Tuesday, April 29, 2014

"I was just diagnosed with Pulmonary Fibrosis. What do I do now?"

What advice do you have for people who were just told they have PF? Here's my list.

First, get yourself an appointment at an ILD center. You can find a list of ILD Centers on the Pulmonary Fibrosis Foundation's website. It is important that you work with your doctor to come up with the most accurate diagnosis and the best possible treatment recommendations early in the course of your disease. A few years back, I published a research study that found that people with IPF who show up at an ILD Center early after the onset of symptoms seemed to have better outcomes in the long-term.


Second, get yourself a pulse oximeter. You can get one in your local drugstore or online for less than $30. A pulse oximeter is a small device that you place on your fingertip to measure oxygen levels. It tells you the percentage of blood in your arteries that is carrying oxygen. Normal oxygen levels (or "sats") are over 97%. When oxygen levels drop below around 90%, we often prescibe oxygen to be used at home. Early on, most people with PF often have normal or near-normal oxygen levels 24 hours per day. As the disease progresses, people with PF initially maintain these near-normal oxygen levels during periods of inactivity (sitting or laying down), but during exertion, oxygen levels start to drop under the 90% mark. These low oxygen levels can contribute to breathlessness and can have other negative health effects (see this post and this one too). Using oxygen at home and when out of doors to help prevent oxygen levels under 90% can be beneficial in PF. Take a look at Dr. Swigris' post on using pulse oximeters at home.

Third, talk to your doctor about pulmonary rehabilitation. Pulmonary rehabilitation is a structured outpatient exercise program for people with lung disease. This is a critically important part of staying healthy and active for people with PF. Dr. Swigris has a great post on exercise in PF.

Fourth, get accurate information about PF. There's a lot of confusing information about PF online. I strongly recommend that you reach out to the Pulmonary Fibrosis Foundation for information online at www.pulmonaryfibrosis.org. You can also call the PFF's Patient Communication Center, which is staffed by wonderful people who can give you accurate and helpful information about PF. Their phone number is 844-TalkPFF (844-825-5733). You can also email the Patient Communication Center at pcc@pulmonaryfibrosis.org. My blog and Dr. Swigris' blog also have accurate information about PF.

Fifth, get support. Involving others in you disease can help improve health outcomes and your emotional well-being. This includes your family and close friends, but also includes reaching out to others with PF. There are support groups for PF throughout the US. If you are in the US, check out the PFF's list of support groups. There are also online support groups that you and your caregivers can join.

Sorry to everyone for the two-month lapse since my last post. Just busy at work. Will be posting more in May and June.

Go ahead and add your suggestions for the newly diagnosed in the comment section below. Also feel free to share this post online through Facebook, Twitter, and other websites so other people can find this information. 

32 comments :

  1. You've been missed. Great subject matter!

    In case someone misses it, this is the link to the full article on people with IPF who show up at an ILD Center early after the onset of symptoms seemed to have better outcomes in the long-term. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3208648/. The link you gave is to the Abstract that does contain the link for the Full Text.

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  2. You know the newly diagnosed have read a lot of the dire predictions our prognosis so I always recommend they read “The Internet Said I Have 3 Years to Live” and if they are on, or close to being on oxygen I recommend your posts "Five reasons you should use oxygen if you have pulmonary fibrosis" and "The Sixth Sense: Why you should use oxygen if you have Pulmonary Fibrosis!".

    You and Dr. Swigris really are a blessing to those of us living with this diseasee.

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    1. Now, you have another post to recommend. You are so funny JR.

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  3. I think there is much confusion because organizations, patients and support groups seem to use PF and IPF interchangeably. It's very misleading and frightens people unnecessarily. I think the definition and distinction between the two should be included in this post.
    Thank you for writing this blog. It is very informative. KimD

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    1. Kim, I agree about the confusion. On the support groups, when people say I just saw the doctor and I was diagnosed with IPF, the red flag goes up for me. Many of us know that it is not that simple and generally not a single point in time or one test. And many people think that IPF is the same as PF and that just because they don't know what the cause is that it's IPF. It is definitely more than the cause being unknown. But I think that is the point of this post and why it is the very first thing that Dr David lists -- get thyself to the ILD/PF experts FIRST, FAST, and FOREMOST!

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  4. David,
    I always think, why the Columbia University is not with your personal views. You are flying solo. Your posts are indeed interesting, but not confirmed by any Organization/University.
    How do I know your information is accurate?

    Just a question for you Dr. Lederer?

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    1. This comment has been removed by the author.

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    2. Well since you're Anonymous and not willing to put your name to your comments, then I guess you're flying solo and your personal opinions are just that -- your personal opinions. And how many years have you spent treating ILD/PF patients????

