Diagnosis: constrictive bronchiolitis Help!

I am new. First time ever posting on a website. Recently diagnosed with constrictive bronchiolitis after having an open lung biopsy done. That was a painful surgery but at least I got a diagnosis. I was/am devastated. This diagnosis is basically telling me that there is no cure, no surgery, nothing that can be done for this disease. The only help would be finding meds that can stop/slow the progression of the disease. Eventually, it will kill me. I don't know how much time is left--there is no way to know how much damage has already been done. They also do not know why or how I got it!! My pulmonary doc says he has only seen a handful of cases in his life and that it is very rare and that there is much they don't know about it. Since the surgery, I have been on oxygen 24/7. Prior to the surgery, I was not on oxygen. Had damage to my left lung from radiation last year (radiation pneumonitis) but doc says he doesn't think that it had anything to do with this diagnosis. I'm not so sure about that! If I could get off this oxygen, I know I would feel so much better. I feel like I'm in jail and can't get out! My freedom has been taken away. Any info, comments, solutions would be welcome. Currently on low dose of prednisone until middle of December. Will see doc again and he's going to try a new med (experimental) to see if it helps to slow down the progression of the disease. We are waiting for some blood tests to come back before he starts the new med.

4 Replies

  • jrsteiger9 I also have a diagnosis of constrictive bronchiolitis which I received after an open lung biopsy in May 2013. I am on oxygen (2 liters) 24/7. Please do not fight it. Instead learn how to embrace it. It is life saving. Get a portable oxygen concentrator (I have Inogen One G3) and resume your life. I was given a three - five year prognosis from severe onset of symptoms (which was December 2011), so I have reached my five year mark. I am now actively listed for a double lung transplant. My lung function was 54% in 2011 and it is now 21% after numerous bouts of pneumonia and two hospitalizations so for pneumothorax. My daily regimen includes Spiriva, Symbicort, 5 mg of prednisone, azithromycin (M, W, F), N-Acetyl Cysteine, ProAir for bronchiospasms, and the nebulizer with albuterol when needed. The key is EXERCISE! You must keep moving. You have to stay strong and in good physical shape to qualify for a lung transplant, so use the oxygen and move forward. I'm 59 and have four young grandsons that I fully intend to make some memories with to carry through their lives. I am still able to drive, shop, even play a few holes of golf. Set your goals and avoid depression. Push through when you feel like quitting; it is just trapped air (use pursed lip breathing); you are not having a heart attack. I will answer any questions that you might have, so feel free to ask. My email address is ldycatcoach@yahoo.com. That is easier because sometimes it is easy to lose people on this sight. May God bless you, Breathe easy, Jeanne

  • Thanks so much for responding to my post. I will email you with specific questions I have. I ended up in emergency last March 30th. Symptoms were a very dry hacking cough, a little shortness of breath, and loss of all energy. I could barely get out of bed. They diagnosed me with an A-typical pneumonia and loaded me up with antibiotics. They didn't help at all. I was admitted because they could not get my oxygen level up. While in the hospital, they discovered the radiation pneumonitis. The treatment for that was steroids. Was put on 60 mil. of prednisone for two weeks and then gradually went down to 20 (10 a week). When I reached 20mil. I started to only go down 5 mil. a week until I reached 2-1/2mil. That process took almost 3 mos. The prednisone was/is like a miracle drug for me. I feel so much better within hours of taking it. Of course, they don't want me on it any longer then absolutely necessary because of the side effects. Within 7 to 10 days off the steroid, all my symptoms returned and we went through the whole process again. Same thing happened a third time. We caught it before it got too bad and went on 20mil. of the prednisone and down 5 mil. a week from there until I reached 2-1/2 mil. That's when my doc said I needed the lung biopsy because he wasn't exactly sure what was causing this to happen. It was acting like an autoimmune disease. Then the diagnosis of constrictive bronchiolitis. My doctor was not expecting this. So now we are at the point of how to handle/live with this diagnosis. I tried to condense this as much as possible. I have lots of questions and really appreciate your willingness to answer them. I will send the questions to your email address. Thanks in advance for your help. Janet

