
Cystic fibrosis is a genetic and autosomal recessive disorder that affects various body systems. It is caused by a mutation in the CFTR gene, which is responsible for the production of a protein that acts as a chlorine ion transmitter between specific cells and extracellular fluid. The balance of ions and water in the cells' mucosa is disrupted in patients, leading to inflammation and infection.
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 cystic fibrosis 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.
Several studies have found a correlation between exposure to air pollution and increased morbidity in individuals with cystic fibrosis. For example, a study by Goss et al. found a significant relationship between the amount of airborne fine particles of soot from sources such as power plants and motor vehicles and the number of CF-related lung infections requiring special medical care. Another study by Psoter et al. found that exposure to fine particulate matter was associated with an increased risk of initial Pseudomonas aeruginosa acquisition in young US children with cystic fibrosis.
In addition to air pollution, 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. For instance, a study by Collaco et al. showed that lower income is linked to a higher CF mortality rate, and a study by Collaco et al. found that secondhand smoke exposure corresponds to lower FEV1 in CF patients.
Overall, the available evidence suggests that exposure to air pollution and other environmental factors can have adverse effects on individuals with cystic fibrosis, leading to increased morbidity and mortality.
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Effect of air pollution on cystic fibrosis | Cystic fibrosis (CF) is a lethal and widespread autosomal recessive disorder affecting over 80,000 people worldwide. It is caused by mutations of the CFTR gene, which encodes an epithelial anion channel. Although CF is genetically determined, the course of the disease might also depend on multiple other factors. Air pollution is one environmental factor suspected to modulate the disease severity and influence the lung phenotype of CF patients. |
Effect of outdoor air pollution on cystic fibrosis | Outdoor air pollutants are numerous (several hundreds to several thousands of chemical species), for most of them at trace levels (from few ppb—parts per billion, to ppm—parts per million), and are gaseous (nitrogen dioxide—NO2, carbon dioxide—CO2, methane—CH4, etc.) but also under particular phases: these atmospheric particles, or aerosols—also referred to as particulate matter—are a suspension of liquid or solid in the gas phase and are categorized according to their size—coarse particles (PM10), fine particles (PM2.5) and ultrafine particles (PM0.1). |
Effect of indoor air pollution on cystic fibrosis | Exposure to higher levels of outdoor air pollution is associated with worse lung function and greater rates of pulmonary exacerbations in CF, but limited data on exposure to indoor air pollution exist. |
What You'll Learn
Air pollution and CF
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.
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.
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.
Useful tools and methods for CF environmental health studies
"Geocoding" refers to the process of translating addresses into a location on the earth’s surface. Precise geolocation data (addresses) are not always available, and so patient location can often only be determined to the level of county or zip code.
Studies with clear associations between environmental exposures and health outcomes
Studies with clear associations between environmental exposures and health outcomes offer an opportunity to enact environmental health policies aimed at timely interventions and clinical decision-making strategies that can enhance the quality of life and longevity of individuals living with CF.
Looking towards the future
We would anticipate that there are more studies with more precise measurements of environmental exposure. Smart apps may be helpful in determining an individual’s proximity to fixed exposure sources (e.g., a coal-fired power plant).
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Environmental factors and CF
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.
Air pollution
There is a well-established link between air pollution and other environmental exposures on respiratory disorders, but significantly less is known regarding how these environmental factors impact patients living 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.
A study conducted in Belgium demonstrated that the risk of CF exacerbations may be associated with short-term increases in ozone, particulate matter with an aerodynamic diameter of 10 microns or less (PM10), and nitrogen dioxide.
Climate
Potential associations with climate have been studied with both reference to long-term exposure (over years) and short-term exposure (generally seasonal). Overall, the data suggest that living in regions with warmer temperatures is associated with lower lung function in CF, largely (but not completely) mediated by higher prevalence of specific pathogens.
Second-hand smoke
Multiple studies have demonstrated the deleterious effects of secondhand tobacco smoke on lung function and nutritional status among individuals with CF, even after accounting for socioeconomic status.
Household characteristics
While outdoor air pollution has been demonstrated to have an effect on lung function and exacerbations, many children, particularly younger ones, spend much of their time indoors. While outdoor air pollution can penetrate the indoors, combustible indoor sources (SHS, gas or wood burning stoves, fireplaces, gas furnaces) also contribute to indoor concentrations of PM2.5 and NO2. The relationship between these sources of indoor air pollution and CF has not been reported; however, exposure to these pollutants have been associated with respiratory morbidity in children with asthma.
