CF- Ivacaftor DI Flashcards

1
Q

Ivacaftor brand name

A

Kalydeco

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2
Q

Ivacaftor MoA

A

Facilitates opening of the chloride channel

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3
Q

Is ivacaftor a CFTR potentiator or corrector?

A

Potentiator

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4
Q

What class CF mutations does ivacaftor work on?

A

Class III and Class IV

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5
Q

Ivacaftor indication

A

Patients ≥4 months with ≥1 of 97 ivacaftor-responsive CFTR mutations

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6
Q

CF Class III mutation

A

CFTR protein is created and gets to the cell surface, but the channel doesn’t open

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7
Q

CF Class IV mutation

A

Opening in the CFTR protein ion is faulty

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8
Q

Ivacaftor, along with all other CFTR modulators, must be taken with what?

A

High-fat meal

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9
Q

When to dose adjust for ivacaftor (and all CFTR modulators)

A

Moderate-severe hepatic dysfunction

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10
Q

Benefits of CFTR modulators in CF

A

Improve QoL, decrease APEs, decrease morbidity and mortality

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