Non-invasive Ventilation and Oxygen Therapy in Cystic Fibrosis Patients With Nocturnal Oxygen Desaturation

Study Overview
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Brief Summary
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The purpose of this study is to determine whether correction of low nighttime oxygen (O2) levels and/ or high carbon dioxide levels in patients with cystic fibrosis improves their quality of life. The treatments being used overnight are (1)O2 (2)pressurised air which assists breathing (non-invasive positive pressure ventilation, NIPPV)

Detailed Description
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Cystic fibrosis is the commonest life-limiting genetic disorder in the Caucasian population with a median survival of 31 years. Lung disease is responsible for the majority of morbidity and mortality and correlates with declining quality of life. Respiratory failure is the primary cause of death. Daytime respiratory failure (hypoxia with pO2<55 and/or hypercapnia with pCO2>50) is associated with a worse prognosis with a 2-year survival of 50%. Nocturnal respiratory failure (greater than 5% of the night spent with SpO2<90% and/or rise in PtcCO2>10mmHg overnight) is a precursor to the development of daytime respiratory failure. It has been postulated that earlier treatment of respiratory failure may improve outcome and quality of life. Intervention: Nocturnal O2 and bilevel NIPPV in CF patients with nocturnal respiratory failure, compared to nocturnal placebo (air). Crossover trial utilising patients as their own control. Aims: (1) To assess the effects of non-invasive ventilation (NIV) and oxygen (O2) therapy on quality of life, hospital admission rate, sleep quality and exercise tolerance in CF patients with NRF (2) To identify a level of severity of NRF where treatment with NIV is effective

Official Title
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Non-invasive Ventilation and Oxygen Therapy in Cystic Fibrosis Patients With Nocturnal Oxygen Desaturation

Conditions
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Cystic Fibrosis

Intervention / Treatment
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* Device: Nocturnal oxygen , nocturnal bi-level positive pressure ventilation


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: proven diagnosis cystic fibrosis, age 18 years or older, FEV1< 70% predicted normal, clinically stable (no admission or antibiotics last 2 weeks, OR end of admission where further clinical improvement not expected), nocturnal respiratory failure (SpO2<90% for > 10% of night or rise in PtcCO2 > 5 mmHg in REM), daytime hypercapnia (PaCO2> 45 mmHg) Exclusion Criteria: Previous home O2 or NIV use, Sedative medications, Cardiac/renal/endocrine/neurological disease likely to compromise ventilatory control

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 75 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Crossover Assignment
Masking: Single


Arms and Interventions

| Intervention/Treatment |
| --- |
|Device: Nocturnal oxygen , nocturnal bi-level positive pressure ventilation|nan|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Quality of life questionnaires: |  |  | 
| CFQoL questionnaire (Gee,Thorax,2000)(a priori chest, physical function, treatment, emotion domains) |  |  | 
| Epworth Sleepiness Scale |  |  | 
| Pittsburgh Sleep Quality Index |  |  | 
| CF Subjective Symptoms Sleep disturbance Questionnaire (CSQ-in house) |  |  | 
| Medical Research Council Dyspnea Scale |  |  | 
| Baseline Dyspnea Index, Transitional Dyspnea Index |  |  | 
| Work or Study status |  |  | 
| Physiological: |  |  | 
| Nocturnal SpO2, nocturnal rise in transcutaneous CO2 |  |  | 
| Daytime arterial blood gases (PaCO2, PaO2) |  |  | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Admission rate |  |  | 
| Lung function tests (FEV1, FVC, RV/ TLC) |  |  | 
| Modified CF shuttle walk test |  |  | 
| Neurocognitive testing (psychomotor vigilance task, Stroop, Controlled Oral Word Association Test, Trails A and B, digit recall forwards backwards) |  |  | 
| PSG (sleep efficiency, arousal index, % REM sleep, urinary catecholamines) |  |  | 
| Serum cytokines (IL-6, TNF alpha, IL-1 beta) |  |  | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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cystic fibrosis, hypoxia, oxygen, noninvasive ventilation, quality of life