      If you haven't bothered to check David's credentials, then don't bother worrying about whether David's information is accurate. Just don't bother reading it!!

      Just my humble opinion. And what are your credentials for stating the information is not confirmed by any Organization/University. I can name several who hold the opinions expressed by David.

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  5. Thank you, doctor, for posting this! I wish I had this information when I was first diagnosed in 2009 however I know that this will help 1000's of patients who are diagnosed with IPF/PF.

    Christine

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    1. Please help me and look at the address below

      www.teespring.com/curecountdown

      Thanks,

      Victor

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    2. Victor, IMHO this is really not the place for your personal fundraising. You can put whatever slogan you want on a tee shirt but where is the money really going?

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  6. Doctor Lederer, this is your masterpiece. Thank you.

    Pam Huff

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    1. That is very sweet of you to say, Pam.

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  7. Just received an email from PFF announcing their Disease Education Webinars.

    The Foundation is pleased to announce our new Disease Education Webinar series. This program allows the PF community to virtually learn from, connect with, and pose questions to leading pulmonary fibrosis specialists.

    Register for our first Disease Education Webinar:

    "What is Pulmonary Fibrosis?"

    Presented by:
    Gregory P. Cosgrove, MD
    PFF Chief Medical Officer and Associate Professor of Medicine in the Division of Pulmonary, Critical Care, and Sleep Medicine at National Jewish Health and the University of Colorado-Denver.

    Wednesday, May 14, 2014
    11:00 a.m. - 12:30 p.m. CDT

    Following the presentation, please stay with us for a tour of the "About PF" section of the newly redesigned PFF website and a Q+A session. To submit your questions, please register (https://attendee.gotowebinar.com/register/7374332486609357569?utm_source=An nouncing+Our+New+Disease+Education+Webinars&utm_campaign=Announcing+New+Dis ease+Ed+Webinars+%7C+Register+%7C+05.01.14&utm_medium=email or http://bit.ly/R7hquf).

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  8. Great article David! Thank you for all you do to help patients and families.

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    1. Thanks, Taleena. It was great meeting you in Chicago last week.

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  9. I was told had scar tissue over 30yrs ago from bronchitis,,so Iam scared that I have pf

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    1. Sometimes an infection leaves behind a little bit of scar tissue. If your infection 30 years ago left behind a small amount of scar tissue, you might have nothing to worry about. A doctor can listen to you lungs and can consider ordering a few simple tests to see if you have anything more serious.

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  10. Are there any IDL centers in Canada, in British Columbia in particular? Canadians often feel very smug about our free health care, and it does work quite well for the most part. However I'm feeling envious reading about the many centres of IDL excellence the the US. I could never afford to go to one of those centres. The fact is that if you have lots of money, or good insurance, health care in the US is the best in the world.

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  11. My mom was diagnosed in the last 30 days with both COPD and IPF. She is already on 5 liters of oxygen while resting and she has been given just weeks to live. Is there anyway the diagnosis could be wrong? There is not an ILD center in out town and she can't travel. Do your have any suggestions?

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    1. Hi Theresa. My heart goes out to you and your mother. This must have been devastating news for both of you.

      Seeking the opinion of an ILD expert is always a good idea. Sometimes an ILD expert can provide new information and can help influence treatment decisions. Other times an ILD expert can help to confirm that the diagnosis is indeed correct. I think a good way for you to get hooked up with an ILD expert is to reach out to the Pulmonary Fibrosis Foundation's Patient Communication Center (which I help oversee. I even staff the phones there a few hours a week). You can reach the PFF by email at pcc@pulmonaryfibrosis.org or by phone at 844.TALK.PFF. You can read more about the Foundation's PCC at http://www.pulmonaryfibrosis.org/our-role/signature-programs/pff-patient-communication-center

      I hope you and your mother are able to find some help.

      Dave Lederer

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  12. Dr. Lederer:
    I was recently diagnosed with PF. I am 62, recently retired RN, and am doing well. I had a routine CXR in May 2013, and did not hear from my primary, so assumed it was normal. I went to see my pulmonary doc in Oct. 2013 for a check up as I have sleep apnea/on bipap. He brought up the results of my cxr from May and was surprised. He repeated the cxr that day. He's been following up with me every few months. I recently had PFT's done and my lung capacity is 70%. I asked my pulmonary doc if I needed any more tests, bloodwork, or workup done and he said NO.
    On my recent cxr, my pulmonary vasculature was increased. I had an Echo done last week, no results yet. My last Echo in Jan. 2010 showed a mean pressure of 24.
    I'm a bit overwhelmed with all the information and am not sure how to proceed at this time. I live about 2 hours north of Los Angeles and have been thinking of seeing a PF specialist at least for just a consult/opinion.
    Any guidance as to how I should proceed?
    Thank you!
    Rose

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    1. Hi Rose,

      Thank you so much for sharing your story. I completely understand your frustration. I see people in your situation quite frequently. Often, PF is thought to be an unimportant finding on a chest x-ray or CAT scan. It is only once symptoms develop that some doctors begin to think the findings are important.

