Monday, March 28, 2016

What do people living with pulmonary fibrosis need to know?

I am putting together information for folks living with pulmonary fibrosis. Please consider sharing your list of things you wish you knew when you were diagnosed with pulmonary fibrosis. You can post it on my blog, on facebook, tweet it, or email it. 

Thanks!
Dave Lederer

18 comments :

  1. 1. Get a GOOD pulmonologist who specializes in ILD and not accept one who is a respiratory specialist. This comes with time and talking to people on Inspire!
    2. Ask the BIG question - what can I do to help myself/partner!! Simple things like - get an oximeter, get to Pulmonary Rehab. This should get you started until you learn more yourself and from No 3 below and start to ask more questions!! If you don't get any help - get rid of them and find another ILD specialist.
    3. Join Inspire.com, they are the most amazing supportive group of people you can ever hope to meet! no question to them is ever too big or too small - they will always answer you honestly according to their experience; and according to their experience, they will also tell you whatever happens is different for each and everyone of us. You will learn everything you need to know, especially at 2am when there is no one around and you are having a bad day/night with disease or worry. Very supporting to the carers too!! I just love them!!
    4. Don't expect your GP to know the answers - they don't! If a pulmonologist doesn't know, how can they? But do make sure you get a doctor who CARES!!!
    5. As a carer - look after yourself too and learn to accept and respect the patient's wishes and limitations.
    6. a good PDF file which explains spirometry readings - abbreviations, what each reading is telling about the patient, ranges and their results, which ones are most important to look for and when to worry!!

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    1. Dear Dr. Lederer: When I was first diagnosed, I wish my doctors mentioned that IPF is not a "one size fits all illness." Second, I would like to see some hard core, blunt posts about the importance of good nutrition, exercise, and a good night's sleep are to our wellness. And ones discussing the serious, negative impact (damage) obesity, lack of exercise etc. do to me/us physcially.

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    2. Yes I like to know if you know a doctor Natalie I have a pulmonary fibrosis and I need a specialist and I wonder you fall into that category

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    3. I was diagnosed with pulmonary fibrosis about 13-14 years ago when I had double pneumonia everything was well until the last two years where my oxygen level drops when I walk to about 85 when I sit down its about 94 my blood oxygen is 92 my pulmonary doctor says I have Progressive pulmonary fibrosis in both lungs and inflammation on the right lung then he needs to do a biopsy to find out what treatment I need. I don't feel shortness of breath unless I walk for a long time are exercise on a treadmill oxygen drops otherwise I feel good unbeknownst to me for three months I was exercising in the gym running for 6 miles lifting weights and using the StairMaster and bicycle I feel good when I got off shortness of breath but not real bad and then I will get my brother real quick so I had my heart checked doctor said my heart is good real strong and I had a can I scan with dye is show information on my right lung and my left lung progression of pulmonary fibrosis

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    4. I wish the doctor had told me what pulmonary fibrosis was and that I needed to be checked every year because I'm not knowing what it was and not being checked every year there was a seven years been where I did not have my lung check.

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    5. Yes I like to know if you know a doctor Natalie I have a pulmonary fibrosis and I need a specialist and I wonder you fall into that category

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    6. You can search for a medical center here: http://pulmonaryfibrosis.org/life-with-pf/find-medical-care

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  2. Hi Doc
    What I want to know is whether a biopsy is necessary to confirm a diagnosis and what are the risks.
    First I was told I had a PF in 2012 in Morocco treated with Prednisone for 3 years then I went to Belgium for a second opinion I was told that it's rather Sarcoidosis . I had a scan then a Pet scan for the whole body in 2016 and the doc said that he still needs to do a biopsy for a final diagnosis. I have no cough, no pain , no need for oxygen but an extreem fatigue and SOB after effort. What should I do ?
    Please Doctor Leadrer I need your advice

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    1. The best way to figure out if a biopsy is needed is to see a doctor who specializes in interstitial lung disease/pulmonary fibrosis. I am not certain where you are in the world, but you can find medical centers around the world specializing in this condition at http://pulmonaryfibrosis.org/life-with-pf/find-medical-care (be sure to click the orange "international search" text if you looking for medical centers outside of the U.S.) Best of luck to you.

