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Pharmacology Exam #3 > Cystic Fibrosis > Flashcards

Flashcards in Cystic Fibrosis Deck (48)
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1

Cystic Fibrosis etiology

autosomal recessive disease

2

What is the gene mutation in CF?

Long arm Chromosome 7

3

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

F508

4

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

G551D

5

List bronchodilators we would use as anti-obstructives

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

6

Why do we use hypertonic saline as an anti-obstructives

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

7

what is the hallmark of CF?

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

8

What is the MOA of Dornase alpha (rhDNase)

Selectively cleaves DNA in mucous secretions to reduce mucous viscosity

9

Dornase alpha SEs

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

10

What is a downside to Dornase alpha?

You can produce antibodies to the medication

11

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

S. aureus

12

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

Pseudomonas aeruginosa

13

What is the main benefit of Macrolides (Azithromycin)

Anti-inflammatory properties
-->Supres excessive inflammatory response

14

Macrolides (Azithromycin) MOA

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

15

Aminoglycosides (nebulizer Tobramycin) application

1. Improves lung function
2. Reduces acute pulmonary exacerbations chronically infected with pseudomonas

16

What is unique about Aminoglycosides (nebulizer Tobramycin) dosing?

Alternate with 28 days of treatment to try and reduce resistance

17

Aminoglycosides (nebulizer Tobramycin) SEs

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

18

List the monobactam (beta-lactam)

Inhaled Aztreonam

19

Inhaled Aztreonam MOA

inhibits bacterial cell wall synthesis

20

Inhaled Aztreonam application

Abx with antipseudomonal activity

21

Inhaled Aztreonam SEs

1. Fever
2. Cough
3. Rash

22

What is unique about Inhaled Aztreonam dosing

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

23

Who we do use HIGH dose ibuprofen in?

<18 y.o. with FEV1 >60%

24

Ibuprofen SEs

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

25

Ivacaftor MOA

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

26

What does Ivacaftor improve?

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

27

Ivacaftor SEs

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

28

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

F508 gene mutation
88%

29

What is the combo effect of Lumacaftor/Ivacaftor

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

30

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

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

31

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

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

32

What do we need to monitor with Tezacaftor/Ivacaftor?

ALT and AST

33

When do we discontinue Tezacaftor/Ivacaftor treatment?

LT or AST >5 x upper limit of normal

34

What is a major SE of Tezacaftor/Ivacaftor?

Cataracts

35

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

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

36

What is Palivizumab?

Monoclonal antibody against RSV
Kids younger than 24 mos

37

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

FEV1<30%

38

What do proton pump inhibitors increase your risk of?

Aspiration pneumonia

39

What are the SEs of Omeprazole?

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

40

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

85%

41

What are the main pancreatic enzymes?

1. Lipase
2. Amylase
3. Protease

42

What is the dose calculated by?

According to the Lipase

43

What are the major ADEs of Pancreatic Enzyme Supplementation

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

44

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

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

45

What are fat soluble vitamins?

A, D, E & K

46

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

Gallstones
Bile can back up and obstruct GB

47

What can we give our CF patients with Gallstones?

Ursodeoxycholic acid (UCDA)
-Gallstone dissolution agent

48

UCDA ADEs

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