Cryptogenic Organizing Pneumonitis

Let me tell you a story.

A little over a month ago, on September 9, my Mom emailed me a short, panicked message: “Call Me.” She couldn’t just call me herself, of course, since she lives in Utah and I live in Germany, an 8-hour difference, and her mid-afternoon email arrived long after I should have been in bed. But I’m never in bed when I’m supposed to be, so I was awake, and I gave her a call. The news was bad, but not really shattering: my Dad was sick, and had been for weeks, and now it was worse than ever and he refused to go to a doctor. This is not surprising, because Dad never goes to doctors when he can help it. There was a lot of “Oh, you know Dad,” and “It’s probably just his asthma,” and so on, but my Mom wasn’t mollified. She’s known this man for decades, and she can tell when he’s legitimately sick. He could barely walk without turning gray and gasping for air, and she was worried.

The next day Dad went to work, walked up the stairs to his office, and called the doctor. That’s how we knew it was serious–he called the doctor on his own, voluntarily. He went in and was almost immediately diagnosed with pneumonia, and “maybe a heart attack,” which is a weird thing to hear from a doctor. You want to know if it is or isn’t a heart attack, none of this “maybe” nonsense. The doctor did ever test he could think of, held Dad as long as he could without actually hospitalizing him, and made another appointment for the following day. Dad’s condition worsened, and when he went back in the next day the doctor did some more tests and sent him straight to the hospital; the pneumonia had now been upgraded to “double pneumonia,” which makes about as much sense as “maybe a heart attack,” but at least now the “maybe” had been downgraded to “probably not,” so that was something.

All through this, of course, my Mom would call me–or email me to call her–every day, because she needed someone to talk to. Of her three children, my sister lives in Ohio and has crippling health problems of her own, and my brother, the only one still left in Utah, has a panic disorder: not exactly the kind of person you can unload all your troubles on. She needed someone she could call and say “I think he’s really sick this time, and I don’t know what to do,” and getting those calls and knowing there was nothing I could do from the other side of the world was maddening.

Dad ended up on oxygen, three liters of pressure, which was low enough that he could leave the hospital and push around one of those little tanks of wheels. This was just in time for my grandparents’ 60th anniversary cruise, which is one of the more surreal parts of the story: in the middle of his mysterious “double pneumonia,” my Dad took off for a week long cruise to Mexico. This sounds luxurious, but in hindsight it may have been a lifesaver, because the move from Utah’s thin, high-elevation, full-of-smoke-from-forest-fires air down to Mexico’s warm, rich, humid air did wonders for his lungs. He could even walk around the boat a bit without the oxygen, which was a bigger deal than we realized.

(The cruise, by the way, was hilarious: it was my grandparents’ 60th anniversary, and my grandpa’s 80th birthday, so they’d invited all their children–my aunts and uncles–and bought the tickets a year in advance. Then my aunt broke her leg, and my dad got pneumonia, and my uncle had a cold, and in the end my 80-year-old grandpa and my Mom with MS were the healthiest people in the group.)

With two days left to go on the cruise, my Dad got suddenly worse–still healthy enough that he could fly home, but barely. Instead of three liters of oxygen and leisurely walks around the block, he was at ten liters of oxygen just to lie in bed doing nothing; fifteen if he had to get up to use the restroom. He was back in the hospital full time, but in a better one this time, and my complete inability to do anything about it was driving me up the wall. My mom would send out positive updates, assuring us all that everything was fine, but the situation only got worse. The doctors would try a sure-fire pneumonia cure and it would do nothing; they’d try another, and the same thing happened. On Sunday, October 7, his lungs failed completely. He required 60 liters of oxygen pressure just to live–the machine was literally breathing for him. He was rushed to the ICU, and my Mom put on her bravest face, but there’s only so many ways you can spin “his lungs have failed and nothing works and he’s getting worse faster than the hospital can keep up.” I decided to hell with the Atlantic ocean, I was going home, and I booked a flight for the next morning. I was home within 24 hours of hearing the news, and my sister soon after, and though none of us would say it out loud, we were all starting to wonder if this was it. If we were going home to help, or to say goodbye.

