Saturday, October 5, 2013

“The Internet Said I Have 3 Years to Live”

I’ve been having a hard time writing this post about “prognosis”, or how long you might expect to live with Pulmonary Fibrosis. It’s a tough subject. After struggling for about 30 minutes, I realized two things:

Number one – my difficulty writing about prognosis is a pebble in comparison to your mountain of difficulty living with Pulmonary Fibrosis. So I’m going to stop complaining right now.

And number two – it’s difficult to write about prognosis because…. Well, because prognosis is a lie. A myth. Let me explain.

I first need to tell you that all that stuff on the internet about a short survival time in Pulmonary Fibrosis comes from studies of patients with one specific form of Pulmonary Fibrosis called Idiopathic Pulmonary Fibrosis (abbreviated IPF). So, if you don’t have IPF, none of that stuff applies to you – your prognosis might be substantially better than the prognosis in IPF. If you haven’t been to a Pulmonary Fibrosis center (also called an ILD center) to sort out exactly which kind of Pulmonary Fibrosis you have, then go get yourself an appointment. Check out the Pulmonary Fibrosis Foundation's website for a list of ILD centers.
Ok, if you’ve been to an ILD center and the team there told you that do have IPF, then we can start talking about how prognosis in IPF is just a myth. Now, I don’t mean that IPF isn’t a serious disease – it IS quite serious and people do die from Pulmonary Fibrosis. But the idea that a doctor can tell you how many months or years you have left before your die is simply ridiculous. How can they know exactly when you are going to die? That would be magic.

You are probably thinking “Well, I don't really expect my doctor to know exactly how long I am going to live – I agree that is kind of silly -- but I would like an idea of what to expect.” And I agree with you – that is very reasonable. But, giving you a single landmark in time is simply misleading. Here’s why:

Many of you have seen something like this on the internet: “Patients with IPF live about 3 to 5 years from the time of their diagnosis.” Most people interpret this to mean: “I have 3 to 5 years left before I die.” But, that’s a false interpretation. It doesn’t mean that everyone with IPF lives 3 to 5 years. It means that “on average” people with IPF live 3 to 5 years. 

Are you average? No, you’re not. No one is average. Everyone is either better than the average or they are worse than the average. Saying “you have about 3 years left” is like saying that people are 5’ 7’’ tall (the average adult height in the U.S.). Kind of unhelpful and ridiculous.

There is a lot of variation in how long people live with IPF – in fact, in some studies 1 out of 5 people live more than 10 years! Some doctors now think that there may be a very long “silent” period where there is scarring (fibrosis) in the lungs without any symptoms at all – and that the “3 to 5 years” really only begins when people become quite ill – so someone diagnosed in a very early stage might remain well for many years – we don’t really know how many years – before the disease begins to progress. One study even showed that “on average” people with IPF lived 7 years if you started the clock at the time that their symptoms began (like when they first developed short of breath with activities and exercise). That means that half of those people lived more than 7 years!

There’s also a whole other level of complexity here. Doctors can look at how sick you are today and they can use that information to refine their prognosis. Some of the things that predict a better prognosis are the need for less oxygen (or none at all), having better lung function test results (tests like the “FVC” and “DLCO”), walking farther during a walking test (usually a six-minute walk test), and how much shortness of breath you have during your day-to-day activities.

On top of all of this, the studies that found the average survival was 3 years were done more than 10 to 15 years ago. Nowadays, the survival seems to be better – at least “on average.”

And none of these numbers account for people who get a lung transplant – that’s a game changer. Lung transplantation is really to only treatment for IPF that seems to prolong life – again, at least “on average”.

Let me drive the point home by showing you an example of how I talk about prognosis with my patients. Realize that this is one scenario tailored to an individual (and hypothetical) patient. I would change the range of numbers used depending on my patient’s individual characteristics.

“No one can tell you how long you are going to live. You may have read on the internet that the average survival with IPF is 3 years, but that means that half of patients with IPF actually live longer than that – some live a lot longer. But, it also means that the other half don’t even make it 3 years. Also, I don’t know if the average is really that helpful, since no one is average. I find it more helpful to think about the range of possibilities. Is it possible that you are going to live longer than 10 years? Without a lung transplant, the truth is that that does seem unlikely to me. I am worried that you will become much sicker before 10 years were up. Could you live 5 years if you don’t undergo lung transplantation? Yeah, I could see that happening, but you might be quite ill 5 years from now, and while it is possible, it is kind of a stretch. I think the most likely thing is that before 5 years are up, your disease will have already progressed to the point where you need oxygen all the time – perhaps up to 10 or 15 liters of oxygen through a face mask, you are very limited in what you can do – each day will be a struggle, and sometime in the next 5 years, your lungs are likely to fail, which is a life-threatening event. I don’t know if all of those things are going to happen in 5 years, 4 years, 3 years, 2 years or even in the next year, but based on how you are doing today, it is unlikely that any of those things are going to happen in the next 6 to 12 months. So, let’s work together on figuring out how to prevent things from getting worse, and let’s get you as healthy as possible so that when your lungs are sicker, you don’t necessarily have to be so limited by your lungs. We can look into some of the clinical trials of new treatments for IPF – maybe one will work for you. And if lung transplantation is an option, let’s start looking into that now too.”

So, don’t let ANYONE tell you that they know how long you are going to live. You are alive today. You are living with pulmonary fibrosis. Living. Treasure that.


197 comments :

  1. Doctor, thanks so much for this blog! When I was diagnosed in 2006 w/scarring and told not to worry I did nothing, no research into 'scarring'. Until my biopsy in 2013 I was always told not to worry about the scarring from a Pulmonary Doctor I had seen. When my current Dr. diagnosed me with IPF I went searching and found that 3-5 years. I spoke to my current Pulmonary doctor about that and he said what you did, he said no one knows when you will die, no one has that crystal ball and he added especially him. So I don't have that doom and gloom because of that comment, the blogs on a couple sites and now with your blog. I live for today and who knows I might get run over by a bus instead one never knows. I believe part of my "I" of the PF is genetic but unproven at this time.
    This month I'm going to Yale's COE. I saw an article where they are involved in a study that may aid in the prognosis?
    Thanks so much for your posts they are so helpful !
    Laura

    ReplyDelete
    Replies
    1. Hi Laura my, I first would like to say that I'm deeply touched by the positive ness in your reply to your diagnosis and 2ndly how much I wish my mom was surrounded by people like you. My mom was diagnosed with ipf in 2013,she had been coping fairly fine up until June this year when she was admitted at hospital for 15days.she came out with an oxygen tank and has to be on the machine for 12 hours daily,she has reacted well and has since recorved but no matter how positive i try to be, another part of me just says you can't really prolong the inevitable. How do i help my mother? What do i do for, what have you found to be bedt in your case? Thank you. If you do not mind you can reply to me on my email qvmbatha@gmail.com

      Delete
  2. Bravo! Some of the frustration I have had are addressed in this post. First of all, many people think that PF and IPF are synonymous. They, or their doctors (not PF specialists) casually throw out the IPF dx because "they don't know how or why the fibrosis occurred". I've been trying to increase awareness that IPF is a dx of exclusion (excluding all known/identifiable causes) BUT ALSO there are definitive inclusions that must be met such as UIP pattern on HRCT and that if the UIP pattern is not seen on HRCT, then confirmation by biopsy.

    I have familiarized myself with the 2011 Guidelines and understand why the biopsy was necessary. I don't want the IPF diagnosis without a very high degree of confidence in the diagnosis because I also understood from my research that the 3-5 years was largely based on IPF, not the general PF population. I also realized that those stats came from a much earlier time and the shape of the normal curve has probably changed.

    I also wondered how much of an effect the old standby treatment of prednisone and immune-suppressants on those earlier stats, especially for IPF. Since these are no longer routinely prescribed for IPF, I personally feel that my prognosis is better than it would have been 10 years ago -- kind of makes me glad I wasn't diagnosed in 2008 if I would have been put on that regimen.

    ReplyDelete
    Replies
    1. What medication(s) is/are routinely prescribed for IPF now?

      Delete
    2. Thanks for your question. While there are no medications that are FDA-approved to treat IPF in the U.S., this is an exciting time in the IPF world because 3 large clinical trials of drugs to treat IPF will be completed shortly. We should have answers about these drugs in the next few months!

      Delete
    3. Has any of these drugs come out on the market? Do you know any answers yet?

      Thank you

      Delete
    4. There are still no drugs approved by the FDA to treat IPF. Hopefully we will be hearing something this Spring.

      Delete
    5. Hi Dr.David, which website i can to get info about the new IPF drugs treatment if it is coming out soon on the market this year! Is the FDA has web-bag?Thanks

      Delete
    6. Since the FDA has not approved any drugs for the treatment of IPF, there unfortunately is no online source of information about which drugs might be coming on the market. You can search on clinicaltrials.gov to find clinical trials of IPF and learn more about the drugs that are being studied.

      Delete
    7. Hasn't the FDA now approved 2 drugs Ofev (nintedanib) and Esbriet (pirfenidone)? I know this is old but I wanted to update this thread.

      Delete
    8. Correct! Thanks for this. Here are links to updated information:
      http://www.pfdoc.org/2014/10/the-fda-approved-pirfenidone-and.html
      http://www.pfdoc.org/2014/05/clinical-trial-results-for-pirfenidone.html

      Delete
    9. I am so glad I found this Blog and thank you for taking the time to write it Dr. Lederer.
      My Mum had ILD and passed away in 2015 just 2 years after diagnosis, she was 74.
      In November I was diagnosed with Sarcoidosis and ILD and ironically now seeing the same specialist as my Mum was. He doesn't seem concerned and said we are monitoring things for now, I have another appointment with him on 27th April.
      Obviously my Mum's suffering and passing is very fresh in my mind. I am 51, never smoked, I have ILD with Fibrotic Changes.
      I have a few questions I would be extremely grateful if you could answer.
      Is there a family connection for this disease?
      Fibrotic Changes, is this early onset of IPF?
      I read that Sarcoidosis just leaves and I will be fine as long as it hasn't left permanent scarring, does the fibrotic changes mean it's scarring?
      Thank you again.
      Alison

      Delete
    10. Hi Alison. In general, the word "fibrosis" means "scar tissue," although sometimes doctors might use the term "fibrosis" in a more casual way for "interstitial lung disease." I suggest that you ask your doctor exactly what the "fibrotic changes" in your lungs are.

      IPF and sarcoidosis are two different diseases. If you have sarcoidosis with "fibrotic changes," you probably do not have IPF.

      Sarcoidosis, IPF, and other ILDs do have family connections. While most people with one of these disease do not have anyone else in the family with the disease, some do.

      Best wishes.

      Dave

      Delete
  3. What a great, and wise, look at the curse of too much knowledge that most of us (IPF patients) face. Ultimately most of us turn to Dr. Google as our primary research tool but your postings are a breath of fresh air (no pun intended) for us. Thank you Dr. Lederer.

    ReplyDelete
    Replies
    1. Thanks for your words, Bill! Keep up the great work on spreading the message about IPF.

      Delete
    2. Hi my name is Marisa my father was diagnosed with ipf over 15 years ago and is still going strong he is definitely not average so there is hope! He rides his bike three miles a day and golfs four days a week his main struggles are his pride and not being able to carry heavy things and help my mom walk up hills or walk up stairs without being out of breath. Currently he is not on any oxygen and just recently started on ofev. Has anyone had any success with ofev or any experience personally? He seems to be having some depression since starting it but I am not sure if it's because he went online to research the medicine and disease and read life expectancy and now is down in the dumps. I'm afraid his attitude will cause him to deteriorate very quickly. Also what are some information on lung transplants are they seen as a good form of treatment? Thanks so much!

      Delete
    3. Hi Marisa. It is great to hear that your father is doing well after 15 years with IPF. Ofev is certainly a suitable treatment for people living with IPF. I haven't seen any of my patients develop depression from Ofev. Lung transplantation is a treatment option for some people with IPF. You can find some information here:
      http://www.pulmonaryfibrosis.org/life-with-pf/pulmonary-fibrosis-treatment-options
      http://www.pfdoc.org/2013/10/the-honest-truth-about-lung.html

      Delete
  4. My mother had it. She lived 5 years with it.

