated for which gene mutation?
F508 delta
