Thursday, September 5, 2013

The Pulmonary Fibrosis Checklist: Do these 8 things!

This is a quick post to introduce the Pulmonary Fibrosis Checklist. If you've been diagnosed with any form of pulmonary fibrosis, talk to your doctor about these 8 things...

The Pulmonary Fibrosis Checklist

  1. Use enough oxygen to keep your oxygen saturation >90% 24 hrs/day.
  2. If eligible, participate in pulmonary rehabilitation.
  3. If you have idiopathic pulmonary fibrosis (IPF), consider treatment with pirfenidone or nintedanib, and avoid long-term use of prednisone (updated January 23, 2016)
  4. Consider a sleep study to check if you have sleep apnea.
  5. See if you are eligible to enroll in a clinical trial of experimental therapies.
  6. Obtain a consultation from an ILD Center for help establishing your specific diagnosis and providing management advice
  7. See if lung transplantation is an option for you. 
  8. Visit the Pulmonary Fibrosis Foundation online for information, resources, and support.
What do you think should be added to this list?

I will focus on each one of the Checklist items in future posts.

24 comments :

  1. I was diagnosed with IPF in May 2013 - since modern medicine really has nothing to offer me in terms of treatment, I have been working with a natural therapist, as well as a 105-year old retired osteopath and physicist, who has been researching natural (Native American) therapies for over 60 years. My treatment includes natural anti-inflammatories, natural immune system support supplements, hydro-colon therapy, and an extremely high alkaline diet, with no wheat, no dairy, no sugar, no caffeine, and no alcohol. My follow up with my pulmonologist showed a "marked improvement" in lung function. What are your thoughts regarding diet and natural therapies?

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    1. Elaine, Do you have an email address? I would love to talk to you about your supplements as I feel my husband (who has ipf) does extremely well on the diet regimen and herbal supplements. Just like to compare notes. My email is tammy.stafford@hotmail.com. Thanks!

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  2. Oh - and PS, Dr. Lederer - thank you a million times over for publishing this blog - the information is so much more clear and understandable than other sites I've found - and I think I've found them all! Nearly all of the information out there is extremely depressing. By the way - UA at Birmingham is doing some interesting research with Fasudil as a protein inhibitor, enabling the apoptosis process to take place in the lung. What are your thoughts on their research - it sounds promising - the most promising I've seen so far? Thank you again for such a great blog!

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  3. Hi Elaine,

    Thanks for your comments on my blog. I am so happy that people are finding this information helpful.

    The research being done at UA Birmingham is indeed exciting - I agree. But, as with most research that hasn't been tried out in people yet, we won't really know if treatments that target this particular protein will help people with PF. Many drugs that seem to work in the laboratory have failed when studied in people. But don't lose hope! There are a number of exciting possible new treatments (including the one from UAB) that might turn out to work for PF!

    I can't make any specific recommendations for you about the natural therapies you are pursuing. I encourage you to speak with your health care team about their role. I can tell you that I do not recommend these therapies for my patients in large part because we don't know the benefits and harms of these therapies. I also always have concerns about the out of pocket costs that are charged for some of these therapies.

    Best of luck with everything.

    Dr. Lederer

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  4. Dr. Lederer I'd like to thank you for this blog.
    I was diagnosed in 2006 w/scarring (The Pulmonary Dr. told me not to worry about it) and make a long journey short I had a biopsy this year that confirmed IPF. I believe my Mother died from this disease. In reading all that you have posted I wish once someone is diagnosed that the Pulmonary doctors who do not specialize in IPF would send their patients to an ILD Center immediately. It has taken me since 2006 to even get to where I am today and that is not right. The word needs to get out to those of us who have this disease. My current Pulmonary doctor did not recommend an ILD Center, instead he gave me prednisone, NAC and this week azathioprine which I will not be taking.
    I researched and found the PFF website and found online support groups that lead me to seeking out an ILD center. I have an appointment in October.
    This website I found through a breathe-support post. What you have posted here has confirmed that I am doing the right thing in seeking a COE. I like my Pulmonary Dr. but I'm frustrated at the process
    Thank you Dr. Lederer for the information here.

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    1. Thank you so much for sharing your experience with pulmonary fibrosis. You have described a very common experience for those suffering from this disease. Very happy to hear that you are being seen by a COE and using the PFF resources on their website. Best of luck!

