CF3 Flashcards
what anti-inflammatory drug are not recommended for CF patients
inhaled corticosteroids; no benefit
oral corticosteroids; risks outweigh benefits
when can corticosteroids be used as part of therapy for CF patients
- asthma
2. allergic bronchopulmonary aspergillosis (ABPA)
what CF population are NSAIDs recommended for
6-17 year olds
what did NSAID studies in CF patients show
high dose ibuprofen decreases pulmonary decline but has no difference in exacerbation rate
what are 2 negative effects of NSAID (ibuprofen only) use in CF patients
lower doses may worsen pulmonary inflammation
GI and renal adverse effects
when are inhaled antibiotics used for CF patients
suppressive therapy for patients infected with pseudomonas
inhaled tobramycin is recommended for whom
- older than 6 years of age with pseudomonas
2. younger than 2 years of age with pseudomonas
what is the one exception for using antibiotics for pulmonary exacerbations only (this one drug can be used when a culture is found positive; without exacerbations)
inhaled tobramycin for eradication of pseudomonas (use for one month then stop)
what must be monitored with inhaled tobramycin
Sr Creatinine, BUN, urinalysis, audiogram, frequency of hospitalizations and IV antibiotic use
what are the 2 dosage forms of inhaled tobramycin
- nebulizations
2. dry powder inhaler
what are the two main differences in DPI inhaled tobramycin and nebulized inhaled tobramycin?
- DPI is only indicated for people older than 6 with pseudomonas
- DPI can’t be used for eradication of pseudimonas
what are 3 common adverse effects of DPI tobramycin
cough
dysphonia
dysgeusia
What is another antibiotic used for suppressivbe therapy in CF patients with chronic pseudomonas other than tobramycin
inhaled aztreonam (Cayston)
what is the time for nebulization of inhaled aztreonam
3-5 minutes (much shorter than others)
what age group is inhaled aztreonam approved for?
6 years old or older
list the order of 4 different inhaled therapies for CF
- albuterol
- mucolytic agent
- airway clearance
- antibiotic
what are CFTR modulators two classes?
what do they do?
- CFTR potentiators = help keep cell membrane open
2. CFTR correctors = helps defective CFTR to reach cell membrane without being destroyed
what is the only CFTR modulator approved for use?
ivacaftor (Kalydeco)
what class of drug is ivacaftor (Kalydeco)?
CFTR potentiator
what is ivacaftor approved for
CF patients 6 or older with a gating mutation on at least 1 allele
what is a gating mutation example
G551D
what are 4 benefits of ivacaftor
- improved lung function (around 10% increase of FEV1)
- increased weight
- increased time to next exacerbation
- increased quality of life
why is ivacaftor have so many drug interactions
CYP3A4 substrate
P-glycoprotein inhibitor
what drugs would cause a decreased dose of ivacaftor to be recommended
erythromycin, fluconazole (moderate CYP3A4 inhibitors), ketoconazole, clarithromycin (strong CYP3A4 inhibitors)
what should be avoided when taking ivacaftor
strong CYP3A4 inducers (rifampin, St. John’s Wort)
what are some side effects and monitoring of ivacaftor
increased liver enzymes
annual eye exam needed due to cataracts in juvenile rats
what is the CFTR corrector that will be available soon in combo with ivacaptor
lumacaftor
