Tuesday, December 6, 2016

Your PF Story

Many of you have emailed me or reached out through social media to share your PF story or the story of a loved one. There seem to be many common themes: why did my loved one go undiagnosed for so long? is it IPF or something else? what treatments are available?, and many others.

If you wish, please share your story in the comments section below. And please respond when others post their story -- perhaps we can all learn from each other.

DJL

17 comments :

  1. I have had one HR CT Scan at a Center for Excellence where my physician is very experienced in IPF and ILD.Interstitial markings were found on routine X-ray during annual physical. I was diagnosed with PF Dec. 2015. I am 70 yrs old, no SOB or coughing. I have been to Pulmonary Rehab and continue on maintenance with 160 min. of exercise a week.
    As long as I'm doing well my doctor does breathing tests every 4-6 months. Had a bronchial lavage which showed 10%. So there is no definitive diagnosis yet. My doctor says the HRCT shows not the typical pattern for IPF. My sister died at age 81 with IPF.
    How long should I wait for a diagnosis? Seems if I have IPF I should go ahead and be taking medication. Patient in Alabama

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    1. Thank you for sharing your story. It is fairly common that a firm diagnosis cannot be reached. Sometimes continued monitoring of the disease over time can clear things up, and in that case a firm diagnosis can be reached. I strongly encourage you to bring up your concerns with your doctor and discuss whether there are any other options to make a diagnosis.

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  2. I am 41 years old, I was diagnosed with MCTD in 2010. In Feb. of this year after feeling short of breath I asked my Rhematologist for a chest x ray. They found markings of interstitial lung disease. I had a bronchoscopy and a lung biopsy. Diagnosis finally several months later, UIP (Usual Interstitial Pneumonia). I have visited the University of Michigan with their specialized clinic. They have put me on the immunosuppresent cellcept in hopes of calming down my immune system. Prior to Univ. of Mich. my pulmonologist put me on Esbriet which made me horribly sick. I go every 4 months to redo my pfts. The last one showed no lung function change in 5 months.

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    1. Thank you for sharing! Readers, have you had a similar experience?

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  3. My husband was diagnosed in May of 2013 after several months of illness. It started in January of 2013 with him just feeling ill. 2 rounds of antibiotics and a false diagnosis of allergies brought us to about March of 2013. Primary dr. finally decided to a CT scan, so we did that. However the guy who read the scan said "nope, nothing is wrong" / misdiagnosis. Primary dr. recommended a visit to a lung dr. Finally got in to a lung dr. in May of 2013 - dr. comes into room pulls us out and takes us straight to his private office. Apologizes and said "Mr. Leal, you are very very sick" "You need to leave my office right now and go straight to the ICU!!". We were in shock. After deciding to go to the hospital where both the primary and lung doctor work out of (which was an hour and 15 minutes from our residence) we headed there. The hospital was in flux because no patient has ever bypassed the ER to head straight to the ICU - paperwork needed to be done and permissions granted - which just drove me crazy. As soon as he got in the room and they placed the oximeter on his finger his O2 levels were only at 73% - super bad. The following day they did the VAT procedure but by the time I arrived at the hospital (after getting 3 kids off to school) he was already on a vent. VAT procedure wasn't easy on him and his body needed help breathing. I was "like what the heck happened??". I was unsure of what to do because I know my husbands wishes and they are not to be hooked up onto machines. After about 7 days, his peep numbers finally reached some specific level and they lowered his propofal levels so we can regain consciousness. I let them know that they should harness his arms but they didn't listen and my husband de-tubed himself. He was required to be under watch after that. They sent his biopsy to the Mayo clinic but the lung dr. already let us know that it was a disease called IPF. He was released after being in there for about 14 days. Doctor recommended an oxygen company, and it's been like that ever since. That summer was awful because my thoughts all lead to death. Research on the web all said 3-5 year life span, blah blah blah. 2015 finally brought on the testing for transplant - all that went well but the doctors wanted his weight to be at 190. The Prednisone that he was on prevented weight loss - but once he was weaned off he dropped down to a good 225 and with watching his diet he did drop to 208 but then 2016 started and since it had been a year - they required another round of repeat transplant tests. He is still all good but his weight / body won't drop past 208 - so we are stagnant. Oh, he's 52 years old now, it's been 3 1/2 years since his diagnosis. He was just recently switched to liquid oxygen - is that good / bad - I really don't know.

