Cystic Fibrosis Treatment

Question 1

What is HFCWO?

Answer 1

High frequency chest wall oscillation a.k.a. vest therapy

Question 2

Name the 4 different types of physiotherapy used in non-pharmacologic treatment of CF Lung Disease?

Answer 2

1. Vest therapy (HFCWO)
2. Postural drainage (PD)
3. Positive Expiratory Pressure (PEP)
4. Exercise

Question 3

Name the order in which inhaled medications for CF should be given.

Answer 3

1. Beta-agonist
2. Hypertonic Saline
3. Dornase Alfa
4. Airway Clearance Techniques
5. Inhaled Antibiotics

Question 4

How do you adjust the dose frequency of Ivacaftor if co-administered with grapefruit juice?

Answer 4

BID-> QD

Question 5

How do you adjust dose frequency of TEVA/IVA or ETA/TEVA/IVA when given with fluconazole?

Answer 5

QAM/QPM -> rotate AM and PM doses daily

Question 6

How do you adjust dose frequency of Vanza/Teva/Deutiva when given with erythromycin?

Answer 6

QD to QOD

Question 7

How do you adjust the dose frequency of Ivacaftor, Teza/IVA, Elexa/Teza/Iva when given with ketoconazole?

Answer 7

BID -> 2x weekly

Question 8

How do you adjust the dose frequency of veza/teva/deutiva when given with clarithromycin?

Answer 8

QD to weekly

Question 9

What are some strong CYP3A4 inducers?

Answer 9

-rifampin
-rifabutin
-phenobarbital
-carbamazepine
-phenytoin
-St. Johns

Question 10

What effect on FEV1 and FVC does hypertonic saline have?

Answer 10

none

Question 11

What effect does dornase alfa have on FEV1?

Answer 11

improves FEV1

Question 12

What is the most common ADR of dornase alfa?

Answer 12

voice hoarseness

Question 13

What is the most common ADR of hypertonic saline?

Answer 13

bronchospasm

Question 14

Which inhaled antibiotic is preferred for pseudomonas coverage?

Answer 14

Tobramycin

Question 15

Which anti-inflammatory agent is preferred?

Answer 15

azithromycin

Question 16

When do you use Aztreonam?

Answer 16

-when tobramycin can’t be tolerated
-worsening despite tobramycin use
-pregnant
-patient preference

Question 17

What is the treatment regimen for inhaled antibiotics?

Answer 17

Tobramycin: 300 mg BID x 28 days then repeat sputum culture

Question 18

How many times can you repeat inhaled Tobramycin treatment regimen before switching over to IV antibiotics

Answer 18

1 time (2 treatments total)

Question 19

If the second course of TOBI fails, what do you do?

Answer 19

1. treat with IV antibiotics x 14 days
2. repeat TOBI course x 28 days

Question 20

What inhaled antibiotics do you use for early MRSA eradication?

Answer 20

1. bactrim + rifampin (or minocycline, if sulfa allergy) x 14 days
2. nasal, skin, and oral decontamination x 14 days (chlorhexidine rinse/wipes, mupirocin intranasal for 5 days)
3. environmental decontamination x 21 days

Question 21

What is the age indication for azithromycin?

Answer 21

age ≥ 6 months w/ PA

Question 22

What is the age indication for high dose ibuprofen?

Answer 22

age ≥ 6 years

Question 23

What is the goal BMI for children with CF?

Answer 23

BMI ≥ 50th percentile

Question 24

What is the goal BMI for adult males with CF?

Answer 24

BMI ≥ 23

Question 25

What is the goal BMI for female patients with CF?

Answer 25

BMI ≥ 22

Question 26

What kind of diet should CF patients follow?

Answer 26

moderate fat, high protein, and high caloric diet

Question 27

When should PERT be initiated in infants?

Answer 27

all infants with: -two CFTR variants associated with pancreatic insufficiency -fecal elastase value <200 mcg/g of stool AND/OR -signs and symptoms of malabsorption

Question 28

What is PERT dosing based on?

Answer 28

lipase

Question 29

What minerals should CF patients be supplemented with?

Answer 29

-calcium -iron -sodium chloride -zinc

Question 30

What are some signs of APE?

Answer 30

-decreased FEV1 or FVC -cough -increased sputum production -SOB -chest pain -loss of appetite -weight loss

Question 31

What classifies an APE as mild?

Answer 31

FEV decrease of less than or equal to 10%

Question 32

What classifies an APE as moderate/severe?

Answer 32

FEV1 decrease of more than 10%

Question 33

Where is mild APE treated?

Answer 33

outpatient

Question 34

What do you increase dornase alfa frequency to for treatment of APE?

Answer 34

BID

Question 35

What do you increase hypertonic saline frequency to for treatment of APE?

Answer 35

BID

Question 36

What do you increase bronchodilator frequency to for treatment of APE?

Answer 36

QID

Question 37

What needs to be done to the dose of systemic antibiotics for the treatment of APE due to altered PK in CF patients?

Answer 37

increase doses due to increased Vd and Cl