    ReplyDelete
  5. We don't come into this world with and expiry date!! Well said Dr. David.
    Back in Aug you detailed IPF (interstitial pulmonary fibrosis) and PF (pulmonary fibrosis), as well as IIP(idiopathic interstitial pneumonia); can you please clarify the difference between pneumonia's that scar and IPF? I have fibrotic NSIP(non specific interstitial pneumonia) confirmed by VATS(video assisted thorascic surgery) lung biopsy.
    Also a terminology blog would be great.
    So many sites use acronyms, that once you are diagnosed you have to become an expert to read most posts!! I too now discuss in that manner, but am still unclear between the pneumonia and the PF's.
    thanxs Melbourne Australia

    ReplyDelete
    Replies
    1. Thanks for your questions. I will indeed discuss the different types of "interstitial lung disease" or PF in an upcoming post.

      Delete
    2. My sister was diagnosed with ILD no scarring and her pulmonary doctor told her it will turn into fibrosis in 5 years and she may have 5 years to live after she develops fibrosis. Will ILD definately turn into fibrosis? She is waking up thinking she may have 20 years to live.

      Delete
    3. Sorry typo 10 yrs to live

      Delete
    4. There are dozens of different kinds of ILD (or "interstitial lung disease"). Some people with ILD have scarring in their lungs (pulmonary fibrosis). Others don't Only your sister's doctor can tell her whether she is at risk to develop pulmonary fibrosis. I am sorry I can't be more helpful than that. You can also encourage your sister to search for a medical center that specializes in ILD and PF at http://pulmonaryfibrosis.org/life-with-pf/find-medical-care. She can also contact the PFF at pcc@pulmonaryfibrosis.org or by calling 844.Talk.PFF

      Delete
  6. I am so glad to find this blog, Dr. Lederer. Hope all is well.

    ReplyDelete
  7. This is a wonderful article! I am sharing it on my Breathe Support facebook page as well as with all 5 of my Breathe Support groups (for those affected by PF). My mom was diagnosed with IPF in 2003 and given 2 years - at best - to live, at that time, given how sick she was. She actually had 6 great years after that! We learned to not look at averages. I now work with the PF community quite a bit and I have met people who have been "living with IPF" and other forms of PF for 5, 10, 15 and even 20 years. The one thing I have noticed with PF (of any kind) is that is so completely different in everyone. Thank you for the wonderful information you post.

    ReplyDelete
    Replies
    1. Hi Taleena. Thanks for sharing my blog on the Breathe Support facebook page. Very happy that you found this information helpful.

      Delete
  8. I was diagnosed in June of 2008. At the time the average was 1-3 years. I think at the time PF was really only being found in older people and late in the stages of the disease. I was diagnosed early compared to many people. I am past the 5 year mark. Things are getting worse but I am still here and I am currently on a lung transplant list. Gailinpa

    ReplyDelete
  9. There is some research being done with DNA- Gnome where they think they will be able to tell if your IPF will progress quickly or slowly. This is exciting because it will help drs know how to track a patients IPF progression, what signs they need to really be watching for and when to have their patients evaluated for transplant. Sometimes people seem to be doing ok then all of sudden they get very sick quickly and there is not enough time to have the evaluation. Gailinpa

    ReplyDelete
  10. Dr Lederer I am trying to understand my disease and how my body works. I'm over 60 and do not remember much of what I learned in school. I need to keep things simple. Can you tell me if these facts are correct?

    The right side of the heart pumps blood though your lungs where the red blood cell pick up oxygen 02) molecules take it to the left side of the heart where it is pumped out to the rest of the body. At the same time carbon dioxide (CO2) in the red blood cells goes into our lungs to be expelled.

    It is not the absents of oxygen that tell the brain there is a problem but and over abundance of carbon dioxide remaining in your blood.

    Does the exchange of o2 and co2 happen equally and at the same time to and from the same red blood cell? Are there move than one kind of red blood cell? If the exchange is one to one how can the balance get so out of whack?

    I am trying to understand this......I suffer these episodes where my o2 level gets low and then plummets to where I think I am suffocating. I feel panicked and my heart races. I start trying to breath deeply using pursed lip breathing. This brings my o2 levels up but the sensation of suffocating last for quite a while. No matter how many times it happens it is very so scary. I have never felt so close to dying. As my disease progresses these episodes are happing more often so I am looking for the best way to deal with them. Thanks for any input any of you can provide. Gailinpa

    ReplyDelete
    Replies
    1. Hi Gailinpa.

      I'm very sorry to hear about the way you are suffering with PF. It sounds like you might not be using enough oxygen. Some people with PF need up to 10 to 15 liters of oxygen when they are active. These high oxygen flow rates often need to be delivered through a face mask instead of a nasal cannula. Please talk to your pulmonologist about assessing your oxygen requirements. Hopefully your oxygen supplier can provide you with enough oxygen at home -- Some people need a 10 liter concentrator or even two concentrators connected together to achieve high enough oxygen flows. You may find that your breathing is easier with more oxygen, but please do discuss this with your doctor before making any changes to your oxygen use at home.

      Your second paragraph about the right and left sides of the heart is correct -- good memory! Your brain is indeed more sensitive to an overabundance of carbon dioxide than to low oxygen levels. However, once the oxygen saturation is less than 90%, the brain senses this and many people have great difficulty breathing with low oxygen levels.

      The transfer of oxygen from the air sacs of the lung into the blood stream is much more difficult than the transfer of carbon dioxide from the blood stream into the air sacs of the lung. This difference is due to differences in the chemical properties (solubility) of these two gases.

      Please let me know how things go after you talk to your pulmonologist. I'd like to hear how things turn out.

      David Lederer

      Delete
    2. I do have 2 concentrators at home. I run 8 liters in my cannula for when I am sitting and have a mask set on a 10 for when I get up to move. I forget that I still have to move very slowly.. When I go to pul rehab I am on 10 liters cannula and flush open on a oxiarm. It looks like and old fashion head set a telephone operator might use. It blows the oxygen in the area of my nose and mouth. This allows me to walk on the treadmill. I developed a heart shunt due to the pul hypertension which I developed because of the IPF. If blood is moving from the right to the left that would mean the blood that has not been oxygenated gets in with the blood that has so I am actually getting less oxygen and the blood with the CO2 is not getting through my lungs to be expelled. Does that make sense? Could that be part of why I feel like I am suffocating even though my saturation numbers are fine. Of course when I am active my heart beats faster which I guess probably means more blood is escaping from the right side of my heart to the left. The adrenaline rush of the panic doesn't help either. I don't know why but understand what is happening in there brings me some kind of comfort. There is so little you can control with this disease. I guess I am one of the knowledge is power people. Thanks for all the information you are sharing with me. I truly do appreciate it. Gailinpa

      Delete
  11. Doctor I very much appreciate your taking the time from probably a very busy schedule to discuss this illness with those of us who have or are close to someone with PF. My wife is currently on the transplant list and we are told that since she is 5-2" tall, the percentages are much less that she will be called for new lungs. Is that true and why??

    ReplyDelete
    Replies
    1. It is true.

      People with Pulmonary Fibrosis have smaller-than-average sized lungs because the scar tissue makes the lungs stiffer (and scarred lungs are difficult to expand -- so the body let's them collapse down a bit). So, lung transplant surgeons need to find a donor that's about the same size or perhaps even smaller than the patient, so that the lungs "fit" into the chest at the time of lung transplantation. If someone is shorter-than-average to begin with, they will already start out with smaller-than-average lungs even before the Pulmonary Fibrosis sets in. So combining Pulmonary Fibrosis with being shorter-than-average height means that the donor has to be quite small with normal sized lungs -- and there aren't a lot of donors like that. This means that on average, the waiting time for lung transplantation will be longer for people who are shorter-than-average and who have Pulmonary Fibrosis.

      Some new studies suggest that we should try using lungs from donors who are taller than the recipient (http://www.atsjournals.org/doi/abs/10.1513/AnnalsATS.201301-008OC#.UmZ2uCQXjEE) and surgeons can use larger lungs and then "cut them down to size" to make then fit inside of the recipient's chest.

      I'll be blogging about Lung Transplantation for Pulmonary Fibrosis shortly.

      Thanks for your question!

      Delete
    2. Thanks Dr Lederer. I knew there had to be a reason and you explained it so well. Now I understand better why I was listed early (with a low LAS score) and have been on the list a while. I talked to a surgeon at my tx center, he said they are getting more successful with trimming lungs and asked if I would accept lungs that would need to be trimmed. I said yes. Gailinpa

      Delete
  12. Hi I am on the transplant list too. I am 5'1" tall. My center does trim lungs but I still have been waiting 26 months. Short lungs do seem to be hard to come by. I see a lot of short people but many seem to be teens. Adults sign up to be donors and talk to their families. I am not a parent. I don't know if the idea of having to make that decision about my child is ever thought about. It is a lot to ask of a parent during such a difficult time. Don't give up hope, the smalls may be hard to find but that does not mean they won't be found for both of us. Hang in there.

    ReplyDelete
  13. I'm 5' 1/2" and I didn't know it was harder to get a lung when one is short. I always wanted to be taller!

    ReplyDelete
  14. And to think we get shorter as we age, YIKES! LOL I don not claim to know or understand things completely, I am not a dr or tx coordinator so take what I write as a way to form questions of your professional medical team. Do not believe everything you read on the internet especially if it is not coming from a professional. I believe..... It is not only about height. I have a friend who is 5'7 and needs a short lung. She is short from shoulder to waist most of her height is her long legs. Scar tissue has tightened and pulled in her rib cage making the space inside smaller. It could be very possible that you do not need short lungs even though you are not what would be considered tall. I think shape as well as size of the chest cavity could determine if lungs are usable for your tx. They have been very successful trimming lungs to fit. I think that may be called a lobar transplant. Don't know if all centers do them or not. The easiest way to know is to ask your tx center. Anything is possible. Stay strong. Gailinpa

    ReplyDelete
    Replies
    1. There is also a situation where although a double lung transplant is the best option for the recipient but only one lung is suitable, another narrative is the size of the chest cavity has reduced through the hardening of the lungs that only one lung will fit.
      I currently need to be referred to the transplant team as I have had constant deterioration since diagnosis in Aug 2015 - 1 year 4 months ago, I had breast cancer in 2013 which disqualifies me from the required criteria until 2023 - I am in hospital just now with a chest infection & very low saturations - my treatment (Rituximab) finished 6 wks ago but could take 2-4 months to work(If it does as there are no guarantees). My ILD specialists hope is he can stabilise me until 2018 (5 year cancer free) then he can fight my case with the transplant team. I'm 54 previously very fit & healthy apart from asthma & a bad pneumonia 30 years ago. I hope & pray I can get my transplants before it's too kate.
      Elaine

      Delete
  15. THANK YOU THANK YOU THANK YOU Dr.!!! My Mom was diagnosed with IPF in January of 2013 she was told that she had 3 to 5 years to live, she passed away on July 30, 2013. I pray for all who have IPF and I pray a treatment and cure can be found SOON.

    ReplyDelete
    Replies
    1. Mom's are so special. I am sorry for your loss. Gailinpa

      Delete
    2. Sorry to hear about your Mom. The same thing happened to my brother . He was diagnosed late February 2013 and passed away after being on Maximum Life Support for about a week. The 3 lung specialists didn't tell us he was dying and only had such a short time to live. He was on oxygen in May and ended up in respiratory failure early in August

      Delete
  16. I would like to thank you for your honesty. My Pulmonary Doctor at the Center of Excellence in my city has said the same things to me. I have fibriotic NSIP and nobody can tell what may happen. Thus the NS..no cause but I am "lucky" that Prednisone has kept me stable for 8+ years and counting. My O2 have increased and my energy levels have lessened BUT, I'm here.

    ReplyDelete
  17. Thank you for this very friendly Blog on PF. I'm 4 yrs out since my transplant. I am so grateful, but I must admit, it was a very rough recovery. I feel I am still managing my energy level everyday . I'm very active, but not consistent energy level.

    ReplyDelete
  18. Doctor Lederer,

    I was diagnosed with lung cancer in August of 2012 using a CT scan (15 months ago). In March 2013 I was diagnosed as having IPF from a follow up CT scan for the lung cancer issue (8 months ago).