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    1. Hi Cynthia. Thank you for sharing your husband's story. I am sorry to hear about the ordeal he went through.

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  4. My husband was diagnosed at age 60 with IPF and HP (by biopsy) in May 2007 after having problems with numerous bouts of bronchitis (had never had that in his life) and SOB for several years prior. In those two years he was variously told he had emphysema, asthma, a bladder infection (yes really!!!!), a cold, and a few other things. We were relieved to have the diagnosis despite the gravity of it. Larry was exposed early in his Air Force career to toxins while working on the flightline crewing KC-135's. He was regularly soaked to the skin in Benzene, JP-4 jet fuel, trichlorethylene, and some other chemicals. They have all been tied to lung disease and HP. Of course it lay dormant for many years, and we know it can never be definitively proved that the exposure is what caused the IPF and HP - but in our hearts we know that is what it was. We have been lucky because Larry has outlived the prognosis and continues to do pretty good. He was started on oxygen immediately at night and on an as needed basis during the day. After a bout with bronchitis in 2013, he went to oxygen 24/7. He was also started on prednisone at 20 mg at the beginning - it was slowly titrated down to 5 mg where he remained for many years. After the bronchitis he needed 10 mg which is where he remains. He has not experienced any of the side effects of prednisone other than having prednisone skin...we're lucky that way too. His oxygen needs have increased a little - he is now at 5 lpm continuous on a 25 foot hose; 6 lpm pulse when out of the house and on his D tank; and 3 lpm hooked into his CPAP for sleep. His doctor at the COE in Seattle started Larry on generic CellCept last month but he hasn't done well on it - lots of nausea and diarrhea plus fatigue. We are waiting on a prescription for an anti-nausea drug to arrive and then he will try the CellCept again. Larry opted to not go for transplant - it was a tough decision for him - and hard for me to understand. Ultimately though - he has to do what is right for him. We are both thankful for the extra time we have had together, and the time Larry has had with our kids and grandkids. We will celebrate our 50th anniversary next July and plan to have a big celebration. I encourage all those who are newly diagnosed to get themselves to a Center of Excellence as quickly as possible...even if its a distance away. We drive 3 1/2 hours each way to get to the Seattle COE...it is so worth it! I also suggest that they not put a lot of time into the prognosis - it is different for everyone - and a prognosis is just a guess. Also get into a support group - both the patient and caregiver. Find one near you and if there isn't one locally, check out Facebook - there are PF support groups online that are a tremendous help. And keep coming back here to check out Dr. Lederer's posts - they are extremely helpful...I'm so glad we have this resource.

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    1. Thank you so much for sharing! And I agree with many of your comments -- a center of excellence can be very helpful -- even if it's just one or two visits to firm up a diagnosis and develop a treatment plan. Support groups are a terrific resource. You can also search for a support group near you at http://pulmonaryfibrosis.org/life-with-pf/find-medical-care?show=support. Best wishes!

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  6. For quite a few years my lung ct scan was showing ground glass opacities mostly in the upper lobes and two right lung nodules. I had been complaining of constant muscle pain and weakness and shortness of breath just bringing in my groceries. I also could not lay flat or I’d get breathless. My primary doctor and my specialist told me I was fine, lose weight and quit smoking, I had COPD.

    Recently I went in for a surgery consult for an unrelated matter and the surgeon said based on my last PFT and current state he would not touch me because I would likely end up on a vent long term. My pulse ox that day was in the 80’s sitting there. I contacted my “specialist” who again said I was fine and recommended a different surgeon. For a few months now I was also dealing with bilateral feet and leg edema with no known cause.

