Pulmonary magnetic resonance imaging in cystic fibrosis [V1.0]
Research type
Research Study
Full title
Pulmonary magnetic resonance imaging in cystic fibrosis: accuracy and reproducibility
IRAS ID
73137
Contact name
Nicholas J Screaton
Contact email
Sponsor organisation
Papworth Hospital NHS Foundation Trust
Research summary
Research Summary
Cystic fibrosis (CF) occurs in 1 of 2500 live births, and is the most common fatal genetic disorder. The disorder involves malfunction of several organ systems, but lung disease is the principal cause of the severely reduced life expectancy. Irreversible lung damage and respiratory failure result from repeated and eventually chronic lung infections, which are caused by an inability to clear inhaled bacteria that manifests in childhood. Despite improved treatments, average life expectancy is only 37 years.\n\nTreatment of chronic and acute deteriorations in lung function in CF is directed by clinical tests. The optimal test is high-resolution computed tomography (HRCT), which is very sensitive to even subtle lung changes. There are, unfortunately, important concerns about exposing this young population to the high doses of ionising radiation involved in repeated HRCT. Therefore, tests that are less sensitive to subtle or regional changes are routinely used, including pulmonary function tests and CXR. A more sensitive way of detecting subtle lung disease without exposing the individual to radiation is crucial in order to improve outcomes, and the radiation free magnetic resonance imaging (MRI) has been indicated to be a better tool that provides valuable structural and functional information. \n\nThis study compares MRI to HRCT and CXR in CF, with the aim to confirm MRI as a valuable tool that may facilitate more targeted and timely treatment with the benefit of improving life expectancy. The study is open to individuals with CF over 18 years who are referred for routine HRCT, and they will in addition to the HRCT undergo two MRI studies (performed within one week) and one CXR. A group of healthy volunteers of the same age will also be recruited (for one MRI). The study is performed at and funded by Papworth Hospital, Cambridgeshire.
Summary of Results
This study was planned to take place at Papworth Hospital, Papworth Everard, Cambridge from 2014 – 2017. It was hoped that this study would provide the basis for ongoing research into diagnosis and treatment of cystic fibrosis (CF). The plan was to scan the lungs of 40 patients with CF and 20 healthy volunteers in an MRI scanner and compare the results to ‘standard of care’ imaging in a CT scanner or using X-rays.
The main advantage of PMR imaging over CT or X-ray imaging is that the latter involves giving the mainly young CF patients repeated doses of radiation which can in turn cause secondary diseases such as cancer later in life. PMR imaging involves no harmful radiation.
The researchers encountered some problems performing this research. Firstly, the study was paired with a sister project to image CF patients’ lungs using hyperpolarized Xenon gas. There were financial, logistical and technical problems in securing hyperpolarization equipment, delaying both projects. In 2019 Papworth Hospital relocated to a new site in central Cambridge and new MRI scanners were purchased, setting technical adjustments back to a new starting point. Several clinicians involved in the project had by then left the hospital and, finally, the COVID epidemic made all research recruitment difficult.
As a result of these difficulties, we only recruited 2 patients and 2 volunteers to this study. These participants underwent a single MR examination, each with administration of a gadolinium contrast agent, which provided information on how blood flows to the lungs. All participants also had standard lung function tests (spirometry) which involved breathing into a tube to measure air pressure generated and answered a brief questionnaire on their current health. The patient group had CT scans and X-rays performed as part of their standard clinical care, but they gave consent for these images to be used as part of the study.
No formal results can be reported from this study since the number of participants recruited was very small.
A future similar study is planned at the new site of Royal Papworth Hospital in Cambridge in which hyperpolarized Xenon will be administered to patients with CF and other lung diseases whilst they are being imaged in an MRI scanner. These images will allow calculation of the degree of blockage of the lung passageways (‘ventilation defects’) and the flow of blood to different regions of the lung (‘perfusion abnormalities’). The aims of this future study will be similar, to reduce the reliance on ionising radiation imaging techniques in diagnosis and treatment evaluation in this cohort of patients.
REC name
East of England - Cambridgeshire and Hertfordshire Research Ethics Committee
REC reference
13/EE/0401
Date of REC Opinion
27 Nov 2013
REC opinion
Favourable Opinion