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.
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.
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.
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, mindfulness/meditation, 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.
14 comments
Josette Kathleen Esquivel
Posted on November 16, 2019 at 12:01 pmOMG… My baby sister was just diagnosed today as a result of radiation from Breast CANCER!! I thought she had Beat Cancer & now this outcome…..
SOMEONE PLEASE HELP HER, SHE’S A SINGLE MOTHER with 2 young kids!
Please
Please
Bert Kortegaard
Posted on November 24, 2019 at 4:35 amMy doctor says omeprazole seems to increase longevity. It’s cheap, and at least cures acid reflux. This does prevent aspirating stomach acids through the asophagus, which might make the disease progress faster otherwise.
I am 90 and was just diagnosed a few months ago but my trajectory right now is 1-3 year survival, or less.
My life has been full, but your sister has been cheated.
I hope your sister and her kids enjoy every minute they have left together.
I hope you all remember the good times.
Terry Jackson
Posted on March 1, 2020 at 11:39 pmFor Burt: I’m seeing my Pulmonologist tomorrow so was searching around the web, and whose name should I see but my old office mate from 50some years ago at LBL. So if you’d like to connect again give me a shout, Terry
PF NOW
Posted on November 24, 2019 at 8:03 amWe are so sorry to hear of your sister’s diagnosis and everything she has gone through. There is no cure for pulmonary fibrosis yet but there are several things she can do to support her lungs. Please visit our website:
https://pulmonaryfibrosisnow.org/ where you can find information about natural and supplemental therapies for treating pulmonary fibrosis. You can learn about nutrition, breathing exercises, enzyme supplements and other strategies to cope with this diagnosis. Please let us know if you need any other information. We are here to support you.
linda
Posted on March 31, 2020 at 12:52 pmI would like to know the age limit for a transplant of the lung, for PF
PF NOW
Posted on April 2, 2020 at 3:43 amAs per the general guidelines for recipient selection, an upper age limit has not been established. Age is considered along with the patient’s other medical conditions, overall health status and expected outcome of the transplant. The number of lung transplant recipients older than 65 has increased annually in the US, this age group accounted for 33% of all lung transplant recipients in 2017. Adults over age 75 are unlikely to be candidates.
DEBORAH MCMILLAN
Posted on August 12, 2020 at 2:11 amI was diagnosed with “lung scarring” in 2012. I am 68 years old. In 2017 I developed breast cancer and hat a malignant tumour removed with one month of daily radiation. Within 6 months I collapsed with Organizing pneumonia and nearly died. This pneumonia is autoimmune based and stays in the body flaring from time to time. Now on my 4th pulmonary specialist, it has been diagnosed as advancing IPF. I have many comorbities as a result, I believe, I have a very damaged body. So far, I have lived 8 years with IPF plus varying complications and plan to keep on for as long as possible. One specialist told me that I should be dead by now. News to me! Quality of life is everything to me. I keep mentally active and love to help others. I don’t want a wasted life.
PF NOW
Posted on August 14, 2020 at 7:56 pmThank you Deborah for sharing your experience with us. Your story gives people hope and shows that with a positive outlook, you can have the best quality of life possible despite the so called “odds”. We really appreciate your sentiment of wanting to help others and making your life meaningful. Please reach out if we can be of support in any way.
Pam Stevens
Posted on December 11, 2021 at 9:32 amI was diagnosed in July 21 with IPF. We have a holiday booked at Warner’s carried over from last year, should I be going, whilst not wanting to spoil it for the rest of the family the Omicron COVID variant is worrying me although I am in good health at the present. Thank you.
PF NOW
Posted on January 11, 2022 at 4:19 pmHello Pam, I’m sorry we missed responding to your message earlier. Did you go on your vacation? Omicron is definitely more transmissible as compared to the delta variant and it is important to continue to protect yourself and minimize exposure. Glad to hear you are in good health.
Dreama
Posted on February 28, 2022 at 1:53 pmI went to the emergency room in 2016 for abdominal pain and they did a CT Scan and when the doctor came back in the room she said we didn’t find anything with your stomach but did you know that you had fibrosis and I said the disease? Lol so after that I knew that they would send the report to my primary care doctor but then when I did see my primary he never said anything so I didn’t either and I don’t know why it’s not like it was going to go away so it wasn’t till 3 years later when my legs kept swelling up and I couldn’t walk very far at all and I was so out of breath that my primary care doctor said anything about the fibrosis and sent me for X-Ray, then to the lung doctor when everything was said and done the final analysis is I have fibrosis, emphysema, Gerd and severe sleep apnea. My Pulmonary Lung doctor told me last year that if I didn’t quit smoking and use my sleep apnea machine that I would be on oxygen in a year and I would be dead within 5years but here I am and I haven’t been able to quit smoking yet or use that machine so far I am very claustrophobic and I have been so stressed out plus my mother lost her husband 4years ago and was then diagnosed with dementia and she has gotten alot worse in the last year so there is always family drama! I wanted to say how sorry I am to Josette about her baby sister! I have 2 younger sisters so I understand how hard that would be especially with young children, I am going to be 62 in April so I am definitely filing for my social security early because I don’t know how long I have although I have already been living with IPF for like 6years and everything I have ever read says 3 to 5 years! Also my hat is off to Bert 90 years young and still going IPF and all and to Deborah she sounds like she has been thru it already with the cancer and now the IPF which really sucks because you can beat cancer but there is really no good diagnosis with IPF so far anyway but I have hope that maybe that will change and we will beat the odds!! Thanks everybody for listening to my really long message and please reach out anytime I’m usually up all hours of the day and night!!
Santos
Posted on August 8, 2022 at 10:24 pmMy husband was diagnosed with pulmonary fibrosis on 3 /2020 and at this point and time he is with 10 litters of oxigen and is starting to have coughing every time he walks or shower he has his fingers and toes rounded he has hart failure and fluid retention I have no idea at what stage or how severe this is but I’m worried because I try to buy life insurance and is either high or denied and have no idea how much life time he has. I wish I could have at least an expected time to be little prepaired thank you any information will help.
Dee
Posted on January 16, 2023 at 6:59 pmMy husband has had IPF for 10 years. Recently his symptoms have become much worse. It’s like he fell off a cliff! He’s been on oxygen since May and his breathing and activity levels have gotten considerably worse. He’s a trooper and does as much as he can each day. I’m going crazy trying to find out how much longer he’s going to be with us. Is it going to be a few years or not even a few months? Are there any signs that would suggest a timeframe?
Herman Holcombe
Posted on February 27, 2023 at 10:35 pmMy Mother was diagnosed 9 years ago and just turned 86. She has done quite well considering the diagnosis. She has been struggling a bit more in the past few months, probably because she did have a bout with COVID about 6 months ago. She had the vaccine and boosters, but still, it took a toll on her. She’s a tough lady, mother of 12, Grandmother or 29, so she is well taken care of. Just wanted to offer hope to anyone dealing with this diagnosis. Mom went on and a stayed on the oxygen, doing what the doctors told her, of course drugs, etc, her least favorite being the prednisone, but she has and still does follow doctors orders and has made the most of a difficult situation.