    In this time I saw two different neurologist and had a lumbar puncture with no reason for the muscle pain, weakness or balance issues found.

    I then found a new specialist om Worcester. MA who told me it was bad asthma. She did do an oxygen test but when I got dizzy when stats dropped to 90 they stopped the test, so no oxygen. Not even a week later I was in the hospital being told I might need to be vented. I avoided that but they tried prednisone and antibiotic because my CT scan was so abnormal. Those didn’t work, they sent me home with no oxygen and on discharge said likely ILD. Two days later I saw a doctor in the same office as my new specialist, he said I had to be admitted right away, I was too sick to go home. This was a teaching hospital and they did a ton of tests including another ct scan to determine likeliness of it being an ILD (at my request since it runs in my family). Those results said findings not consistent with ILD. I was discharged there after I failed trending oxygen test but no oxygen was given. They told me in a couple more days my oxygen level would be fine. Back at the second specialist I still was complaining of being short of breath. The testing showed me dropping to 86% at the 3 minute mark of walking. I was sent home with a script for 2L of oxygen to be used on exertion and when sleeping.

    I pushed for a thoracic surgery consult. The surgeon agreed it needed to be done and was going to do a lung resection via VATS in my upper left lung. After I woke I found out he took part of upper, middle and lower parts of my left lung. I left using 3L oxygen 24/7. I went to rehab for a couple of weeks. The biopsy results determined it was ILD-DIP, the 2nd specialist said high doses of prednisone was the only treatment. Now I had spent the better part of the last two years using prednisone and it was not fun. I searched for a new specialist.

    The new, larger hospital in Boston, MA, specialized, ILD/cancer doctor and his diagnostic team read my biopsy slides and determined all three biopsies, about a dozen slides, showed I had pulmonary scarring along with DIP. This doctor feels there is more going on as I have significant muscle pain and weakness. (Also, surgeon didn’t biopsy any nodules or lymph nodes) He is due to call me today after he had met with his team to tell me the next step or course of action. I know he wants a current CT scan as my last one showed multiple bilateral predominantly upper lobe ground glass nodules and opacities, reticular scarring and enlarged lymph nodes up and down my wind pipe up to 2.2cm as well as edema in the lungs.

    I will update once I hear something. I am a 43 year old woman.


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  7. I am 78 years old, smoker since a teenager but am relatively healthy otherwise. I exercise regularly and eat healthy. I was diagnosed in 2012 with PF (shortness of breath, coughing and fever) using a CT scan and started taking Prednisolone, Azathioprine and N-acetylcysteine. I have been on these meds since 2012 but with very low dosages now. I have regular (every 3 months) lung function tests, X Rays and blood tests to monitor my condition. Things have been stable for me.

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  8. Dear Dr.Lederer,

    I would like to share with immense grief that I lost my beloved father to ILD just 4 months back. He was 79 and was a very fit person even in his seventies before he was diagnosed with ILD. From what I understand he was suffering from IPF which is a disease under the category of ILD. All his vital statistics like BP , Sugar levels were perfect even after he was diagnosed with ILD.Although he was a long time heavy smoker for about 27 yrs ,he had given up smoking totally since 1984.

    I wanted to know your view if past smoking habits has any relation to ILD/IPF and if so, is there any correlation with respect to degree of deterioration in the condition of ILD patients who have been past smokers.

    Your feedback would be highly appreciated.

    Regards

    Shiv

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  9. Pieces of my story: IPF diagnosis May 2014 age 44. Only noticeable symptom at time of diagnosis was chronic cough. My wife and I have 4 daughters. Completely healthy otherwise, never smoked, rarely even a cold and no sick days in 20+ year career. Fairly fit and healthy my adult life. Double lung transplant July 3, 2016 age 46 @ UNC Health Care. Doing well...to date. No expectations...just thankful for each next breath.

