Could the environment be having an effect on cystic fibrosis?

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Find out about some of the research the Cystic Fibrosis Trust are funding to unravel the links between cystic fibrosis (CF) and the environment, and what affect this work could have on the lives of people with the condition.

Last month, Bianca Nicholas who has cystic fibrosis (CF), spoke to Channel 4 news about the affect pollution has on her health. To improve our understanding of how much of an impact environmental factors could be having on the health of individuals with CF, the Cystic Fibrosis Trust is co-funding research into the links between CF and environment factors – for example, where people with CF live and what they are exposed to.

This research is conducted by the Small Area Health Statistics Unit (SAHSU), an internationally recognised research team at Imperial College London. Hear from Dr Fred Piel, a lecturer in SAHSU, and Olivia Murrin, a Medical Research Council Doctoral Training Partnership PhD student, about how this research works and the impact it could have.

How can the environment have an impact on people with cystic fibrosis?

There is growing evidence that large portions of the population of England are exposed to unhealthy levels of a range of pollutants. For example, air pollution may reduce the diversity of the bacteria in our airways, which can lead to infections.

Because the lungs, digestive system and other organs of individuals with CF are already affected by the condition, it is possible that the impact of exposure to various environmental factors could have more adverse effects on them than on people without cystic fibrosis.

Alongside air pollution, there is some evidence from the US, Canada and Europe that other factors such as climate, humidity, temperature, dust and second-hand smoke exposure, could also have an effect on the health of individuals with cystic fibrosis. A study in the United States showed that exposures to air pollution could cause an increased risk of pulmonary exacerbations and a decline in lung function in people with the condition. The conclusions from studies conducted in different countries are not always consistent and few studies on these associations have so far been conducted in England.

As well as the effects of purely environmental factors (eg pollution and climate), various other factors such as age, sex, socio-economic status and proximity to a CF centre could potentially also have an influence on the health of people with CF and need to be accounted for in studies of environment-health associations.

How will the research work?

We are working on this Trust-funded project to use vast amounts of information available in the UK Cystic Fibrosis Registry alongside detailed data on environmental factors to study possible associations and improve our understanding of the extend by which environmental factors may affect the progression of CF, and how this could change throughout the lifetime of individuals.

The research will assess whether there are differences between groups of individuals with CF across the country that cannot be explained by genetic variations and socio-demographic parameters and explore the possible reasons for these differences. For example, the detection of a group of individuals with CF identified with more frequent complications – such as an increased risk for pulmonary exacerbations and lower FEV1 – than in other parts of the country could suggest the presence of a specific environmental risk factor such as high levels of exposure to air pollution.

Using the UK Cystic Fibrosis Registry

The study will use Registry data for the analyses. The Registry includes a range of information on clinical complications for a large number of individuals over a period of more than 20 years. For example, FEV1 data from the Registry will allow for an examination of the effect on lung function in the longer term and the effect of long-term exposure from living in highly polluted environments.

With 99% of individuals with CF in the UK on the database, and 9,432 out of 10,400 people listed with a valid postcode, the Registry offers a unique resource for small-area studies, and will help the SAHSU team to complete the research with great accuracy. Using detailed geographical information is essential in such studies to attribute a good approximation of the actual exposures experienced by individuals. This research nicely complements other projects already funded by the Trust such as CF EpiNet (read more about the CF EpiNet study in the Spring 2017 issue of CF Life).

What impact could the study have on people with CF?

A better understanding of the impact of external and environmental factors could potentially have an immediate influence on the quality of life of individuals with CF by informing them about specific risk factors and therefore preventing complications and exacerbations.

By identifying which environmental factors might have an impact on individuals with CF, and in what ways, the study could help inform recommendations for people with CF in the future about where they live or visit and what they can do to keep themselves as healthy as possible.

Identifying when and where risk increases due to air pollution or other risk factors could also inform health management. In the future a system could be put in place where a forecast or a sudden measured rise in pollution could send an alert to individuals with CF in areas affected so that precautions can be taken.

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