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Drug Action I > Block 1 - Cystic Fibrosis + PAH Pharmacology > Flashcards

Block 1 - Cystic Fibrosis + PAH Pharmacology Flashcards

1
Q

List the ideal therapies of CF?

A
  1. Airway clearance
  2. GI enzyme and nutritional supplements
  3. Control inflammation
  4. CFTR correctors and patentors
  5. Lung/organ transplants
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2
Q

Can you replace only one lung?

A

No you need to replace both cause they both contain infection

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3
Q

How can drugs help with airway clearance?

A
  1. ALbuterol
  2. Hypertonic Saline (Hypersal) Neb
  3. Dornase alfa (Pulmozyme)
  4. NAC (Mucosal)
  5. Chest physiotherapy
  6. ICS, LABA, Inhaled antibiotics
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4
Q

What help with GI enzymes and nutrition?

A
  1. PERT
  2. PPI or H2 antagonist
  3. ADEK
  4. Electrolyte replacement
  5. High calorie/fat diet
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5
Q

How would you control inflammation in CF?

A
  1. OCS (benefit needs to outweigh the risk)
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6
Q

How does albuterol help with airway clearance?

A

Proventil allows bronchodilation and increase ciliary efficacy

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7
Q

How does hypertonic saline help with airway clearance?

A

Hyper-sal hydrates the lung surface

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8
Q

How does dornase help with airway clearance?

A

Pulmozyme is a recombinant human deoxyribonuclease that breaks down DNA in the sputum decreasing its viscosity

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9
Q

How does N-acetycysteine help with airway clearance?

A

Mucosal is a mucolytic that thins out and dissolves lung mucus

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10
Q

How does chest physiotherapy help with airway clearance?

A
  1. Postural drainage and percussion
  2. High frequency chest wall oscillation (vest)
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11
Q

What drugs can be used to clear airways for CF?

A

ICS, inhaled LABA, inhaled antibiotics

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12
Q

How does CF affect mucous?

A
  1. Salt transport dehydrates mucous causing it to become sticky and thick
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13
Q

How do you resore airway surface liquids? MOA?

A

Bronchitol (Mannitol) is a dry powder rehydrates airways and lungs promoting productive coughing (modest effect)

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14
Q

What is the difference between CFTR correctors and potentiators?

A

C: Facilitates the folding and presentation of the mature CFTR protein increasing the trafficking of CFTR proteins to the outer membrane
P: Facilitates Cl- transport by increasing CFTR channel effect increasing the probability of Cl- channels opening

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15
Q

What are examples of CFTR Correctors?

A
  1. Lumacaftor
  2. Tezacaftor
  3. Elexacaftor
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16
Q

What are examples of CFTR potentiators?

A
  1. Invacaftor
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17
Q

What is the MOA of ivacaftor?

A

Kalydeco facilitates Cl- trasport by increasing its CFTR effectiveness to increase Cl- channel opening

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18
Q

Who qualifies for Kalydeco? Dose?

A

2 or older, with 1 mutation (G551D)
150 mg PO BID w/ fatty foods

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19
Q

What are the ADRs of Kalydeco?

A

Ivacaftor: Abdominal pain, increased hepatic enzymes, HA, URTI, congestion, nausea, rash rhinitis, dizziness, arthralgia

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20
Q

What is the MOA of Orkambi? Age of patients?

A

Lumacaftor+Ivacaftor stabilizes F508del-CFTR in proving folding of the protein (Luma) -> processing and trafficking of mature protein cell surface

6 YO w/ 2 copies of F508 del mutation

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21
Q

What is the Symdeko MOA? Age of patients?

A

Tezacaftor+Ivacaftor is a corrector

12 YO w/ 2 copies of F508del mutation or at least 1 mutation in CFTR gene responsive to therapy

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22
Q

How does Orkambi’s combo promote its efficacy

A

Lumacaftor allows more CFTR to reach the surface while Ivacaftor keeps the CFTR Cl- channel open

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23
Q

What is the MOA of Ivacaftor?

A

Allow more chloride ions to pass in and out the cells helping keep a balance of salt and water in organs (lungs)

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24
Q

What is the ADR of Luacaftor/Ivacaftor

A

Orkambi brings chest discomfort, dyspnea, respiratory discomfort requiring liver toxicity monitoring

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25
Q

What are the ADRs of Tezacaftor/Ivacafotor?

A

Symdeko brings nausea, HA, sinus congestion, DIOS, cataracts requiring liver toxicity monitoring

26
Q

Trikafta qualifications for treatment?

A

Elexacaftor/tezacaftor/ivacaftor require patients to have at least 1 f508del mutation or at least one other mutation responsive to drug (e and t, correctors; i, potentiator)

27
Q

Describe the outcomes and disadvantages of using CFTR modulators?

A
  1. Don’t cure or restore full function of protein
  2. Effective in relieving symptoms increasing lifespan
28
Q

What are disadvantages of the elexacafotr/texacaftor/ivacaftor combo?

29
Q

What is another treatment alternative to no CFTR protein CF?

A

Gene therapy

30
Q

What is the goal of gene therapy for CF?

A

Replace the defective CF gene in the lungs to cure CF or slow the progression of the disease

31
Q

How is gene therapy administered? Barriers?

A

Inhaling a spray that delivers normal DNA to the lungs

Involve delivery of vectors

32
Q

What are the goals of treating PAH?

A
  1. Alleviate symptoms, improve exercise and quality of life
  2. Improve cardiopulmonary hemodynamics and prevent RVHF
  3. Delay time to clinical worsening
  4. Reduce morbidity and mortality
33
Q

What are the targets of PAH therapies?

