The Effects of Long Term Inhalation of Hypertonic Saline in Subjects With Cystic Fibrosis
Cystic Fibrosis
About this trial
This is an interventional treatment trial for Cystic Fibrosis
Eligibility Criteria
Inclusion Criteria: Confirmed diagnosis of CF (sweat tests/genotype) The subject, or their legal guardian for children under 18 years old, must provide written informed consent. The subject must be in stable clinical condition at the time of and for a period of 14 days prior to their recruitment into the study. Age > 6 years old FEV1 > 40% predicted for height, age and gender Proven or anticipated compliance with therapy or study protocol Regular attendee at a Cystic Fibrosis Clinic (> 2 visits per year) Able to reproducibly perform lung function tests (spirometry) Relatively stable nutritional status (< 2 kg weight loss in last 6 months and < 5 kg weight loss in last year) Known to have "normal" (for CF subject) laboratory tests - haematology, biochemistry, immunology, coagulation, etc. Exclusion Criteria: Requiring home oxygen (pO2 <55mmHg or pCO2 >50mmHg) or assisted ventilation. Considered "terminally ill" or listed for transplantation (either lung or liver). Subjects that are listed for transplant after being enrolled in the trial are eligible to continue in the trial. Subjects colonised with Burkholderia cepacia. However, if a subject becomes colonised with B. cepacia during the trial, they should continue in the trial. Subjects should be considered to be B. cepacia positive if they have had even a single lifetime isolate. In these subjects, spirometry should be measured on a dedicated spirometer. Cigarette smoker. Exposure to investigational drugs within the past 30 days. Major haemoptysis (> 60 mL in a single episode) within the last twelve months. Concurrent illnesses eg. cor pulmonale, clinically significant liver disease (portal hypertension, varices). Known allergy to quinine sulphate, Glucose 6-phosphate dehydrogenase deficiency. Immune thrombocytopaenic purpura. Pregnant or lactating females. At risk females unwilling to use appropriate contraception to prevent pregnancy for the duration of their enrolment in the study.