But a lot, it turns out, can happen in 24 hours. After days and weeks of diagnosing and treating and trying and failing, the doctors figured out what it was: Cryptogenic Organizing Pneumonitis, which is a fancy way of saying “there’s something growing in your lungs but we don’t know what and none of our medicines work on it.” Most pneumonia is bacterial, and the rest is viral, and all the drugs they use to treat it work on those two causes, but with COP the muck in your lungs is something else–I don’t know what, fungus maybe, or some kind of evil spirits. It’s very rare, and very dangerous, and it’s called “organizing” because it literally gets in and starts remodeling your lungs to suit its own purposes, none of which are breathing. But in every other way it looks and acts like normal pneumonia, so the only way to diagnose it is to try and fail with every other pneumonia in the book, and when none of them work you know it’s COP, and you know exactly how to treat it. This happened while I was in the air, and the doctors started him on prednisone, and by the time I landed and raced to the hospital he had turned a corner. He had gotten slightly–every so slightly–better, the first time in over a month that a change had been positive.

My sister finally made it (her flight itinerary on US Air involved not one but two broken planes, a full day of delays, and an overnight stay in Phoenix), and together we set about trying to help. I cleaned some of the house. My sister took our Mom shopping. We spent hours in the hospital talking to Dad–he rarely talked back, of course–and hours more hanging out with Mom, watching movies, doing laundry, doing whatever we could to make life easier, or better, or at the very least more normal. Inspired by Mary Robinette Kowal, who reads her manuscripts aloud as part of an editing pass, I read my latest manuscript to my Dad, who loved it. We watched him progress through various benchmarks of breathing ability–using less pressure, using different masks, taking longer walks, achieving higher levels of oxygen saturation. We became intimately familiar with the minutia of the equipment, and the nurses, and even the terminology.

The first day I arrived I talked to the doctor, who introduced COP to my Dad as “that long confusing word we talked about before.” I asked what the word was, and he asked if I had a medical background. “Not really,” I told him, “but I’ve published five medical thrillers,” which is only a slight exaggeration: the John Cleaver books are not overtly medical, but they are directly concerned with psychiatric evaluation and profiling; PARTIALS is at least one third medical thriller, with a very detailed study of virology, and THE HOLLOW CITY is set in a mental hospital with a plot centered around diagnosis, neural chemistry, and drug interactions. I didn’t mention BLACKER DARKNESS. The doctor was impressed enough that he started taking me much more seriously than the rest of the family, opening up about that their theories and treatments and even inviting me to their meetings. I had a front row seat to everything they thought and tried and did, and it was awesome. And every day, my Dad got a little bit better.

My last night in Utah was Sunday, October 14. He was on a canula now instead of a mask, and they were giving him 40% oxygen, and he was maintaining a steady 91% saturation rate. The feeding tube was gone, and he could have real food again. He was still in the ICU, but only because they were concerned that something COULD go wrong, because he’d been so bad for so long they didn’t dare believe that he was good again. I set up my laptop on the little rolling hospital table, and we watched THE AVENGERS and ate pumpkin pie. It was the most normal thing he’d done in over a month. The next day I flew home, and the day after that they moved him out of the ICU, and somehow, whether through prayers or miracles or drugs or sheer force of will his numbers went up. Instead of 91% saturation he was maintaining a strong 95%, and even when he got up to walk it didn’t drop below 90%. I suspect that the simple act of moving out of the ICU–the most tangible sign of progress yet–gave him a renewed vigor, and as he cheered up his body started fighting harder. This morning I had another message from my Mom, not urgent but jubilant, saying that he is probably going home tomorrow. You have to realize that this is amazing: even with the progress we’d seen the week before we expected him to be in the hospital at least another week; his improvement was steady, but it was slow. And then for some reason it wasn’t slow anymore. The doctor was almost in tears, and Mom said she could see him physically trying not to say “just one week ago we thought he was gone,” because everybody thought he was gone. In ten days he’d progressed from “alien monsters are eating your lungs and we can’t do anything about it” to “you’re great, we’ll send you home tomorrow.” It was shocking, but it was exactly the kind of shocking we love.

My Dad’s health isn’t perfect, and it might never be again. The disease had a whole month to remodel his lungs, and they never did figure out what was up with his heart. Life is crazy, and anything can happen, and if there’s one thing I learned from the doctors it was “Don’t make predictions because they’re almost always wrong.” I don’t know what will happen next, but my Dad’s alive, and breathing, and going home, and for now that’s the best news ever.

23 Responses to “Cryptogenic Organizing Pneumonitis”

  1. NM Whitley says:

    Good to hear your dad is OK. I know the frustration that is living overseas and having family and parents with serious ailments and not really being able to do anything about it. ¡Ánimo!