    I have no symptoms of lung cancer or IPF to date. I have a question for you if you can answer for me.

    How long after being diagnosed for there diseases, should I expect to develope symptoms?

    I must add - I walk every day for about 2 kms. and yet my oxygen level maintains at 96 - 97 and I do not experience any meaningfull shortness of breath after my walks.

    Thanking you in advance,

    Gordon

    ReplyDelete
    Replies
    1. Hi Gordon. You bring up a very important question, but it is one that doesn't have an answer. Even if I had an answer about the "average" time until symptoms develop, that answer would only mean that half of people develop symptoms before than time point and the other half develop symptoms after that time point. You still wouldn't know which half you were in. I do want you to talk to your pulmonologist about your condition and be sure to discuss optimal management strategies and whether or not you should be seen at an ILD center. Best of luck with everything!

      Delete
  19. is there any connection between age ( my age ) an how many years left to live with ipf ?

    ReplyDelete
    Replies
    1. Thanks for that comment.

      It's a tricky question to answer. Let me answer it by ignoring the PF part of it for a moment. I'm changing your question to "Is there any connection between age and how many years I have left to live?"

      The answer is yes.... and no. Do people who are 70 years old die sooner than people who are 60 years old? Some of them do. But some of them don't. When I talked about this with my wife, she said "well that's not what he meant. of course some of the 70 year olds will live longer than some of the 60 year olds." She thought I was being "snarky". But I am not trying to be snarky. I am being 100% honest and plain and true. Some will and some won't. Your question really ought to be: "Can I use my age to figure out how long I have left?" The answer to that question is No. You cannot use someone's age to predict the time of someone's death with any accuracy at all. It's the same message that's in this post: No one is average. Demographics are not destiny. Doctors are not fortune-tellers.

      I find great comfort in that. I hope you do too.

      David

      Delete
    2. Dr. Lederer;

      What is your take on using the GAP Risk Assessment System as a tool to help determine a basic prognosis for the IPF patient?

      Thanks

      John R.

      Delete
  20. Doctor L: I love the last three lines of your remarks. Perfect. ( Also - individuals in their 70s have already lived longer than those in their 60s....)

    Ragtop

    ReplyDelete
  21. I have IPF and Emphysema. I was diagnosed with IPF May of 2006, and emphysema before that. I've been on oxygen since July 2009. I'm on 2 lpm for exertion, and I raise it to about 3 lpm when I am exercising. I am not eligible for trials because the trial for IPF won't except me because I have Emphysema and visa versa. Is it possible that my lungs may be close to average size because I have two conflicting diseases? Although my aveoli may be disappearing faster. I did smoke for 44 years and after I quit smoking I got pneumonia and that's when they found the IPF.

    ReplyDelete
    Replies
    1. Thanks for your question. When emphysema and pulmonary fibrosis co-exist, it is indeed common for the lungs to remain nearly average in size instead of shrinking (common in pulmonary fibrosis) or enlarging (common in emphysema). We call this condition "combined pulmonary fibrosis and emphysmea" or CPFE. But, I should point out that the normal size of the lungs in CPFE does not mean that the disease is not serious. In fact, the normal size of the lungs in CPFE can sometimes mislead one to think that the lungs are normal, when in fact two serious conditions are present. Best of luck battling your PF!

      Delete
  22. In 2004 at age 64 I was diagnosed with IPF and my doctor said that I probably had only 5 years or less to live and there wasn't anything that could be done worldwide. For me to just go home and wait to die. I found a doctor on the internet in Germany who had a laboratory and he analyzed my blood, making a serum that destroyed a fungus in my blood by June 2008. I've been fine until a few months ago and I feel that my PF is again progressing. I am going to go back to Germany this month for a serum. I feel that he can help me again as he did 8 years ago. I've been to several Pulmonary doctors here in Dallas and they tell me that they can't help me. I was the doctors first PF patient ever and he was reluctant to work with me but eventually he did and he's treated several hundred others since. He is writing a medical journal about PF and his treatment of me. I have never been on oxygen and am not yet on it.

    ReplyDelete
    Replies
    1. Jerry,
      My father has IPF and I am interested in finding out who you went to see in Germany and exactly what it was you were given if you could.

      Sincerely,

      Heather

      Delete
    2. Jerry,
      My father has IPF and I am interested in finding out who you went to see in Germany and exactly what it was you were given Please.

      Regards,

      Daniel

      Delete
    3. Hi Jerry,
      I just wondered whether you could share the name of your German doctor with us in order to see if he could still be of help to some of us?
      Thanks
      Jennifer

      Delete
  23. I also am very interested in knowing more about the Doctor from Germany and if he has had success in helping other patients with IPF. Thank you for sharing this information.

    ReplyDelete
  24. Dr. Lederer

    I have limited scleroderma with PF. My last PFT and spirometery said my diffusion capacity was 64% but the pulmonologist said the HRCT scan didn't show active inflammation and hasn't changed too much from 6 months. I am not on treatment or oxygen but if diffusion capacity should be 100 percent then isn't 64 % pretty bad ? Thank you for sharing your information

    ReplyDelete
    Replies
    1. Hi -- it looks like I never replied to your question. Very sorry about that.

      For many people with PF, a diffusing capacity of 64% is actually not so bad. Most people with PF with a diffusing capacity in that range have mild disease, do not need oxygen around the clock, and often have mild symptoms.

      I hope things are going well for you.

      Dave Lederer

      Delete
  25. My father has diabetes too along with IPF. My mother was told by Doctor that due to diabetic condition, lung transplant is not an option for him.

    Dr. Lederer, can you tell us, if you think this is true ?

    -Neeraj Vasudeva

    ReplyDelete
    Replies
    1. Hi Neeraj. Thanks for reaching out to me. Diabetes can be a barrier to lung transplantation for some people, but others may be able to safely undergo lung transplantation even if they have diabetes. Damage to the eyes, kidneys, heart, legs, nerves, or brain from diabetes can be enough to make lung transplantation unsafe for some people. Also, if your father's diabetes has been difficult to control, then some transplant centers would defer offering transplantation until his diabetes is under better control. If one transplant center has turned your father down, then it would be reasonable to consider evaluation at other centers.

      Please give your father my best wishes.

      Dave Lederer

      Delete
  26. EDIT with information on tests. Hello Dr. I hope you are still answering questions on this blog. My 87 year old father was recently diagnosed with both IPF and emphysema. He also has occasional issues with racing heart beat and has had a valve replacement and bypass surgery (one operation). He has had shortness of breath issues for several years. But in June, things seemed to turn worse. He began falling because he was light headed. A couple of times he blacked out completely. After multiple doctor visits - regular doc, cariologist, and now a lung doc, along with multiple tests, HRCT's, blood work, etc., we have this diagnosis. The doc showed us the images... both of the emphysema scaring, but the "ground glass" fibrosis. It's in both lungs, but seems worse in the right. He tried to go off O2 but within 3 days was falling again. He wants to drive, but my siblings and I don't want that and the pulminary doctor said no... He's stubborn enough to sneak out, I'm afraid. He does not understand the seriousness of what he is fighting. His o2 is 24 hours a day. 2-3 litres at rest, 6-7 while active. The doctor is reluctant to give us a prognosis, and I do understand why. But at the same time, I think it impacts my dad's understanding of this progressive disease. He already is confused about things, and this doesn't help. From the little I've told you, do you have any observations? Any thoughts on what me might expect over the next year or two? Does the presence of emphysema complicate his prognosis? Also, from his last tests he had 74% lung capacity and 50% diffusion.
    Thank you for your help
    Mark

    ReplyDelete
    Replies
    1. Hi Mark. Thanks for reaching out to me. I am sorry to hear about your father's illness. I very much understand your desire for clear information and guidance about your dad's condition. As you can imagine, it is very difficult for me to make any specific conclusions about your dad's condition, since I haven't met him or examined him. What I can tell you is that the presence of emphysema does indeed make things more serious (at least in my experience) than having PF without emphysema. One reason this is true is that the breathing tests can be falsely reassuring when PF and emphysema exist together. Most people who have a combination of PF and emphysema seem to have only slightly reduced lung function tests (like "forced vital capacity" or FVC) and sometimes they are told their condition is not serious, when it fact it is. The other problem that I see in my patients who have both PF and emphysema is that they commonly develop pulmonary hypertension (high blood pressure in the blood vessels that connect the heart and the lungs) as a complication of their condition. They also typically require more oxygen with exertion that those with PF alone.

      I can't give you specific advice about your dad's condition other than that it would be a good idea to consult with a physician at an interstitial lung disease (ILD) center. You can find a list of PFF Care Center Network sites and a list of ILD centers in the US and elsewhere on the PFF's website at http://www.pulmonaryfibrosis.org/life-with-pf/find-medical-care

      Best of luck, Mark. Please give your father my best wishes.

      Dave Lederer

      Delete
  27. After having been diagnosed with COP in 2012 and treated with Prednisone, I was off meds until this summer, when it was thought I was having a relapse. I did not respond to Prednisone this time, and a CT was inconclusive but IPF was a possibility. So I am weaning off the Prednisone to prepare for an open lung biopsy in November. I am a nurse, and I am terrified of the ramifications of this. I did hear that 2 new drugs have been approved this week for IPF, but the diagnosis alone scares me to death. Can you give me any insights into this?

    ReplyDelete
    Replies
    1. Hi Ellen, I am sorry to hear that you are going through this. I agree that it is very important for your doctors to sort out exactly which kind of lung disease you have. If you do turn out to have IPF, then pirfenidone or nintedanib might be helpful for you. Here are my recommendations for people diagnosed with any form of PF: http://www.pfdoc.org/2014/04/i-was-just-diagnosed-with-pulmonary.html. Best of luck with everything, Ellen.

      Dave Lederer

      Delete
    2. Thank you so much for your reply. The information you suggested is very helpful. I am seeing Dr Leslie Tolle at Cleveland Clinic, and he has been very good - I am just a bit overwhelmed at this point, and the waiting period is so very difficult. I am so grateful for your insight.

      Delete
  28. This comment has been removed by a blog administrator.

    ReplyDelete
  29. Thanks for your article. I was diagnosed with IPF after lung biopsy in 2000, my older sister had died of this disease in 1991. Was hospitalized for Bronchitis Pneumonia in 2006. My O2 readings were down to 72 and I was using Oxygen at least 12 hours a day and was in line for a lung transplant.

    Since that time, my IPF symptoms have disappeared, my breathing is normal, my O2 readings are 98 resting and 95 after a brisk walk. I have been told that this is an unique situation and that the disease will come back. My questions to you are as follows:

    1. Can a person stay alive over 20 years after being diagnosed with IPF?

    2. Can a medicine like Valcyte help in controlling IPF?

    3. My doctor cannot explain how my breathing and oxygen saturation rate is back to normal
    when in reality I should be dead.

    ReplyDelete
    Replies
    1. Thanks for sharing your experience, which is pretty amazing. I've seen folks with very early PF that was stable for many years before they became sick, but I haven't yet met someone who truly had idiopathic pulmonary fibrosis and got better (without a lung transplant). Some people think that valcyte might indeed help IPF (although it's never been studied) and I've had many patients with IPF who were on valcyte and their lung disease did not improve. were you on valcyte? It is also possible that you had a different form of PF (not IPF, but one of the other kinds of PF) that might have responded to other medications (like prednisone -- were you on prednisone?) or might have responded to getting rid of mold, birds, or other triggers of scarring in the lungs in some people. Maybe you have all of the answers. I'd love to see your CAT scans. All the best!

      Delete
    2. Dear Dr. Lederer,

      Thanks for for your reply. I do have the results of the lung biopsy (VATS) that was done to confirm the IPF diagnosis. I also have MRI/CAT scans that were done. I have not had any tests done over the last year as I was feeling good. I was put on prednisone for 6 weeks before the biopsy, but my body reacted to it with swollen legs, face etc. I took Valcyte for 6 months after the confirmed diagnosis through VATS and stopped it when I was feeling better.. I will connect with you separately to provide all the data you need to help me understand my 'situation'!
      Thanks

      Delete
  30. I had an open lung biopsy this month, and just received the diagnosis of early stage IPF. I will be starting on one of the new drugs as soon as possible. My question is: the "I" in IPF denotes Idiopathic (unknown origin). I am a 29 yr breast cancer survivor - bilat mastectomies in 1985 and bilat implants (silicon and saline) in 1988. Some material I have read suggests that older implants may have an inflammatory effect on the lungs, and some places specifically indicated a link to IPF. Have you heard of any such link? The implants are now 26 yrs old, and I really no longer care about the cosmetic effects. I am thinking about removal, and I know it is a simple outpt procedure. Can you comment?

