Gene therapy administration under managed access programme

GMO Register No: G0726-01

SNIF Notification No: B/IE/20/01

On 25th February 2020, the EPA received a notification from The Clinical Research Facility at St James’s Hospital, James’s Street, Dublin 8, and Children’s Health Ireland at Temple Street Hospital, Temple Street, Dublin 1, seeking consent under Article 14 of the GMO (Deliberate Release) Regulations, S.I. No 500 of 2003, to carry out the deliberate release of a Genetically Modified Organism (GMO).

Objective of the proposed deliberate release

The objective of this proposed deliberate release is to administer gene therapy product AVXS-101 or onasemnogene abeparvovec-xioi (marketed by Novartis in the US as Zolgensma), to paediatric patients with spinal muscular atrophy (SMA), under a managed access programme. AVXS-101 is a recombinant adeno-associated virus-based vector which has been designed to express the survival motor neuron-1 (SMN-1) in paediatric patients less than 2 years of age with SMA.

Location and Duration

It is proposed that this treatment of patients with AVXS-101 will take place at St James’s Hospital (in the Wellcome-HRB Clinical Research Facility) between March - December 2020.  Patients will be followed up at Children’s Health Ireland at Temple Street Hospital, Temple Street, Dublin 1 as part of the AveXis global Managed Access Programme. 

Legislation

Any person or organisation proposing to carry out a deliberate release of a GMO for purposes other than placing on the market, must comply with the legislative requirements set out under Part II of the GMO (Deliberate Release) Regulations S.I. No 500 of 2003.

Role of the EPA

In accordance with the Regulations, it is the role of the Agency to evaluate the risks posed by the proposed deliberate release for human health and the environment.

The Agency must respond to the applicant in writing within 90 days of receipt of the notification.  This does not include any period during which the Agency is awaiting receipt of any further information on the notification, which it may have requested from the applicant.
In its response, the Agency may indicate that consent to the proposed deliberate release is:

  • granted with or without conditions; or, 
  • refused, and the reasons for the refusal.

In reaching its decision, the Agency will take into consideration the views expressed by:

  • the GMO Advisory Committee, a statutory body set up to advise the Agency on any aspect of its functions in relation to GMOs; and,
  • members of the public in representations forwarded to the Agency in accordance with Article 16 of the Regulations (see also “How to make representations to the EPA about a proposed GM release”);
  • competent authority of another Member State of the European Communities.

Here below we provide access to the notification documentation:

  • Summary Notification Information Format (SNIF); 
  • Environmental Risk Assessment.

Newspaper notice

The applicant published a notice of the proposed deliberate release, in the Irish Times (27th February 2020) inviting members of the public to make representations to the EPA within 28 days of publication of the notice in a newspaper (i.e. up to 5pm on 25th March 2020). 

GMO Register Entry

The proposed clinical trial has been assigned GMO Register No G0726-01.  The Register provides significant details about the trial.  The register entry will be updated as further details become available.


Relevant Documentation

Summary Notification Information Format (SNIF)

How to make representations to the EPA about a proposed GM release

Newspaper Advert  

GMO Register Entry G0726-01

Inspectors Report

Consent Conditions 

Board Minute Extract