Lung Clearance Index (LCI) Track is a multi-centre longitudinal study designed to monitor the changes in LCI in a cohort of cystic fibrosis patients and healthy controls at The Hospital for Sick Children and at the Riley Hospital for Children in Indianapolis. This study is an extension of a preschool longitudinal study that ended in the summer of 2016.

We plan to follow this cohort of cystic fibrosis patients and healthy children for two years to delineate changes in LCI associated with disease progression between the preschool years and school age. We are also interested in measuring LCI when cystic fibrosis patients experience a pulmonary exacerbation and after treatment. The purpose of this study is to determine whether LCI can be used to monitor cystic fibrosis patients clinically during episodes of worsening pulmonary symptoms.

This study is currently recruiting participants.

The Lung Clearance Index (LCI) is a breathing test that has been recently developed and has been shown to be a more sensitive test than traditional PFTs in mild cystic fibrosis. However, LCI does not provide any information about the location of disease within the lung. Hyperpolarized xenon magnetic resonance imaging (Xe-MRI) of the lung is a radiation-free imaging technique that has been shown to be useful for identifying structural lung abnormalities in various adult diseases, but has never been investigated in children with cystic fibrosis.

The combination of lung MR imaging and LCI could be a very powerful tool for quantifying and localizing ventilation inhomogeneity in lung disease while concomitantly assessing for local anatomical changes in the lung. At The Hospital for Sick Children, we have a pilot study underway to investigate this combination of techniques in children with stable cystic fibrosis. We propose to study this further by investigating the ability to identify improvements in lung structure and function after treatment for a common complication of cystic fibrosis lung disease called a pulmonary exacerbation (PEx).

The aim of this study is to assess whether the Lung Clearance Index (LCI) can be used to track lung function in patients with mild lung disease. LCI will be assessed when a child is well, then at the onset of a pulmonary exacerbation (PE), after PE treatment has been completed, and lastly when the child is well again. This will allow us to evaluate LCI as a measure to track lung function during these clinically important events.

This study is currently recruiting participants.

Implementation of intervention strategies to prevent lung damage early in cystic fibrosis requires objective outcome measures that capture and track lung disease. In this longitudinal 12 month observational study, we demonstrated that the Lung Clearance Index (LCI) deteriorates in preschool cystic fibrosis children over time and during episodes of cough as well as pulmonary exacerbations, whereas similar symptoms in healthy children did not change the LCI. The findings of this study suggests that LCI may be a useful marker to track early disease progression as well as guide therapies in young patients with cystic fibrosis.

The Toronto Cystic Fibrosis Registry (TCF) is a detailed clinical summary of patients with cystic fibrosis followed at Toronto Cystic Fibrosis Centre. The TCF has been in existence since the early 1970s, when it was designed to collect basic demographic details and vital statistics. In the early 1980s, clinical data such as lung function, nutrition markers (e.g. height and weight), and microbiology were added to the Registry. In the late 1990’s detailed information about antibiotic treatments were added as part of an auxiliary study.

TCF are incredible resources for cystic fibrosis clinicians, researchers, as well as individuals with cystic fibrosis and their families. The TCF data have been used to publish multiple manuscripts in peer-reviewed journals and have enhanced our understanding of disease progression in cystic fibrosis.

The aim of this study is to develop in vitro models of therapeutic response to CFTR modulators. The program will generate model cell systems from 100 cystic fibrosis patients, as they are accessed by the Cystic Fibrosis Canada research community. This study is a collaboration between Cystic Fibrosis Canada, SickKids Foundation and The Hospital for Sick Children to develop a nationally accessible resource that will accelerate the development of precision medicine for the treatment of each individual living with cystic fibrosis.

Hereditary Hemorrhagic Telangiectasia (HHT) is a condition with blood vessel malformations, called telangiectasia and arteriovenous malformations (AVMs), occurring in organs of the body such as the brain, nose, lungs, stomach, bowels and liver. Brain AVMs (BAVMs) in HHT are difficult to study because they are rare, affecting approximately 10% of people with HHT. We are joining with 12 HHT Centers of Excellence in North America to collect information about HHT to construct a database to help us find risk factors for this bleeding.

We plan to study risk factors for rupture of BAVMs, including primarily genetics and imaging characteristics of the BAVMs. Knowledge about risk factors will help in the care and management of HHT patients. This will be achieved through the collection of health information to construct a HHT database, and through genetic analysis at the University of California San Francisco.

This study is currently recruiting participants.