A
  1. Prostaglandin pathways
  2. Endothelin pathways
  3. NO pathway
  4. Vasodilators and anticoagulants
34
Q

What are therapeutic classes for PAH?

A
  1. Prostaglandin analogs
  2. Endothelian receptor antagonist
  3. PDEi
  4. Soluble guanylate cyclase stimulator (sGC)
  5. Calcium channel antagonists
35
Q

What are prostacyclin receptor agonist MOA?

A

Bind to prostaglandin I2 receptor targeting 3 of the pathologic changes that occur in PAH
1. Vasodilation
2. Inhibits Platelet aggregation
3. Inhibits smooth muscle proliferation
4. Inotropic effects

36
Q

What are the prostacyclin analogs? Dosage forms?

A
  1. Epoprostenol (Flolan, Veletri) - IV
  2. Treprostinil (Remodulin - IV, SC; Tyvaso - INH; Orenitram - Oral)
  3. Iloprost (Ventavis - INH)
  4. Selexipag (Uptravi - Oral)
37
Q

What are some endothelin receptor antagonists? Dosage forms?

A
  1. Bosentan (Tracleer - PO)
  2. Macitentan (Opsumit - PO)
  3. Ambrisentan (Letairis - PO)
38
Q

What are the PDE5is? Dosage forms?

A
  1. Sildenafil (Revatio - PO)
  2. Tadalafil (Adcirca - PO)
39
Q

What are examples of soluble guanylate cyclase stimulators? Dosage forms?

A

Riociguat (Adempas - PO)

40
Q

What are examples of CCBs?

A
  1. Amlodipine (Norvasc)
  2. Diltiazem (Cardizem, Tiazac)
  3. Nifedipine (Adatta, Procardia)
41
Q

What is the difference between the brands of Treprostinil?

A

Dosage forms

42
Q

What is the MOA of Iloprost? How is it eliminated? ADRs?

A

Ventavis
Synthetic drug of prostacyclin
70% in urnine and 10% feces
Cough, flushing, jaw pain, syncope

43
Q

How does Uptravi differ from other prostacyclin receptor agonists? CIs?

A

Selexipag is a prodrug metabolizes by the liver to activate, 13 fold affinity higher at the IP receptor and half life of 8hr

CYP2C8 inhibitors

44
Q

What is the MOA of epoprostenol?

A

Derivative of PGI2 that causes vasodilation and platelet aggregation inhibiton

45
Q

How is epoprostenol absorbed?

A
  1. Requires central venous circulation to achieve selective pulmonary vasodilation and a portable infusion pump
  2. Due to 6 minute half life
46
Q

How do endothelial cells play a part in the pulmonary vascular structure? Mechanism?

A

Produce vasoconstrictors and catalyzes conversion of angiotensin 1 -> 2

ET-1 and TXA2

47
Q

Differentiate the 2 endothelial vasoconstrictors? What do they do?

A

ET1: potent, prolonging vasoconstriction, increasing vascular tone and PVR
TXA2: Stimulates platelet aggregatio -> thrombosis and increased PVR

47
Q

Differentiate the 2 endothelial vasoconstrictors? What do they do?

A

ET1: potent, prolonging vasoconstriction, increasing vascular tone and PVR
TXA2: Stimulates platelet aggregatio -> thrombosis and increased PVR

48
Q

What is the function of Endothelian 1 antagonists?

A

Target ET1 by blocking receptors on endothelium and vascular smooth muscle

49
Q

What is the MOA Bosentan? How is it administered? ADRs?

A

Tracleer is a ompetitive antagonist of ETA and B, CYP2C9 and 3A4
PO BID
Hepatic toxicity, teratogenicity

50
Q

What is the MOA of Macitentan? How is it different than Tracleer? ADRs?

A

Opsumit is competitive at ETA and B and has less hepatoxicity
QD
Teratogenicity

51
Q

What is the MOA of Ambrisentan? Administration? ADRs?

A

Letairis is competitive specific ETA blocker
PO QD
Hepatic toxicity and teratogenicity

52
Q

What is the Black bow warnings associated with ET1 antagonists

A

Teratogenicity

53
Q

What is the function of PDE5? Where are they found?

A

Regulates intracellular cGMP in the arterial walls

54
Q

What is the MOA of PDE5i in general?

A

Inhibiting PDE5 facilitating the effect of NO increasing cGMP levels and relaxing pulmonary vascular smooth muscle

Increases blood flow to heart

55
Q

What are reported ADRs of PDE5is? CIs?

A

HA, congestion, cardiac events, HTN

Nitrates

56
Q

Describe the mechanism of soluble guanylate cyclase?

A
  1. NO binds to sGC mediating synthesis of cGMP
  2. cGMP-dependent protein kinase (PKG) increases cytosolic calcium ion concentrations -> decreased actin-myosin contractility -> vasodilation
57
Q

How is sGC affected in PAH patients?

A

No synthase -> lower endothelial cell-derived NO -> reduced vasodilation

58
Q

What is the MOA of Adempas? ADRs?

A

Stimulate sGC activity independent of NO increasing cGMP -> vasodialtion

Can cause brith defects and can only be prescribed through the Edemas REMS program

59
Q

What drug classes are sometimes combined for PAH treatments? Why are they considered beneficial? Cons?

A

PDE5 and ERA

Enhance efficacy and compliance

Increase ADRs

Drug Action I (32 decks)

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