You have just been diagnosed with pulmonary fibrosis(PF), a family of more than 200 serious lung diseases characterized by scarring and thickening of the lung tissue. Undoubtedly, you’ll have many questions about this disease and how it will affect you and your family. Chief among those questions will be “How quickly will this disease progress?” and “What should I expect to happen?”

The answers to these questions are as many and varied as the estimated 200,000 people suffering from the most common form of this disease (idiopathic pulmonary fibrosis (IPF)). While every person diagnosed with PF has a unique experience, there are some broad trends among the many paths PF can take. This summary of existing knowledge should help you understand the progression of this disease and, perhaps more importantly, what you can do to improve your quality of life while living with PF.

physical exam

Factors affecting the progression of pulmonary fibrosis

Most people diagnosed with PF will experience a progression of the disease over time. In this context, “progression” means your lungs are developing more scar tissue and your symptoms are worsening. It is important to note that there is no typical rate of progression, and the rate will vary depending on a number of factors.

The cause of the disease is one such factor. There are several possible causes of PF:

  • Environmental factors such as mold or bird droppings
  • Occupational exposure to certain toxins or pollutants
  • Radiation therapy for the treatment of cancer
  • Certain drugs or medications known to affect lung function
  • Autoimmune diseases that affect joints and connective tissue like rheumatoid arthritis and scleroderma
  • Genetics or hereditary factors

In most instances, however, the cause of PF is not known, in which case the disease is referred to as idiopathic pulmonary fibrosis (IPF) which is the most common type of PF.

Other factors affecting the incidence and progression of PF include age, gender, general health, duration of exposure to any causative agents, lifestyle choices, the effectiveness of any prescribed treatment and the length of time between onset of the disease and its diagnosis.

Rate of progression and life expectancy

The rate at which PF progresses can differ significantly from one person to the next. Some people may experience mild to moderate symptoms that worsen slowly over the course of several years; whereas, others may experience “acute exacerbation” in which their symptoms worsen quickly over the course of days or weeks. Still, others may experience extended periods of stability followed by periods of more rapid disease progression.

Physician looking at radiograph

Life expectancy also varies among those suffering from PF. Different life expectancies may be associated with varying types of PF, so the proper diagnosis of the disease by a pulmonary specialist is critical to determining your prognosis. In general, however, most people with IPF rarely survive more than three to five years following their diagnosis.

While there is no known cure for PF, there are several promising research studies and clinical trials that may lead to more effective treatment of PF and possibly even a cure. There are also existing treatments that can slow the rate of progression and improve your quality of life.  These include both medicinal and non-medicinal treatments.

What to expect as pulmonary fibrosis progresses

Like any serious disease, PF will affect you physically as well as emotionally. Having been diagnosed with PF by your doctor, you are already aware of some of its symptoms. These include shortness of breath, fatigue, hacking cough, painful joints and muscles and sometimes clubbing (rounding and widening) of your fingers and toes. As the disease progresses, you may experience a worsening of some or all of these symptoms. Pulmonary fibrosis may also lead to serious complications such as pulmonary hypertension, partial heart failure, lung infections, lung cancer and ultimately, respiratory failure.

Depending on the type of PF you have, your doctor may prescribe certain FDA-approved drugs such as nintedanib or pirfenidone to slow the progression of the disease; however, these can have undesirable side effects, such as nausea, diarrhea and loss of appetite. Other drug treatments for certain types of PF may include corticosteroids (or other immunosuppressants) and antibiotics. There is some evidence that certain systemic enzymes may be helpful in treating PF as well.

Old couple sleeping together in bed man with nasal cannula

Oxygen therapy can improve your quality of life by making it easier to breathe and exercise, improving sleep patterns and reducing complications resulting from low oxygen levels in the bloodstream. In cases of acute exacerbation, patients may be placed on a mechanical ventilator.

Lung transplantation can be a viable option, but it’s available to relatively few people since the number of potential recipients far exceeds the number of donors. There are also strict eligibility requirements that may present an obstacle to your being selected as a candidate for lung transplantation. As your disease progresses and symptoms worsen, however, you are likely to move up on the list of eligible candidates, assuming you meet the other criteria.

Lifestyle changes and emotional support to improve your quality of life

There are several non-medicinal and non-invasive ways to relieve your symptoms and improve your overall quality of life as you learn to live with PF. Your doctor may recommend a program of pulmonary rehabilitation involving physical exercise, controlled breathing exercises, improved nutrition and cessation of smoking or other harmful habits.

Equally essential to improving your quality of life are family support, counseling, education and participation in PF support groups. The importance of your family’s support and connecting with others who live with PF cannot be overstated.

Hopefully, as you come to accept the reality of living with pulmonary fibrosis, you will become less focused on the rate at which your disease is progressing and more determined to lead as full and rewarding a life as possible.

Never disregard professional medical advice or delay in seeking it because of any information received from us.

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