Long-Term Effect of LIAM on Respiratory Performance in NIV Patients Suffering From Neuromuscular Disease

NCT ID: NCT02288299

Last Updated: 2019-11-18

Basic Information

• Recruitment Status: UNKNOWN
• Clinical Phase: NA
• Total Enrollment: 30 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2015-10-29
• Study Completion Date: 2021-12-31

Brief Summary

The long-term effect of LIAM (Lung Insufflation Assist Maneuver) on respiratory performance in home non-invasively ventilated (NIV) patients suffering from neuromuscular disease will be assessed in a prospective, randomized, cross over, open label study

Detailed Description

In advanced neuromuscular disorders, respiratory complications represent the main cause of morbidity and mortality. Beside chronic respiratory insufficiency, necessitating a ventilatory support, mostly performed by non-invasive ventilation (NIV), cough is impaired due to the muscle weakness, and respiratory physiotherapy becomes an essential part of the management.

Various techniques have been proposed to improve lung recruitment and cough in neuromuscular patients. The assisted techniques based on a positive pressure insufflation maneuver have shown an improvement in physiological variables on the short term, but there is to date no good-quality prospective study allowing to evaluate the long term efficacy of mechanical cough assistance devices in neuromuscular patients.

We designed a randomized, cross over, open label study to assess the long-term effect of LIAM (Lung Insufflation Assist Maneuver) on respiratory performance in NIV patients suffering from neuromuscular disease.

Conditions

Neuromuscular Disease

Study Design

• Allocation Method: NA
• Intervention Model: SINGLE_GROUP
• Primary Study Purpose: TREATMENT
• Blinding Strategy: NONE

Study Groups

non invasive mechanical ventilation

Patients suffering from neuromuscular disease with NIV indication and cough inefficiency

Group Type: EXPERIMENTAL

non invasive mechanical ventilation

Intervention Type: DEVICE

instrumental increase of inspiratory capacity and cough

Interventions

non invasive mechanical ventilation

instrumental increase of inspiratory capacity and cough

Intervention Type: DEVICE

Eligibility Criteria

Inclusion Criteria

• o neuromuscular disease ( progressive muscular dystrophy, for example; Duchenne muscular dystrophy, spinal muscular atrophy)

• age ≥ 18 years
• indication to NIV (ongoing ventilation or new patients)
• vital capacity ≤ 50% pred
• peak cough flow < 270 l/min
• "LIAM Responder" = increased insufflation capacity (≥ + 50%) and Peak Cough Flow (PCF ≥+ 50%) with LIAM

Exclusion Criteria

• o acute respiratory failure (respiratory acidosis)

• home treatment by instrumental cough assistance in the preceding 12 months
• ongoing medical treatment of the neuromuscular disease (for ex: corticosteroids in Duchenne disease, enzyme therapy in Pompe disease)
• previous pneumothorax
• plan of legal protection
• pregnant or breastfeeding women
• failure to cooperate
• no affiliation to a social security scheme

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Centre d'Investigation Clinique et Technologique 805

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

David Orlikowski, MD, PhD

Role: PRINCIPAL_INVESTIGATOR

Assistance Publique - Hôpitaux de Paris

Locations

Home ventilation Unit , Raymond Poincaré hospital

Garches, Paris Area, France

Site Status: RECRUITING

Countries

France

Central Contacts

David Orlikowski, MD, PhD

Role: CONTACT

david.orlikowski@rpc.aphp.fr

+33(0)147107777

Frederic Lofaso, MD, PhD

Role: CONTACT

frederic.lofaso@rpc.aphp.fr

+33(0)147107941

Facility Contacts

David Orlikowski, MD, PhD

Role: primary

david.orlikowski@rpc.aphp.fr

33147107776

Sandra Pottier, CRA

Role: backup

sandra.pottier@rpc.aphp.fr

33 1 47 10 44 69

Other Identifiers

2014-A01005-42

Identifier Type: -

Identifier Source: org_study_id