Thursday, August 22, 2013

Why a blog about pulmonary fibrosis?

Why a blog about pulmonary fibrosis? You already know why.

There is a world of confusion out there about PF. What is it? What's the difference between IPF, PF, and ILD? What causes it? Will my kids get it? Which treatments work? Should I try stem cells? prednisone? NAC? Do I need a lung transplant?

Top that off with the nearly universal problem that in the months and years before you are finally told that you have PF, you were probably told that your cough or difficulty breathing was due to asthma, COPD (chronic obstructive pulmonary disease), emphysema, heart disease, or sleep apnea, or that "you just need to lose weight and exercise." 

It's no surprise that just about every single one of my patients with PF is frustrated and confused when he or she walks in the door. 

I created this blog to try to clear up the confusion about PF. Here we go...

15 comments :

  1. Thank you sooooo much for starting this. My husband was diagnosed with IPF in mid July by a lung specialist. Prior to meeting with the doctor they did a Lung Function Test. He then discussed with my husband what IPF was, showed him on the ex-rays and his CT scan the things that also indicated it. It was the one occasion I didn't go with him. He's been to see a number of specialists for different things and that first appointment typically is just a meet and greet and he'd leave with a requisition to have a bunch more tests done. How sorry I am that we made that assumption this time as my husband had an extremely hard time taking everything that he heard in. It was a total shock for him as what they thought was going on was a stubborn case of pneumonia or some other lung infection.

    While he was there the receptionist booked a Bronchoscopy for him (coming up on Sept.16th) and then a follow up meeting with the doctor to discuss the results. Unfortunately that appointment isn't until Oct. 3.

    Naturally the first I did when he told this was do a "google" search. Big mistake as everything we read and could find to read was very depressing. Since then I have found only one site that talks about the survival outlook based on some the things you have already mentioned.

    Your posts have been by far the easiest information I have found on this topic and it's reassuring to know that there is a spectrum of types and mortality rates. At this point my husband is completely convinced that he has been misdiagnosed and that the Bronchoscopy will prove that out. I can understand his "denial" as at this point we still don't know very much about the state of my husband's lungs etc.

    Again, thanks so much for doing this. I know that there is a whole community of sufferers and their family/caregivers scratching there heads and feeling totally overwhelmed by it all.

    Shelly Rasmussen

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    1. Hi Shelly,

      I am sorry to hear about your husband's situation. I hope everything turns out ok for him. Thanks for sharing your thoughts about my website. I will be posting more information every few days.

      David Lederer

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  2. Hi David

    Thanks a lot for starting this wonderful blog. I lost my dad to IPF in March 2013. I'm from India and to be honest doctors in India aren't very clued up on IPF (or, they don't take the time to guide patients right). Most of my information on IPF came from rigorous online research.

    In India options also tend to be limited. While Pirfenidone (still under clinical trial in the US...) is approved for usage in India, it's not very effective. Also, lung transplants in India have just started about 2 years ago so the technology/techniques are very new and the teams are not very experienced. We have a few hospitals in the south performing Single Lung Tx but so far even 6 month survival rates have been in the low 70's. (72% last time I checked..)

    I started a blog on pulmonary fibrosis (www.pulmonaryfibrosis.in) to share information and to create a sort of support group for Indians who have this dreadful disease. I'll be following your blog and advice closely as well so that I can maybe share it with some of my readers. It's nice to have a qualified voice talking about IPF as I am not a medical professional.

    Thanks for creating this blog!

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    1. Hi Ashray,

      Thanks for commenting on my blog. I am sorry to hear about your father's battle with IPF.

      I enjoyed your blog. Keep up the good work!

      David

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  3. Dr David. This is a wonderful thing that you are doing and doing it so well. You are writing for the audience you are reaching. I am very fortunate in that I have been going to the Toronto General Hospital's ILD clinic since being diagnosed almost 3 years ago -- I wanted to be with the experts within this field and my doctor is marvelous. Not everyone has been so fortunate and I really feel that you reaching out in this way will fill a gap that is larger than the Grand Canyon and help thousands of people world-wide.

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    1. Thanks for your words of encouragement. I am very happy to hear you found a great doctor. That's a very important part of puzzle when dealing with pulmonary fibrosis. Best of luck with everything!