  • Wow! Really sounds familiar - except I started out on 60 mg of prednisone for four weeks and then eventually tapered down to 5 mg. I have never been able to reduce it to 2.5 mg. I started out with a hypersensitivity pneumonitis diagnosis and found out that I am an Alpha One Antitrypsin carrier (mz). However, my antitrypsin levels are within normal range although it is possible that being a carrier has resulted in some susceptibility. After two bronchoscopies did not yield a diagnosis, we opted for the open lung biopsy. I cannot be exposed to any strong chemical odors, strong cooking odors, perfumes, or smoke. They will instantly set off bronchiospasms and cause an exacerbation (usually landing me in the hospital). Baking soda, Dawn, and vinegar have become my only cleaners. Vanilla and coconut are my only tolerable scents. How did this happen? Other than being an alpha one carrier, our best guess is exposure to gym floor cleaners and varnish (I coached basketball for twenty -five years), floor cleaners and waxes used in the school (I taught for thirty-five years), fumes from diesel fuel (I drove a school bus to athletic events for twenty-five years), and chlorine from an indoor pool and hot tub (we were on vacation and I came home with pneumonia), oh yes, and Clorox - no Clorox, it's deadly! My first hospitalization came on Christmas Eve of 2011. We had just arrived home from a family cruise and I honestly thought I was having a heart attack. The emergency room doctor said the CT scan showed "glasslike shards" in my lungs and possible pneumonia. After a heart catheterization with no issues other than mitral valve prolapse, my cardiologist turned me over to a pulmonologist. And the story began .... Please stay in contact. You and two others are all that I have found in five years of battling this disease. One is also listed for a lung transplant and the other has been deferred until further testing can be done. I hope you are able to enjoy the holidays this week. My transplant team tell me "no large crowds in small spaces", walk two fifteen minute intervals on the treadmill each day and do my "leg squats"! That's what keeps us going... Breathe easy and may God bless...

  • Thanks for your background info. I'm afraid I don't have any idea what an Alpha One Antitrypsin carrier is. I have never heard of it. Would you mind telling me? Thanks for the tip about baking soda, Dawn and vinegar. I am still using commercial products which I will stop. When I spray them, I walk out of the area for a few minutes. My friend who gave me this website said that she makes her own. (She does not have what we do but cannot be exposed to odors, etc.) I will try and find out her recipes. The main difference between you and me is that I have not been exposed (that I know of) to any of the chemical odors you list. I worked mostly in an office setting my entire life. I was in banking on and off for about 15 years but even part of that was in an office. That's why my pulmonary doc did not expect this result. I just don't have a background in any type of exposure to chemicals. I was diagnosed with breast cancer in 2015. It was a nodule that was encapsulated. Because of that, I only had to have the radiation treatments and did not have any chemo treatments. They removed the nodule and also did a surgery (a month later) to remove some lymph nodes just to make sure the cancer had not spread. It was fine and so I just did the radiation as a precaution. Because of my circumstance, they actually reduced the number of treatments to only 20. Most cases are 30 and above. All was well except I did develop pleursey (inflammation of the lining around your lungs- not sure of correct spelling), the last week of radiation. Ended up in emergency. Had no idea what was going on, only that I had terrible chest pain and thought I was having a heart attack. They said the radiation caused it. I was fine for 4 months and then got sick with the symptoms I told you about. I was told the radiation pneumonitis only shows up 4 to 6 months after finishing radiation. Prior to all this starting, no one ever mentioned that this could happen after radiation. I also had never heard of this before. And that's how it all started. I trust you will try to answer my questions that I sent to you in an email. Please take your time and don't feel that you have to respond immediately. I know it takes time to write all this stuff out. I look forward to hearing from you and learning from you. There is so little info on the web. What's there keeps repeating on other sites and offers little hope for this diagnosis. Thank you again for responding to my inquiry.