When you are diagnosed with pulmonary fibrosis, it is normal to have many questions about what life is like living with this disease. Below are expert answers to some common questions from patients about living with PF. Be sure to learn about the basics of PF, how it is treated, how to live well and where to get support. If you have a question that is not answered, call our free Lung HelpLine at 1-800-LUNGUSA (1-800-586-4872).
- Why did I get pulmonary fibrosis?
If you are diagnosed with idiopathic pulmonary fibrosis (IPF), physicians do not know what caused you to get the disease. Doctors try and rule out risk factors like hazardous chemicals in the workplace, exposure to certain medication, medical procedures and family history. There are also people who have other diseases such as rheumatoid arthritis or lupus that can lead to pulmonary fibrosis. Learn more.
- Is there a difference between pulmonary fibrosis and idiopathic pulmonary fibrosis (IPF)?
"Pulmonary fibrosis" is a general term that can apply to scarring or stiffening of the lung tissue from a number of different reasons. Adding the term "idiopathic" means none of the known causes of PF have been found and that the patient's symptoms, physical exam and high-resolution CT (HRCT) scan finding are consistent with IPF. The other distinction is treatment. IPF has two FDA-approved medications to slow the progression of the disease. These drugs aren’t approved to treat other types of PF.
- How is pulmonary fibrosis treated?
There is no cure for pulmonary fibrosis. People with IPF may benefit from a drug that slows the progression of the disease. Oxygen therapy and pulmonary rehabilitation are key components of maintaining a good quality of life with PF. Some patients will be candidates for single or double lung transplants. Learn more.
- Does pulmonary fibrosis spread? Is pulmonary fibrosis contagious?
PF progresses. It gets worse with time but the length of time it takes to progress varies from patient to patient. The disease is usually just in the lungs but if it is the result of other diseases, the fibrosis (scarring) can be other places like in the joints. Pulmonary fibrosis is not contagious. If someone with PF is wearing a mask, it is because they want to avoid exposures that could make them sick or trigger a coughing fit.
- Is pulmonary fibrosis hereditary? Should siblings and children be evaluated?
PF can run in families. There are several genes associated with familial pulmonary fibrosis, but those genes aren’t present in everyone with a family history of the disease. There is currently no genetic test that can show who is a carrier of the disease or who is at risk of developing the disease if there is a known family history. Genetic testing is not recommended in most cases because it doesn’t change the recommended treatment or prognosis. If there is a family history of PF, everyone in the family should tell their doctor about it and keep an eye out for PF symptoms.
- What do the breathing test (pulmonary or lung function tests) numbers mean?
The numbers are usually measuring lung size or capacity and flow rates. The results of a pulmonary function test are measured in comparison to the average performance of other individuals of the same age, height and weight. Test results can be measured over time to see if your lung function is getting worse. Learn more.
- If I am coughing more or more short of breath does that mean my pulmonary fibrosis is getting worse?
The short answer is: maybe. Worsening cough or shortness of breath are symptoms that should prompt your doctor to reevaluate you. Always talk to your doctor if your symptoms get worse. It could be a cold or bronchitis or a more serious lung infection such as pneumonia. It could also mean your disease is progressing. Make sure you stay up to date on your vaccinations, try and stay as healthy as possible and talk to your doctor right away if you notice a change in your symptoms.
- Does vigorous exercise slow the progression of PF?
No. Exercise can make you less short of breath by keeping your heart, lungs and breathing muscles strong. It can also help you use oxygen more efficiently. It is important to stay active and not become sedentary. Exercise doesn’t change the fact that pulmonary fibrosis will get worse over time, but it makes a huge difference in how patients feel. Learn more.
- What should I do to get my affairs in order?
It is important for everyone (regardless of whether or not they have a health issue) to take steps to plan for serious illness or disability and end of life. This involves writing a will, completing an advance directive and durable power of attorney and making funeral or memorial arrangements. It also involves organizing any financial documents and making sure all of your important paperwork is in one place. It is also a good time to reflect on what you want out of life. A member of your palliative care team or a social worker can help you fill out important documents and help you work through any emotional issues that come up when you are life planning.
