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Pharm I ( Melicia) > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

Transmission of Cystic Fibrosis?

A

autosomal recessive

most common life limiting genetic disorder in white population

life span ~41

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2
Q

Genetic abnormality in CF?

A

long arm of chromosome 7

88% delta F508

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3
Q

Mutations of CFTR (cystic fibrosis transmembrane conductance)

A

Class I: defective protein production

Class II: Defective protein processing (protein not properly folded)

Class III: defective regulation (gets to channel but doesn’t work well)

Class IV: defective conductance

Class V: reduced number of active CFTR

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4
Q

Pathophys of CF?

A

2 defective CFTR genes >

defective CFTR protein >

abn Cl permeability altered ionic transport >

decreased water content in airway surface liquid >

mucus obstruction >

inflammation and scarring >

progressive loss of lung func.

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5
Q

Classic features of CF?

A

chronic sinusitis, severe chronic bi infx of airways, pancreatic exocrine insufficiency, meconium illeus at birth , sweat chloride value usually 90-110, obstructive azoospermia

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6
Q

Approach to care for CF?

A

good nutrition

pancreatic enzymes and vitamin supplementation

airway clearance and anti-inflammatory therapies

antipseudomonal agents

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7
Q

Pulmonary tx for CF?

A

chest physiotherapy, abx, pancreatic enzyme sup., multivitamins, antiobstructives, anti-inflammatory, CFTR modulators, vaccinations, supplemental O2, BiPAP, lung transplant

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8
Q

What is the recommended sequence of clearance therapy for CF (pulmonary toilet) regimen?

A

~concurrently with percussion therapy

  1. Bronchodilator (Albuterol)
  2. Hypertonic Saline
  3. Dornase alfa (pulmozyme)
  4. Aerosolized abx ( Aztreonam) indicated based on severity of lung disease and sputum cultures
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9
Q

Dornase alfa MOA?

A

enzyme cleave EC DNA, results in decreased viscosity of mucus

  • airway in the lungs is improved
  • risk of bacterial infection may be decreased

$$$

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10
Q

Effect of hypertonic saline?

A

administered to hydrate mucus –> draws water from the airway to re-establish the aqueous surface layer that is deficient in CF

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11
Q

What is the hallmark of CF?

A

presence of abundant, purulent airway secretions composed primarily of highly polymerized DNA

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12
Q

ADE of dornase alpha?

A

fever, rash, pharyngitis, rhinitis, dyspepsia, conjunctivitis, laryngitis

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13
Q

Should CF pts be treated with abx chronically to control infection?

A

NO

but there is an exceptions if using for anti-inflammatory properties:
-Azithromycin, Nebulized tobramycin, nebulized aztreonam

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14
Q

Most common bacteria in respiratory secretions of CF pts?

A

s. aureus

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15
Q

How does Azithromycin target pseudomonas bacteria?

A

reduces the ability of pseudomonas to produce biofilms

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16
Q

Effect of nebulized tobramycin?

A

improves lung func.

reduces acute pulmonary exacerbations chronically infected w/ P. aeruginosa

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17
Q

Nebulized tobramycin MOA?

A

interferes w/ bacteria protein synthesis by binding to 30S and 50S ribosomal subunits, resulting in a defective bacterial cell membrane

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18
Q

How is nebulized tobramycin dosed?

A

BID for 28 days, then alternate with 28 days off tx

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19
Q

ADEs of inhaled tobramycin

A

sputum discoloration

rales, wheezing, voice alteration

cough, abnormal taste,

eosinophilia

tinnitus

20
Q

Inhaled Aztreonam drug class? MOA?

A

Monobactam (beta lactam)- abx with antipseudomonal activity

inhibits bacterial cell wall synthesis

21
Q

ADEs of inhaled aztreonam?

A

fever, cough, rash, abd pain, vomiting

22
Q

Dosage of inhaled aztreonam?

A

Alternate 28 days on, 28 days off

often Tobramycin and Aztreonam are alternated to provide continuous coverage

23
Q

Ibuprofen MOA

A

reversibly inhibits cyclooxygenase 1 and 2 (COX 1 and 2 enzymes)

24
Q

Who is high dose ibuprofen recommended for?

