The purpose of this study is to determine whether correction of low nighttime oxygen (O2) levels in patients with cystic fibrosis improves their qualtiy of life. The treatments being used overnight are (1)O2 (2)pressurised air which assists breathing (non-invasive positive pressure ventilation, NIPPV)

Condition	Intervention	Phase
Cystic Fibrosis	Device: Nocturnal oxygen , nocturnal bi-level positive pressure ventilation	Phase I Phase II

Further Study Details: 

Primary Outcomes: Quality of life questionnaires:; CFQoL questionniare (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; Work or Study status; Physiological:; Nocturnal SpO2, nocturnal rise in transcutaneous CO2; Daytime arterial blood gases (PaCO2, PaO2)
Secondary Outcomes: 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)
Expected Total Enrollment:  18

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

Ages Eligible for Study:  18 Years   -   75 Years,  Genders Eligible for Study:  Both

Criteria

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)

Exclusion Criteria:

Previous home O2 or NIV use, Sedative medications, Cardiac/renal/endocrine/neurological disease likely to compromise ventilatory control

Please refer to this study by ClinicalTrials.gov identifier  NCT00157183

Alan C Young, MBBS, FRACP      613 9276 2000  Ext. pager 4576    alan.young@med.monash.edu.au
Matthew T Naughton, MBBS, FRACP      613 9276 2000  Ext. 3770    m.naughton@alfred.org.au

Australia, Victoria
      The Alfred, Melbourne,  Victoria,  3181,  Australia; Recruiting

Study chairs or principal investigators

Matthew T Naughton, MD,  Principal Investigator,  The Alfred Hospital   

Study ID Numbers:  35/03
Last Updated:  September 10, 2005
Record first received:  September 9, 2005
ClinicalTrials.gov Identifier:  NCT00157183
Health Authority: Australia: Therapeutic Goods Administration
ClinicalTrials.gov processed this record on 2005-09-13