    ReplyDelete
    Replies
    1. Hi Ellen. I have never heard of a link between implants and IPF, but people who have received chemotherapy or radiation for breast cancer can sometimes develop pulmonary fibrosis. Please talk to your doctors about this possibility if you did receive either of these treatments. All the best.

      Dave Lederer

      Delete
  31. Dr. Lederer, this blog is wonderful. My father has end phase IPF. However, he is still mobile and walks around. When he is at rest, he can be on 5 LPM. However, when he moves around in the house, he probably needs 12-13 LPM. Is there a downside to hooking 2 concentrators together to get this flow if he uses a mask? Also, my dad has been off of prednisone for about a year because of the risk it posed to infection. Is there a point at which one might consider using it again? He has had a couple of set backs as of late, and his O2 reqs when he walks has doubled. Thanks!!

    ReplyDelete
    Replies
    1. Thanks for your post. I often recommend to my patients that they connect 2 concentrators at home to obtain enough oxygen so they can maintain an oxygen saturation over 90% during in-home activities and exercise. If your dad's doctor has recommended this for your dad, then I think it is a good idea. Your question about prednisone is trickier to answer. There is no single right or wrong answer about different treatment approaches -- your dad's doctors will need to answer that one. Sorry. All the best.

      Dave Lederer

      Delete
  32. Dr. Lederer, thank you very much for your reply. I also wondered whether there was any danger to too much oxygen for someone who has IPF, and if many of your patients require 10 LPM at night to maintain adequate saturation? What happens if more than 10 LPM is required?

    Thanks again.

    ReplyDelete
    Replies
    1. Dr. Lederer, I also wanted to share that the below mask is really really decent for people who use high flow oxygen, and need to give their nasal passage a break. My dad has used it, and it is very effective. it is called Oxymask, and is at http://southmedic.com/products/oxymask-adult/. It can be purchased online.

      Delete
    2. Dr. Lederer, my father is at end phase IPF. I was reading about PRM 151. He suffers from mylofibrosis and IPF. Apparently it treats both. Is this drug something that could be considered? He was on the generic equivalent of pirfenidone (pirfinex) for a few months but it seemed to cause bone marrow suppression. So we stopped. Thanks very much.

      Delete
    3. Hi MPJ. Sorry for the delayed response. We often work with oxygen suppliers to make sure people with PF are able to get as much oxygen as they need at home -- between tanks, concentrators, nasal cannulas, and masks, we can usually get things to work for each person.

      I haven't heard about PRM 151. Will look into it.

      All the best.

      Dave Lederer

      Delete
  33. My mom was diagnosed with ILD in 2013 we had been managing it till a week ago suddenly she wasn't even able to move talk or think straight.. we where hesitant to take her to hospital fearing they would intubate her right away.. but seeing her pain we took her to the emergency room there they gave her multiple antibiotics and put her on prednisone four days later she's back home on 3_4 liters when sitting,sleeping and 6_7 ltrs when active to keep her 02 levels at 95% or higher how us it she was so close to death but now seems much better.. tomorrow we have appointment at cedar sinai transplant center to see if she is candidate for lung transplant she is 63 she is our world our rock our mom my heart goes out to everyone going through this plz stay positive please now that your not alone and there is hope always hope never give up use this experience to love and cherish everyday. This my num if you ever need to talk 8186266946

    ReplyDelete
  34. I was diagnosed in 2008 with pulmonary fibrosis , pleural effusions, scarring , plaques and calcification took doctors 34 years to admit there was something wrong. After rolling in asbestos for 4years in Navy 1970 to 1974. I looked like feathered and dusted chicken at end of day several days a week. after denial all those years I found when you can't breathe don't panic and hunt for air relax and wait on or your emotions will kill you . I sat in emergency room parking lots for years till I got my breath knowing I couldn't go in . I would tell them I couldn't get a deep breath no more they would say yes you can just look at yourself . Now after over they say you can have pulmonary fibrosis for 20 years before showing up in x-ray. Took 35 years before they put pulse -ox on finger and said walk instead of pulse-ox on finger and sit down. Because I looked healthy they said there was nothing wrong

    ReplyDelete
  35. Recently, I was diagnosed with Advanced Progressive Massive Fibrosis due to talc granulomatosis (talc depositions in the capillaries of the lungs due to IV drug use) and possibly concurrent Sarcoidosis. I am 36 years old, and I have shortness of breath upon exertion (1 flight of stairs), a dry hacking cough, and mild chest pain beneath the center of my sternum. My pulmonologist gave me Advair and Ventolin to help manage my symptoms, and while those medications do help with breathing somewhat, they do not prevent the cough. I noticed that since I've left the hospital my symptoms seem to be getting slightly worse each day. My pulmonologist told me that once they have completely ruled out infection (some fungal infections take up to 30 days to grow in the lab), he would start me on Prednisone, 45mg.

    Is it possible for PMF due to talc granulomatosis to enter complete remission, thus allowing me to live a somewhat normal life (I'm only 36) for an extended period (20-30 years)?

    PMF is usually considered a very slowly progressing disease. What does that mean?

    Do 'normal' people have oxygen saturation levels that drop below 90% on vigorous exercise (playing soccer, basketball, aerobics, etc.). Do very sedentary people experience low oxygen saturation upon moderate exercise? Are low oxygen levels a characteristic of fibrosis ONLY?

    ReplyDelete
    Replies
    1. Been clean for 8 years. No drugs, alcohol, or smoking.

      Delete
    2. Hi. Thanks for sharing your story. I completely understand your desire to have a sense of what the future holds, but the truth is that I would need a lot of medical information to know whether your condition might improve. There is no simple answer. I strongly recommend that you discuss this with your pulmonologist, since she or he knows your condition best.

      People with healthy lungs and hearts (even sedentary folks) never drop their oxygen level even during routine vigorous exercise.

      A number of conditions can lead to a drop in oxygen levels during exertion. Some examples are interstitial lung disease (which includes all the forms of pulmonary fibrosis plus many other conditions), certain blood vessel problems in the lungs, and certain problems in the heart.

      Best of luck with everything.
      Dave Lederer

      Delete
    3. Dr. Lederer,

      Thank you for all your work on this blog. It is clear that your engaging with so many folks provides many with much needed insight and therefore alleviates much fear and anxiety associated with these illnesses.

      I have a question as far as "never drop their oxygen level even during vigorous exercise." Is that absolutely true? What I mean is that, as one walks up 5 flights of stairs, for example, would it be abnormal to drop a few percent as breathing and heart rate increase? I ask because I have been using a pulse oximeter during hiking and other exercises, and I notice that at times my sp02 drops from a norm of about 96 down to 94, but then might recover. Are some fluctuations in saturation levels not normal?

      Delete
    4. Hi. Thanks for your comments and your question. Pulse oximeters can vary by up to 3% (or more in some cases) just by chance. A change from 96% to 94% might not be a "real" change (although you should certainly discuss this with your doctor). Also, if you hand is moving while you are measuring your oxygen saturation, your saturation reading may also be inaccurate. Elite athletes can indeed drop their saturation during "supramaximal" exercise, but others without lung disease (and without other certain conditions) typically should not drop their oxygen levels during exertion.

      Delete
  36. I know for a fact there's no cure for pulmonary fibrosis no medicine that will help my mom has dealt with this for years I know it's genetics my aunt and uncle just got diagnosed I lost a aunt from it over 17 years ago so I know there's no hope and there's no medicine to help thispolish doctors would stop giving people hope like that it's wrong it's not right I pray for the best for everybody with pulmonary fibrosis my mom is struggling she's not doing good she was diagnosed over 12 years ago stop giving people hope when there really is no hope

    ReplyDelete
  37. Dr. Lederer,
    How does CPFE affect the transplant listing process? My sister has a very serious case of CPFE and is undergoing transplant evaluation but her docs have suggested that she might not list well because she does not use O2. At rest, her sats her pretty good (95-96%) but upon exertion she suffers severe shortness of breath. Her PFTs are not terrible either. I've looked at the LAS process and other than the 6MWT, her LAS eval might seem OK. And I cannot tell how the CPFE would be factored into the LAS. Thank you.

    ReplyDelete
    Replies
    1. I would add that I cannot find CPFE as a diagnostic code in the LAS calculators I've seen on-line. Thanks.

      Delete
    2. Hi and thanks for your question. I consider CPFE (combined pulmonary fibrosis and emphysema) and type of pulmonary fibrosis. For purposes of calculating an LAS score, it would be reasonable to select "Pulmonary Fibrosis, Other" (or something along those lines) in the online calculator. If her doctors believe she is "too well" to be placed on a waiting list for lung transplantation, I would consider that good news. Please share my best wishes with your sister.

      Delete
  38. Is Pulmonary Fibrosis the same thing as Farmer's Lung? My grandpa's doctor told him that he has Pulmonary Fibrosis, which has also been called Farmer's Lung. I've looked up Farmer's Lung, and it doesn't seem to be the same thing, although it looks as though Farmer's Lung can lead to PF. Can you please give some clarification?

    I also wanted to say that I was thrilled to read this article. The thought of losing my grandpa in 3-5 years is devastating. You've at least given me a little bit of hope! I am not sure if the doctor told him IPF or PF, but I do know that he associated it with Farmer's Lung (my grandpa was a farmer for most of his life-- he is 80 years old).

    Thank you for your help!

    ReplyDelete
    Replies
    1. Farmer's Lung is one type of lung disease called "Hypersensitivity Pneumonitis" -- which means that the lungs are overly sensitive to certain airborne particles breathed into the lungs. In some cases, Hypersensitivity Pneumonitis (and Farmer's Lung) can lead to Pulmonary Fibrosis. Please share my best wishes with your grandfather.

      Delete
  39. Hello Dr. David. I am a lady aged 33 and in July 2012 I was diagnosed with NSIP and my lung capacity was 40%. An open lung biopsy was done and treatment of prednisone - 20mg was prescribed. I managed to get better and was really look fresh and vibrant on the outside. The lung capacity improved after about about 1 year to 80%! The predisolene was reduced to 10 mg. I have been taking it on any other day. Then just last month, October 2015, I went for a check up and the results were not good. The scan showed that my lungs are deteriorating faster and they have become smaller. The results on breathing showed that my lung capacity has gone below 50%! The doctor/pulmonologist said the scarring is increasing. He didnt say that I am dying but his quiteness on the diagnosis had lots of question marks! I feel like am going to die soon becasue it seems the scarring has covered the lungs. If you see me in appearance I look good. I only fall short of breath when am on a flight of stairs, and I need about 5 mins to relax and be normal. I try to walk. Currently, am back on 20 mg prednisolene and in a week i willl be on 10mg. I had lost almost 8 kg within a year. All I do now is to hope and pray for a miracle. I have a question, I am looking forward to getting married and have a baby. Will that be possible. I so much was a baby and my fiance too. I have surrendered it all to Jesus now since the Doctor seemed shocked with the deteriorating condition. I am in Africa and I travel for days to see my pulmonologist.

    ReplyDelete
    Replies
    1. Thank you for reaching out to me and sharing your story. I am sorry to hear about everything you are going through. Only your own doctor can give you medical advice about pregnancy. Please ask him or her at your next visit. I am struck that you have to travel for days to see your pulmonologist. Do you have another doctor closer to your home that you can see? Where in Africa are you? A colleague of mine here in New York developed a training program for pulmonologists in Addis Ababa, Ethiopia. Africa is very large -- my guess is you are far from Addis. I wish you all the best. Please keep me posted on your health.