  2. Kristina K says:

    I’m so very, very glad that your Dad is going home and recovering so well. What wonderful news!

  3. Mom says:

    The last 6 1/2 weeks have been such a wild roller coaster ride! Thanks for all of your help and support. And being the liaison with Dr. Pearl. (I think you can go ahead and kill him in a book).

  4. Becca says:

    May I speak for everyone when I say 1) we’re glad your dad’s on the mend and 2) your mom is completely fabulous?

  5. Melanie Jacobson says:

    Glad to hear he’s doing better. I think I have a little PTSD from reading this post, though. My dad died a few years ago from respiratory failure. When I was little he had cancer and somehow all the radiation treatment had scarred up his lungs significantly so he had fibrosis (not cystic, though). So he couldn’t be a runner. Big deal. But then a few years ago, the dummy decided to move a file cabinet down a flight of stairs, tripped, fell, broke his neck, and had to have surgery. Because of his lungs, they had to keep him on a special vent during the operation. When they attempted to take him off of it, he vomited and aspirated and got aspiration pneumonia. Six weeks in the ICU. ICU psychosis. PEG tubes. Life support. Ultimately he came home and got to where (after A LOT of work and time) he could be on oxygen only at night. But then my mom got cancer, and he knew she wasn’t going to make it and he got sad and quit eating, kind of. I mean, when it’s already hard to breathe and then life tries to take your wife away and that thought steals what breath you have left, maybe that’s what you do. And then his lungs started failing more. And then one day he got a stomach flu, but he was only 104 pounds by then. So he died. And I have no idea why I told you all of this, except I lived your hospital experience for weeks of my life and it all came rushing back. And also, his birthday was Monday and he’s been on my mind a lot. And none of that is to say to worry about your dad. All of his progress points to an excellent prognosis, and if he chooses to keep being stubborn, he’ll be just fine. Glad all is well.

  6. Peter Hansen says:

    Great news that your Dad is pulling through this. Best wishes for him to finish his recovery. Happy thoughts to you and your family to help get through the last of the troubles and back to enjoying life.

  7. Paul Genesse says:

    Dear Dan,

    So sorry to hear about your family’s big medical scare. I’m so glad he made it home. I’ve had patients very similar to him, as I work in the Heart and Lung Building at Intermountain Medical Center as a nurse. Anyway, I wonder what medicine they gave him that finally worked–besides the prednisone?

    Paul Genesse RN

  8. Kristine N says:

    That is wonderful news, Dan. I’m glad your dad’s illness was finally figured out and he’s recovering so well.

    Melanie–I don’t know you, but I’m so sorry for your loss. That sounds like a particularly traumatic end of life experience, both for your dad and for his loved ones. My heart goes out to you.

  9. Megan W. says:

    So glad to hear your dad is doing better. Sounds like he, and the rest of your family, went through hell. I’ve never heard of COP, but you learn something new every day.

  10. admin says:

    Thanks, everyone, and thanks to Melanie for making my trials seem pretty easy in comparison. Perspective is a great thing. You have my condolences and love.

    And yes, Becca, my Mom is indeed awesome.

  11. Natalia says:

    Love your mom’s comment above. :) And, along with your dad’s health improving, I’m so happy you had the time and means to make it out to Utah and spend a week with your family. Sometimes being surrounded by family helps just as much psychologically as getting out of the ICU. :)

  12. Melanie Jacobson says:

    To clarify, I’m not taking anything away from the experience your family endured. I commented earlier only to say that I’ve been where you were, I get it, and I understand the profound sense of relief and wonderment that comes with a miraculous recovery. I’m truly happy for you and your family that your dad pulled through. What I dealt with wasn’t worse, only different. But similar enough that I get it. And for the record, I think hopping on a plane was the smartest thing you could have done for you and your mom. And even for Rob who had his own business to attend to. It speaks a lot to your dad’s character and parenting that you all made the effort to be with him and your mom. I’m truly so glad for you that it all shook out in the end.

  13. HamletMonkey83262 says:

    Best wishes to you and your family for recovery.

  14. BC Woods says:

    Hey Dan,

    Glad to hear your father is doing well.

    Also, this may be one of those examples of someone saying “Hey, please take this highly specific bit of medical knowledge I have and try to utilize it in an unrelated area” but my grandmother had something similar happen back in March and it would have been useful for her to be aware of, so here goes for whatever it’s worth.

