NAGS Deficiency Non-Interventional, Qualitative Study v2.0

  • Research type

    Research Study

  • Full title

    International Study to Explore N-acetylglutamate Synthase Deficiency Patients’ Compliance with their Treatment and the Impact of the Disease and Treatment Non-Compliance on Patients’ and Caregivers’ Quality of Life

  • IRAS ID

    228661

  • Contact name

    Charlotte Dawson

  • Contact email

    Charlotte.Dawson@UHB.nhs.uk

  • Sponsor organisation

    Orphan Europe

  • Clinicaltrials.gov Identifier

    35465, CPMS ID

  • Duration of Study in the UK

    1 years, 0 months, 2 days

  • Research summary

    Carbaglu®, manufactured by Orphan Europe, is currently the only available product indicated for the treatment of acute hyperammonemia due to N-acetylglutamate Synthase (NAGS) deficiency, and as maintenance therapy for the treatment of chronic hyperammonemia due to NAGS deficiency. Thus far, no study has been conducted with NAGS deficiency patients and caregivers to understand perspectives about treatment and impacts of treatment non-adherence. The objective of this study is to conduct interviews to gain a better understanding of the symptoms and impacts of the disease and to explore reasons for treatment non-adherence and its impact on the health-related quality of life of patients and caregivers. This study will involve one-to-one, 60-minute, phone interviews with patients 16 years or older with a confirmed NAGS deficiency diagnosis who are being treated with Carbaglu® and/or their caregivers. No investigational drugs or devices will be administered or evaluated. The study population will include approximately 11-13 patients and 11-13 caregivers from 6 European countries, including the United Kingdom. Participant recruitment will only begin after approval of the protocol and supporting study documents is obtained from the local site research ethics committee. Prior to participation, all potential participants must be able to read, write, and speak English and be willing and able to comprehend and sign a written informed consent form. Clinicians will recruit and obtain consent from potential participants. Demographic and health information will be analyzed using descriptive statistics, and qualitative data will be analyzed using ATLAS.ti. Findings will be summarized in a final report and may be published or presented by the investigator(s) after review by, and in consultation and agreement with, the sponsor. Results will be presented in such a way that confidential or proprietary information is not disclosed. The estimated overall duration of the study is one year from the time of recruitment initiation.

  • REC name

    East Midlands - Nottingham 1 Research Ethics Committee

  • REC reference

    17/EM/0354

  • Date of REC Opinion

    29 Nov 2017

  • REC opinion

    Further Information Favourable Opinion