- What is the average survival time once diagnosed with pulmonary fibrosis?
A diagnosis of PF can be very scary. When you do your research, you may see average survival is between three to five years. This number is an average. There are patients who live less than three years after diagnosis, and others who live much longer. It is important to recognize that PF is a chronic condition that is going to progress and can lead to premature death. But it is also important to remember that no one can predict your individual experience. You are not a statistic.
- Does life expectancy change after a lung transplant?
Yes. Life expectancy after a lung transplant is based on the success of the transplantation. It takes into account how your body handles the transplant and the medications you need to be on after the transplant.
- I feel really depressed. Should I talk to someone?
Yes. It is not unusual for patients with PF and their families to feel depressed or anxious. You may experience feelings you wouldn’t necessarily label as depression but coping with these feelings is still difficult. Talk to your doctor about what you are feeling. Your doctor can recommend a therapist or counselor who can help you work through your emotions. Learn more.
- Where can I find support from other patients?
Support from other people facing PF is very important. You can join an online support community and attend an in-person support group. Pulmonary rehabilitation is also a great place to connect with others facing lung disease. Learn more.
- If I start on oxygen therapy, will it make my pulmonary fibrosis worse? Will I become addicted to oxygen therapy?
Using oxygen therapy will not make your PF worse and it is not an addictive drug. The oxygen may make you feel better and able to do more and you may prefer to be on it.
- What is the difference between continuous flow and pulse flow?
Pulse flow delivers oxygen when you inhale. Portable oxygen concentrators deliver this type of flow. Continuous flow delivers oxygen at a steady rate, no matter how you are breathing. Home oxygen tanks provide continuous flow. The flow you need depends on how short of breath you are and what activities you are doing. Many people find pulse flow works well during the day and continuous flow works better at night.
- Can I get a lung transplant if I am over 65?
Maybe. Every transplant center has their own specific criteria. Some centers are moving the upper age limit to 70 or 75 years. Your eligibility for a transplant depends on many factors, not only your age. Learn more.
- When should I get on a lung transplant list?
The sooner you are evaluated at a transplant center, the better you will understand the process. The transplant center will become familiar with you and able to identify when you need to get on a list. If you're evaluated, it doesn't mean you need to get a transplant, but it is good to gather information shortly after you are diagnosed. Learn more.
- What is the role of diet in living with pulmonary fibrosis?
The main role of your diet is to help you maintain an ideal weight. Being over or underweight can impact your ability to breathe. There are no specific supplements or vitamins that have been shown to make a difference for patients with PF. Ask your doctor for a referral to a registered dietitian who can help you understand your nutritional needs. Learn more.
- Should I move somewhere so I can breathe better if I have pulmonary fibrosis?
It is more difficult to breathe at higher altitudes. It is recommended that pulmonary fibrosis patients live at a lower altitude. Try and avoid areas where air quality is poor. Talk with your doctor about your concerns and consider the potential pros and cons of moving before making a decision.
- Will I need to stop working if I have pulmonary fibrosis?
You should be able to continue working until your symptoms make it too difficult. If your job is very physical, you may need to stop working sooner.
- How important is pulmonary rehabilitation in treating pulmonary fibrosis?
Pulmonary rehab will help you stay in good physical shape which will improve your quality of life. It also offers psychosocial support, which is so important to quality of life. Learn more.
- Can I travel with pulmonary fibrosis?
Yes. Flying might require the use of oxygen during the flight, and you will need to check with the airline about arrangements. Driving should pose no issues. If you use oxygen, make sure you’ve done your homework so you know where you are getting oxygen during each leg of your trip. There are more logistics involved, but many people with pulmonary fibrosis travel well with no problems. Learn more.
Page last updated: March 22, 2020