Socioeconomic status
The most commonly used proxy for individual-level socioeconomic status in CF linked to geography is insurance status as coverage for specific therapies may differ by state or country. Several association analyses using this measure, adjusted for age, genotype and other variables, have been performed.
Geocoding
“Geocoding” refers to the process of translating addresses into a location on the earth’s surface (often latitude and longitude coordinates). Precise geolocation data (addresses) are not always available, and so patient location can often only be determined to the level of county or zip code.
Interventions
Looking towards the future, we would anticipate that there are more studies with more precise measurements of environmental exposure. Smart apps may be helpful in determining an individual’s proximity to fixed exposure sources (e.g., a coal-fired power plant).
Our questionnaire-based data suggest that exposure to sources of indoor air pollution increase morbidity in both the pediatric and adult cystic fibrosis populations. Future studies with quantitative indoor air quality assessments are needed.
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Geographic factors and CF
Geographic factors play a significant role in the prevalence and exacerbation of cystic fibrosis (CF) symptoms. Here are four to six paragraphs on the topic:
The distribution and prevalence of CF are influenced by geographic factors such as air pollution, climate, and socioeconomic status. Air pollution, particularly particulate matter (PM2.5 and PM10), nitrogen dioxide (NO2), and ozone (O3), has been linked to increased risk of CF exacerbations and reduced lung function in patients. The impact of air pollution on CF is particularly notable in urban areas with high traffic density and industrial processes.
Air Pollution and CF
Air pollution, including PM2.5, PM10, NO2, and O3, has been associated with increased risk of CF exacerbations, reduced lung function, and higher prevalence of respiratory pathogens in patients. Studies have shown that exposure to air pollution, especially traffic-related pollutants, can lead to increased airway inflammation, oxidative stress, and impaired microbial clearance in CF patients. The impact of air pollution on CF is most pronounced in areas with multiple air pollutants.
Climate and CF
Climate factors, such as temperature and humidity, also play a role in CF prevalence and exacerbation. Higher mean annual ambient temperatures have been linked to lower lung function and increased prevalence of respiratory pathogens in CF patients. Warmer temperatures may contribute to the acquisition and prevalence of specific pathogens, such as Pseudomonas aeruginosa and non-tuberculous mycobacteria. However, the specific climate factors influencing CF are still under investigation.
Socioeconomic Status and CF
Socioeconomic status, including income, education, and access to healthcare, can also impact CF outcomes. Lower socioeconomic status has been associated with increased risk of respiratory pathogens, such as methicillin-resistant Staphylococcus aureus (MRSA), and poorer lung function in CF patients. Additionally, lack of access to healthcare services and specialized CF care centers can negatively affect CF outcomes.
Geographic Variation in CF Distribution
The distribution of CF varies geographically, with higher prevalence in certain regions. For example, the incidence of CF is higher in Western Asia compared to Eastern Asia. Environmental factors, such as air pollution and climate, may contribute to these geographic variations in CF prevalence.
The Impact of Geographic Factors on CF Management
Understanding the impact of geographic factors on CF is crucial for developing effective management strategies. Geographic information systems (GIS) and geocoding techniques have been used to map the distribution of CF and identify areas with higher risk of exacerbations. This information can help healthcare providers and policymakers develop targeted interventions and policies to improve CF outcomes in specific geographic regions.
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Community characteristics and CF
A community is a group of people who share a common interest, location, or identity. Communities are formed when people come together to share ideas, learn from one another, and work towards common goals. They provide a sense of belonging and enable members to deepen their understanding of a particular subject.
When it comes to Cystic Fibrosis (CF), community characteristics can play a significant role in understanding and managing the disease. Here are four to six paragraphs discussing the impact of community characteristics on CF:
Geographic Community and CF
CF is a genetic disorder that affects individuals worldwide. However, its prevalence varies across different geographic regions. For example, CF is more common in individuals of European descent, particularly those from northern Europe. The distribution of CF cases within a country can also vary based on geographic factors such as climate, altitude, and pollution levels.
In a study by Hassanzad et al. (2022), the distribution and prevalence of CF in Tehran, Iran, were investigated using a geographic information system (GIS). They found that the southern and central parts of Tehran had higher rates of CF, which were associated with lower altitudes and drier climates. Additionally, these areas had higher levels of air pollutants such as nitrogen dioxide (NO2) and particulate matter (PM10 and PM2.5).
Social Community and CF
Social communities provide a sense of fellowship and support, which can be crucial for individuals with CF and their families. Support communities, in particular, play a vital role in providing emotional and practical support to those affected by CF. For example, the Cystic Fibrosis Trust in the UK funds research and raises awareness about the condition.