A

pts under 18 y/o with FEV1 > 60%

Effects:
-less decline in pulmonary func.

  • able to maintain weight and less hospital admission
  • freq. blood draws for PK monitoring
25
Q

Which pts is Ivacattor effective in?

A

those with G551D gene mutation, ~5% of CF pts

and additional CFTR gene mutations

26
Q

ADEs of Ivacaftor?

A

HA, abd pain, nasopharyngitis, hyperglycemia, transaminases increased, arthralgia

27
Q

ADEs of Ibuprofen

A

edema, HA, fluid retention, epigastric pain, GI bleed, tinnitus

28
Q

CFTR Modulator - Ivacattor MOA?

A

cystic fibrosis transmembrane conductance regulator potentiator

  • potentiates epithelial cell chloride ion transport of defective cell surface protein -> allows us to make that protein
  • improves regulation of salt and water absorption and secretion in various tissues
29
Q

what has a major interactive with Ivacaftor?

A

substrate of CYP3A4

30
Q

What should you monitor in a pt taking Ivacaftor?

A

monitor blood glucose and LFTs

31
Q

What is the drug class of Lumacaftor and Ivacaftor combination (Orkambi)?

A

cystic fibrosis transmembrane conductance regulator potentiator

32
Q

Lumacaftor/Ivacaftor combination MOA?

A

Lumacaftor: partially corrects the CFTR misfolding

Ivacaftor: improves the Cl gating abnormality

33
Q

Tezacaftor/Ivacaftor (symdeko) - Dose should be reduced in who?

A

in pts with mod/severe hepatic impairment. When co-administration with drugs that are moderate or strong CYP3A inhibitors

34
Q

ADEs of Lumacaftor/Ivacaftor?

A

nasopharyngitis, fatigue, menstrual disease, increased creatine phosphokinase, URI, rhinorrhea

35
Q

Tezacaftor/Ivacaftor (symdeko) MOA?

A

Tezacaftor: move the defective CFTR protein to the proper place in the airway surface cell (similar to LUmacaftor)

Ivacaftor: improves the Cl gating abnormality

36
Q

What is the most common gene mutation in cystic fibrosis?

A

F508del

37
Q

Who is Tezacaftor/Ivacaftor approved for?

A

> 12 y/o with two copies of F508del or those with single copy of certain specified mutations

38
Q

Tezacaftor/Ivacaftor (symdeko) should be taken with…

A

fat containing food

39
Q

ADEs of Tezacaftor/Ivacaftor?

A

HA, nausea, sinus congestion, dizziness

40
Q

What should be monitored in pts taking Tezacaftor/Ivacaftor?

A

ALT/AST every 3 months during first yr, then annually

cataracts

41
Q

What vaccinations should CF pts receive?

A

influenza vaccine, pneumococcal vaccine

Palivizumab in children <24mos

42
Q

When should pt be referred for lung transplant?

A
  • FEV1 < 30% or rapid decline in FEV1 particularly in young female pts
  • increasing freq. of exacerbations requiring abx tx
  • refractory and or recurrent pneumothorax
  • recurrent hemoptysis not controlleld by embolization
43
Q

ADEs of UCDA?

A

Alopecia

leukopenia, thrombocytopenia

serum creatinine increased

44
Q

ADEs of Omeprazole?

A

Acid regurgitation, constipation, back pain, weakness, cough

45
Q

ADEs of pancreatic enzyme supplementation?

A

prolonged contact with oral mucosa may cause ulcers

administer with food, rinse mouth after administration

excessive dose: fibrosing colonopathy, characterized by inflammation and strictures

46
Q

What vitamins should be supplemented in CF pts?

A

fat soluble vitamins

A, D, E, K

(pancreatic insufficiency and CF related liver disease lead to fat malabsorption)

47
Q

Tx for CF related liver disease?

A

Urosodeoxycholic acid (UCDA)

gallstone dissolution agent

-MOA: reduction of the secretion of cholesterol from the liver and the fractional reabsorption of cholesterol by the intestines

Pharm I ( Melicia) (18 decks)

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