Cystic Fibrosis and Pulmonary Fibrosis may sound similar, but they’re actually quite different. Both conditions include the term “fibrosis,” which means thickening or scarring tissue, and each one can affect a person’s lungs and airways, making breathing more difficult.¹
However, cystic fibrosis and pulmonary fibrosis are distinct conditions with individual causes, symptoms, and treatments.
In this guide, we’ll explore the differences between cystic fibrosis vs. pulmonary fibrosis in greater detail. Particularly how they impair airway clearance and what treatments are available to help patients find relief from symptoms and improve their quality of life.
What Is Cystic Fibrosis (CF)?
CF is a genetic disease that affects a person’s lungs, pancreas, and other organs. It’s caused by a mutation in the CF transmembrane conductance regulator (CFTR) gene, which produces a protein that regulates the flow of salt (i.e., chloride) and water in and out of cells. However, when the CFTR gene is mutated, it can lead to thick, sticky mucus buildup.²
The excess mucus can then block airways, making it difficult to breathe, and can also interfere with the function of the pancreas, leading to digestive issues.³ CF is an inherited condition, meaning it is passed down from parents to their children. Approximately 1 in 30 Americans is a carrier of the mutated gene.4
What Is Pulmonary Fibrosis (PF)?
Pulmonary Fibrosis is a lung disease that causes thickening and scarring of the lung tissue and air sacs.5
The condition is caused by the formation of scar tissue in the lungs, which can be triggered by various factors, including smoking, occupational exposure, environmental toxins, autoimmune diseases, and reactions to certain medications or treatments (i.e., radiation).6
Although external factors can cause PF, it can also be inherited.7
Symptoms of Cystic Fibrosis vs. Pulmonary Fibrosis
For the purpose of this article, we’ll focus on symptoms that directly impact the lungs and airways.
Symptoms of CF, for example, can range from persistent coughing and wheezing to poor weight gain and digestive issues (as noted earlier). However, because it causes mucus buildup in the lungs, it leaves patients susceptible to the spread of bacteria and germs; therefore, other respiratory symptoms may include:
- Shortness of breath
- Inflammation
- Infection8
Common symptoms of PF include:
- Shortness of breath
- Dry cough
- Fatigue
- Fast, shallow breathing9
How Are CF and PF Diagnosed?
Cystic fibrosis is typically diagnosed through a combination of family history, newborn screening, and specialized tests.10
A sweat test is a common diagnostic tool for CF, which measures the amount of chloride in a person’s sweat, as CF patients will have higher levels than those without the condition. Genetic testing can also be used to confirm a diagnosis by identifying mutations in the CFTR gene.
To diagnose pulmonary fibrosis, your healthcare provider may perform a physical exam, order imaging tests, or conduct pulmonary function tests to measure how well your lungs work.11 For example, lung scarring on a CT scan or X-ray helps rule out other chronic lung conditions, while a High-Resolution CT (HRCT) scan may provide a more detailed image for accurate diagnosis.12
In some cases, a lung biopsy may be necessary to confirm a PF diagnosis.13
If you or a loved one has been diagnosed with cystic fibrosis or pulmonary fibrosis, it’s important to work closely with your healthcare provider to develop a treatment plan that meets your individual needs.
Treatment Options for CF and PF
Currently, there is no cure for CF or PF; however, several treatment options are available to help manage symptoms and slow the progression of each disease.
Treatment for CF
Individuals with cystic fibrosis can manage their condition daily by following a comprehensive treatment plan that includes airway clearance techniques, taking prescribed medications,14 and maintaining a healthy diet and exercise routine.15
It’s also important for individuals with cystic fibrosis to avoid exposure to respiratory infections and other environmental factors that can worsen symptoms.
Regular check-ups with a CF care team are also crucial for monitoring the progression of the disease and adjusting treatment plans as needed.16
Treatment for PF
Treatment for scarred lungs may include a combination of medications and oxygen therapy to help improve breathing.17 In some cases, pulmonary rehabilitation programs can help patients improve their lung function and overall quality of life. In severe cases, lung transplantation may be considered another treatment option.18
It’s important to work closely with your healthcare provider to determine the best course of treatment for your individual needs.
