Tezacaftor / Ivacaftor (5) – Symkevi®

Cystic fibrosis (CF), combination therapy with ivacaftor in patients 6 to < 12 years (heterozygous for F508del and RF mutation)

Characteristics

Start date 01.12.2020 – Marketing authorisation: 25.11.2020
Resolution 20.05.2021
INN Tezacaftor/Ivacaftor
Brand name Symkevi®
Pharm. company Vertex Pharmaceuticals (Ireland) Limited
G-BA Procedure ID D-609
ATC code R07AX31 Other respiratory system products (R07AX)
DDD 1 U O
Therapeutic area Metabolic diseases Orphan (turnover limit)
Reason for procedure New therapeutic indication
Specialty Bundling

Studies and Results

  • Clinical trials
    • In addition, the pharmaceutical manufacturer provided the results of the 8-week RCT VX16-661-115 (TEZ/IVA + IVA vs. IVA; hereinafter referred to as Study 115) and the single-arm extension study VX17-661-116 (hereinafter referred to as Study 116).

Children with cystic fibrosis aged 6 to < 12 years who are heterozygous for the F508del mutation and carry one of the following mutations in the CFTR gene: P67L, R117C, L206W, R352Q, A455E, D579G, 711+3A→G, S945L, S977F, R1070W, D1152H, 2789+5G→A, 3272–26A→G and 3849+10kbC→T

  • For children with cystic fibrosis aged 6 to < 12 years who are heterozygous for the F508del mutation and carry one of the following mutations in the CFTR gene: P67L, R117C, L206W, R352Q, A455E, D579G, 711+3A→G, S945L, S977F, R1070W, D1152H, 2789+5G→A, 3272–26A→G, and 3849+10kbC→T, the additional benefit of tezacaftor/ivacaftor in combination with ivacaftor compared with the appropriate comparator therapy is not proven.
  • In its dossier for the assessment of the additional benefit of TEZ/IVA + IVA, the pharmaceutical manufacturer does not present any direct comparative studies against the appropriate comparator therapy, Best Supportive Care (BSC).
  • Furthermore, no indirect comparisons were submitted to address the issues raised in the benefit assessment.
  • The single-arm study 113 is not relevant to the present benefit assessment, as no data are available for an assessment of TEZ/IVA + IVA compared with the appropriate comparator therapy.
  • Consequently, the pharmaceutical manufacturer did not submit any study for this patient population that would have been suitable for assessing the additional benefit of TEZ/IVA + IVA compared with the appropriate comparator therapy.
  • Overall assessment
    • For patients aged 6 years or older but under 12 years with cystic fibrosis who are heterozygous for the F508del mutation and carry one of the following mutations in the CFTR gene: P67L, R117C, L206W, R352Q, A455E, D579G, 711+3A→G, S945L, S977F, R1070W, D1152H, 2789+5G→A, 3272–26A→G, and 3849+10kbC→T, additional benefit is not proven.

Courtesy translation only, please refer to the German original.

Associated procedures

Tezacaftor / Ivacaftor (5) Symkevi® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination therapy with ivacaftor in patients 6 to < 12 years (heterozygous for F508del and RF mutation) 50 100% additional benefit not proven Orphan (turnover limit)
Tezacaftor / Ivacaftor (4) Symkevi® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination therapy with ivacaftor in patients 6 to < 12 years (homozygous for F508del) 470 100% additional benefit not proven Orphan (turnover limit)
Tezacaftor / Ivacaftor (2) Symkevi® Vertex Pharmaceuticals Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor in patients ≥ 12 years (homozygous for F508del) 2,400 100% additional benefit not proven Orphan (turnover limit)
Tezacaftor / Ivacaftor (3) Symkevi® Vertex Pharmaceuticals Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor in patients ≥ 12 years (heterozygous for F508del) 200–300 100% additional benefit not proven Orphan (turnover limit)
Tezacaftor / Ivacaftor (1) Symkevi® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), F508del mutation, ≥ 12 years 0
2,600–2,700
91% considerable additional benefit Orphan repealed


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