# 一名囊性纖維化青少年開始使用ivacaftor/tezacaftor/elexacaftor。哪一項服藥指導對正確吸收最重要？

> source: MyMerci (mymerci.kr)  
> url: https://mymerci.kr/pages/nclex_q.php?qn_id=391794&lang=zh-tw  
> language: zh-TW  
> subject: Medical Emergencies  
> category: PA

## 題目

一名囊性纖維化青少年開始使用ivacaftor/tezacaftor/elexacaftor。哪一項服藥指導對正確吸收最重要？

## 選項

1. 空腹服用藥物
2. 將錠劑磨碎以方便吞嚥
3. 早晚劑量都與含脂食物一起服用以確保吸收；不要吃葡萄柚 **✔ 正確答案**
4. 停用dornase alfa和氣道清除治療

**正確答案: 3**

## 解析

正確 (2)：CFTR調節劑具親脂性，每次給藥都需要含脂食物來確保吸收。葡萄柚/塞維亞橙會抑制CYP3A4並增加藥物濃度，需避免。(1) 空腹會明顯降低吸收。(3) 通常不建議磨碎錠劑。(4) Dornase alfa與氣道清除治療應持續。

## 深入解析

記憶提示 CFTR調節劑（Trikafta、Kalydeco）=「要脂肪、不要葡萄柚」。也需要肝功能監測。

## 臨床情境

情境 16歲囊性纖維化個案（F508del異合子）開始elexacaftor/tezacaftor/ivacaftor（Trikafta）早晨劑量與ivacaftor晚間劑量。正在接受門診衛教。

## 重要概念

- **CFTR調節劑** — 改善囊性纖維化CFTR蛋白功能的藥物；ivacaftor、tezacaftor、elexacaftor、lumacaftor。
- **含脂食物** — 親脂性藥物吸收所需；是CFTR調節劑的標準衛教。
- **CYP3A4葡萄柚交互作用** — 葡萄柚抑制CYP3A4，使CFTR調節劑濃度上升；需避免。

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