      David

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  4. Could you perhaps do a discussion on weight and transplant. Many people when they hear they have to lose weight in order to get listed tend to view it as punishment and exclusion without understanding the reasoning behind the need to be at a more ideal weight. There are of course the interim benefits with breathing but the increased transplant risks such as graft dysfunction. Me, I view it as positioning myself as the best possible candidate for transplant with the best possible outcome.

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    1. I absolutely will, Barbara. I just put up a post about lung transplantation -- It's an overview of the prospect of lung transplantation for people with Pulmonary Fibrosis. I plan to put up a few more posts about lung transplantation, including a discussion about indications and exclusions, including body weight.

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  5. Could you comment on post transplant and the challenges they face day to day? I had a fantasy, that I would return to a pretty normal life. I have in some ways, but there is always something to manage. Is all of this pretty normal? Energy particularly, wax and wanes. I am very active, but some days I am just knocked out. I am 4 + yrs out. Thank you

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    1. Thanks for your question. Everyone is different, so it's hard for me to comment specifically on your situation, since I don't have the details of your condition. Low energy is common after lung transplantation. Sometimes this is due to effects of the medications you have to take. Sometimes this is related to sedentary lifestyle and lack of exercise. Sometimes this is due to medical conditions (such as anemia, thyroid disease, depression, and other sometimes more serious conditions) that need to be evaluated and treated. I recommend that you speak to your transplant doctor about your waxing and waning energy level and see if there is anything that can be done. In some cases, people might begin to have more energy after working out some of these problems. I hope things improve for you.

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  6. Dear David,

    Today I breath a sigh of relief after seeing your blog, from the past three months I have been hunting for information from the net and really confused. I am Swati from India and my father has been diagnosed with IPF just before three months, he is in his early stage of fibrosis, he had dry cough and weight loss before medication. He is now on mucinac 600 and Pirfenex 200. His coughing has disappeared and weight has not improved but is not rapidly decreasing. But just 15 days back he was having problems like short term memory loss, confusion, insomnia, sometimes blurred vision.. He has been having Hypothyroid since 40 years and suddenly now his TSH is 0.06(hyperthyroid). Are these symptoms because of his thyroid problem or may be related to IPF.
    At this stage I just want to know how fast or slow the progression of IPF is in my father's case. What are the factors to determine. Truly I want to know how long will my father live. HELP.
    Thanks and Please reply
    Swati

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    1. Hi Swati. I am very sorry to hear about your father's condition. Your question about how quickly your father's condition will worsen is an important one, but it can be a difficult question to answer. Each person experiences their own unique illness as a result of Pulmonary Fibrosis. I wrote about the challenges of predicting the future in Pulmonary Fibrosis in a previous post (http://www.pfdoc.com/2013/10/the-internet-said-i-have-3-years-to-live.html). I hope you find it helpful.

      Additional helpful resources available online are:
      The Pulmonary Fibrosis Foundation: pulmonaryfibrosis.org
      Pulmonary Fibrosis in India blog: http://www.pulmonaryfibrosis.in/
      Online patient and caregiver support groups: http://www.inspire.com/partners/pulmonary-fibrosis-foundation/
      Dr. Jeff Swigris' Blog at PF: http://pulmonaryfibrosisresearch.org/

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  7. Would you consider doing a blog on cause? I understand what ILD is but I also understand that not all of the ILD types develop pulmonary fibrosis.

    I feel that the myriad of questions and tests searching for a "cause" is two-fold.

    1. classifying the type of ILD and therefore helping to determine treatment, prognosis, etc. If the cause is an automimmune disease, then treating the underlying disease.

    2. elimination or avoidance of the cause in the hope of stopping or at least slowing down the progression.

    For #2, is this only a reasonable expectation when pulmonary fibrosis is involved or most likely to develop?

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    1. Yes! I will do definitely do that. Sorry I haven't been posting too much lately. Bogged down in work. I also hope to post about the pirfenidone results shortly. Thanks for being such an an active voice here and elsewhere in the PF community.

      David

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  8. The ASCEND results post would be great. And I understand work takes priority.

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