Subject: Tegsedi 2773-A SGM P2023

Drug

TEGSEDI  ((inotersen)

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.

          FDA-Approved Indication
          Tegsedi is indicated for the treatment of the polyneuropathy of
          hereditary transthyretin-mediated amyloidosis in adults.

          All other indications are considered experimental/investigational
          and not medically necessary.


    II.  DOCUMENTATION

          Submission of the following information is necessary to initiate
          the prior authorization review:
          A.  For initial requests:
               1.  Testing or analysis confirming a mutation of the TTR gene
               2.  Medical record documentation confirming the member
                    demonstrates signs and symptoms of polyneuropathy
          B.  For continuation requests: Chart notes or medical record
               documentation confirming the clinical benefit of Tegsedi

  III.  PRESCRIBER SPECIALTIES

          This medication must be prescribed by or in consultation with a
          neurologist, geneticist, or physician specializing in the treatment
          of amyloidosis.


   IV.  CRITERIA FOR INITIAL APPROVAL

          Polyneuropathy of Hereditary Transthyretin-mediated
          Amyloidosis
          Authorization of 12 months may be granted for treatment of
          polyneuropathy of hereditary transthyretin-mediated amyloidosis
          (also called transthyretin-type familial amyloid polyneuropathy
          [ATTR-FAP]) when all of the following criteria are met:
          A.  The diagnosis is confirmed by detection of a mutation of the
               TTR gene.
          B.  Member exhibits clinical manifestations of ATTR-FAP (e.g.,
               amyloid deposition in biopsy specimens, TTR protein variants
               in serum, progressive peripheral sensory-motor
               polyneuropathy).
          C.  The member is not a liver transplant recipient.
          D.  The requested medication will not be used in combination
               with patisiran (Onpattro), tafamidis (Vyndaqel, Vyndamax) or
               vutrisiran (Amvuttra).


     V.  CONTINUATION OF THERAPY

          Authorization of 12 months may be granted for the continued
          treatment of ATTR-FAP when all of the following criteria are met:
          A.  The member must have met all initial authorization criteria.
          B.  The member must have demonstrated a beneficial response
               to treatment with Tegsedi therapy compared to baseline
               (e.g., improvement of neuropathy severity and rate of
               disease progression as demonstrated by the modified
               Neuropathy Impairment Scale+7 (mNIS+7) composite score,
               the Norfolk Quality of Life-Diabetic Neuropathy (QoL-DN)
               total score, polyneuropathy disability (PND) score, FAP
               disease stage, manual grip strength).

Place of Service:

Outpatient

The above policy is based on the following references:

1. Tegsedi [package insert]. Waltham, MA: Sobi, Inc.; June 2022.
2. Benson MD, et. al., Inotersen Treatment for Patients with Hereditary Transthyretin Amyloidosis. N Engl J Med. 2018 Jul 5; 379(1):22-31.
3. Ando Y, Coelho T, Berk JL, Cruz MW, Ericzon BG, Ikeda S, Lewis WD, Obici L, Planté-Bordeneuve V, Rapezzi C, et al. Guideline of transthyretin-related hereditary amyloidosis for clinicians. Orphanet J Rare Dis. 2013; 8:31.
4. Sekijima Y. Hereditary Transthyretin Amyloidosis. 2001 Nov 5 [Updated 2021 June 17]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1194/. Accessed March 15, 2023.

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.

October 22, 2023