Respiratory Physiotherapy Performed by Simeox In Patients With Primary Ciliary Dyskinesia
Condition: Primary Ciliary Dyskinesia
MeSH terms: Ciliary Motility Disorders; Dyskinesias
Study type: Interventional

Summary: 
      Primary ciliary dyskinesia (PCD) is characterized by impaired airway clearance and mucus
      stagnation. This results in recurrent upper and lower respiratory tract infections often
      leading to chronic inflammation and, if not treated early and properly, to irreversible
      functional and structural changes of the respiratory tract. As there is no causal treatment
      of PCD yet, airway clearance techniques (ACT) provide fundamental care for these patients.

      Simeox is a new airway clearance device, recently developed by the French company
      PhysioAssist. This technology is based on pneumatic vibrations generated by the device
      itself. Vibrations are induced by rapidly alternating between atmospheric and negative
      pressure as the patient exhales, providing the most effective clearance of mucus from the
      lungs. Vibrations of different intensity and frequency are known to alter the rheological
      properties of mucus in the airways, whilst the negative pressure during exhalation helps to
      mobilise and drain the mucus to the central bronchi.

      Although there have not yet been any evidence based papers published clarifying the effect of
      Simeox specifically in patients with PCD, using up-to-date information, experience, and
      positive feedback from our patients, we assume that there could be a significant benefit for
      the effectiveness of ACT.
    

Detailed description: 
      In this study the effectiveness of ACT will be evaluated based on the short-term effect(s) of
      respiratory physiotherapy with Simeox on changes in lung function and thoracic expansibility
      paediatric patients with PCD.

      An interventional randomised non-inferiority crossover trial will be conducted in Motol
      University Hospital; the project was developed by the multidisciplinary team (MDT) of the
      Division of Paediatric Pulmonology of the Department of Paediatrics, 2nd Faculty of Medicine,
      Charles University in Prague and Motol University Hospital; and the Department of
      Rehabilitation and Sports Medicine, 2nd Faculty of Medicine, Charles University in Prague and
      Motol University Hospital.

      Eligible patients will be randomly assigned into 2 arms - "Simeox-first group" and
      "Pari-O-PEP first" group. Randomisation will be done using computer generated code for each
      patient. The protocol will include 3 study visits - K0, K1 and K2 planned 3 months apart.
      After 6 months (second visit - K1), each patient will be switched to the other interventional
      group (crossed over). The first session (K0) includes an initial lung function assessment
      using spirometry, nitrogen-Multiple Breath Washout test (N2-MBW), Electrical Impedance
      Tomography (EIT), 6 Minute Walk Test (6 MWT), respiratory amplitudes (RA), and a theoretical
      introduction to therapy with the Simeox device. The second (K1) and third (K2) sessions will
      be interventional, including a respiratory physiotherapy session in between each measurement,
      excluding 6 MWT, which will be measured just once per session.

      According to randomisation criteria, participants will initially undergo either
      physiotherapist-administered 20-minute ACT session using the Simeox device or 30-minute
      therapy with PARI O-PEP. In accordance with the rules for crossover study design, each
      patient will undergo therapy with both ACT devices, ideally within six-month duration of the
      study. These interventions will take place in between each measurement (spirometry, MBW, EIT,
      RA) during the second (K1) and the third (K2) session.
    

Intervention name(s): Simeox; PARI O PEP
Intervention type(s): Device; Device
Intervention description(s): A respiratory physiotherapy session with the Simeox device will be performed in accordance with the official PhysioAssist recommendations, which are;
clearance of the upper airways before the physiotherapy session
correct position of the mouthpiece in patient's mouth, with the lips placed on the thinnest part of the mouthpiece and the tongue positioned underneath the mouthpiece
slow nasal inhalation without too much recruitment of the inspiratory residual volume (IRV) and an exhalation with real deflation of the thorax
step-by-step shifting of the patient's tidal volume towards the expiratory residual volume (ERV), in order to target the most distal regions of the lungs
comfortable, relaxed sitting position with straightened spine
controlled cough, which will be encouraged only when it is productive; ACT with PARI O-PEP will be performed in an upright sitting position, using four positions of the device for the most effective clearance of mucus from the lungs. These positions will be:
horizontal;
low;
transition of the device from the low position to the horizontal position;
upside down.
The mouthpiece will be placed between the patient's teeth as per standard procedure, and enclosed properly within the lips. Patients will perform slow and deep inhalation via the nose, then hold their breath for about 1 to 2 seconds at the end of inhalation. Exhalation will be performed slowly and completely into the PARI O-PEP through the patient's mouth. At the end of the session, the patient will perform "huffing" with the PARI O-PEP device in the upside down position in order to maintain open airways, and, if possible, expectoration sputum.
Allocation: Randomized
Intervention model: Crossover Assignment
Intervention model description: First session - initial medical and physiotherapy assessment
Second session - medical and physiotherapy assessment, respiratory physiotherapy with Simeox/PARI O PEP (according the randomization)
Third session - medical and physiotherapy assessment, respiratory physiotherapy with Simeox/PARI O PEP (according the randomization)
Masking: None (Open Label)
Primary purpose: Supportive Care
Keywords: primary ciliary dyskinesia; respiratory physiotherapy; airway clearance techniques; lung function testing; ventilation inhomogeneity; nitrogen multiple breath washout test; spirometry; electric impedance tomography