IPF - early stages of diagnosis...so many questions

Posted by Renee941 @renee941, Jan 1 10:48pm

I don't even know where to start to help my spouse through this. We are writing questions down as they come up but other than some obvious questions that can be answered on Mayo's website, I feel like we're forgetting something. There is the sound of crackle when listening to the lungs and ground glass on the 1st HRCT scan about 6 months ago. Another will be performed this month to check progression, if any. Spouse is experiencing low o2 levels with walking and exertion but they are brought right back up with o2 machine and rest. It seems to be progressing and he is becoming less active. I'm keeping tabs on the tale-tell signs of late stages IPF like fatigue and appetite and its scares me the fatigue is increasing and his appetite is decreasing.

We are told there is a 50/50 survival rate during transplant but what happens down the road if the organ(s) fails. Will we have a heads up physically if something is wrong or will it/they just give out? Can we get back on a transplant list after receiving one already? Is there a chance that all the damage is done and will be stable, meaning we just treat the symptoms going forward? Or does IPF always get worse and force a decision of getting on a transplant list?

We have small children and just lost at the moment. I'm looking for a support group to check in with, share questions that come up and compare dr recommendations etc…anyone going through this or on the other side of a transplant?

Thanks in advance.

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I had IPF before getting my bilateral lung transplant. My first advice is ask if he should be on the machine all of the time. Try to keep walking as much as possible even if it is just going to get a glass of water. It is like a spiral effect, the less you do, the less you can do. I am not sure how low his numbers are getting. I was on level 2 for a few months only when walking or doing any type of activity. Then I needed more oxygen as time went on. However, I kept pushing until I wasn't able to anymore (needed level 30, which is only available in the hospital).
Since having my transplant, I have had regular bronchoscopes. During 2 of the bronchoscopes, there was inflammation noticed, which can be a sign of early rejection. Due to having issues with my esophagus (had hiatal hernia repair year before transplant), it can cause issues with the new lungs, so I need more bronchs than usual. However, they just had me go in for therapeutic infusions for 3 days in a row each time to combat the inflammation. It's almost like a reset.
I also had the issue of losing weight, becoming malnourished and had a protein deficiency. My doctor had me drinking ensure high protein shakes (4 a day) to combat this before and after the surgery.
Try to get all of the tests done prior to him getting too bad. Since I was already at level 5 and still wasn't getting enough oxygen by the time my regular pulmonologist referred me to the transplant team, they fast tracked me. I have spoken to others and it seems to go much smoother if referred earlier. When getting the tests at the hospital I was on level 8 and wasn't able to speak in a full sentence (not enough oxygen).
I would also advise, if your husband likes pomegranates, grapefruit, or their juices have them now when he still can. Other things that he will not be able to have are: sushi or anything made where it is prepared (due to possible cross contamination), anything less than medium well done meat, unpasteurized cheeses, goat or sheep cheese other than pasteurized feta (need to make sure it is pasteurized), buffets or smorgasbords, and unwashed vegetables or fruit. For the first 3 months, no outside meals. It has been almost 8 months and I still can't have bananas or plantains (too high in potassium), papaya, mangos (too high in sugar), or more than 1 cutie a day.
I believe that if the lungs fail, you are put back on the list. However, from my understanding, it's not that you drop if it happens. If you have any other questions, please ask.

REPLY

I was diagnosed with pulmonary fibrosis one year ago based on CT scans and breathing tests. One doctor immediately told me to start taking a very expensive pill called Ofev. With Medicare Advantage insurance it cost me $3,000 for one month supply and then dropped to about $899 per month. Worse than the cost, however, were the possible side effects. So I decided NOT to start on this drug. Another specialist agreed to hold off on the drug and my second breathing test indicated to him that I was "stable." I am scheduled for another breathing test in the next week so I'm keeping my fingers crossed that I remain "stable." I do not hook up to oxygen at all. I walk the golf course (9 holes) even in the winter months when the fairways are very wet in western Oregon. I take several medicines already for other issues — allergies, thyroid, cholesterol — so I really don't want to start on Ofev. Any advice from this support group.

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@chickytina

I had IPF before getting my bilateral lung transplant. My first advice is ask if he should be on the machine all of the time. Try to keep walking as much as possible even if it is just going to get a glass of water. It is like a spiral effect, the less you do, the less you can do. I am not sure how low his numbers are getting. I was on level 2 for a few months only when walking or doing any type of activity. Then I needed more oxygen as time went on. However, I kept pushing until I wasn't able to anymore (needed level 30, which is only available in the hospital).
Since having my transplant, I have had regular bronchoscopes. During 2 of the bronchoscopes, there was inflammation noticed, which can be a sign of early rejection. Due to having issues with my esophagus (had hiatal hernia repair year before transplant), it can cause issues with the new lungs, so I need more bronchs than usual. However, they just had me go in for therapeutic infusions for 3 days in a row each time to combat the inflammation. It's almost like a reset.
I also had the issue of losing weight, becoming malnourished and had a protein deficiency. My doctor had me drinking ensure high protein shakes (4 a day) to combat this before and after the surgery.
Try to get all of the tests done prior to him getting too bad. Since I was already at level 5 and still wasn't getting enough oxygen by the time my regular pulmonologist referred me to the transplant team, they fast tracked me. I have spoken to others and it seems to go much smoother if referred earlier. When getting the tests at the hospital I was on level 8 and wasn't able to speak in a full sentence (not enough oxygen).
I would also advise, if your husband likes pomegranates, grapefruit, or their juices have them now when he still can. Other things that he will not be able to have are: sushi or anything made where it is prepared (due to possible cross contamination), anything less than medium well done meat, unpasteurized cheeses, goat or sheep cheese other than pasteurized feta (need to make sure it is pasteurized), buffets or smorgasbords, and unwashed vegetables or fruit. For the first 3 months, no outside meals. It has been almost 8 months and I still can't have bananas or plantains (too high in potassium), papaya, mangos (too high in sugar), or more than 1 cutie a day.
I believe that if the lungs fail, you are put back on the list. However, from my understanding, it's not that you drop if it happens. If you have any other questions, please ask.

