Symptomatic Treatment of Excessive Dynamic Airway Collapse Using Daytime Portable Continuous Positive Airway Pressure
Condition: Excessive Dynamic Airway Collapse; Tracheobronchomalacia
MeSH terms: Tracheobronchomalacia; Bronchomalacia; Shock
Study type: Interventional

Summary: 
      The aim of this study is to assess the benefit of a portable ventilator generating positive
      end-expiratory pressure on exercise tolerance in patients with Excessive dynamic airway
      collapse (EDAC) and tracheobronchomalacia (TBM).
    

Detailed description: 
      Excessive dynamic airway collapse (EDAC) and tracheobronchomalacia (TBM) occur due to
      abnormal weakening of the walls of the central airways leading to central airway collapse on
      expiration. This collapse is responsible for breathlessness, cough or syncope. In adults,
      this weakening can be idiopathic (Mounier-Khun syndrome), secondary to respiratory diseases
      such as chronic obstructive pulmonary disease (COPD), secondary to systemic diseases such as
      relapsing polychondritis (RP) or secondary to invasive ventilation or trauma. The incidence
      of EDAC and TBM varies from 12% in all patients undergoing bronchoscopy to 44% in patients
      with chronic bronchitis undergoing bronchoscopy. Gold standard for the diagnosis of EDAC and
      TBM is bronchoscopy which identifies and quantifies the narrowing of the airway. Non-invasive
      technique such as inspiratory and expiratory chest computed-tomography (CT) can also be used
      to diagnose EDAC / TBM.

      Currently, in addition to management of the underlying disease, treatment options for EDAC /
      TBM are limited. Surgical tracheoplasty can be offered but can be associated with severe
      post-operative complications. Airway stenting can also be offered but, even if this treatment
      improved quality of life, it fails to improve exercise capacity. Airway stenting is also
      associated with infectious complications as well as stent migrations. Other endoscopic
      treatment such as Yttrium Aluminium Perovskite laser can be offered with good results but
      have not yet been validated in a randomised trial. Nocturnal non-invasive ventilation (NIV)
      can also be used, especially in patients with associated obstructive sleep apnoeas but again
      there is not randomised clinical trial evidence that validates this approach in adults.
      Expiratory Positive Airway Pressure (EPAP) provides a pneumatic stenting that prevents the
      expiratory collapse of the airway. But, by giving the NIV during the night, patients are left
      without any support during the day whilst their respiratory demand is higher and when they
      are more symptomatic. Currently, NIV is only given at night or at rest because current
      non-invasive ventilators are not suitable for ambulatory use as they are heavy. Recently, a
      new portable ventilator with built-in battery has been issued (Z1®, Breas®). This ventilator
      is light (500g) portable and has a working duration of 8 hours. Therefore, it can be easily
      carried and used while walking. By providing a nasal pillow interface (Nasal swift®, Resmed®)
      to patients, it will allow them to walk safely with the device on.
    

Intervention name(s): Experimental: Treatment; Experimental: Run In Phase; Sham Comparator: Stand Care Arm
Intervention type(s): Other; Other; Other
Intervention description(s): Participants will be established on portable CPAP during the day with nasal pillows as an interface and requested to use it at least 8 hours/day.; Participants will undergo a run-in phase during which they will undergo baseline assessments. If they tolerate the portable CPAP and if their 6-MWT improves by more than 30m when performed on CPAP, they will be randomised into the trial.; No change will be made to participants care
Allocation: Randomized
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Treatment