CF Pharmacotherapy

Question 1

CF medications are given ____

Answer 1

Lifelong - adherence is VERY IMPORTANT

Question 2

Ideally CF patients should be…

Answer 2

Followed + managed by a multidisciplinary team specializing in CF care

Question 3

The goal of pulmonary therapies is to…

Answer 3

Increase airway clearance

Question 4

The administration order of inhaled therapies is important. This order is…

Answer 4

Bronchodilator
Hypertonic saline
Dornase Alfa
Physical airway clearance
Inhaled antibiotics
Inhaled corticosteroids

Question 5

Pulmonary therapies are indicated in all CF patients ____ and have been shown to…

Answer 5

6+; shown to improve FEV1, QOL, and decrease rate of exacerbation

Question 6

Bronchodilators include…

Answer 6

SABA
SAMA

Question 7

The purpose of bronchodilator usage is to…

Answer 7

Decrease bronchospasm associated with other inhaled therapies (hypertonic saline, antibiotics)

Question 8

The purpose of hypertonic saline is to…

Answer 8

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

Question 9

Unique administration with hypertonic saline is…

Answer 9

A nebulizer machine is required

Question 9

Adverse effects with hypertonic saline include…

Answer 9

Coughing
Sore throat
Wheeze
Bronchospasms

Question 10

MOA of dornase alfa is to…

Answer 10

Decrease mucous viscosity and decrease inflammation in lungs

Good for maintain FEV1

Question 11

These are the IV antibiotics usually used for pulmonary management…

Answer 11

Levofloxacin
Aztreonam
Colistimethate
Tobramycin

Question 12

Inhaled antibiotics are used for patients with ____ on sputum culture

Answer 12

Pseudomonas

For ealy eradication treatment, chronic suppressive therapy

Question 13

Usual regimen for IV antibiotics is ____ to help prevent resistance

Answer 13

28 days on, 28 days off - cycle

Question 14

Pancreatic enzyme replacement therapy is initiated in most patients in order to…

Answer 14

Break down fats + enhance vitamin and mineral uptake

Question 15

There are many types of products with various lipase units. These products…

Answer 15

Are NOT interchangeable

Question 16

Adverse effects from pancreatic enzymes include…

Answer 16

Bloating, flatulence, pain, loose stools
Steatorrhea
Dyspepsia

Question 17

These vitamins are likely the ones that will need to be replaced…

Fat-soluble !

Answer 17

A,D,E,K

Question 18

CFTR modulators work to…

Answer 18

Partially restore CFTR function in patients with specific mutations

SPECIFIC !!!

Question 19

CFTR modulators should be initiated…

Answer 19

At the youngest age possible

Question 20

A “corrector” in CFTR modulators works by…

Answer 20

Fixing flaws in CFTR protein, so it can form right shape + traffic to cell surface

Question 21

A “potentiator” in CFTR modulators works by…

Answer 21

Binding to defective protein at cell surface - opens chloride channel + holds it open so chloride ions can flow through

Question 22

3 drugs in Trikafta, a CFTR modulator include…

Answer 22

Elexacaftor - Corrector
Tezacaftor - Corrector
Ivacaftor - Potentiator

Question 23

Trikafta needs to be taken with…

Answer 23

Fat containing foods

Question 24

Safety concerns with Trikafta primarily include ____, hence monitoring is primarily via...

Answer 24

Elevated LFT's Behavioural/psychiatric changes Cataracts Monitoring: routine LFT monitoring, opthalmological exam

Question 25

Trikafta DI's are usually with...

Answer 25

CYP 3A4 inhibitors and inducers

Question 26

Efficacy of Trikafta shows...

Answer 26

Significantly increased FEV1 + evidence of sustained FEV1 improvements with long-term usage Evidence of improvement in lung function trajectory

Question 27

GERD and CF-related diabetes are treated...

Answer 27

Similarly as non-CF populations

Question 28

Azithromycin as chronic use may be considered for...

Answer 28

Patients with bacterial colonization - potential anti-inflammatory action | NOT common

Question 29

Pharmacist role in CF treatment is to...

Answer 29

Assess inhaler technique, adherence, and monitor for adverse effects/ signs of worsening disease Assure drug coverage

Question 30

A CF exacerbation should be treated with... ## Footnote Exacerbation = significant + sustained change from baseline in respiratory symptoms, pulmonary function, or chest X-ray

Answer 30

A minimum of 2 weeks IV antibiotic therapy ## Footnote Pharmacist role = appropriate drug selection, therapeutic drug monitoring, etc.

Question 31

If there are NO previous cultures available to guide antimicrobial choices, empiric therapy is...

Answer 31

Piperacillin/tazobactam Ceftazidime with IV tobramycin | Coverage for most CF pathogens and pseudomonas

Question 32

Some PK of drugs may be altered in CF. Factors that need to be considered include...

Answer 32

Extent/speed of oral absorption may be delayed Distribution changes - malnourishment, hypoalbuminuria Enhanced clearance of medications