Table 1.

Drug and metabolite concentrations in plasma and sputum in CF patients receiving Kalydeco and Orkambi therapy.

Medication Management
Patient 1	150mg/q12h# Ivacaftor, 750 mg ciprofloxacin bdΔ, inhaled tobramycin 112mg/bd 250/25µg bd seretide, mometasone nasal spray 2 sprays/bd, 100 µg salbutamol 2 puffs bd plus prn§, pancreatic extract tdsǂ with meals, 500mg/d Calcium/400IU/d Vitamin D bd, 600mg salt prn
Patient 2	200mg/q12h Lumacaftor and 125mg/q12h Ivacaftor Pancreatic extract, 25µg/d vitamin D, 20mg/d pantoprazole, 300mg/d nizatidine, 500mg/m/w/f azithromycin, hypertonic saline 6%, 5mL bd, 250/25 mg/d salmeterol, 2x/d Vitamin ABDECK, 2 salt tablets, 2mg/35mcg /d ethinyloestradiol/cyproterone acetate, 100mcg/prn salbutamol, inhaled tobramycin
Plasma concentrations [16] **
		IVA [µg/mL]	IVA M1 [µg/mL]	IVA M6 [µg/mL]	LUMA [µg/mL]
KALYDECO Patient 1	Plasma css	0.73±0.02	0.41±0.08	0.11±0.03	--
	Plasma 2.5h	0.97±0.31	0.50±0.09	0.16±0.04	--
ORKAMBI Patient 2	Plasma 2.5 h	0.06±0.01	0.07±0.01	0.15±0.03	4.42±0.67

^#Every 12 hours; ^Δ twice daily; ^§ as needed; ǂ 3 times per day; *below the limit of quantification (LOQ)

**Plasma concentrations were first reported by Schneider et al (2016) [16].