REVEALS Registry of Validated Endpoints in ALS

  • Research type

    Research Study

  • Full title

    TRICALS RESEARCH INTO THE COURSE OF ALS: A MULTI-CENTRE STUDY OF RESPIRATORY DECLINE AND ASSOCIATED SYMPTOMS IN PEOPLE WITH ALS/MND

  • IRAS ID

    242722

  • Contact name

    Christopher McDermott

  • Contact email

    C.J.McDermott@sheffield.ac.uk

  • Sponsor organisation

    Sheffield Teaching Hospitals NHS Foundation Trust

  • Duration of Study in the UK

    1 years, 9 months, 13 days

  • Research summary

    Motor neurone disease is a life shortening disorder, in which the nerves that connect the muscles to the nervous system die. This leads to a progressive weakness of the muscles needed for movement of the arms and legs, chewing and swallowing, and breathing. Usually individuals affected by MND succumb to the illness and die within 2-3 years. There is no cure and treatment is aimed at easing symptoms.

    Therapies that improve breathing have the potential to improve both quality of life, and possibly survival. This is evidenced by the beneficial effect of NIV (airway support) on survival (up to 9 months). Drugs that enhance breathing muscle strength therefore have the potential to significantly enhance quality of life and survival, both by delaying the time to introduction of NIV, and by enhancing cough and secretion management. However, definitive measures that evaluate the impact of changes in lung efficiency and the ability to cough are lacking. This is a barrier to the development and identification of effective new treatments improving breathing muscle strength.

    The overall aim of this study is to identify the most appropriate outcome measures to detect meaningful clinical differences in breathing and cough muscle strength which can be used in future studies to evaluate the effectiveness of new treatments.

    We aim to recruit 300 patients from six MND clinics around Europe. We will carry out four respiratory assessments: sniff nasal inspiratory press (SNIP); slow vital capacity (SVC); forced vital capacity (FVC); and peak cough flow (PCF). We will administer questionnaires to assess respiratory symptoms, quality of life, sleep, fatigue, secretion management, and caregiver burden. Patients will be followed up for 18 months at 3 monthly intervals. We will also contact patients remotely every 2 weeks to record the details of any chest infections.

  • REC name

    South Central - Hampshire A Research Ethics Committee

  • REC reference

    18/SC/0174

  • Date of REC Opinion

    29 Mar 2018

  • REC opinion

    Further Information Favourable Opinion