      Delete
  40. Hello Dr. David. I am a lady aged 33 and in July 2012 I was diagnosed with NSIP and my lung capacity was 40%. An open lung biopsy was done and treatment of prednisone - 20mg was prescribed. I managed to get better and was really look fresh and vibrant on the outside. The lung capacity improved after about about 1 year to 80%! The predisolene was reduced to 10 mg. I have been taking it on any other day. Then just last month, October 2015, I went for a check up and the results were not good. The scan showed that my lungs are deteriorating faster and they have become smaller. The results on breathing showed that my lung capacity has gone below 50%! The doctor/pulmonologist said the scarring is increasing. He didnt say that I am dying but his quiteness on the diagnosis had lots of question marks! I feel like am going to die soon becasue it seems the scarring has covered the lungs. If you see me in appearance I look good. I only fall short of breath when am on a flight of stairs, and I need about 5 mins to relax and be normal. I try to walk. Currently, am back on 20 mg prednisolene and in a week i willl be on 10mg. I had lost almost 8 kg within a year. All I do now is to hope and pray for a miracle. I have a question, I am looking forward to getting married and have a baby. Will that be possible. I so much was a baby and my fiance too. I have surrendered it all to Jesus now since the Doctor seemed shocked with the deteriorating condition. I am in Africa and I travel for days to see my pulmonologist.

    ReplyDelete
  41. Thank you Dr. I will keep you updated after my next visit. I am far from Addis! I am in Southern Africa - Zimbabwe and I travel to South Africa.

    ReplyDelete
  42. Hi Dr Lederer! My father was diagnosed with IPF last month via a scan of his lungs after a bad case of pneumonia. It is currently only showing as effecting one lung. Does IPF always/usually spread to the other lung? Is the prognosis typically better for someone with IPF in only one lung? I thought someone could live with one healthy lung... Maybe I am just grabbing at straws for some extra hope. Thank you

    ReplyDelete
  43. Hi Dr Lederer! My father was diagnosed with IPF last month via a scan of his lungs after a bad case of pneumonia. It is currently only showing as effecting one lung. Does IPF always/usually spread to the other lung? Is the prognosis typically better for someone with IPF in only one lung? I thought someone could live with one healthy lung... Maybe I am just grabbing at straws for some extra hope. Thank you

    ReplyDelete
    Replies
    1. It is not uncommon for people with IPF to have "lopsided" disease, with more scarring on one side and less scarring on the other. A very small number of people (like your father) have almost all of the scarring on one side. When I see this, I often wonder if there is a specific trigger causing it to occur on just one side (such as reflux from the stomach dripping into the lungs during sleep -- consider discussing this with your doctor).

      I would love to be able to reassure you that scarring on one side means that his other lung is safe and that your father's disease is not going to progress rapidly. But, IPF is a disease that has few rules -- everyone has their own disease, with their own rate of progression -- and in my experience the lopsidedness of the scarring is not something that helps me predict what will happen to an individual person with IPF. More important factors would be (1) how much scarring there is overall, (2) how much oxygen he needs, and (3) what his breathing test results look like. I encourage you to discuss all of these results with his ILD doctor to see if he or she can give you any insight into what to expect in the future.

      Please share my best wishes with your father.

      Delete
  44. HI All,

    from last one month I am having shortness of breath wile climbing steps or taking bath or playing badminton and brisk walk too, till 2 months back I use to do cross trainer for 1 hr and play cricket and bowl 5 or 6 over spell continuously , I consulted doctor today he recommended for Blood test, chest X-ray and ECG , even now my blood pressure is high 150/105, do you think any symptom of ILD or IPF ,

    ReplyDelete
  45. Hello Doctor,
    My mom who is 52 had severe chest pain and had her CT scan.The results said tht features represent UIP. Is UIP same as PF ?
    What is the next best option we have for her treatment?
    1. Reticular opacities noted at lung periphery predominantly at subplueural and perifissural distribution involving bilateral lung fields.
    2.areas of increased attenuation noted at posterior segment of right upper lobe,medial basal segments of both lower lobes and perihilar regions.
    3. Insignificant mediastinal lymphadenopathy
    The above are the observations from her CT Scan.
    Is her condition very severe?
    What shall I do next for treatment?
    Please guide me sir..
    We are from India. I have no idea what to do next?

    ReplyDelete
    Replies
    1. Hi Ram. I am sorry to hear about your mother's condition -- I hope her severe chest pain resolved. I cannot really comment on whether her condition is severe or on an appropriate treatment. My strong advice to you is to bring your mother to a pulmonologist (respirologist/lung doctor) who can help figure out what is going on. It doesn't appear to have been updated recently, but this blog may be helpful: http://www.pulmonaryfibrosis.in/tag/ipf.html

      All the best,
      Dave Lederer

      Delete
  46. hi , david my wife had a ct scan and she was told its inflamery lung conditions can this be interstitial sacring as she has the same symptoms like short breath .

    ReplyDelete
    Replies
    1. Hi. There is a big family of lung diseases called "interstitial lung diseases" often abbreviated "ILD." ILD refers to any lung disease where there is inflammation, scarring, or other changes in the walls of the tiny little air sacs of the lung. While inflammation and scarring often occur together in the lung, they are different types of problems. Inflammation can sometimes be treated with medications that make the inflammation go away. Scarring can be slowed down in some cases, but does not go away. Both inflammation and scarring certainly can cause breathlessness. I encourage you to talk to your wife's doctor about the cause of her lung inflammation and whether or not there is also scarring. You might want to consider taking your wife to a specialist in "ILD." You can find a list of medical centers that specialize in ILD (and pulmonary fibrosis) at http://pulmonaryfibrosis.org/life-with-pf/find-medical-care

      All the best,
      Dave Lederer

      Delete
  47. hi doctor , my wife when for ct scan , and the report reads inflammatory lungs i wish to know if this is scarring and if its related to intestial lung disease .

    ReplyDelete
    Replies
    1. Hi. Your question is very similar to the question asked right above yours here on December 29. Please take a look at my above response. if you still have questions, please let me know.

      Delete
  48. My mother has IPF and is on oxygen all the time now. When moving she uses about 3/3.5 and is on 1 0r 1.5 at other times.She has completely lost her appetite which in turn has made her very very tired all the time. She gets mixed advice about the oxygen use. Some say to keep it as low as possible as too much can cause problems and others advise using more when doing anything. Her cheeks are often purple even when just sitting. She is on a low dose of prednislone. They also have her on mirtazapine. I feel more could be being done to help her have a better quality of life.Do you have any advice about use of oxygen

    ReplyDelete
    Replies
    1. Thanks for this question about oxygen use. First, please understand that I cannot provide medical recommendations over the internet. I can however provide you with general medical information that you can then discuss with your mother's health care provider. For my patients with PF who have normal carbon dioxide levels in their blood, I recommend that they use as much oxygen as possible to keep their oxygen saturations (measured by a pulse oximeter) over 90% particularly when they exertion themselves. Please talk to your mother's health care provider about whether this kind of strategy is a safe one for her. You can also search for medical centers that specialize in pulmonary fibrosis as http://www.pulmonaryfibrosis.org/life-with-pf/find-medical-care

      Delete
  49. Hi I'm 30 years and was diagnosed last year of pf.i have a scar on my lungs. I don't have ifp. I have rheumotid athritis too. Iv currently slowly came off the steriods prednisone because of weight gain. But I feel I am getting more breathless without them. Iv had to lung function test. One beginning of 2015 and then treatment of 2 Infusion of rituximab and then another lung test and they both came bk as pretty much same which meant the scarring hasn't progressed due to rituximab which controlled my joint for rheumatoid athritis. My joints are better but iv started feeling breathless bit more bcz I don't take steriods. I feel I shud re start again could u help. I'm very worried abt dying in next 3 years my doctor says the prognosis which is 3 to 5 years wud b for some1 over 60 years and this was set for people over 20 years or more and things are different now Dr say I'm only 31 I have age by my side and I don't smoke or I'm not fat or I dnt have that type lung disease but of course it's stil serious I just need some help and very scared.

    ReplyDelete
    Replies
    1. Hi Sofia. I am sorry to hear about your medical condition and the way in which you are suffering. I cannot give you medical advice on the internet, but I do strongly suggest that you consider getting medical care at a center that specializes in pulmonary fibrosis. Please take a look at the PFF website for details: http://www.pulmonaryfibrosis.org/life-with-pf/find-medical-care

      Delete
  50. Hi Dr.,
    My father is 79 years old and has mild emphysema due to smoking. He quit smoking over 40 years ago. About 2 months ago, he contracted what the doctors initially thought was pneumonia. Interstitial fibrosis on the bottom of the lungs was evident. Antibiotics were not helpful but prednisone was. After 2 months he has most of his energy back and is having fewer episodes of breathlessness after exertion. The doctors said he has IPF after giving him a CT scan. No biopsy was done. Prior to 2 months ago he was able to walk up 9 flights of stairs without any breathing issues. He is anticipating getting a new medication for the IPF. My question is twofold - is it possible he doesn't have IPF? And secondly, if they put him on the new medication(OFev)would it be harmful if he didn't have IPF? I find it curious he had no symptoms prior to this episode and that he is improving like he is...

    ReplyDelete
    Replies
    1. Thanks for your questions. I hope you understand that I won't be able to answer your first question --- only his doctors can figure out if he has IPF or a different disease. We also don't have any solid information about whether drugs like nintedanib (OFEV) or pirfenidone (Esbriet) work for diseases other than IPF. I am not aware of any harms from these medications that would only affect those who don't have IPF -- their side effects probably apply to everyone equally. Please also consider getting a second opinion from a team at a medical center that specializes in his condition. you can find a list on the Pulmonary Fibrosis Foundation's website: http://www.pulmonaryfibrosis.org/life-with-pf/find-medical-care

      Delete
  51. I had a tough case of the flu in Dec. 2013 I was left with a cough that lasted over 3 months. My PCP xrayed my lungs and sent me to a local Pulmologist. He, in turn, sent me to Duke who confirmed IPF. I've had Fibromyalgia, before it was even called that, since 1990. Between these 2 conditions I've planned my funeral and met with my adult children. My numbers are still good, I use 3 liters at night, 2 rarely during the day. The fibro keeps me from doing much of the things I used to enjoy. I take Ofev, lately I've had violent gastric side effects so I'm not sure I will continue it. Although my Doctor monitors me closely I do not believe I have long to live. I'm exhausted all the time.
    Now for my question. Have you had any experience with Fibro and Ipf patients and can you offered me any advice? Thank you, Jeanne

    ReplyDelete
    Replies
    1. Thanks for sharing your experience. I have seen a few patients who have both fibromyalgia and PF. I don't think there's any relationship there, except that since both conditions can lead to fatigue, people can feel pretty lousy. Best to see experts in both conditions to make sure you are getting the best advice and therapeutic options.

      Delete
    2. Hi Dr.
      Amazing blog you have here.I have had copd for probably 8 yrs. now. I had a 11mm. lung cancer removed 3 yrs ago. I went to a walk in clinic for uti last week.While I was there, got a chest x-ray. Results as follows- Small right lung secondary to prior surgery.No focal infiltrate or evolving mass. The costophrenic angles are sharp, without evidence for effusion.Heart size and media's Tina contours within normal limits. There are no Osseo us abnormality identified.
      IMPRESSION: No evidence of active pulmonary disease.
      Top of page says: Post wedge resection, hyperinflation lungs, interstitial dz, no pax, no effusion, no infiltrate.
      As you see, it mentions - interstitial dz.
      I am freaking out over this. Can you explain my x-ray to me? This is wonderful what your doing to help people. Thank you so much

      Delete
    3. Hi Dr.
      Amazing blog you have here.I have had copd for probably 8 yrs. now. I had a 11mm. lung cancer removed 3 yrs ago. I went to a walk in clinic for uti last week.While I was there, got a chest x-ray. Results as follows- Small right lung secondary to prior surgery.No focal infiltrate or evolving mass. The costophrenic angles are sharp, without evidence for effusion.Heart size and media's Tina contours within normal limits. There are no Osseo us abnormality identified.
      IMPRESSION: No evidence of active pulmonary disease.
      Top of page says: Post wedge resection, hyperinflation lungs, interstitial dz, no pax, no effusion, no infiltrate.
      As you see, it mentions - interstitial dz.
      I am freaking out over this. Can you explain my x-ray to me? This is wonderful what your doing to help people. Thank you so much

      Delete
    4. Hi Ruby. Sorry to hear about the distress you are experiencing with this. The impression of "No evidence of active pulmonary disease" is not consistent with having interstitial disease. Is the information at the top of the page the "clinical information"? Clinical information is the information that the doctor gives to the radiologist (not the radiologist's interpretation). I suggest you reach out to your doctor to ask her or him to explain why the report includes the words "interstitial disease". Also, it is usually not possible to confirm the presence of interstitial disease on a chest x-ray -- particularly when someone also has COPD. Hope this all goes well for you.

