Overview

Respiratory disease affects one in five people and is a leading cause of global morbidity and mortality. Chronic obstructive airways diseases encompass conditions characterised by expiratory airflow limitation, exertional dyspnoea, activity limitation and impaired quality of life. The most common conditions include chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, cystic fibrosis and primary ciliary dyskinesia. In recent years, there has been concerted effort in the scientific and respiratory medicine community to improve the diagnosis and management of chronic obstructive airways diseases using personalised or precision medicine (i.e., tailoring therapies and interventions according to specific "treatable traits") and identifying phenotypes or endotypes using validated biomarkers. To date, however, research in this setting has primarily focussed on people with COPD and asthma, with limited studies in other forms of chronic obstructive airways diseases. The aim of this study is therefore two-fold; first, to compare pulmonary physiology (i.e., large and small airway involvement) and extra-pulmonary manifestations across the spectrum of chronic obstructive airways, and second, to determine how disease-specific treatable traits associate with physical activity and health-related quality of life.

Eligibility

Inclusion Criteria:

• Prior diagnosis of airways disease in accordance with European Respiratory Society guidelines.
• Non / ex-smokers (packs recorded as cigarettes per day / years smoked).
• Male or female aged 18-65 years
• Ability to provide written informed consent.
• Full comprehension of spoken and written English language.
• Cystic Fibrosis patients on triple CFTR modulators (90% cohort)
• Cystic Fibrosis patients on no CFTR modulators
• Healthy controls - entirely asymptomatic, no prior history of inhaler medication use and free from respiratory disease.

Exclusion Criteria:

• Severe exacerbation requiring hospital admission or oral corticosteroids (OCS) in the past two months.
• Absolute or relative contraindications to cardio-pulmonary exercise testing or submaximal exercise testing.
• Absolute or relative contraindications to pulmonary function testing .
• Absolute or relative contraindications to dual energy x-ray (DEXA) scanning (i.e., pregnancy, recent contrast media administration or subject weight).
• Non-ambulant or musculoskeletal impairment that may affect activities of daily living or maximal exercise testing.
• Significant cognitive impairment (i.e., unable to provide written informed consent or safely / successfully perform tests).
• Currently receiving oxygen therapy.
• Inability to consent.
• Burkholderia Cepacia Complex, mycobacterium tuberculosis or mycobacterium abseccus infection.
• Lung transplantation
• Diagnosis of cardiovascular disease.
• Abnormal blood screening (anaemia, moderate / severe renal failure etc.)