Please select the eligibility criteria that correspond to the clinical trial below.
Title: Non-Invasive Ventilation (NIV) For Positive Pressure Support. A Randomised Cross-Over Trial To Evaluate The Short-Term Effects of NIV As An Adjunct To Airway Clearance Techniques in Adults With Cystic Fibrosis.
Brief Summary: Cystic Fibrosis (CF) is a genetic disorder of altered ion transport across cell membranes which is characterised by the production of thickened bodily secretions, affecting the function of organs such as the pancreas and the lungs. Within the lungs, thickened sputum is very difficult to clear, which can results in recurrent chest infections, which can lead to lung damage. therefore it is important to optimise the removal of sputum to try and prevent these complications. Traditionally, a variety of approaches are usually combined including inhaled medications to thin or hydrate secretions, and chest physiotherapy to mobilise secretions and improve sputum clearance.

There are many chest physiotherapy or airway clearance techniques (ACT) available including breathing methods such as the Active Cycle of Breathing Techniques (ACBT) or Autogenic Drainage (AD) and adjuncts such as Positive Expiratory Pressure (PEP), High Frequency Chest Oscillation (the "Vest"), or oscillatory devices such as the Flutter or Acapella. When people with CF have an infection or have severe disease often the effectiveness of ACTs can decrease due to fatigue, shortness of breath or having an overwhelming amount of sputum. At this time it is necessary to re-assess ACTs and the addition of positive pressure to airway clearance techniques has been shown to be helpful in decreasing fatigue during chest physiotherapy.

At present no research studies have reported an increase in sputum cleared with the addition of positive pressure, however it is thought that the ability to take a deeper breath when using positive pressure would help to improve sputum clearance. With clinical experience of the use of NIV with adult CF patients, the investigators aim to explore this objectively in this study.

Research Question:

Does the addition of non-invasive ventilation (Breas, I-Sleep 25) as supplementary positive pressure to normal airway clearance techniques improve sputum clearance in stable adult patients with cystic fibrosis?

Hypothesis

The inclusion of non-invasive ventilation in addition to a patient's normal airway clearance technique will lead to improvements in subjective ease of clearance and work of breathing during airway clearance and objectively increase sputum clearance, as well as being well tolerated in patients as an adjunct to airway clearance.


Options:
A: Inclusion Criteria:

* Pass the inhalation test
* Chronic sputum production, at least ≥ 10 ml /24h
* Cystic Fibrosis (CF) diagnosed ( established by genotype or sweat sodium\>70 mmol/l or sweat chloride of\>60 mmol/l)
* Clinically stable at the time of recruitment (defined as no requirement for antibiotics or change in respiratory medication in the preceding 4 weeks)
* Trained in the use of autogenic drainage technique (at least 6 months)
* Inhaling hypertonic saline since at least 6 months
* To be able to provide written, informed consent and perform the protocol and the evaluations

Exclusion Criteria:

* Active massive hemoptysis during the previous 2 months
* Patient in transplantation or retransplantation list
* Patient already participating in another study at the same time
B: Inclusion Criteria:

* Diagnosis of CF (confirmed by standard criteria)
* Sixteen years of age or over
* Patients in a stable state (characterised by no required therapy changes within 28 days of visit and patient symptoms)

Exclusion Criteria:

* Current infective exacerbation or reduction in lung function requiring therapeutic intervention
* Current moderate haemoptysis (greater than streaking in the sputum)
* Current dependency on positive pressure support with ACT
* Previous history of spontaneous rib fractures
* Pregnancy
* Inability to give consent for treatment or measurement
* Current participation in another interventional study
* Current dependency upon non-invasive ventilation
* Current dependency upon oxygen therapy
C: Inclusion Criteria:

* Diagnosis of cystic fibrosis (confirmed by genotype or a sweat sodium concentration of \>70mmol/l or sweat chloride of \>60mmol/l)
* Sixteen years of age or over
* Patients admitted to the Royal Brompton Hospital with a pulmonary exacerbation of which is resolving. Patients will be considered for inclusion from day 7 of treatment to 3 days prior to discharge (as determined by a member of the cystic fibrosis medical team and have spirometric values within 20% of the mean of the last two stable recordings (at least 1 month apart)
* Patients with an established airway clearance regime that they have used for 3 months or more

Exclusion Criteria:

* Current moderate haemoptysis (greater than streaking in the sputum)
* Current pneumothorax or history of pneumothorax in the 3 months prior to consideration for the study
* Current dependency on positive pressure support with airway clearance via the IPPB (Intermittent Positive Pressure Breathing) machine or NIV
* Previous history of spontaneous rib fractures
* Pregnancy
* Inability to give consent for treatment or measurement
* Current participation in another study
* If the patient requires more than 2 airway clearance sessions a day
D: Inclusion Criteria:

* Acute or chronic disease requiring secretion mobilization as per hospital protocol

Exclusion Criteria:

* Absolute contraindications for positional changes
* Absolute contraindications for chest percussion
* Women visibly pregnant
* Previous enrollment in study
* Previous CPT or Vest treatment

Answer: C