      David

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  5. Has there been any update to the Panther trials especially in terms of the triple therapy arm (Prednisone, Azathioprine, and N-Acetylcysteine). I've been hearing that some pulmonologists (not necessary PF/ILD experts) feel it was the high dosage of prednisone that was the problem.

    My ILD expert won't even touch Prednisone and Azathioprine together for my IPF. He had no problem with me trying NAC but I tried it for 6-weeks and didn't notice any difference either during or after I stopped.

    I respond extremely well to short high to tapering dose of prednisone for exacerbations and that is the only time we plan to use it -- to get me back on my feet and functioning again but not as maintenance.

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    1. Hi Barbara,

      Terrific question! There will be an update soon from the PANTHER-IPF trial, which will hopefully finally answer the question of whether or not NAC (N-Acetylcysteine) helps people with IPF. Once the trial results are announced, I discuss the results (in a way that everyone can understand!) in a post.

      I will also discuss prednisone and other treatments in an upcoming post.

      David

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  6. Are visits to ILD Centers primarily for those who have IPF? I ask as my PF is secondary to Mixed Connective Tissue Disease (Scleroderma and Lupus). I see a local rheumatologist and pulmonologist and am now wondering if I should be going to an ILD center.

    Thank you for sharing your knowledge with us through this wonderful blog!

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    1. Thanks so much for your kind words!

      Yes, patients with all kinds of pulmonary fibrosis (not just IPF) are seen at ILD Centers. You can find a list of ILD Centers on the Pulmonary Fibrosis Foundation website: http://www.pulmonaryfibrosis.org/MedicalCenters

      Best of luck!

      David

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  7. Perhaps in a future post you could expand upon pulmonary rehabilitation such as the benefits, what to look for in a good program (education and exercise component), etc.

    Also why don't pulmonologists refer patients to rehab as a matter of course? In some cases is it because in order to qualify, that is have the rehab paid for, you need to meet certain requirements and the patient isn't anywhere near that yet?

    I personally felt I totally benefited from rehab. I was aware of pulmonary rehab basically from diagnosis almost 3 years ago and should have pushed for it sooner instead of at the 2 year mark.

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  8. Hi, I was diagnosis in June of 2008 with IPF. I also have an unidentified connective tissue disease but plenty of symptoms. I have been on 10mg of Prednisone for a very long time to control the symptoms. This was listed as one of the 8 things to not do. Would you explain why? How dangerous is my 10mg a day? Thanks

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    1. Thanks for your comment. I cannot give individual medical advice through my blog. I can tell you that stopping prednisone all of a sudden can be harmful -- so please don't do that. I can also tell you that if you have an unidentified connective tissue disease, then prednisone might be a very reasonable treatment for you. The fundamental question that you and your doctor will need to figure out is whether your "IPF" is really "IPF" or if it is an autoimmune condition that is mimicking IPF. Not easy to figure that out in some cases. Bottom line: talk to your doctor about the benefits and risks of prednisone. Best of luck!

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    2. Hi, thanks for your response. I would never change how I take a med without discussing it with my doctor and transplant coordinator. I know Prednisone is not a treatment for IPF, there is no drug treatment (yet). I have many symptoms that point to a connective tissue disease but do not test positive for any of the types. Some specialist say I do have a connective tissue disease other say I don't. I did have a lung biopsy and know I have PF (and developed PH in the last year). The idiopathic part is in question. For now I am listed as and IPF patient. Was the reason you had Prednisone listed as something not to do because it so hard on the body and has been proven useless in treating PF?

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    3. Prednisone certainly has a role in some forms of Pulmonary Fibrosis. The reasons I cautioned about the use of prednisone for Idiopathic Pulmonary Fibrosis (IPF) is because high-quality clinical trial showed that people with IPF who received prednisone incombination with two other medications (azathioprine and N-acetylcysteine) had an increased risk of complications and even an increased risk of dying compared to people with IPF who were taking placebo pills. Here is a link to the abstract of the article reporting the results of that trial: http://www.nejm.org/doi/full/10.1056/NEJMoa1113354

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  9. What to add to this list: Whether on oxygen or not, purchase a finger oxymeter and use it to verify/quantify your body's feedback/symptoms of SOB. First opportunity, take the finger oxymeter and test it against a professional version to be sure of it's reliability. As Dr Swigris' Blog (http://pulmonaryfibrosisresearch.org/pulse-oximeter-what-do-i-do): "We don’t care if the two pulse oximeters read exactly the same numbers; we care only that they track together."