      Delete
  52. Thank you for your reply. The top of the page says- History/prelim Diagnosis; pt.presents sob- post wedge resection as per Dr. Furiato-post-op rules, hyperinflation lung, interstitial dz, no put, no effusion,no infiltrate. I guess that's the drs. Diagnosis. The rest I told you is from the radiography person. What do you think? This is not my Dr. Its a walk in clinic. I've not had insurance and wanted to check on my lungs because of past lung cancer. Quick question- Hubby has O2 level of 93-94 when sitting. Doesn't have lung disease. Shouldn't be. Right? Thanks again, Ruby

    ReplyDelete
    Replies
    1. Hi Ruby. It is difficult for me to answer either of your questions -- both situations require a visit with your (and your husband's) doctor to sort things out. I am sorry that I can't help.

      Delete
  53. Thank you for this valuable blog. My Mother was diagnosed with IPF, a blocked valve in her heart as well as leukaemia in March of 2014. To our surprise we had no idea that the IPF would be her biggest threat given we had no idea what it was. Initially, they did not believe they would be able to replace the valve was she was unable to have open heart surgery given her lung condition. After much consultation with many specialist they agreed to do the surgery but through a TAVI procedure. Her surgery was a success and she is now the owner of a cow valve. The doctors hoped that this would improve her breathing however that part was not a success as she is still on 24 hrs oxygen. In regards to her leukaemia she was unable to receive direct chemo so she takes a chemo tablet every 2nd day which her specialist has said has been in remission. Now comes the questions. LAST week my Mother was not feeling well so she was taken to hospital on a wed and told she had pneumonia and given prednisone and another antibiotic. By Monday she was no better. Her RT visited at home and called for ambulance as thought she was in congestive heart failure. Another emergency Doctor did more tests and said no it was not that not was it pneumonia it was in fact progression of her IPF. She is having a lot of difficulty getting her pills down and has been bringing up ALOT of white mucus almost like strings of it very thick but clear. But we are being told this is not common with IPF usually it is dry and a dry cough which she does not have either. Her breathing is not worse but she is tired and incontinent last few days. Please help our family. I have read through and we too want that magic ball that doesn't exist but would appreciate any input you may have on the above. Our Mother was told 2 years ago she had advanced severe IPF so we feel blessed for everyday!

    ReplyDelete
    Replies
    1. I am sorry to hear that your mother is suffering through all of this. Since your mother is not my patient, I can't provide any specific medical advice. I do, however, recommend that you talk to your mother's doctors about her symptoms (why does she have white mucus? what can be done about her swallowing, fatigue, and incontinence?). Even when we don't have a cure, we often have treatments that can help people feel a whole lot better. Best wishes.

      Delete
  54. Dr. Lederer, thank you for your service to the human kind. My sister (33 yrs, South Asian race) has been diagnosed with Fibrosis NSIP (in April 2016) and SLE (in May 2016). Her lungs capacity was at 85-90% in February and in April it has come down to 30%. She is currently on no medication as the doctor was awaiting further test results and her breathlessness has reduced to a level where she feels tired while eating and thus cannot finish her meal. We have been running from pillar to post and have gotten hundred's of tests performed to finally get this diagnosis. I am not sure what am I looking from you here, but this blog is definitely a source of positivity for me and my family and hence writing to you. If you need any more details regarding her tests, please let me know. Any word of advice or guidance is all I am looking from you.

    With deepest regards!
    Tripathy Family from India

    ReplyDelete
    Replies
    1. I hope your sister is not suffering terribly with this. I know that some of the suffering from PF is from the uncertainly of exactly what is going on. It sounds like she has been through so much. Please give her my best wishes. I can't give medical advice over the internet, but I can give you information: Talk to her doctors about whether oxygen, medications, and exercise would help. if you aren't happy with the information you are getting from your doctor, ask for a second opinion from another pulmonologist. I wish you and your sister all the best.

      Delete
  55. Is OFEV recommended for heart transplant patients wit IPF ?

    ReplyDelete
    Replies
    1. As far as I know, there have not been any studies using Ofev to treat IPF in folks who have undergone heart transplantation. Ofev could interact with some of the drugs used to treat people who have undergone heart transplantation -- but the decision to treat (or not treat), should be made between you (if you are the patient), your heart transplant doctors, and a pulmonologist who specializes in IPF.

      Delete
  56. Thanks you so much sharing.

    ReplyDelete
  57. Thank you for this article. My husband has COPD, emphysema and now this month has been diagnosed with IPF- I am frightened. His latest breathing tests had terrible numbers. He uses oxygen 24/7 and nebulizer 4 X a day- I feel helpless- we have a wonderful pulmonologist-

    ReplyDelete
  58. Laura, I am sorry to hear about your husband. Stay strong, and be resourceful, as hopefully he will come out the woods. I have read that there are drugs out there which are antifibriotic in nature, and may help IPF. The drug names are Pirfenidone, Esbriet, and the generic equivalent is called Pirfenex which can be purchased at a low cost abroad. There may be side effects to these drugs, and i do not know how effective any may be, but you may want to inquire about them. Best of luck to you and your husband.

    ReplyDelete
  59. This blog is fantastic and possibly thr only ray of hope for people who have given up. Thank you for sharing this and may God Bless your wisdom.

    My dad recently (2 years ago) got diagnosed with Pulmonary Fibrosis. After having seen three specialist who all tried Panafcort steriod (ranging 5mg to 25 mg) were unable to stabalise his decline. He has lost a lot of weight now down to 50 kg. He has no energy and motivational levels are very low.
    I'm very worried about him. We recently saw a specilaist at the hospital who have recommended a trial drug Pirfenidone also called Esbriet.

    This drug is not FDA approved in Australia yet. I'm hoping to get some advise to help stablize his condition and get him active.

    Any thoughts and advice would be very much appreciated.

    ReplyDelete
    Replies
    1. Hi Sandeep. I can't make medical recommendations on the internet, but I can give you medical information. Both pirfenidone and nintedanib have been shown to slow the rate of decline of lung function. I use these medications routinely in my patients who have idiopathic pulmonary fibrosis. Please consider bringing your dad to see a pulmonologist/respirologist who specializes in pulmonary fibrosis. Perhaps they will be able to obtain pirfenidone or nintedanib for your dad. I also have my patients enroll in pulmonary rehabilitation and use supplemental oxygen (as described elsewhere on this website). These things seem to help people with PF live healthier and more normal lives. All the best - Dr. Lederer

      Delete
    2. Thank you for responding Dr. Lederer.
      We met with a pulmonologist at the respiratory clinic and they have recommended Pirfenidone.
      Most importantly I wanted to know more about the effectiveness. It seems to get good reviews online and you also perscribe it, makes me confident.

      My dad has tried pulmonary rehabilitation but he seems to lose all the motivation to try further. I believe he is depressed especially with this disease and thr steriods he has been taking.

      Once again thnaks for replying we are all hoping that the new medication will help him gain strength. We have also got home oxygen and will start to get him more activity at home.

      This is a great blog and has much needed information on pulmonary fibrosis.
      Wish everyone gets the right information at the right time.

      Delete
  60. Recently a pulmonary specialist stated: "It is impossible to make a specific prognosis (the rate of progression, or time to recovery or the time to death) in an individual patient with IPF." Would you agree with this? I have been led to believe that it is not possible to "recover" from IPF.

    ReplyDelete
    Replies
    1. Thanks for your question. There seem to be two elements to your question: (1) can we predict when IPF will progress and/or when someone with IPF might pass away, and (2) can we predict if someone with IPF will recover. I can see how these two might be seen as two sides of the same coin, but in the case of IPF, recovery (without lung transplantation ) has never been observed. Your doctor may have been trying to tell you that they can't predict #1 -- that is, they can't tell you when your disease is going to get worse and they can't tell you how long you have to live. That is the same message I was trying to convey with this blog post above. Does this answer your question?

      Delete
    2. Thanks for replying. But, with respect, it doesn't answer my question. The prognosis in question i.e. 3/5 years to live (per the GAP Index), was given based on 1 x HDCT scan and 1x PF test both done in June 2013. So arguably a doctor saw fit to make an adamantine prediction of death. Notwithstanding no symptoms have or do exist viz., breathlessness, clubbing, GERD's weight-loss, in fact there has been a couple of pounds gained. I work as a soccer technique and movement coach. I am 75 y.o. Now, numerous tests and over 3 years later, my most recent PF was apparently that of a fit fifty y.o. male. A doctor I had until recently was purportedly treating me for Bronchiectasis Uncomplicated. He notes: The patients Sprirometry is improving. I have suffered from acute chronic sinusitis since two consecutive bouts of serious "Flu", for which I didn't seek medical treatment.

      Delete
  61. Wonderful post. My father just received an abdnormal CT scan that shows scarring in the lungs. The radiologist has labeled it "IPF" but I am keeping my hopes up and will seek out the exact diagnosis from a Pulmonologist. It was heartbreaking to read "IPF" a few days ago but the more I research pulmonary fibrosis, the more I understand we have a long way to go. The next step is an appointment with your colleagues. In the meantime, your blog is giving me light in very dark times. It is truly appreciated.

    ReplyDelete
    Replies
    1. Hi Nina. I am glad your found my blog helpful. I hope the appointment goes well.

      Delete
    2. I can't believe it's been a year! We've been seeing Dr. Brenner since then and your colleagues have been amazing. I'm sure you've taken a peak at my father's biopsy, medical notes and so much more. Dr. Brenner has explained it's a team effort when it comes to this disease and especially for my father who is showing some not so classic results in his biopsy and scans. His PFT has improved slightly so we are holding on to that and the fact he's incredibely active from morning until night. More so than his children in their 30s! Hope you hold an info session for families soon so I may attend!

      Delete
  62. I had breast radiation in 1998 and ever since have had breathing problems, n
    But only on the right side (right bc, did) a lung coffee I saw told me I had lung scarring, and fibrosis and that part of my lung will not expand. Everytime I got a chest x-ray for five years 2010-2019 and two CT scans they always say no changes. But the coffee just looks at my results and shakeshis head. Needless to say I'm hunting for a new doctor. My question is, will this scarring from radiation spread like the one you are describing. I have difficulty getting mucus, phlegm whatever it is out from right side. But once it breaks loose, breathing if fine again.

    ReplyDelete
    Replies
    1. Excuse the typos, hopefully you understand it

      Delete
    2. I think that only your doctor can answer your question. A lot depends on what your CT scan looks like. I am sorry I can't be more helpful.

      Delete
    3. What do you mean when you use the word: "coffee" as in "lung coffee"?

      Delete
  63. hello, can you tell me the symptoms to look for when a patient with IPF is entering the "end stage" of the disease. her oxygen intake is set at 8 while sitting and 12 when moving. is that considered a high intake? she has become panicky, anxious and has been put on an anti-anxiety. the oximeter reads above 90 but I understand that is not always indicative of final stage IPF. I just don't know what to expect or watch for. Can you help me please? her appetite is fine and very little weight loss.

    ReplyDelete
    Replies
    1. Hi. Thanks for reaching out to me, and I am sorry for the delay in responding. I completely understand your concern and I am sorry to hear that your loved one is suffering in this way. Doctors don't have a definition for "end stage" -- largely because everyone is different in how they experience IPF and how IPF progresses. I also can't really provide any medical advice (sorry!) -- but please do talk to your loved one's pulmonologist for guidance. You may also wish to make an appointment with a palliative medicine doctor who can help manage symptoms. I wish you and your family all the best.

      Delete
  64. Dr. Lederer, my name is Daniel. Thanks very much for the valuable knowledge you're sharing with us.
    If I may, I would like to share my dad's situation and would be most grateful if you can share your thoughts and let me know what you think.