    Prednisone is kind of a miracle drug, but also extremely disruptive to your Adrenal system. That’s why you get such a big kick out of it at the start. But if you’re on it for more than a couple of days, you can get dependent on it very quickly. My grandmother’s lungs were so bad they let her stay on it for longer than they really ought to have. They’ve been trying to wean my grandmother off of it for months and it’s almost like she has some kind of cartoon illness where something is stealing her “life force” or something. Not really a super medical description (and I’m sure not really confidence building) but it’s completely stolen her “get up and go.”

    Not to add to what I’m sure are your family’s already numerous worries but it’s something to be aware of if he’s still on prednisone or hasn’t talked about it with his physician already.

    Oh, and thanks for killing me in “The Hollow City.” That kind of came at a good time for me in my life.

  15. Susan V. says:

    I feel for you and what your family has gone through. I am currently on a high dose of Prednisone also for COP (BOOP LIKE). Unlike your father it took four years and many hospitalizations for pneumonia before I was finally correctly diagnosed. I would wake up with right sided chest pain and a fever and go to the emergency room all within 3 hours. I would tell the Doctor when it started and that I had pneumonia; they would think I was crazy until a CT scan or chest x-ray was done. I have been on so many different antibiotics; sometimes 3 different ones at a time. Last month while in the hospital my Pulmonary MD brought in a Heart and Vascular Surgeon to perform surgery for Lung Biopsies: as my CT scan shows more scar tissue and infiltrates. The results gave me a difinitive diagnosis and finally some peace of mind. I had gotten so paranoid about always being sick that I was afraid to go anywhere or leave the area. I have to Thank my Husband Mike and the rest of my family for always being there for me and helping with almost everything at times. Sorry if I just kept rattling on, but just writing about it to someone that actually knows what COP is is cathartic.

  16. Becky says:

    Hey Dan,
    I was perusing some books and came across yours which led me to this blog. Glad your Dad is doing better. Say hi to the fam!
    Take care!

  17. Angela says:

    So glad that your father is improving. It’s hard when you live so far away.

    A friend’s father was diagnosed with the same COP condition a couple of years ago. He was also a long-time asthma sufferer. Turns out the mold growing in his lungs was caused from not washing the mouthpiece of his inhaler every time he used it. Just a thought… and as BC Woods mentioned above, prednisone is not a medication that you want anyone on any longer than absolutely necessary. Good luck!

  18. Denis Wong says:

    I contracted COP from an overdose of antibiotics prescribed for prevention of infection from a simple gum surgery. A year and a half prior to this procedure, I had a stroke due to atrial fibrillation (AFib) possibly due to depression due to my wife’s passing. I had been on amidoxirone and Pradaxa, listed on the dental pre-examination forms. Upon filling the antibiotics: amoxicillin, clindamycin, and cipro for 20 days, the CVS pharmacist reminded the dental surgeon that I was on amoxidarone and Pradaxa. I never did receive a call from his office to stop or modify the prescription.

    I did not recognize that I was getting weaker due to shortness of breath, until the 20th day of antibiotics. I could walk about 50 yards, and had to rest before getting to my car. I immediately fell asleep at home, and upon rising, decided to check on GOOGLE on the interatction of drugs I had been taking.

    Summarily, I called the National Poison Center’s 800 #. After describing I was taking, the tecnician shouted 3 words: “Call 911 immediately!” Notwithstanding,I called for help and my son rusned me to the ER at Queen’s Medical Center (Honolulu). After a series of X-Rays, CAT scans, brachialotomy, the images showed cloudy lungs. I was treated for pneumonia with aythromycin and something else(?) and Discharged after 4 days. 5 days later, bloody stools appeared.

    Again, I was back in the hospital. Dypsnea (shortness of breath) returned. This time I was put on high doses of predisone and 5(?) liters of oxygen. Dishchard after 6 days of hospitalization, with declining dosage of prednisone from 60, to 40, to 20, to 15, 10, and 5 mg monthly. I had 3 liters of oxygen for 24 hours for 15 days. I was discharged on my last appointment, and was “good to go!”

    Nevertheless, during the interim treatmentI decided to go to a pulmonary specialty clinic at Stanford Medical Center. They reviewed the scans, and the treatment. So far you are OK, but if you have “organizing pneumonitis” we want you back in 3 months. Upon returning, new imaging indicated that there was no improvement, actually getting slightly worse over the past 3 months. That is how pervasive that the disease is. I am now on 20, 15, and 10mg each month and Bactrim (generic sulfamthoxazole-TMP DS) on MWF, before I return to the clinic. Doctor recommends using a mask visiting a hospital or limited air travel and absolutely no foreign travel.

    What is going on in my lungs is a mystery, but it iw worth taking careful precaution as this type of disease is on the upswing. Respectfully submitted, Denis Wong

  19. Kris says:

    I am so thankful I found this blog . I just left the ICU where my mom has been for a week and the pathology reports from her lung biopsy came back with”cryptogenic organizing pneumontis”. She has been on a vent for five days and last night they put her on the big gun vent ” the oscillator” which forces 100 breaths per minute. While on it she is on a paralytic med so she can’t move. I am a wreck.
    The dr who told me this diagnosis is a hospitalist and seemed uncomfortable sharing more info bout this. Now I know why. They don’t see this very much!
    This blog has given me the hope I needed to believe that she won’t die today and she may get ff the vent altogether and even live a little longer.
    She is all I have left. Both my siblings and dad have all passed away and I need her so badly . Thanks again !

  20. Wayne Westman says:

    Samething happened to me, unknown origin, infection, weakness, Finally i said i had to see a dr. Only a ct scan showed what was wrong, predisone was what made me better. no problems since, nobody knew what i had. Just to let everybody know out here.

  21. Naomi Macorrison says:

    This is a great read. My Dad passed away 3 weeks ago from this disease. Its so good to hear your Dad survived it, and then it ,makes me upset, why didn’t my Dad survive.

  22. Paul B. says:

    it’s so encouraging hearing about your dad’s progress. It echoes my situation to a T. I was diagnosed with COP on July 5th after a bronchoscopy. They took a large piece of my lung and also a vessel with it and I bled on liter of blood in my lungs and this invited staff aureus. I was intibated and I spent 27 days in ICU. And was battling both COP and Staff. I was goner. Within 29 days and hi doses of antibiotics at first to solve the staff problem, and then then the starts of antibiotics I began improving with steroids and went from a 60 liters, to 40 to 5 just as I entered rehab for three weeks an acute care hospital aimed at physical rehab. I never smoked or drank and had an otherwise healthy body at 50 years old. 2 months later I am breathing room air at 98% saturation. Sleep without oxygen and can walk for ten minutes without oxygen going below 93. I’m supposed to use one liter of oxygen with exertion and working out , but often cheat and
    “forget” to put it on., I work out for an hour and a half dally with 2 days a week rest. My doctor was amazed at the recovery and said at my last visit two weeks ago ” there is no,permanent fibrosis or permanent scarring and all things lead to happy”.., I may be off of oxygen completely. We hope. Am I out of the woods? Not really. I am not gaining any weight. I’m on 50 mgs of prednisone and my vital capacity has not improved in months. But if my baseline ends up being where it is now, and I have to live with occasional oxygen at 1 liter, is be a happy man. Something miraculous did happen. Prayers and good family support helps! The trick is to be be careful and avoid relapses, work out steadily, eat proteins to build muscle, watch your salt so you don’t bloat, work on muscle strength to help get oxygen flowing, stay away from large crowds, smoke, barbecue smoke, coughing and sick people while on prednisone. Relapses do occur in about 50% of COPers. Lastly, not every case of COP is a death sentence and each individual is different. Weight, age, last history are all factors in the prognosis. Just know and be inspired by the positive stories here. It is important to keep mentally strong and willful in your quest to get better and live a long life. It can be done. I don’t know how long I have to live but for now I have hope. Good luck to all of you and stay positive and germ free!

  23. Katherine says:

    My husband is in the ICU right now and has been in the hospital since last Tuesday. We finally got a firm diagnosis on Thurs Dec 12. First they did a biopsy and that went to Pathology which wasn’t conclusive so it was sent to the Mayo Clinic in Arizona and they sent Cryptogenic——- I am about ready to go crazy with worry. My husband is only 62 years old and healthy. Tonight they are going to check his heart in the morning for possible prep for a lung transplant. When you are in the hospital it is so frustrating because there are so many different doctors and you just get bits and pieces of information. I am not sure I trust one doctor that I think is making the decision for my husband to have the heart procedure in the morning. I don’t know what to do think. I am very happy to see some encourging outcomes. We also live in Utah. I would be so grateful for any advise or suggestions.

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