Developing best practice guidelines for management of mouthpiece ventilation in Neuromuscular Disorders

Location: Hoofddorp, The Netherlands

Title: 252nd ENMC workshop on Mouthpiece ventilation in Neuromuscular disorders

Date: 6 – 8 March 2020

Organisers: Dr. Michel Toussaint (Belgium), Dr. Michelle Chatwin (UK), Prof. Jesus Gonzales (France) and Prof. Miguel Goncalves (Portugal)

Participants: Dr. Michel Toussaint PT, PhD (Belgium), Dr. Michelle Chatwin PT, PhD (UK) Prof. Jésus Gonzalez-Bermejo MD (France), Prof. Miguel Gonçalves PT, PhD (Portugal), Prof. Joshua O. Benditt MD (USA), Prof. Doug McKim MD (Canada), Ms. Brit Hov PT, PhD student (Norway), Dr. Jesus Sancho MD (Spain), Dr. Valeria Sansone, MD (Italy), Prof. Hélène Prigent MD (France), Dr. Annalisa Carlucci MD, PhD (Italy), Prof. Peter Wijkstra MD, PhD (The Netherlands), Dr. Tiina Andersen PT, PhD (Norway), Ms. Barbara Garabelli PT (Italy), Dr. Joan Escarrabill MD, PhD (Spain), Mr. Tiago Pinto PT, MSc (PhD Student) (Portugal), Mr. Nicolas Audag PT, Msc, (PhD Student) (Belgium), Mrs. Laura Verweij-van den Oudenrijn (The Netherlands), Dr. Adam Ogna, MD (Switzerland), Mrs Wendy Hughes (UK), Mr. Christian Devaux, PT (France), and Mr. Johann Chaulet PhD (France).

Translations of this lay report by:

Dutch by Peter Wijkstra

French by Christian Devaux

Italian by Annalisa Carlucci

Spanish by Joan Escarrabill and Jesus Sancho

Norwegian by Tiina Andersen

Finnish by Tiina Andersen

Twenty-two participants with full representation from the multidisciplinary team, along with one patient representative two patient advisory groups. Participants from Europe, USA and Canada attended the 252nd ENMC sponsored workshop, focusing on mouthpiece ventilation (MPV) in neuromuscular disorders (NMD’s).

Respiratory insufficiency and pneumonia are primary causes of mortality and comorbidity in many NMDs. Studies show mechanical ventilation improves symptoms, gas exchange, quality of life and survival in NMD’s. Ventilatory support is usually offered via non-invasive techniques such as the use of a nasal or full-face mask during sleep. As the disease progresses, daytime ventilatory support becomes necessary. In some conditions, tracheostomy may be proposed when non-invasive strategies are no longer possible (ventilator dependence, severe bulbar insufficiency or mask related complications). An alternative to having a mask on the face during the daytime is to receive ventilation via a mouthpiece ventilation (MPV). Currently there is no consensus with regards to the timing, equipment or settings for the use of MPV in NMD’s.

In order to have a up to date information on current worldwide practice of the use of MPV, a pre workshop survey was sent to individuals working with NMD’s. The survey gathered information around current practice i.e. which countries/regions/type of hospital utilise MPV well, to identify the preferred mode, re-imbursement and aim of MPV delivery and identify any other perceived barriers to MPV.

During the ENMC workshop, there were dynamic discussions between clinical researchers, clinicians and patient representatives on issues related to MPV in order to improve the understanding and formulate recommendations and an algorithm to produce a consistent approach to MPV in individuals with NMDs.

Based on the survey and the experience of the delegates, concluded that MPV is currently underutilised. The attending experts discussed barriers and limitations to the provision of MPV including lack of understanding around the equipment required, the ventilation modes and settings. There are differences between countries regarding provision of MPV and its funding. Several bench studies were described to state the knowledge of technical issues, physiological aspects and ventilator alarms. The workshop group expressed their expectations that industry should develop further dedicated portable devices, modes and equipment for the MPV.

Enthusiastic discussions were facilitated with regards to the physiology of MPV, how to initiate, and monitor MPV. These sessions were based on the available evidence, clinical experience and expertise. The participants and MPV users were in agreement that MPV has several advantages for example, for speaking, eating, swallowing, and coughing. MPV has a huge impact on quality of live as it allows mobility and decreases social isolation as there is not mask on the face acting as a barrier to social inclusion. However, issues of safety and caregiver training were highlighted and recommendations and teaching tools were devised to support this. In further discussions, both the cost/benefit of this technique were explored along with what individuals with NMD’s would benefit the most.

During the workshop, treatment protocols and algorithms were prepared to provide practical recommendations and detailed information about the technique, and answer key questions about when to start; what ventilator; what mode; what mouthpiece; what patient; what monitoring, and follow up is required for individuals using MPV. The attendees hope that this will support clinicians to initiate individuals with MPV and have excellent outcomes.

A full report is published in Neuromuscular Disorders (PDF)