Lifestyle Changes and Coping Strategies
In addition to medical treatments, lifestyle changes and coping strategies can help patients with pulmonary fibrosis manage their symptoms.
These may include quitting smoking, avoiding exposure to environmental irritants such as dust and pollution, and exercising regularly to improve lung function.
Patients may also benefit from joining support groups or seeking counseling to help them cope with the emotional challenges of living with a chronic illness. Finally, patients and caregivers must work together to develop a comprehensive treatment plan that addresses medical and lifestyle factors.
Support and Resources for Lung Diseases
Resources are available to help patients and caregivers navigate CF and PF. Support groups—like the American Lung Association’s Better Breathers Club—can provide a sense of community and connection with others who understand what living with your condition is like.
Many organizations also offer educational resources, such as webinars and informational materials, to help patients and caregivers better understand the condition and its treatment options.
Additionally, healthcare providers can offer guidance and referrals to other specialists. By taking advantage of these resources, patients and caregivers can feel more empowered and better equipped to manage the challenges of cystic fibrosis or pulmonary fibrosis.
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Resources
[1] American Lung Association. “7 Things Everyone Should Know about Pulmonary Fibrosis.” Retrieved from https://www.lung.org/blog/7-things-know-pulmonary-fibrosis
[2] Cystic Fibrosis Foundation. “About Cystic Fibrosis.” Retrieved from https://www.cff.org/intro-cf/about-cystic-fibrosis
[3] Cystic Fibrosis Foundation. ” The Digestive Tract.” Retrieved from https://www.cff.org/managing-cf/digestive-tract
[4] American Lung Association. “Learn about Cystic Fibrosis.” Retrieved from https://www.lung.org/lung-health-diseases/lung-disease-lookup/cystic-fibrosis/learn-about-cystic-fibrosis
[5] American Lung Association. “7 Things Everyone Should Know about Pulmonary Fibrosis.” Retrieved from https://www.lung.org/blog/7-things-know-pulmonary-fibrosis
[6] Pulmonary™ Fibrosis Foundation. “About Pulmonary Fibrosis: Causes.” Retrieved from https://www.pulmonaryfibrosis.org/understanding-pff/about-pulmonary-fibrosis/diagnosis
[7] American Lung Association. “7 Things Everyone Should Know about Pulmonary Fibrosis.” Retrieved from https://www.lung.org/blog/7-things-know-pulmonary-fibrosis
[8] Cystic Fibrosis Foundation. “About Cystic Fibrosis.” Retrieved from https://www.cff.org/intro-cf/about-cystic-fibrosis
[9] American Lung Association. “Symptoms of Pulmonary Fibrosis.” Retrieved from https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/introduction/symptoms
[10] National Heart, Lung, and Blood Institute. ” Cystic Fibrosis: Diagnosis.” Retrieved from https://www.nhlbi.nih.gov/health/cystic-fibrosis/diagnosis
[11] Cleveland Clinic. “Pulmonary Fibrosis.” Retrieved from https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis
[12] Pulmonary™ Fibrosis Foundation. “About Pulmonary Fibrosis: Diagnosis.” Retrieved from https://www.pulmonaryfibrosis.org/understanding-pff/about-pulmonary-fibrosis/diagnosis
[13] Cleveland Clinic. “Pulmonary Fibrosis.” Retrieved from https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis
[14] National Heart, Lung, and Blood Institute. “Cystic Fibrosis: Treatment.” Retrieved from https://www.nhlbi.nih.gov/health/cystic-fibrosis/treatment
[15] Cystic Fibrosis Foundation. “Why Fitness Matters.” Retrieved from https://www.cff.org/managing-cf/why-fitness-matters
[16] Cystic Fibrosis Foundation. “Your CF Care Team.” Retrieved from https://www.cff.org/managing-cf/your-cf-care-team
[17] American Lung Associaiton. “How Is Pulmonary Fibrosis Treated.” Retrieved from https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/how-is-pulmonary-fibrosis-treated
[18] Cleveland Clinic. “Pulmonary Fibrosis.” Retrieved from https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis
