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Pharm 3 Shea > Cystic Fibrosis 2- MJ > Flashcards

Cystic Fibrosis 2- MJ Flashcards

1
Q

What are the 10 ADEs of inhaled Aztreonam? (3 were in red)

A

1. *Fever (MC in children)

2. *Cough

3. *Rash

  1. Nasal congestion
  2. Pharyngeal pain
  3. Wheezing
  4. Bronchospasm
  5. Chest discomfort
  6. Abd pain
  7. Vomiting
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2
Q

Do not repeat aztreonam ______ days after completion

A

28 days

(to provide continuous coverage: could do 28d Aztreonam then 28d Tobramycin then 28d Aztreonam….)

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3
Q

The following is the mechanism of which drug?

–Reversibly inhibits cyclooxygenase-1 and 2 (COX-1 and 2) enzymes

–decreased formation of prostaglandin precursors

A

Ibuprofen

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4
Q

Which drug?

–antipyretic

–analgesic

–anti-inflammatory properties

A

Ibuprofen

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5
Q

What is the minimum age that you can give ibuprofen?

A

must be > 6 months

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6
Q

Which patients is high-dose ibuprofen indicated for?

A

Patients under 18y/o w/ FEV >60%

(insufficient evidence for ppl under 18y/o)

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7
Q

Which med?

  • Less decline in pulmonary function
  • Able to maintain weight and had less hospital admissions
  • Benefits exceed the risks of GI complications and nephrotoxicity
  • < 5% of CF patients on this regimen
A

High-dose Ibprofen

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8
Q

What are requirements to obtain a specific therapeutic level of ibuprofen?

A

Frequent blood draws for pharmacokinetic monitoring

  • Target peak plasma concentration between 50-100 μg/ml
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9
Q

What are the 6 main ADEs of Ibuprofen?

A

1. *Edema

2. *Headache

3. *Fluid retention

4. *Epigastric pain

5. *GI bleed

6. *Tinnitus

(also dyspepsia and flatulance can occur)

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10
Q

Interactions of which CFTR modulator?

–Substrate of CYP3A4 (major);

–Inhibits CYP2C8 (weak), CYP2C9 (weak), CYP3A4 (weak), P-glycoprotein

A

Ivacaftor

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11
Q

The following are the main ADEs of which med?

  • *Headache
  • *Abdominal pain
  • *Nasopharyngitis
  • *Hyperglycemia
  • _*Transaminases increased_
  • *Arthralgia

“HAN HAT”

A

Ivacaftor

(a CFTR modulator)

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12
Q

MOA of which med?

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

–improves regulation of salt and water absorption and secretion in various tissues (eg, lung, gastrointestinal tract).

A

Ivacaftor

(a CFTR modulator)

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13
Q

What is the con of Ivacaftor?

A

Very expensive (~300,000/year)

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14
Q

MOA of the combination of which 2 drugs?

  1. ________=
    • F508del mutation interferes with CFTR protein folding and channel gating activity
    • partially corrects the CFTR misfolding
  2. ________=
    • Improves the gating abnormality
A

1. Lumacaftor

2. Ivacaftor

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15
Q

People w/ 2 copies of the ______ mutation represent the largest group of people w/ CF

A

F508del

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16
Q

Data from 2 phase III studies on people w/ 2 copies of the F508del mutation showed statistically significant improvements in lung function when pts were treated with what?

A

Lumacaftor/Ivacaftor

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17
Q

Data from 2 phase III studies on people w/ 2 copies of the F508del mutation showed that patients who took Lumacaftor/ivacaftor also experienced reductions in _____ and improvements in _______*

(in addition to i_mprovements in lung function)_

A

_*reductions in pulmonary exacerbations*_

*Improvements in _BMI*_

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18
Q

>10% of people taking Lumacaftor/Ivacaftor experience what main ADE? (the only red one)

A

Nasopharyngitis

(Also nausea, diarrhea, chest discomfort and dyspnea)

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19
Q

What are the 6 main ADEs of Lumacaftor/Ivacaftor? (the ones in red)

A

1. Nasopharyngitis

2. Fatigue

3. Menstrual disease (amenorrhea, dysmennorhea, etc)

4. Increased creatinine phosphokinase

5. URI

6. Rhinorrhea

“FIN RUM”

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20
Q

Tezacaftor/Ivacaftor is combination therapy used to tx CF…. What is Tezacaftor designed to do?

A

Tezacaftor is designed to move the defective CFTR protein to the proper place in the airway cell surface (similar to Lumacaftor)

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21
Q

Tezacaftor/Ivacaftor is combination therapy used to tx CF…. What is Ivacaftor designed to do?

A

Ivacaftor helps facilitate the opening of the chloride channel on the cell surface to allow chloride and sodium (salt) to move in and out of the cell

22
Q

Who is Tezacaftor/Ivacaftor combination therapy approved for? (2 different types of individuals)

A
  1. individuals >12y/o with 2 copies of 508del (MC CF mutation)
  2. individuals who have a single copy of one of 26 specified mutations – regardless of their other mutation
23
Q

Tezacaftor/Ivacaftor (Symdeko) should be taken with what type of food?

A

fat-containing food

24
Q

The dose of Tezacaftor/Ivacaftor should be reduced in patients with moderate and severe _______

A

hepatic impairment

25
Q

The dose of Tezacaftor/Ivacaftor should be reduced when it is co-administered with drugs that are moderate or strong _______ inhibitors

A

CYP3A

26
Q

Concomitant use of Tezacaftor/Ivacaftor with strong CYP3A inducers (e.g., rifampin, St. John’s wort) may substantially decrease the therapeutic effectiveness of __________thus co-administration is not recommended

A

Tezacaftor

27
Q

Avoid food containing grapefruit or Seville oranges when giving what med?

A

Tezacaftor/Ivacaftor

28
Q

What are the 4 common adverse drug rxns of Tezacaftor/Ivacaftor?

A
  1. HA
  2. Nausea
  3. Sinus Congestion
  4. Dizziness

“Some Headaches Never Die”

29
Q

How should you monitor a patient on Tezacaftor/Ivacaftor?

A

ALT and AST prior to initiating treatment and every 3 months during the first year of treatment, annually thereafter

30
Q

Monitoring parameters for Tezacaftor/Ivacaftor:

When should you discontinue treatment?

A

If significant elevations of transaminases

(ALT or AST >5 x upper limit of normal (ULN), or ALT or AST >3 x ULN with bilirubin >2 x ULN)

31
Q

Why is baseline and follow-up examinations recommended in pediatric patients prior to initiating treatment with Tezacaftor/Ivacaftor?

A

Cataracts: Non-congenital lens opacities/cataracts have been reported in pediatric patients

32
Q

Which 2 vaccines should be given to patients with CF?

A

_1. influenza vaccine (anyone older than 6mo)**_

_2. Pneumococcal vaccine**_

  1. Palivizumab
33
Q

The following describes which recommended vaccine for patients w/ CF?

  1. Monoclonal antibody against respiratory syncytial virus
    1. Children younger than 24 months of age
  2. Not a firm recommendation
A

Palivizumab

34
Q

When should a CF patient be given oxygen and BiPAP?

A

Oxygen: progressive CF accompanied by worsening hypoxemia

BiPAP: advanced CF and hypercapnia

35
Q

According to the International Society for Heart and Lung Transplantation, when should a pt be referred to a transplant center? (4 things)

A
  1. FEV <30% predicted or a rapid decline in FEV1, particularly in young female patients
  2. Increasing freq. of exacerbations requiring abx
  3. Refractory/recurrent pneumothorax
  4. Recurrent hemoptysis not controlled by emobilization
36
Q

What 4 GI treatments are used in CF?

A
  1. GERD tx
  2. Pancreatic enzyme supplementation
  3. Vitamin supplementation
  4. Prevention and tx of cirrhosis
37
Q

What disease is 6-8x more common in CF children than those w/o CF? How do you tx this?

A
  • GERD
  • Proton Pump Inhibitors
38
Q

Which group of meds?

–act by irreversibly blocking the hydrogen/potassium adenosine triphosphatase enzyme system (the H+/K+ ATPase, or more commonly, the gastric proton pump) of the gastric parietal cells.

–The proton pump is the terminal stage in gastric acid secretion, being directly responsible for secreting H+ ions into the gastric lumen.

A

Proton Pump Inhibitors

39
Q

The following are apart of which group of meds and what do they treat? (“-prazole”)

  1. Omeprazole
  2. Lansoprazole
  3. Dexlansoprazole
  4. Esomeprazole
  5. Pantoprazole
  6. Rabeprazole
A

Proton Pump Inhibitors

Used to tx GERD

40
Q

What are the 5 main ADEs of Omeprazole? (the ones in red)

A

1. Acid regurgitation

2. Constipation

3. Back pain

4. Weakness**

5. Cough

“AW CBC”

41
Q

What is the “backbone of GI therapy” in patients w/ CF?

A

pancreatic enzyme supplementation

42
Q

Dosage of Pancreatic enzyme supplementation is calculated using ______ units

A

**Lipase units

43
Q

ADEs of pancreatic enzyme supplementation:

Prolonged contact of the enzyme supplements with oral mucosa may cause ______, especially with the powdered form

A

_*ulcers*_

44
Q

Which med?

_–Administer with food (eg, applesauce), even in infants*_

_–Mouth should be rinsed after administration, particularly in infants*_

A

Pancreatic Enzyme Supplementation

45
Q

*Excessive doses of pancreatic enzymes can cause what?*

A

_**Fibrosing colonopathy_

_(characterized by inflammation and strictures)**_

(risk reduced by limiting dose to < 2500 lipase units/kg)

46
Q
  • Pancreatic insufficiency and CF related liver dz lead to ____ _______
  • This predisposes to what?
A
  • Fat malabsorption
  • predisposes to deficiencies (Vitamins A, D, E, K)
47
Q

Decreased calcium absorption and intake in CF can lead to what?

A

Bone loss

48
Q

Dosing of Vitamin A, D, E, and K supplementation is based on what?

A

age

49
Q

The following describes what med, which is used to tx CF- related liver disease:

–Decreases the cholesterol content of bile and bile stones

–Mechanism is reduction of the secretion of cholesterol from the liver and the fractional reabsorption of cholesterol by the intestines

A

Ursodeoxycholic acid:

Gallstone dissolution agent

50
Q

What are the 4 main ADEs of Ursodeoxycholic acid (Gallstone dissolution agent)

(the ones that are blue on the slide)

A

1. Alopecia

2. Leukopenia

3. Thrombocytopenia

4. Serum creatinine increased

“LAST”

Pharm 3 Shea (17 decks)

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