CF Pharmacotherapy Flashcards
CF medications are given ____
Lifelong - adherence is VERY IMPORTANT
Ideally CF patients should be…
Followed + managed by a multidisciplinary team specializing in CF care
The goal of pulmonary therapies is to…
Increase airway clearance
The administration order of inhaled therapies is important. This order is…
Bronchodilator
Hypertonic saline
Dornase Alfa
Physical airway clearance
Inhaled antibiotics
Inhaled corticosteroids
Pulmonary therapies are indicated in all CF patients ____ and have been shown to…
6+; shown to improve FEV1, QOL, and decrease rate of exacerbation
Bronchodilators include…
SABA
SAMA
The purpose of bronchodilator usage is to…
Decrease bronchospasm associated with other inhaled therapies (hypertonic saline, antibiotics)
The purpose of hypertonic saline is to…
Increase mucous clearance from airways, by thinning out secretions - reduce viscosity + improve mucus flow
Usually start with 7% but can decrease to 3% if intolerable
Unique administration with hypertonic saline is…
A nebulizer machine is required
Adverse effects with hypertonic saline include…
Coughing
Sore throat
Wheeze
Bronchospasms
MOA of dornase alfa is to…
Decrease mucous viscosity and decrease inflammation in lungs
Good for maintain FEV1
These are the IV antibiotics usually used for pulmonary management…
Levofloxacin
Aztreonam
Colistimethate
Tobramycin
Inhaled antibiotics are used for patients with ____ on sputum culture
Pseudomonas
For ealy eradication treatment, chronic suppressive therapy
Usual regimen for IV antibiotics is ____ to help prevent resistance
28 days on, 28 days off - cycle
Pancreatic enzyme replacement therapy is initiated in most patients in order to…
Break down fats + enhance vitamin and mineral uptake
There are many types of products with various lipase units. These products…
Are NOT interchangeable
Adverse effects from pancreatic enzymes include…
Bloating, flatulence, pain, loose stools
Steatorrhea
Dyspepsia
These vitamins are likely the ones that will need to be replaced…
Fat-soluble !
A,D,E,K
CFTR modulators work to…
Partially restore CFTR function in patients with specific mutations
SPECIFIC !!!
CFTR modulators should be initiated…
At the youngest age possible
A “corrector” in CFTR modulators works by…
Fixing flaws in CFTR protein, so it can form right shape + traffic to cell surface
A “potentiator” in CFTR modulators works by…
Binding to defective protein at cell surface - opens chloride channel + holds it open so chloride ions can flow through
3 drugs in Trikafta, a CFTR modulator include…
Elexacaftor - Corrector
Tezacaftor - Corrector
Ivacaftor - Potentiator
Trikafta needs to be taken with…
Fat containing foods
Safety concerns with Trikafta primarily include ____, hence monitoring is primarily via…
Elevated LFT’s
Behavioural/psychiatric changes
Cataracts
Monitoring: routine LFT monitoring, opthalmological exam
Trikafta DI’s are usually with…
CYP 3A4 inhibitors and inducers
Efficacy of Trikafta shows…
Significantly increased FEV1 + evidence of sustained FEV1 improvements with long-term usage
Evidence of improvement in lung function trajectory
GERD and CF-related diabetes are treated…
Similarly as non-CF populations
Azithromycin as chronic use may be considered for…
Patients with bacterial colonization - potential anti-inflammatory action
NOT common
Pharmacist role in CF treatment is to…
Assess inhaler technique, adherence, and monitor for adverse effects/ signs of worsening disease
Assure drug coverage
A CF exacerbation should be treated with…
Exacerbation = significant + sustained change from baseline in respiratory symptoms, pulmonary function, or chest X-ray
A minimum of 2 weeks IV antibiotic therapy
Pharmacist role = appropriate drug selection, therapeutic drug monitoring, etc.
If there are NO previous cultures available to guide antimicrobial choices, empiric therapy is…
Piperacillin/tazobactam
Ceftazidime with IV tobramycin
Coverage for most CF pathogens and pseudomonas
Some PK of drugs may be altered in CF. Factors that need to be considered include…
Extent/speed of oral absorption may be delayed
Distribution changes - malnourishment, hypoalbuminuria
Enhanced clearance of medications
