Sunday, June 22, 2014

A good article on prognosis

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.


But, before I get to the myth of prognosis, 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 really sort out exactly which kind of Pulmonary Fibrosis you have, then go get yourself an appointment tomorrow.Check out the Pulmonary Fibrosis Foundations website for a list of ILD centers. 

Ok, if you’ve been to an ILD center and the team there said you do indeed 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.

Posted by at 
    

45 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

    Reply
  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.

    Reply
    Replies
    1. What medication(s) is/are routinely prescribed for IPF now?
    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!
    3. Has any of these drugs come out on the market? Do you know any answers yet? 

      Thank you

    4. There are still no drugs approved by the FDA to treat IPF. Hopefully we will be hearing something this Spring.
    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
    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.
  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.
    Reply
    Replies
    1. Thanks for your words, Bill! Keep up the great work on spreading the message about IPF.
  4. My mother had it. She lived 5 years with it.
    Reply
  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

    Reply
    Replies
    1. Thanks for your questions. I will indeed discuss the different types of "interstitial lung disease" or PF in an upcoming post.
  6. I am so glad to find this blog, Dr. Lederer. Hope all is well.
    Reply
  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.
    Reply
    Replies
    1. Hi Taleena. Thanks for sharing my blog on the Breathe Support facebook page. Very happy that you found this information helpful.
  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
    Reply
  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
    Reply
  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

    Reply
    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

    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
  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??
    Reply
    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!

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

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

    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.

  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

    Reply
    Replies
    1. Thanks! And you are totally right!
  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. 


    Reply
    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!
  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.
    Reply
    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

  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.


    Reply
  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

    Reply


Subscribe to: Post Comments ( Atom )
Posted by at

No comments:

Post a Comment

Subscribe to: Post Comments (Atom)