      If your pulmonologist thinks you might have PF, then I strongly encourage you to see a PF specialist. You can go to the Pulmonary Fibrosis Foundation's website and search the "find medical care" section at http://www.pulmonaryfibrosis.org/life-with-pf/find-medical-care to find a medical center in LA. I know Dr. John Belperio at UCLA and Dr. Paul Noble at Cedars-Sinai -- they are both excellent. I am certain there are also other terrific ILD docs in LA.

      Best of luck with everything. I'd love to get an update from you down the road.

      Dave Lederer

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    2. Thank you Dr. Lederer. I'll let you know when I find anything else. Also thank you for the names from UCLA. That's very helpful!

      Rose

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    3. Hi Rose, I know this is an older post but I am hoping it will still connect me with you. My husband and I are just relocating to Oxnard from Northern California. My husband was recently diagnosed with IPF via a biopsy at UCSF. We are looking for a pulmonogist in Ventura County that is experienced in IPF and I was wondering if you have found someone that you might recommend? I Amy be reached at Lorinlee@aol.com

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  13. Dear Dr. Lederer,

    First of all, thank you for the invaluable resource you have provided to your readers! I am a 40ish year-old survivor of H1N1 influenza/pneumonia which resulted in ARDS this past January-February 2014. I was put into a medically-induced coma, trached and on a vent (and on one of those "astronaut" tables) for around 30 days, and discharged from inpatient rehab at the very beginning of March (I'm a feisty girl and *had* to get out of there). I have been using oxygen almost 24/7 and bounce easily into the mid to low 80's when out and about or during exercise, so I basically use it all the time to get back to my active lifestyle. A recent HRCT done in August showed "patchy areas of interstitial lung disease and scarring which can be seen with constrictive bronchiolitis" (in addition to mild bilateral bronchiectasis, mild parenchymal banding, and some air trapping). My question is -- at what point in general do people "top out" in terms of healing from the damage from ARDS and the kind of illness I had? We are around 9 months out from my date of discharge, and my lung function has remained relatively unchanged over the last few months (there was initial general improvement over the first few months as I regained strength). My absolutely wonderful pulmonologist, who was with me through the critical as well as the recovery part of this journey, is taking a wait-and-see where we end up approach (we did discuss prednisone, but I refused to try it), along with fairly aggressive exercise and possibly pulmonary rehab. Is PF a common outcome from ARDS/H1N1 pneumonia? Given that this isn't supposed to progress the way that IPF does, is it possible to gain back enough lung function to get back to being active in daily activities without needing supplementary O2, or will the current condition of my lungs be a relatively stable thing? I know that no one holds that crystal ball, but I would be curious to hear about long-term healing you've seen from patients in similar situations.

    Thanks very much -- Deb

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  14. I was diagnosed with IPF last Thursday at Cleveland Clinic. My son lives in Minnesota, and my docs encouraged a second opinion at Mayo Clinic since it is so close to his home. Do you have any recommendation for a doctor to request for a second opinion at Mayo? Thank you so much for your time. ellen dublo

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    1. There are many terrific doctors at both Cleveland Clinic and Mayo Clinic. I don't think you can go wrong with either center. Best of luck!

      Dave Lederer

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  15. Dr. Lederer,

    Thank you for your blog! I was recently diagnosed with ILD but have not had a biopsy yet. My biopsy will be next month. Currently, I wear a pulse oximeter each evening and see spo2 levels in the mid-80s throughout the night. Should I ask my doctor for nighttime oxygen?
    Thank you again for your blog!
    Dan

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    1. Hi Dan,

      Thanks for your question. You should certainly discuss your findings with your pulmonologist. The next step might be a formal test of your oxygen levels overnight at home (also called nocturnal oximetry) or a sleep study (called polysomnography). Best of luck with everything!

      Dave Lederer

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  16. My friend was just diagnosed with IPF and I have been researching all I can on it. Found your blog off Pulmonary Fibroses Foundation site. Thank you for the information. I knew about the pulse ox and getting to a specialized med facility but reading your blog it was easier to present to my friend in a way he thought it was a good idea. Look forward to more from your blog.

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