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  3. Dr. Lederer,
    When I was diagnosed, I already had some knowledge of the disease because a brother and sister were diagnosed before me. While I appreciate your mantra, "No one is average. Demographics are not destiny. Doctors are not fortune tellers," as the positive message it is, but I would appreciate more information on outcomes. I am a scientist and I make many life decisions with data. The lack of data about disease progression is frustrating. An analysis of PFT variables (e.g., FVC, DLCO) over time of the IPF population, for example, could be enlightening and allow reasonable planning for what's ahead. It would permit a person to see where one might fall on an outcome curve (because everyone is not average, but some people are above and some people are below, and we should have a right to understand that if we want). I am sure this information is out there because many papers seem to reference statistics that must be derived from the data. Nevertheless, the raw data is hard to find. Thank you.

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    1. Great comment! I answered you here: http://www.pfdoc.org/2016/03/i-would-appreciate-more-information-on.html

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  4. Dear dr Lederer, would like to know whats the difference on CT scan between IPF fibrosis and fibroses of hypersensitive pneumonitis. We live in a country where the medicine is not good, so every doctor says different. I got ct scans made every year and every doctor reads and sees different things on it.
    Is there any new treatments for HPF apart from steroids and Mycophenolat Mofetil? Thank you!

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    1. Thanks for your question. IPF and the chronic form of "hypersensitivity pneumonitis" can look similar on a CT scan. When the CT scans are performed as "high resolution CT scans", then doctors can sometimes find certain clues that can help distinguish one from the other. But doctors also use other clues to help distinguish one from the other -- largely based on your responses to our questions about your health and exposures in your environment, plus the sounds your lungs make when you breathe in and out. Sometimes we also perform a biopsy of the lung to sort things out. There are no specific treatments that have been proven to be effective for hypersensitivity pneumonitis. In addition to trying medications to weaken the immune system, many doctors will try to find and eliminate the trigger from your environment. The trigger is sometimes exposure to mold, birds/other animals. Please talk to your doctors about which treatments might be right for you. Best regards.

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  6. Thank you Dr. Lederer. Never having been chronically ill before in my life, the diagnosis of IPF came as a shock. One day you are fine and then you find yourself never the same again. It's as if someone flipped a light switch with your life. This illness is unlike any other. So many courses of treatment, tests, diagnosises, and hopefully you get it right. What works for one patient does not necessarily works for another. Be ready to be your own advocate as you have to do the asking of questions that most docs do not know the answers to. You are so right regarding going to a Center of Excellence. I am seeing a wonderful doctor at the Cleveland Clinic and feel so much better now that my questions are being answered. I guess my main concern is the emotional toll this illness takes from you. You just get through a rough bout of shortness of breath, coughing, etc., then you get a cold, break a rib, the list goes on. Some days are good days and some are bad. When the good days come, my goodness it's the best ever!!! Do you have any advice to help rise above this? Thanks.

    Sandy

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    1. Thank you so much for sharing your thoughts and experience. You are totally right about the emotional toll of PF. I first suggest that you talk to your health care team about strategies to cope with the very personal effects of living with PF. Talk to them about whether mindful meditation might be helpful -- take a look at Jon Kabat-Zinn's website for more information: http://www.umassmed.edu/cfm/. Others find individual or group psychotherapy helpful. Many find PF support groups helpful: http://pulmonaryfibrosis.org/life-with-pf/support-groups. Sharing your thoughts, fears, and experience with your loved ones may also be therapeutic.

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  7. Re your list of things people wished they knew. First I wasn't told. Next time I was told something there but we can't treat it so don't worry, then I was told some scarring - probably from radiotherapy. Finally I was told pulmonary fibrosis probably from radiotherapy. I never had any inkling it was serious until a physiotherapist let something slip. The rest I found in a week of comprehensive Internet searching. My fibrosis has spread from the upper left lobe to across the bottom of both lungs. My main symptom has been steadily increasing breathlessness. All the doctors above were specialists I have now managed to secure an appointment at the Alfred Interstial (sp?) Clinic at the Alfred Hospital in Melbourne - all the PF research in OZ is pretty much generated there I will be able to get perfenidone and who knows what else. I am going to give NAC with C and E a trial. I have been taking Serreptase I* haven't noticed much difference. So far the best has been exercise (P rehab) Exercise is like magic

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