Online communities, such as social media groups and forums, also provide a platform for individuals with CF and their loved ones to connect, share experiences, and offer mutual support. These communities can be especially valuable for those who may not have access to in-person support groups or who prefer the anonymity of online interactions.
Academic and Scientific Communities
Academic and scientific communities play a critical role in advancing our understanding of CF and developing new treatments. Researchers and scientists collaborate globally to study the genetic, environmental, and physiological aspects of CF. Their work informs the development of new therapies and improves clinical care for individuals with CF.
For example, the Small Area Health Statistics Unit (SAHSU) at Imperial College London is conducting research funded by the Cystic Fibrosis Trust to understand the links between CF and environmental factors. This includes studying the impact of air pollution, climate, and other geographic variables on CF progression and exacerbations.
Healthcare Community
The healthcare community, including physicians, nurses, and other healthcare professionals, plays a crucial role in the diagnosis, treatment, and management of CF. Specialized CF care centers provide comprehensive care to individuals with CF, ensuring they receive the necessary medical attention and support.
Proximity to a CF care center can impact an individual's health outcomes. Those living farther from a CF center may experience challenges in accessing specialized care and support, potentially affecting their overall health and quality of life.
Patient Community
The CF patient community comprises individuals living with CF and their families. This community plays a vital role in raising awareness, advocating for better treatments and healthcare policies, and providing peer support.
Patient communities can also contribute to research by participating in clinical trials and sharing their experiences and insights. By collaborating and sharing their stories, individuals with CF can empower one another and ensure their voices are heard in the healthcare system.
In conclusion, community characteristics have a significant impact on our understanding and management of CF. Geographic factors influence the distribution and prevalence of CF, while social communities provide essential support to those affected by the condition. Academic and scientific communities drive research and innovation, and the healthcare community plays a critical role in patient care. Additionally, the patient community advocates for better treatments and supports one another through shared experiences.
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Household characteristics and CF
Cystic Fibrosis (CF) is a genetic disorder that affects the lungs, pancreas, and other organs. It is caused by mutations in the CFTR gene, which encodes an epithelial anion channel. CF is characterised by a great phenotypic variability, which is not yet fully understood. While CF is a genetically determined disease, the course of the disease may also depend on multiple other factors.
Air pollution is one environmental factor that is suspected to modulate the disease severity and influence the lung phenotype of CF patients. This is of particular interest as pulmonary failure is the primary cause of death in CF.
The Impact of Air Pollution on CF
Outdoor air pollutants are numerous, and they can be divided into two groups: primary and secondary pollutants. Primary pollutants are emitted directly from a source, such as nitrogen oxides (NOx) or soot particles from traffic. Secondary pollutants, on the other hand, are not directly emitted but are formed when other pollutants react in the lower atmosphere, such as the production of ozone (O3) from reactions between nitrogen oxides (NOx) and volatile organic compounds (VOCs).
Several studies have investigated the impact of air pollution on CF. These studies have found associations between air pollution and increased risk of pulmonary exacerbations, primary infections, and reduced lung function in CF patients. For example, a study by Goss et al. (2004) linked exposure to particulate pollution (PM2.5 and PM10) with a decrease in lung function and an increased risk of pulmonary exacerbations and mortality in CF patients. Another study by Jassal et al. (2013) found that residential proximity to a major road was associated with an increased risk of pulmonary exacerbations.
In addition to epidemiological studies, there have also been experimental studies using animal and cellular models of CF to investigate the effects of air pollution. These studies have found that air pollution can reduce the expression and function of CFTR, which could lead to a decrease in cell permeability and airway surface liquid secretion, further exacerbating CF symptoms.
Overall, the existing research suggests that air pollution may contribute to the progression of CF and increase the risk of pulmonary exacerbations and infections. However, more research is needed to fully understand the complex interactions between genetic and environmental factors in CF.
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Frequently asked questions
Exposure to air pollution is associated with increased morbidity in individuals with cystic fibrosis. Studies have shown that exposure to higher levels of outdoor air pollution is associated with worse lung function and greater rates of pulmonary exacerbations in cystic fibrosis.
Exposure to higher levels of outdoor air pollution is associated with worse lung function and greater rates of pulmonary exacerbations in cystic fibrosis.
Exposure to secondhand smoke and forced hot air has been associated with a decrease in lung function in individuals with cystic fibrosis.
Exposure to air pollution can lead to a decrease in the quality of life of individuals with cystic fibrosis by informing them about specific risk factors and therefore preventing complications and exacerbations.
Exposure to air pollution can inform healthcare recommendations for individuals with cystic fibrosis, such as where they live or visit and what they can do to keep themselves as healthy as possible.