    My dad who's 52, about a year ago started facing difficulty breathing when doing too much, used to be a heavy smoker (quit last week when he learnt about his case) and lives overseas was diagnosed with lungs fibrosis (believe it's known as PF?). His doctor has explained after a few scans that around 65% of his lungs is still functioning. Could you please share your thoughts about this point, is that good enough?

    Furthermore, his doctor has prescribed him with medication to help and slow the fibrosis. However, if that doesn't work when he sees him again next month, he will need a type of medicine that is not available where my dad lives (known as Pirfenidone).

    My granddad passed away due to PF after spending a number of years on Oxagen. Could it be passed to my dad from him?

    Would being a smoker since an early age worsen his case quicker you think?
    He walks about 2-3k everyday and still rides a bike and drives his car around?

    Also, I am wondering if I can by any chance send you the scans sometimes soon for a double check?

    Thanks in adavance.
    Daniel

    ReplyDelete
    Replies
    1. Hi Daniel. Thanks for reaching out - sorry for the very late response. I am also sorry that your dad is going through this. Your questions are excellent. Unfortunately, I cannot give medical advice on the internet. Instead, I'll try to provide some medical information.

      Lung function tests (for example, a lung function test showing 65% functioning) can be useful to help us understand how mild or severe someone's lung disease is -- but it's hard to look at one number and know how mild or severe someone's disease is. for many people 65% suggests mild to moderate disease but for some people, this number could be misleading (meaning that the number suggests mild to moderate disease, but the truth is that the disease is more severe than the number). so i can't give you a good answer to your question about 65% lung function.

      You asked about whether PF can be passed down. In some cases, family members might have a somewhat increased risk of developing PF, but most family members will never develop PF. Anyone who has a family member with PF should be sure to mention this to their own primary care provider. There are no current medical recommendations on whether we should look for PF in family members, but a routine physical exam is always a good idea.

      Smoking does increase the risk of some forms of PF. and of course, smoking increases the risks of many different diseases.

      According to my employer, I can review a chart and scans for a fee. I am sorry that I cannot do it for free. please call my office for details. 212-305-8203.

      best regards.

      Delete
    2. Hi Dr. Lederer,

      Thanks for your reply.

      Could you please advise of a good time to reach you? A fee to review the charts is absolutely fine. I would be happy to DHL my dad's old and new scans for consultation.

      Best,
      Daniel

      Delete
  65. Hello doctor lederer, I enjoyed your blog even though it was written awhile ago. I recently came across it because I was just diagnosed with ILD. I am 29 years old (male) and have had dermatomyositis since I was 19. Just this past year I noticed some breathlessness and a slight cough so I went to the doctor and they did X-rays and thought it was slight pneumonia. Went on a week of anti biotics but my lungs still didn't feel quite right. Went back and they did more X-rays and said they didn't see any real changes between the X-rays taken a week or so apart. So they brought me in for a CT scan where they were able to see the inflammation and scarring. They put me on 80mg of prednisone and 2500mg of cellcept. Anyways my question more relates to the inflammation aspect of ILD. If for instance my ILD is caused by the inflammation going on in my body either from the dermatomyositis or my immune system, would reducing inflammation help slow the ILD? For instance having a food allergy panel
    done? Avoiding foods that are also aggravating the inflammation. I know it depends on which type of ILD I have but just in theory if my ILD is present because of inflammation would reducing inflammation throughout my body maybe help? Thank you

    ReplyDelete
    Replies
    1. Hi Jesse. Thanks for reaching out. I am unaware of any dietary changes that help ILD. I imagine that even if there were some foods influencing inflammation, the effects of anti-inflammatory medications would be far stronger. I've never seen someone need less medication for their ILD because they changes their diet.

      Delete
    2. Thank you for that reply I really appreciate it. A follow up question I have then is, due to my ILD being caused by my dermatomyositis if I am able to control the dermatomyositis that should also in turn slow or possibly halt the ILD correct? Because from what I understand with DM is my immune system is attacking healthy tissue so if I'm able to stabilize my immune system then wouldn't that also help with with the ILD? I know these are way different questions then my original post but just curious on your thoughts. Thank you so much

      Delete
    3. Everyone's condition is different. I can't give you a good answer to your question. Very sorry.

      Delete
  66. Thank you Doctor for such a good blog!
    My dad developed a small patch of Fibrosis in one of his lungs around 2010, at that time he had just been diagnosed with autoimmune hepatitis and was under treatment for it. Could his lung fibrosis be caused by his immune system just like his liver was at that time? Since then his fibrosis has not progressed and his autoimmune hepatitis has been controlled with no relapses. Could this mean that his lung fibrosis will continue to be non-progressive? (It has not progressed since then)

    ReplyDelete
    Replies
    1. Thanks for your question -- it is a good one, but I can't give you an accurate answer. I would suggest that you discuss this with his pulmonologist. I agree it is important to figure this out.

      Delete
  67. Anon
    I would welcome your thoughts please Dr Lederer
    My son is 52 with IPF
    I am his father aged 80 same height and weight and in good health only medication is amlopidine 5 mg prescribed 2 years ago.
    If a transplant is necessary is there any reason why I would be disqualified from being a donor?
    Thank you your blog is really helpful

    ReplyDelete
    Replies
    1. Good question. Almost every lung transplant performed in the United States uses one or two lungs from a donor who has died. It is extremely rare for a living donor transplant to be performed.

      Delete
  68. Dear Dr. Lederer,
    I was diagnosed with IPF exactly four years ago. My doctor said it was IPF (although I told him that my mother had succumbed to the disease after having it for 10+ years). The first doctor said I had 2 1/2 - maximum 3 years to live. Four years later, I am struggling for breath from time to time and coughing (dry and wet, since I have bronchiestasis as well) hundreds of times a day. I am totally fatigued, althouugh I am usually able to walk my dog 1/2 mile each day)! I just returned from a visit with another pulmonary doctor and after I took another spirometry exam, he said that he doesn't believe that I have IPF, but PF, since I am still alive four years later. He said that I don't need to be on oxygen anymore. However my last doctor told me that I needed to be on oxygen at 4 liters per minute for 16 hours per day. Does that make any sense? Meanwhile, all my doctors (I live in the Republic of Panama) have been prescribing for me Seretide Discus and Spiriva inhaling products which seem to alleviate some of the symptoms. I went for this new check up because, for the last month, I have been experiencing extreme dizziness and lack of equilibrium and, consequently, walk around like a drunken sailor. I figured it was caused by lack of oxygen in my blood from the PF. The doctor said the disorientation/dizziness was not caused by low oxygen since my oxygen level was measured at 98! He said that, in addition to my PF, I may have some other disorder and should visit a neurologist or an ear, noes and throat doctor. Do you concur, Dr. Lederer? I would appreciate any help you can offer since I am self-confined to my bed at this time since it is uncomfortable (and dangerous) being ambulatory. It is difficult to get answers down here living in a Third World country,

    ReplyDelete
    Replies
    1. Thank you for sharing your story. I recommend following your doctors order to have you seen by a neurologist. I am sorry to hear that your are confined to your bed. I hope you are not suffering terribly, and I hope the oxygen is helping you. All best wishes.

      Delete
  69. My father was diagnosed last week with IPF after fighting pneumonia for over a month. He was given Prednisone and the pneumonia has cleared. His oxygen stays in the mid 90's with occasional drops into the 80s. It usually returns to normal within 5min or less of rest. He has supplemental oxygen but his pulmonologist says he doesn't think he needs it. They told him that he has one small area of scarring on his right lung and no honeycombing. He is going to have pulmonary function tests done this week. The doctor told him there's a chance this may not progress fast and there's a chance he can live a regular life expectancy. Is there any chance his diagnosis could be wrong? He had a camera-assisted lung biopsy, ct, and x-rays prior to his diagnosis. He also has a history of triple bypass and sees his cardiologist regularly who advised that his heart is functioning normally.

    ReplyDelete
    Replies
    1. Hi. I wish I could give you a clear-cut answer, but I can't. Everyone's condition is unique, and the answer to your question depends on the medical details of your father's condition. If you are in the US, consider having your father seen at an ILD center. You can search for one here: http://www.pulmonaryfibrosis.org/life-with-pf/find-medical-care

      Delete
  70. Dr. Is all scarring progressive pulmonary fibrosis? I have linear scarring all over my lungs but coe says i am fine. All radiologists also say no pulmonary fibrosis. Is there scarring that shows up one day and never progresses?

    ReplyDelete
    Replies
    1. There are different kinds of scars that can develop in the lungs. Some are progressive and some are not.

      Delete
  71. Hi I am 53 years old diagnosed in April 2016 put on the list December 22 double lung transplant on January 2, 2017 . I waited several years because I thought it was just age And lifestyle . I am doing great still get winded but 1000% better than I was. Just had a sniff test which said one lobe on right side not working . Can't seem to get a straight answer from my team not meeting with mydoctor for two weeks. Is that a common problem that might fix itself

    ReplyDelete
    Replies
    1. Glad to hear transplantation was helpful for you. A "sniff" test can check whether your breathing muscle (diaphragm) is working well. Some people do have problems with their diaphragm after a lung transplant. I can't give you specific medical information about your condition (sorry!). I do recommend that you speak with your doctors about your test results and your breathlessness. all the best.

      Delete
  72. I am 53 years old woman from India. I have been in the teaching profession for the last 28 years.

    I was having cough and shortness of breath for the last two months. Otherwise I have always led a very active life.

    Last week I have been diagnosed with Fibrotic NSIP through HRCT and Lung Biopsy. Now I am taking my treatment from Apollo Hospital, New Delhi, India and on Defcort 30 and Dytor 10 mg.

    My concerns are whether I am taking right medicines, should I continue my job, is Fibrotic NSIP is equvalent to IPF...etc.Pl suggest. Thanks

    ReplyDelete
    Replies
    1. Thanks so much for reaching out and sharing your experience. I cannot give out medical advice over the internet, so I unfortunately cannot tell you whether you are taking the right medicine or not -- only your doctor can do that. I can tell you that fibrotic NSIP and IPF are considered to be two different types of pulmonary fibrosis, and the treatments can be different for each condition.

      Delete
  73. Hello Dr.David,

    I am 27 and recently was diagnosed with Polymyositis. Had a CT done of my lungs to see if I have lung disease and I tested 'positive' for pulmonary fibrosis; also was told my lungs are working at 50%.
    Right after I googled and read the 3-5 year lifespan; I assumed that can be real...seemed way too short.
    So thank you for your insight and knowledge on this; I really appreciate it. Now if i just have slight scarring on my lungs and am only 27, will I most likely need a lung transplant in the future?? And you used the 10-15 year life span...is it 'lucky', so to speak, for someone to live longer than 15-20 years???

    Thank you Greatly

    ReplyDelete
    Replies
    1. Thanks for your question. I completely understand your desire to want to know what will happen to you. I can tell you that I have seen people with small amounts of scar tissue who have little or no progression for years (even 10 year or more) with or without treatment, and I've seen others with a little bit of scar tissue become sicker within 6 to 12 months. In your case, I can't really know what will happen to you. Your doctor may be able to paint a range of possible outcomes more specific to your individual circumstance. I apologize that I cannot. I wish you all the best.

      Delete
  74. Thank you for being so prompt with your response. I appreciate your honesty and professional input.
    Glad to hear that it can be years before any progression starts, but I understand you can never really know 100%. It seems that people can live from 10 months to 10 years...and possibly more; but I guess we aren't really able to know. Well I will be having my first consult with a pulmonary fibrosis specialist in May, so we'll see how that goes.

    Again, thank you very much Dr.Dave
    Many Blessings

    ReplyDelete
  75. hi there i have minor ipf the dr said its only 6ml of it. is it something to worry about at this stage

    ReplyDelete
  76. My husband, 53 years old, was just "preliminarily" diagnosed yesterday with idiopathic pulmonary fibrosis (IPF). A winter infection that hit our household left my husband in a more severe state of shortness of breath. He was subsequently hospitalized for potential pneumonia; in the emergency room they did an X-ray that showed diffuse potential fluid areas, so they followed up with a CT scan. He was admitted for pneumonia and spend several days in the hospital. COPD was listed as a potential underlying factor. He was sent home with O2 and told to see a pulmonologist as soon as possible. Those appts are hard to get and so three weeks after his discharge, he saw a pulmonologist who had him do a series of breathing tests. My husband was expecting a diagnosis of COPD. The doctor told him he had pulmonary fibrosis (based on the XRay, CT, breathing test, and listening). My husband let out a surprised and breathy, "This is what killed my mother!" His mother died at 62 of IPF, 5 years after her diagnosis (circa 2010). His maternal aunt also died of IPF (circa 2007). Can the breathing tests over time give us a predictorial picture of what lay ahead? (He had a series of blood tests to rule out known reasons for PF).

