Subject: Zokinvy 4384-A SGM P2023
Policy:
I. INDICATIONS
The indications below including FDA-approved indications and
compendial uses are considered a covered benefit provided that
all the approval criteria are met and the member has no
exclusions to the prescribed therapy.
A. FDA-Approved Indications
Zokinvy is a farnesyltransferase inhibitor indicated in patients
12 months of age and older with a body surface area of 0.39
m2 and above:
1. To reduce risk of mortality in Hutchinson-Gilford Progeria
Syndrome
2. For treatment of processing deficient Progeroid
Laminopathies with heterozygous LMNA mutation with
progerin-like protein accumulation
3. For treatment of processing deficient Progeroid
Laminopathies with homozygous or compound
heterozygous ZMPSTE24 mutations
Limitations of Use
Not indicated for other Progeroid Syndromes or processing-
proficient Progeroid Laminopathies. Based upon its
mechanism of action, Zokinvy would not be expected to be
effective in these populations.
All other indications are considered experimental/investigational
and not medically necessary.
II. DOCUMENTATION
A. Hutchinson-Gilford Progeria Syndrome
Submission of the following information is necessary to
initiate the prior authorization review for Hutchinson-Gilford
Progeria Syndrome: Genetic testing results confirming the
member has a LMNA mutation.
B. Processing Deficient Progeroid Laminopathy with
Progerin-Like Protein Accumulation
Submission of the following information is necessary to
initiate the prior authorization review for Processing Deficient
Progeroid Laminopathy with Progerin-Like Protein
Accumulation: Genetic testing results confirming the member
has a heterozygous LMNA mutation.
C. Processing Deficient Progeroid Laminopathy without
Progerin-Like Protein Accumulation
Submission of the following information is necessary to
initiate the prior authorization review for Processing Deficient
Progeroid Laminopathy without Progerin-Like Protein
Accumulation: Genetic testing results confirming the member
has either homozygous or compound heterozygous
ZMPSTE24 mutations.
III. CRITERIA FOR INITIAL APPROVAL
A. Hutchinson-Gilford Progeria Syndrome
Authorization of 12 months may be granted for treatment of
Hutchinson-Gilford Progeria Syndrome when all of the
following criteria are met:
1. The member is 12 months of age or older.
2. The member has a body surface area of 0.39 m2 or
above.
3. The diagnosis of Hutchinson-Gilford Progeria Syndrome
has been confirmed with genetic testing indicating the
patient has a LMNA
B. Processing Deficient Progeroid Laminopathy with
Progerin-Like Protein Accumulation
Authorization of 12 months may be granted for treatment of
Processing Deficient Progeroid Laminopathy with Progerin-
Like Protein Accumulation when all of the following criteria
are met:
1. The member is 12 months of age or older.
2. The member has a body surface area of 0.39 m2 or
above.
3. The diagnosis of Processing Deficient Progeroid
Laminopathy has been confirmed with genetic testing
indicating the patient has a heterozygous LMNA
C. Processing Deficient Progeroid Laminopathy without
Progerin-Like Protein Accumulation
Authorization of 12 months may be granted for treatment of
Processing Deficient Progeroid Laminopathy without
Progerin-Like Protein Accumulation when all of the following
criteria are met:
1. The member is 12 months of age or older.
2. The member has a body surface area of 0.39 m2 or
above.
3. The diagnosis of Processing Deficient Progeroid
Laminopathy has been confirmed with genetic testing
indicating the patient has homozygous or compound
heterozygous ZMPSTE24
IV. CONTINUATION OF THERAPY
Authorization of 12 months may be granted for continued
treatment of an indication listed in Section III when all of the
following criteria are met:
A. Member meets all initial authorization criteria.
B. Member is experiencing benefit from therapy.
Place of Service:
Outpatient
The above policy is based on the following references:
- Zokinvy [package insert]. Palo Alto, CA: Eiger BioPharmaceuticals, Inc.; November 2020.
- Progeria Research Foundation (PRF). The Progeria Handbook: A Guide for Families & Health Care Providers of Children with Progeria. Second Edition. PRF. https://www.progeriaresearch.org/wp-content/uploads/2019/03/PRF_Handbook_2019_eFile.pdf. Accessed November 27, 2020.
- Gordon LB, Brown WT, Collins FS. Hutchinson-Gilford Progeria Syndrome. University of Washington, Seattle; 2019.
- AHFS DI (Adult and Pediatric) [database online]. Hudson, OH: Lexi-Comp, Inc.; http://online.lexi.com/lco/action/index/dataset/complete_ashp [available with subscription]. Accessed March 15, 2021.
- Micromedex Solutions [database online]. Ann Arbor, MI: Truven Health Analytics Inc. Updated periodically. www.micromedexsolutions.com [available with subscription]. Accessed March 15, 2021.
Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.
August 18, 2023
