script.reportAU
Rare disease

Lumacaftor and ivacaftor

Brand: Orkambi

PBAC's latest decision on Lumacaftor and ivacaftor: Noted (2021). Considered for Treatment of cystic fibrosis patients homozygous for the F508 deletion mutation in the CFTR gene, across multiple age groups (2 years and older).

PBAC outcome
Noted
Restricted
ICER (AUD/QALY)
Redacted
commercial-in-confidence
Submissions
3
first 2017
Submissions
3
2017 → 2021

Eligible population

Patients with cystic fibrosis aged 2 years and older who are homozygous for the F508del mutation in the CFTR gene, and those aged 12 years and older who are homozygous or carry one copy of the F508del mutation with a residual function mutation.

Therapy area
Rare disease
Line of therapy
Not applicable
Evidence base
Registry
Primary endpoint
PFS
Key trials
PROGRESS, VX15-809-110, Study 129 (VX18-809-129), PASS 108, Flume 2021, Konstan 2017
Comparator
best supportive care
Economic model
CUA
ICER note
ICER redacted in public summary; range provided via footnote corresponding to redacted values in MAP table ($115,000 to <$135,000/QALY). Updated from prior submission ($155,000 to <$255,000/QALY).
ICER (historical)
$115k/QALY–$135k/QALY across 3 submissions (2017–2021) — the latest submission carried no numeric base case. No single PSD states this combined range; see source PSDs.
Risk sharing
Risk-sharing arrangement in place — Managed Access Program (MAP) with subsidisation caps for Years 3, 4, and 5, contingent on sustained relative rate of decline in ppFEV₁. Two options apply depending on whether ppFEV₁ relative rate of decline meets or exceeds 42% threshold.

Submission history

Similar precedents

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