    ReplyDelete
  77. My husband was just diagnosed with UIP from a biopsy. Do you have any information regarding this disease? He is 50 and has no symptoms. The only way we found out he has this is he went in for CT of his kidneys because of kidney stones and they noticed the lower lobe of his lungs to be inflamed. We had a CT of his lungs and were asked to do a biopsy to confirm the results. It came back that it is UIP leaning more to fibrosis. What do we have to expect now? everything that I am reading is heartbreaking. He is very healthy and active. He walks 3 miles 3 times a week and goes to the gym to lift weights as well the other 3 days a week. Please I would appreciate any information that you can give me. Also we have an appointment next week with the pulmonary doctor. Do you have any questions that I should ask our doctor at that time?
    Thank you
    Carol

    ReplyDelete
    Replies
    1. The diagnosis is IPF with UIP. The pathology report although states most likely UIP. We were informed that to get a 100% UIP is almost rare. Any comments or advice to give?
      Thank You
      Carol

      Delete
  78. My HRCT scan for chest on 64 slice scanner is as follow Minimal bilateral apical subpleural fibrotic changes and tiny subcentimeter sized nodule in anterior segment left upper lobe ? Old etiology


    Do I have lung cancer or some serious health issue.

    ReplyDelete
    Replies
    1. Hi. I am unable to make a diagnosis over the internet (so sorry!). Please discuss your CT scan findings with your doctor to find out if you have a medical condition that needs attention.

      Delete
  79. Hi Dr. Lederer,
    Does mild scarring and a small amount of honeycombing seen on a CT at the base of the lungs mean you have IPF?

    ReplyDelete
    Replies
    1. Hi. Sorry for the delayed response! We define IPF as the presence of a very specific pattern of scarring in the lungs. Only your doctor can give you a diagnosis. Sorry I could not help more.

      Delete
  80. Hello Dr. Lederer,
    My 80 years young father passed away 3 weeks ago and I'm struggling to understand why it happened so fast. Approximately 2 years ago he was diagnosed with vasculitis which was primarily affecting his kidneys. His doctor prescribed a combination of steroids and chemo to treat the vasculitis. From this he developed diabetes and had to be put on insulin. In January of this year he started having trouble breathing and tired easily while walking so his doctor referred him to a pulmonologist. The pulmonologist sent him to the hospital and ultimately diagnosed him with pulmonary fibrosis. He was subsequently put on 3-5 liters of oxygen in early February. Last month he was admitted in the emergency room because he was having an increasingly hard time breathing. In the hospital they were administering 20 liters of oxygen. He was there for almost 2 weeks and was transferred to a skilled nursing facility on May 20th. He only spent 1 night at that facility and I stayed with him the whole time. During this stay, at about 3:15 a.m., on May 21st, his massive oxygen tank ran out of oxygen. It took 3 nurses about 10-15 minutes to switch out the tank and in the meantime my father was in distress because he couldn't breathe. Needless to say, I demanded that they move my father out of that place and transfer him to my brother's house. This was done by 12 noon that same day. When the ambulance crew transferred my dad from the gurney to his hospital bed, my dad looked extremely weak and I knew that episode at the nursing facility had taken a toll on him. He fell asleep after a dose of morphine and never awoke until he passed on May 24th. Do you think it Is likely that he would succumb to the pulmonary fibrosis so quickly (within 4 months) or did being deprived of oxygen during that horrible experience at the nursing facility hasten his passing? Thank you.

    ReplyDelete
    Replies
    1. I am so sorry to hear about your father's experience. My heart goes out to you and your family. I know you are looking for answers, and I wish I had them. Unfortunately, I cannot make a medical determination about what happened (or what would have happened), since he was not my patient and since I am not in a position to know the details of his medical case. You may want to reach out to the Pulmonary Fibrosis Foundation about the work they are doing, particularly their "daughters" program (which is designed for both male and female family members, despite the name): http://pulmonaryfibrosis.org/our-role/signature-programs/pff-daughters. They also have a searchable index of support groups.

      Your dad is in my thoughts today.

      Delete
  81. My dad was diagnosed with ipf in May 2016 and June 18, 2017 he passed away. It seemed to happen so fast. He used oxygen at home occasionally. On Thursday he woke my mom up, he was struggling to breathe, she called an ambulance, in the hospital they said he had pneumonia and started him on antibiotics. Sunday I asked for a transfer to Baylor, they are supposed to be a lung specialty hospital but they said he wasn't stable enough to be moved and should spend 24 hours in icu, 30 minutes later, during the move to icu his oxygen mask was removed while waiting for them to put it back on, they "said" he went into cardiac arrest, he passed away. We are devastated, don't know what really caused his death and can't get any answers. When he woke my mom up on that Thursday morning he told her wanted a lung transplant, which he previously said he didn't want. I think the struggle to breathe was way worse by then and I can't even imagine how scary it was to struggle that much. Just a daughter's advice...don't wait for a dr who doesn't seem concerned "right now" go somewhere else, anywhere else, and don't put off any treatment, even a transplant since it seems to take longer when you are on a waiting list, we thought he still had time. I miss him everyday.

    ReplyDelete
    Replies
    1. I am so sorry for your loss. And I am saddened that you dad suffered in his last days. Thank you for taking the time to share your experience here. Your advice is right on target, and I hope your words here will help others.

      Delete
  82. Husband diagnosed with NSIP lung pattern. Doc does not want to put him on any meds because he is stable. Says meds might do more harm then good. Doc feels that he might have plateaued with this disease and that he may remain like this for years. I would like your take on this. Does it plateau and remain? is no medicine good or bad idea? I just would like to hear your perspective about it.

    ReplyDelete
    Replies
    1. Hi and thanks for reaching out. In short, there is no "correct" way to manage NSIP. Some people need treatment right away, and others don't. Yes, some people do remain stable for a while. Many folks look for another formal opinion - you can find a list of ILD specialty centers on the PFF website: http://www.pulmonaryfibrosis.org/life-with-pf/find-medical-care

      Delete
  83. Hi Im very glad I found your blog. I have a question I smoked for many years. I have had pneumonia many times I have scaring now my diagnosis is mild bronchial cuffing and fibrosis....please explain this to me is this the fibrosis your blogging about? Maria

    ReplyDelete
    Replies
    1. Hi Maria. I could only answer your question if I saw your CAT scan myself. Your pulmonologist might be able to tell you whether this is "pulmonary fibrosis" or something else. Sorry I couldn't be more helpful than this. Best Wishes.

      Delete
  84. This comment has been removed by a blog administrator.

    ReplyDelete
  85. My father was diagnosed with UIP last week, and i was very worried because i see poor prognosis is been mentioned everywhere. He got exposed to dust 7 months ago and had to hospitalized for few days but he got recovered. He had a mild cough since then and HRCT showed honeycombing and ground glass appearance in both lungs (R>L). I believe the dust was the course. He was very healthy, did exercise regularly, but now his is mildly breathless. he is on pirfenidone 200mg now as his doc prescribed it. But he is not talking oxygen yet.
    He is 64 years now and he is diabetic. i wonna know whether lung transplantation possible at this age??

    ReplyDelete
    Replies
    1. Thanks for sharing your father's story here. Lung transplantation may be an option for him. The only way to be certain is to have his doctor send his medical records to a lung transplant program to initiate an evaluation. Each transplant program uses different criteria to decide who they will and won't put on the waiting list. Sometimes it is even helpful to seek evaluation at two or even three or more transplant centers. I wish you and your family all the best.

      Delete
    2. Thank you very much for your quick reply..

      Delete
  86. I know you do not give a diagnosis on this website but I would like YOUR professional opinion about a diagnosis of NSIP. The overall distribution of the disease was fairly homogenous in the lungs but there is definitely areas of honeycomb change predominantly in the upper lobes. Is honeycomb a definite diagnosis of IPF or because it is in the upper lobes could it be NSIP. Basically my question is, can you have cellular and fibrotic NSIP pattern with upper honeycombing in the lungs? or is the honeycombing an indicative diagnosis of IPF, no matter the location of it? Please, I would like your professional take on it.
    Every autoimmune test negative as well as hypersensitivity.

    Thank you!!!

    ReplyDelete
    Replies
    1. Honeycombing on a CAT scan of the chest can be seen in a number of ILDs, not just IPF.

      Delete
  87. Hello Dr. lederer,

    Everything points to the fact that honeycombing is the last stage of lung disease. If there is honeycombing in one area, does it spread? Is it just a matter of time till the end when honeycombing is present or can we stop it from progressing.
    Thank you in advance for your reply.

    ReplyDelete
    Replies
    1. This is a tough question to answer. "Honeycombing" does not mean that the lungs are full of scar tissue -- this is a common misconception. Honeycombing is a word doctors use to describe areas of the lung where scar tissue has completed replaced the normal lung tissue in that area. Sometimes there might just be one tiny bit of honeycombing in the lung. Other people may have honeycombing in many areas of the lung. To answer your question, finding honeycombing in the lung does mean that it is the last stage of the disease. Honeycombing just means scar tissue.

      Delete
  88. Can a diagnosis of NSIP later turn into IPF. If it does, would you say that it was misdiagnosed in the beginning? Or does it evolve in some people to IPF?

    ReplyDelete
  89. Hi Dr. Lederer,
    My 45 year old husband has newly diagnosed lung disease from chemo drug Bleomycin. I'm not sure what disease he has just yet but he's been on prednisone at 60 mg and is weaning now at 20 mg. It's been a rough wean for him though. He is no longer using oxygen daily but with latest wean of prednisone his pulseOx is starting to drop more frequently. I've read some interesting articles on the development of stem cell therapy treatment for lungs now and was hoping this could maybe be something he could try. Do you have any background on this with others? I know it's new.

    ReplyDelete
    Replies
    1. Thanks for reaching out. I am sorry to hear about what your father is going through. There are no known effective stem cell therapies for interstitial lung disease or pulmonary fibrosis. The FDA has begun to issue warning to centers that are performing harmful and unproven stem cell "treatments" (https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm573443.htm)

      Delete
  90. Dear Doctor,

    Yours in indeed a well written and informative article.

    I wanted to discuss the case of my father to you. My father had dry cough since January 2016. However it would come and go and he was taking medication for it as well. In Nov 2016 we also got his entire body checkup done with a Chest Xray which was all fine. However in May 2017 along with persistent coughing he also started getting very breathless. On getting a fresh Xray and HRCT done our regular physican sent us to a Chest Physician. The chest physician explained to us that he has fibrosis in his lung and he will need to undergo a bronchoscopy. My father by then was not fit for it and as my mother was also recovering from her own illness we decided not to go for it. He however told us that the treatment is the same and it looks like Sarcoidosis. He took Wysolone 50 mg for a month. His symptoms had however vanished in a week. But after a month when the time came to taper off the steroids his symptoms relapsed when he was at Wysolone 20 mg. On visiting the doctor he prescribed an increase in dose. However that too did not help him. When his condition deteriorated in August he was advised to be hospitalised. He was then on 4 litre Oxygen. Although his cough went away his breathlessness was the same. On doing another HRCT scan the doctors told us that his days were numbered and his lung damage had drastically spread.
    WE did not understand how the lung damage rapidly spread in just 4 months. Is there something wrong we did? Is he allergic to something that caused this? Kindly assist.

    He is currently on 8 litre oxygen and totally bed ridden. I wish to understand if he could undergo stem cell treatment and will need assistance on the same.

    ReplyDelete
    Replies
    1. I am sorry to hear that your father has been going through this. Sometimes fibrosis worsens rapidly without a clear cause. I can't imagine it was something you did. There are no stem cell "therapies" that work for pulmonary fibrosis. The FDA was issued a warning to centers that offer these unproven (and in some cases unsafe) "treatments": https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm573443.htm

      Delete