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  10. Dr. Lederer - first of all, thank you so much for this blog and all the information! I have been at this for almost 4 years and your information is straight forward and easy to understand. I have a suggestion to add to your list - Prevacid/Prilosec (generic). For Global Awareness Day, I attended an event in Atlanta, and one of speakers suggested this as there has been a recent discovery/research between IPF and reflux/GERD. He mentioned that side effects are minimal and it could be beneficial. I checked with my husband's doctors and his current medications, they saw no contraindications.

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    1. Hi Michele. Thanks for your comment. I hope to blog about the role of these medications in a future post. In a nutshell, I agree that these acid blocking therapies should be considered for some patients with Pulmonary Fibrosis. The studies you are referring to have focused exclusively on those with Idiopathic Pulmonary Fibrosis (IPF -- just one type of Pulmonary Fibrosis), and there hasn't been a single clinical trial of these therapies yet. The studies have been "observational" -- meaning that the investigators identified better outcomes among people with PF who were taking these therapies when compared to people with PF who weren't taking these therapies. And since people take (or don't take) certain therapies for important reasons, sometimes it's the reason they were taking a pill (rather than the pill itself) that influences their outcome. So, while I do think it is reasonable to recommend acid blocking therapy for people with IPF in some circumstances (and always under the guidance of a healthcare provider), the jury is still out on whether or not these drugs actually help slow progression. I guess it was more than a nutshell. :)

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    2. Dr. Lederer, Here's a footnote to the above two posts. I have what is considered auto immune PF (Lupus & MCTD). I was diagnosed in May of 2012. The first year I remained stable but the last year I have been on Mycophenolate, 2000 mgs a day. About the same time I was prescribed Omeprazole for GERD and had read somewhere that they shouldn't be used together. When I asked the pharmacist, she said she found no contraindication and to go ahead and use them together. My latest PFTs showed a pretty substantial decline in my pulmonary function, which I also could tell by the way I felt. Months ago I had written to Dr. Swigris about blood work which I felt should have been better after several months on the Mycophenolate. He suggested I have blood work to check the absorption of the Mycophenolate. Which I recently did, and it showed very low absorption. I spoke to another pharmacist at length and he said that the Omeprazole could block the absorption by as much as 70%!! So I feel the whole last year of my being on the Mycophenolate was a waste of time and money and worst of all the decline in my lung function. I was told to stop the Omeprazole (he said the same for any of the PPI drugs) and went back on Zantac for acid control. Also, the Mycophenolate was increased to 3000 mgs a day. I think both doctors and patients should be aware of this possibility and be monitored for absorption rate. It's too late for me as my lung function has dropped into the severe stage and I can't help feeling some resentment. Hopefully things are different for people with IPF and not taking Mycophenolate.

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  11. I have been dealing with scleroderma CREST, for 18 years, Now I am working on transplant at UPMC(I was denied at Cleveland) I have done everything on this list but Pulmonary Rehab has helped the most.

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  12. I have Ehlers Danlos syndrome hypermobility. I was just diagnosed with LPR and I since I am in the medical field I went out and bought a pulse oxometer. I was surprised to see that my O2 saturation never went above 95%. I haven't tried with exercise because I just had hip surgery but when I lye down it drops to 89-92%. The other thing that happens is when I stand I get short of breath but my heart rate jumps up and my blood pressure drops. My BP has dropped as low as 65/55 hr 165. My cardiologist put me on Propanalol (which keeps it around 110-135 standing and drops in the 50's when lying down) I have tried Flournif and Midodrine, but they actually made my standing BP actually drop to low 70's. I was wondering if this could be because my O2 saturation was dropping. I have the horrible cough at night (non smoker, drinker etc) post nasal drip sensation, swallowing issues, feels like someone is sitting on my chest, my lips are always pale, my voice/throat hurt when I sing in lower registers and talk. I tried an inhaler and it helped my singing but it was only temporary. I used to be a very active athlete but can't do anything because of this. I am a Kaiser Patient and I fear this is going to go nowhere. Thank you for your help.

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    1. Hi. Thanks for sharing your story here. I think a good next step would be to see a pulmonologist. He or she can evaluate your symptoms and oxygen levels. I hope everything turns out ok.

      All the best,
      Dave Lederer

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  13. Also, I didn't mention I am 41, 5'6" 130lbs. My weight has dropped as low as 117 and goes up and down.

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