    My contact information:
    andy.nelson@unchealth.unc.edu
    andynelsoncpa@gmail.com
    Cell 919-454-7290

    Speaking at UNC Jason Ray Foundation talk (I can send anyone pdf of slides from talk) and WNCN TV interview...
    http://wncn.com/2016/09/18/at-fundraiser-unc-worker-recounts-how-organ-donation-saved-his-life/

    Thankful for every next breath and every next moment God grants me to invest here. Life itself, just like healthcare, is a sacred trust to be stewarded...every moment, regardless of my feelings or understanding, infused with meaning by God. Thankful for IPF, for lung transplant & any more moments I may have left...reserving hope for many more.

    -Andy Nelson

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  10. My 53-year-old husband was in the hospital for several days for respitory distress. The family had all had gotten sick, but it severly affected him. The doctors did an X-Ray, CT, ECG and admitted him for pneumonia. They thought he may have underlying COPD. They gave him an oxygen prescription and told him to follow-up with a pulmonologist after hospital discharge. Those appointments were hard to get. He did not work between the hospital visit and the three week gap to see the pulmonologist. The pulmonologist had him do a breathing test (FVC 48, DLCO 36) and listened to him (crackles). He said said he had pulmonary fibrosis. I didn't know what that was, but my husband did. His mother died of it at age 62. His aunt also died of it in her 70s. The doc also took many vials of blood to rule out other things. My husband does manual labor, and tried to go back six days after being diagnosed and it was not good. Two days later we had a follow-up visit to the doc. The doctor said the blood work ruled out other things and that my husband was not to go back to work. He is now on oxygen. We immediately filed for the Family Medical Leave Act, Social Security Disability (he has only worked in manual labor all his life), and his employers disability insurance. We are also looking closely at his employers life insurance policy- it has a living care benefit (50% of policy value) but only if he has just 12 months to live or premiums can be paid for if he is totally disabled under their definition. Both tough hurdles to get over. We reviewed the peer-reviewed literature on the new $90,000/yr drugs that our insurance doesn't cover. Neither looks like they work for someone with as advanced PF as he has and the side effects are not of any interest to him (he takes his intestinal fortitude seriously). He is currently debating a lung transplant. He would need to slim down a bit for that. This big reason to do it is that we have two minor children. But it seems that only wealthy people can afford a transplant ($750,000 - $1,000,000). We were already only just barely making it as a two-income household. We both cringe at the "spaghetti-fed" benefit type thing, but if that is what it takes. The other option is to sell our house and move four-hours away to our favorite rural fishing spot (which would exclude the possibility of a transplant). Life is up in the air and the path unclear.

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  11. My name is Belinda.

    I am 47 years old and I was diagnosed with Idiopathic Pulmonary Fibrosis on May 15, 2017.

    I did not know anything was wrong. I had been having shortness of breath and often complained that I could not breathe. I just chalked that up to being out of shape.

    In October of 2016 I visited a podiatrist for an ingrown toenail. The podiatrist discovered that I had clubbing of the digits (fingers and toes). He advised me to see a doctor right away because clubbing of the digits could mean an underlying illness.

    I started with my general practitioner he did several test determined it was not lung cancer or my heart. I did have something showing up on my chest xray so he then referred me to a pulmonologist.

    I was diagnosed with asthma and Nonspecific Interstitial Lung Disease. I tried steroids, inhalers (Breo, Incruse,albuterol), and breathing treatments for several months with no improvement to the PFT’s. In May I had a lung biopsy and was diagnosed with IPF.

    I am taking Ofev and have been referred to the University of Alabama.

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  12. Hi can you interpret this x ray result of my husband only 34 yrs old
    Follow up study compared with the previous done on february 12 2017 shows no significant interval change. There is stability of the fibrotic densities in the right upper lobe. The heart and the rest of the chest structures are intact. No other remarkable findings.
    Impression is pulmonary fibrosis. Stable since feb 12 2017

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    1. Hi Joan. Thanks for reaching out. unfortunately i cannot provide medical care, advice, or recommendations over the internet. You can search for an expert medical center at http://www.pulmonaryfibrosis.org/life-with-pf/find-medical-care.

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