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Thank you so much for sharing and this information. Congrats on your transplant and i pray you continue to do well! He is not going to be happy about diet changes lol. We discussed that last night, in the event he was put on the list and we had to get his bmi in line. His #s dip into the 80s and its even hit 70s but I'm not sure if his monitor is correctly calibrated, even so, it does dip since we have all tried it on ourselves and our #s stayed in the high 90s. He's doing another "home test" at the hospital this week and we're bringing his monitor as well. Right now I believe he's on level 2 with machine and was told to use it as often as possible. I am trying to keep him moving, we used to exercise 3x+/wk but with the fatigue and depression setting in, it's becoming more of a challenge. I am praying for good news tomorrow but to be honest not sure what good news would be? We were told the 2 items up for discussion would be transplant or order a biopsy.

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@renee941

Thank you so much for sharing and this information. Congrats on your transplant and i pray you continue to do well! He is not going to be happy about diet changes lol. We discussed that last night, in the event he was put on the list and we had to get his bmi in line. His #s dip into the 80s and its even hit 70s but I'm not sure if his monitor is correctly calibrated, even so, it does dip since we have all tried it on ourselves and our #s stayed in the high 90s. He's doing another "home test" at the hospital this week and we're bringing his monitor as well. Right now I believe he's on level 2 with machine and was told to use it as often as possible. I am trying to keep him moving, we used to exercise 3x+/wk but with the fatigue and depression setting in, it's becoming more of a challenge. I am praying for good news tomorrow but to be honest not sure what good news would be? We were told the 2 items up for discussion would be transplant or order a biopsy.

Jump to this post

At least for me the biopsies are nothing. They take at least one biopsy every time they do a bronch. They give me anesthesia and I wake up sometimes with a little sore throat, but that goes away within a day.
I would bring up the oxygen level with the doctor, sounds like he needs more. From what my doctors said, better to raise the level and keep up the activity as much as possible than to keep it low. I was on level 5 at home all the time for a while before I got even worse (my O2 was dropping to the 30s). I had major issues with the insurance company not approving a machine that would go above level 5. At least my portable would go up to level 6. I also had major issues with the insurance company approving more than 2 tanks every 8 weeks, which wasn't enough for me to even make all of the doctor's appointments let alone exercise outside the home.
The transplant team said that as long as I didn't loose any more weight (BMI was 18) that I would still qualify. However, I lost more and they still did the transplant for me.

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Did you go through the process of ruling out NSIP early on? I'm guessing that's the 2nd direction (biopsy) if they don't suggest the lung transplant. He said last night that he could definitely go up one more level on the machine and we're going to bring it up today at the appt. Getting ready to head out now, fingers crossed we get some relatively good news today.

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My PC referred me to a pulmonologist due to me having a cough for over 10 years when it started to get worse. I come from a family that doesn't believe in going to the doctors, so I had put it off for a while. He had me go for X-rays and did a full blood work up on me. I did have pneumonia associated with COVID after being diagnosed with IPF, which made things much worse (went from 70% use of lungs to about 40%).
I wish you luck. May god bless the two of you.

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@george6768

I was diagnosed with pulmonary fibrosis one year ago based on CT scans and breathing tests. One doctor immediately told me to start taking a very expensive pill called Ofev. With Medicare Advantage insurance it cost me $3,000 for one month supply and then dropped to about $899 per month. Worse than the cost, however, were the possible side effects. So I decided NOT to start on this drug. Another specialist agreed to hold off on the drug and my second breathing test indicated to him that I was "stable." I am scheduled for another breathing test in the next week so I'm keeping my fingers crossed that I remain "stable." I do not hook up to oxygen at all. I walk the golf course (9 holes) even in the winter months when the fairways are very wet in western Oregon. I take several medicines already for other issues — allergies, thyroid, cholesterol — so I really don't want to start on Ofev. Any advice from this support group.

Jump to this post

I'm glad you're doing well all things considered and can still get out on the course. I'm gathering that the key to keeping the disease at bay is to manage any inflammation. Our Dr said hubbys inflammation is whats causing progression. Im guessing they already had you try prednisone? I did some digging on IPF and found that NSIP is very close in symptoms at first glance and IPF can be misdiagnosed if a biopsy is not done to confirm. Our Dr agreed and has hubby going for a biopsy and if its the cellular version, hes planning to prescribe 60mg of prednisone for about 3mo to kill the inflammation. If its the other version, fibrotic, he will be put on autoimmune/ antifibrotic meds. You're price on the meds is outrageous! I spoke to a woman yesterday who said she worked with insurance and "CVS specialties rx" and they got the price of her meds down substantially. Good luck on your follow-up!

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@chickytina

My PC referred me to a pulmonologist due to me having a cough for over 10 years when it started to get worse. I come from a family that doesn't believe in going to the doctors, so I had put it off for a while. He had me go for X-rays and did a full blood work up on me. I did have pneumonia associated with COVID after being diagnosed with IPF, which made things much worse (went from 70% use of lungs to about 40%).
I wish you luck. May god bless the two of you.

Jump to this post

Thank you! Dr does not believe that it's IPF after all and now we are going down the road of NSIP and Hypersensitivity Pneumonitis, pending the outcome of the biopsy.

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