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Pharmacology Exam #3 > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

Cystic Fibrosis etiology

A

autosomal recessive disease

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2
Q

What is the gene mutation in CF?

A

Long arm Chromosome 7

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3
Q

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

A

F508

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4
Q

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

A

G551D

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5
Q

List bronchodilators we would use as anti-obstructives

A
  1. B-2 agonists- Albuterol
  2. Theophylline
  3. Anticholinergics- Ipratropium (short acting) and Tiotroprium (long-acting)
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6
Q

Why do we use hypertonic saline as an anti-obstructives

A

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

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7
Q

what is the hallmark of CF?

A

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

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8
Q

What is the MOA of Dornase alpha (rhDNase)

A

Selectively cleaves DNA in mucous secretions to reduce mucous viscosity

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9
Q

Dornase alpha SEs

A
  1. Fever- 32% in pt’s with FVC <40%
  2. Pharyngitis, rhinitis
  3. Laryngitis
  4. Dyspepsia
  5. Conjunctivitis
  6. Rash
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10
Q

What is a downside to Dornase alpha?

A

You can produce antibodies to the medication

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11
Q

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

A

S. aureus

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12
Q

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

A

Pseudomonas aeruginosa

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13
Q

What is the main benefit of Macrolides (Azithromycin)

A

Anti-inflammatory properties

–>Supres excessive inflammatory response

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14
Q

Macrolides (Azithromycin) MOA

A

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

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15
Q

Aminoglycosides (nebulizer Tobramycin) application

A
  1. Improves lung function

2. Reduces acute pulmonary exacerbations chronically infected with pseudomonas

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16
Q

What is unique about Aminoglycosides (nebulizer Tobramycin) dosing?

A

Alternate with 28 days of treatment to try and reduce resistance

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17
Q

Aminoglycosides (nebulizer Tobramycin) SEs

A
  1. GI: sputum discoloration, abnormal taste
  2. Respiratory: Rales, wheezes, cough, voice alteration
  3. Otic: Tinnitus
  4. Hematologic: Eosinophilia
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18
Q

List the monobactam (beta-lactam)

A

Inhaled Aztreonam

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19
Q

Inhaled Aztreonam MOA

A

inhibits bacterial cell wall synthesis

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20
Q

Inhaled Aztreonam application

A

Abx with antipseudomonal activity

21
Q

Inhaled Aztreonam SEs

A
  1. Fever
  2. Cough
  3. Rash
22
Q

What is unique about Inhaled Aztreonam dosing

A

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

23
Q

Who we do use HIGH dose ibuprofen in?

A

<18 y.o. with FEV1 >60%

24
Q

Ibuprofen SEs

A
  1. GI: Epigastric pain, GI bleed
  2. CV: Edema
  3. Endocrine: Fluid retention
  4. Otic: Tinnitus
  5. CNS: HA
25
Q

Ivacaftor MOA

A

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

26
Q

What does Ivacaftor improve?

A

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

27
Q

Ivacaftor SEs

A
  1. Hyperglycemia-Monitor blood sugars
  2. Transaminases increased
  3. HA
  4. Abdominal pain
  5. Nasopharyngitis
  6. Arthralgia
28
Q

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

A

F508 gene mutation

88%

29
Q

What is the combo effect of Lumacaftor/Ivacaftor

A
  1. Reduction in pulmonary exacerbations
  2. Improved lung function
  3. Increased BMI
30
Q

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

A
  1. Menstrual disease
  2. Increased creatine phosphokinase
  3. URI
  4. Rhinorrhea
  5. Nasopharyngitis
  6. Fatigue
31
Q

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

A

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

32
Q

What do we need to monitor with Tezacaftor/Ivacaftor?

A

ALT and AST

33
Q

When do we discontinue Tezacaftor/Ivacaftor treatment?

A

LT or AST >5 x upper limit of normal

34
Q

What is a major SE of Tezacaftor/Ivacaftor?

A

Cataracts

35
Q

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

A
  1. Influenza- older than 6 mos.

2. Pneumococcal

36
Q

What is Palivizumab?

A

Monoclonal antibody against RSV

Kids younger than 24 mos

37
Q

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

A

FEV1<30%

38
Q

What do proton pump inhibitors increase your risk of?

A

Aspiration pneumonia

39
Q

What are the SEs of Omeprazole?

A
  1. Acid regurgitation
  2. Constipation
  3. Back pain
  4. Weakness
  5. Cough
40
Q

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

A

85%

41
Q

What are the main pancreatic enzymes?

A
  1. Lipase
  2. Amylase
  3. Protease
42
Q

What is the dose calculated by?

A

According to the Lipase

43
Q

What are the major ADEs of Pancreatic Enzyme Supplementation

A
  1. Oral Mucosa Ulcers with prolonged contact-Administer with food and rinse after
  2. Fibrosing Colnopathy
44
Q

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

A

Fat malabsorption due to:

  1. Pancreatic insufficiency
  2. Liver dz’s
45
Q

What are fat soluble vitamins?

A

A, D, E & K

46
Q

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

A

Gallstones

Bile can back up and obstruct GB

47
Q

What can we give our CF patients with Gallstones?

A 
Ursodeoxycholic acid (UCDA) 
-Gallstone dissolution agent
48
Q

UCDA ADEs

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

Pharmacology Exam #3 (6 decks)

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