Preference of neuromuscular patients regarding equipment for daytime mouthpiece ventilation: A randomized crossover study

Abstract

Background Patients with neuromuscular disorders (NMDs) are likely to develop respiratory failure which requires noninvasive ventilation (NIV). Ventilation via a mouthpiece (MPV) is an option to offer daytime NIV. Objectives To determine the preferred equipment for MPV by patients with NMDs. Methods Two MPV equipment sets were compared in 20 patients with NMDs. Set 1, consisted of a non‐dedicated ventilator for MPV (PB560, Covidien) with a plastic angled mouthpiece. Set 2, consisted of a dedicated MPV ventilator (Trilogy 100, Philips Respironics) without backup rate and kiss trigger combined with a silicone straw mouthpiece. The Borg dyspnea score, ventilator free time, transcutaneous oxygen saturation (SpO2) and carbon dioxide tension (TcCO2) were recorded with and without MPV. Patient perception was assessed by a 17‐items list. Results Carbon dioxide tension measurements and total perception score were not different between the two MPV sets. Dyspnea score was lower with the non‐dedicated versus dedicated equipment, 1 (0.5) versus 3 (1‐6), P < 0.01. All patients with a ventilator free time lower than 6 hours preferred a set backup rate rather than a kiss trigger. Sixty five percent of patients preferred the commercial arm support and 55% preferred the plastic angled mouthpiece. Conclusions Dedicated and non‐dedicated MPV equipment are deemed effective and comfortable. Individualization of arm support and mouthpiece is advised to ensure success of MPV. A ventilator free time lower than 6 hours seems to be a useful indicator to a priori set a backup rate rather than a rate at zero associated to the kiss trigger.

Long-Term Mechanical Ventilation: Recommendations of the Swiss Society of Pulmonology

“…For many NMD, use of NIV will become necessary outside of the home (i.e., adapted to an electric wheel chair), requiring a built-in battery with prolonged autonomy; also, use of interfaces such as mouthpieces may be useful: in this case, multimodal ventilators with performant built-in batteries and specific modes for mouthpiece ventilation are recommended [185]. When dependence to NIV reaches 16 h/day, having a back-up “life support-type” ventilator (i.e., a second device) is mandatory.…”

“…Mouthpiece ventilation must be considered in very dependent patients to facilitate speech and social contacts, and to provide ventilator support while on the move (electric wheelchair). Several ventilators presently provide a “mouthpiece” mode which facilitates implementation of mouthpiece ventilation [185].…”

252nd ENMC international workshop: Developing best practice guidelines for management of mouthpiece ventilation in neuromuscular disorders. March 6th to 8th 2020, Amsterdam, the Netherlands

“…Recently, ventilator companies developed a dedicated MPV mode with dedicated arms and circuits without expiratory valve allowing the patient to exhale outside the mouthpiece [29] . Another advantage of this arms and circuits is the practicality to support and hold the mouthpiece in perfect position and easiest way to adapt it to a wheelchair.…”

“…When looking to the literature there is very little evidence for the use of MPV in other NMDs. Studies have shown MPV to be used in acid maltase deficiency [32] , myotonic dystrophy [32] and limb girdle muscular dystrophy [32] , fascioscapular muscular dystrophy [9] , congenital myopathy [9] , Becker muscular dystrophy [9] , metabolic myopathy [9] , post poliomyelitis [15] , primary adhalinopathy, congenital dystrophies [29] , Pompe's disease [15] and other neuromuscular diseases [9] . Based on the MPV survey we conducted, use of MPV in other NMD's is common in congenital myopathies ( n = 60), cervical spine injury ( n = 46), myotonic dystrophy ( n = 30).…”

The adult multidisciplinary respiratory neuromuscular clinic

“…The interface used with NIV has considerable impact on outcome. Although the majority of studies investigating the impact of interface have focused on patients with acute respiratory failure [45], two randomised studies in patients with chronic hypercapnic respiratory failure have demonstrated that interface can impact on tolerance to therapy as well as physiological markers of therapy success [46,47]. A recent systematic review on NIV in ALS patients has demonstrated the importance of a patient-tailored approach to selection and fitting of the interface to optimise delivery of NIV [48].…”