Ivacaftor is prescribed for a child diagnosed with cystic fibrosis. Before initiating therapy with this medication, which baseline laboratory assessment is most essential for the nurse to verify?

Appeared in: NORCET-11 Prelims

Explanation

Ivacaftor can cause elevations in liver enzymes and hepatotoxicity.
Baseline liver function tests are required to detect pre-existing hepatic impairment.
Ongoing monitoring of LFTs is recommended during therapy for early detection of adverse effects.

Option A: Serum lipase and amylase assess pancreatic function, not directly affected by Ivacaftor.
Option C: CBC is not specifically required before Ivacaftor as hematologic toxicity is not a known risk.
Option D: Serum creatinine and BUN assess renal function; Ivacaftor is not nephrotoxic.

Clinical Relevance

Nursing practice connection: Safe nursing care depends on performing Baseline laboratory monitoring before starting Ivacaftor in cystic fibrosis in the correct sequence, documenting the action clearly, and monitoring for the expected response.
Nurses must verify baseline LFTs before starting Ivacaftor to prevent serious hepatic complications.
Early detection of liver dysfunction allows for prompt intervention, dose adjustment, or discontinuation if needed.
What if? If a child has elevated baseline LFTs, Ivacaftor may be delayed or not started, and further hepatic evaluation is warranted.

How to Approach the Question

Identify the drug and its major side effects or monitoring requirements.
Focus on the most serious or common adverse effect associated with the medication.
Eliminate options that do not relate to the drug's known toxicity profile.
Choose the laboratory test that directly addresses the main safety concern for the drug.

Concept Tested & Keywords

Concept Tested: Baseline laboratory monitoring before starting Ivacaftor in cystic fibrosis
Stem keywords: Ivacaftor, cystic fibrosis, child, baseline laboratory assessment
Lead-in keywords: most essential, verify
Clinical cues: Ivacaftor can cause liver enzyme elevation

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E6 Pharmacology Nursing Lilley 11e Part 3 p. 31-33

Practise the full NORCET-11 Prelims

Attempt every question from this paper in a timed mock, then review the full solution for each one.