Cystic Fibrosis

Question 1

Cystic Fibrosis etiology

Answer 1

autosomal recessive disease

Question 2

What is the gene mutation in CF?

Answer 2

Long arm Chromosome 7

Question 3

What is the gene mutation in Class II, defective protein processing??

Answer 3

F508

Question 4

What is the gene mutation in Class III, defective regulation?

Answer 4

G551D

Question 5

List bronchodilators we would use as anti-obstructives

Answer 5

1. B-2 agonists- Albuterol
2. Theophylline
3. Anticholinergics- Ipratropium (short acting) and Tiotroprium (long-acting)

Question 6

Why do we use hypertonic saline as an anti-obstructives

Answer 6

Hydrates mucous my osmotically pulling water from airway to re-establish the aqueous surface layer that is deficient in CF

Question 7

what is the hallmark of CF?

Answer 7

Abundant/purulent airway secretions made of polymerized DNA from dead neutrophil
Viscous mucous

Question 8

What is the MOA of Dornase alpha (rhDNase)

Answer 8

Selectively cleaves DNA in mucous secretions to reduce mucous viscosity

Question 9

Dornase alpha SEs

Answer 9

1. Fever- 32% in pt’s with FVC <40%
2. Pharyngitis, rhinitis
3. Laryngitis
4. Dyspepsia
5. Conjunctivitis
6. Rash

Question 10

What is a downside to Dornase alpha?

Answer 10

You can produce antibodies to the medication

Question 11

What is the most common bacterial organism in kids/younger adults?

Answer 11

S. aureus

Question 12

What is the most common bacterial organism in adults (25-34 yrs)?

Answer 12

Pseudomonas aeruginosa

Question 13

What is the main benefit of Macrolides (Azithromycin)

Answer 13

Anti-inflammatory properties

–>Supres excessive inflammatory response

Question 14

Macrolides (Azithromycin) MOA

Answer 14

Reduces ability of pseudomonas to produce biofilms so other drugs can kill it

Question 15

Aminoglycosides (nebulizer Tobramycin) application

Answer 15

1. Improves lung function

2. Reduces acute pulmonary exacerbations chronically infected with pseudomonas

Question 16

What is unique about Aminoglycosides (nebulizer Tobramycin) dosing?

Answer 16

Alternate with 28 days of treatment to try and reduce resistance

Question 17

Aminoglycosides (nebulizer Tobramycin) SEs

Answer 17

1. GI: sputum discoloration, abnormal taste
2. Respiratory: Rales, wheezes, cough, voice alteration
3. Otic: Tinnitus
4. Hematologic: Eosinophilia

Question 18

List the monobactam (beta-lactam)

Answer 18

Inhaled Aztreonam

Question 19

Inhaled Aztreonam MOA

Answer 19

inhibits bacterial cell wall synthesis

Question 20

Inhaled Aztreonam application

Answer 20

Abx with antipseudomonal activity

Question 21

Inhaled Aztreonam SEs

Answer 21

1. Fever
2. Cough
3. Rash

Question 22

What is unique about Inhaled Aztreonam dosing

Answer 22

for 28 days, then off for 28 days- DO NOT repeat for 28 days after completion

Question 23

Who we do use HIGH dose ibuprofen in?

Answer 23

<18 y.o. with FEV1 >60%

Question 24

Ibuprofen SEs

Answer 24

1. GI: Epigastric pain, GI bleed
2. CV: Edema
3. Endocrine: Fluid retention
4. Otic: Tinnitus
5. CNS: HA

Question 25

Ivacaftor MOA

Answer 25

Potentiates epithelial cell chloride ion transport of defective (G551D) cell-surface CFTR protein

Question 26

What does Ivacaftor improve?

Answer 26

Regulation of salt and water absorption and secretion in lung, GI tract, etc

Question 27

Ivacaftor SEs

Answer 27

1. Hyperglycemia-Monitor blood sugars 2. Transaminases increased 3. HA 4. Abdominal pain 5. Nasopharyngitis 6. Arthralgia

Question 28

What gene mutation represents the largest group of people with CF? Percentage of people?

Answer 28

F508 gene mutation | 88%

Question 29

What is the combo effect of Lumacaftor/Ivacaftor

Answer 29

1. Reduction in pulmonary exacerbations 2. Improved lung function 3. Increased BMI

Question 30

What are the concerning SEs of Lumacaftor/Ivacaftor combo therapy?

Answer 30

1. Menstrual disease 2. Increased creatine phosphokinase 3. URI 4. Rhinorrhea 5. Nasopharyngitis 6. Fatigue

Question 31

When would we need to adjust the dosing of Tezacaftor/Ivacaftor?

Answer 31

If coconmitant use with moderate and strong inhibitors (antifungals) -->CYP3A interaction

Question 32

What do we need to monitor with Tezacaftor/Ivacaftor?

Answer 32

ALT and AST

Question 33

When do we discontinue Tezacaftor/Ivacaftor treatment?

Answer 33

LT or AST >5 x upper limit of normal

Question 34

What is a major SE of Tezacaftor/Ivacaftor?

Answer 34

Cataracts

Question 35

List the two vaccines we want to administer to our CF patients

Answer 35

1. Influenza- older than 6 mos. | 2. Pneumococcal

Question 36

What is Palivizumab?

Answer 36

Monoclonal antibody against RSV | Kids younger than 24 mos

Question 37

What FEV1 % would indicate a referral for a lung transplant?

Answer 37

FEV1<30%

Question 38

What do proton pump inhibitors increase your risk of?

Answer 38

Aspiration pneumonia

Question 39

What are the SEs of Omeprazole?

Answer 39

1. Acid regurgitation 2. Constipation 3. Back pain 4. Weakness 5. Cough

Question 40

What percentage of CF patients will have pancreatic insufficiency in their lifetime?

Answer 40

85%

Question 41

What are the main pancreatic enzymes?

Answer 41

1. Lipase 2. Amylase 3. Protease

Question 42

What is the dose calculated by?

Answer 42

According to the Lipase

Question 43

What are the major ADEs of Pancreatic Enzyme Supplementation

Answer 43

1. Oral Mucosa Ulcers with prolonged contact-Administer with food and rinse after 2. Fibrosing Colnopathy

Question 44

Why do we need to add vitamin supplementation in our CF patients?

Answer 44

Fat malabsorption due to: 1. Pancreatic insufficiency 2. Liver dz's

Question 45

What are fat soluble vitamins?

Answer 45

A, D, E & K

Question 46

What CF-related liver disease is an issue in CF? Why?

Answer 46

Gallstones | Bile can back up and obstruct GB

Question 47

What can we give our CF patients with Gallstones?

Answer 47

``` Ursodeoxycholic acid (UCDA) -Gallstone dissolution agent ```

Question 48

UCDA ADEs

Answer 48

1. Leukopenia 2. Thrombocytopenia 3. Alopecia 4. Increased serum creatinine