Research Project information
Principal researcher: Dr Peter Merkus
Institute: Radboud University Medical Centre, the Netherlands
Cost: £87,810 over 36 months
Completion Date: 30th August 2024
Project Overview
Many children and adults with Ataxia Telangiectasia (A-T) suffer from recurrent infections and some develop malignancies. This is responsible for a large burden for these patients and their families/caretakers, and may cause severe symptoms and even death. In order to improve treatment, it is necessary to be able to monitor the condition of the lung better. Lung function measurements are difficult to carry out reliably for many people with A-T, and imaging of the lungs with conventional radiology of the chest involves ionizing radiation that should be avoided whenever possible. Magnetic Resonance Imaging (MRI) does not involve ionizing radiation and improved MRI would be the ideal tool to obtain reliable structural information of the lungs. However, MRI scanning used to be less suitable for lung imaging till recently because of poor resolution. Furthermore MRI is not a quick procedure and patients with A-T often have uncoordinated movements that preclude optimal imaging.
Research Methods and Outcome
Dr Merkus and his team, aimed to improve the resolution of the MRI images, to correct for movement during the procedure and to reduce the time needed to obtain images of the lungs. The goal was to enhance and standardize a technique suitable for routine clinical care of patients with A-T, also the severe and the young.
Although the project suffered considerable delays, the team were successful in reaching their stated goals/milestones. Furthermore, they now also have a tool to assess ventilation and perfusion of the lungs during tidal breathing, so not only are they now able to make images of the structure of the lungs, but also of their function.
The quality of the imaging is clearly better than using conventional Chest X-rays. Through optimizing duration of procedure and resolution using the software developed in the past few years, MRI quality is now far better than previously possible. The team believe that their work will contribute to better monitoring and treating people with A-T, thereby enhancing the quality of their lives.
What next?
The team at Radboud University Medical Centre have subsequently decided that they will introduce the MRI as a part of the annual visit to the outpatient clinic. This will require serious planning and preparation of patients but is considered important. They will also develop a protocol aimed at accepting the MRI scanning better (for instance through distraction with MRI – Compatible VR glasses).
Publications
The team are currently working on a manuscript for publication.
