FEV1 and Oxygen needs

[julie61554]

Hi everyone

My lung function has been hovering between 20-24% for the last couple years. I started the process of testing for a lung transplant in August of 2015 at which time I also started the Orkambi. In December of 2015, they determined that I was still too well for transplant but wanted to continue following me for when the time came. Well last year was a bad year. I started with I.V. antibiotics in May. Then again in June and again in July. Then from July through December I was pretty much on continuously with only about a 4 day break between each 2-3 week course. So the transplant center decided to update some of my testing and then they listed me in November of last year. At the lowest point in December my lung function dropped to 15% but I was still only needing oxygen with sleep and activity and my levels still weren't bad. With activity they would drop to about 88%. They hospitalized me at the transplant center at that time. They wanted to treat me with Zosyn which I am allergic to so they needed to desensitize me to it. After that 2 week course, my lung function improved again. At first back to 21%. Then because culturing a mold (fusarium) in intermittent cultures, they decided they wanted to treat the mold. They started me on Voriconazole. Because there is an interaction between the Orkambi and the Vori, they discontinued the Orkambi. 2 weeks after stopping my lung function improved again to 25%. Then they ended up having to stop the Vori because of elevated liver enzymes. They chose not to start me back on Orkambi as they didn't feel it was benefiting me. Since my lung function improved, they decided to put me on an inactive status on the list. I have needed 2 rounds of I.V. antibiotics so far this year--1 round for 2 weeks then 5 days later another round for 3 weeks. But both times my lung function only went down to 24% and no need at all for oxygen with the exception of sleep. They have told me that even if I am on the active list, that because I don't need oxygen 24-7 that I would be very low on the list and they wouldn't even expect me to get a call. Unfortunately, the amount of I.V. use doesn't play a role at all in where you are on the list.

My concern is that the bug I grow (burkholderia gladioli) is resistant to most antibiotics. Of the 11 they tested for, I was resistant to 7, intermediate to 1, and sensitive to 3. However, those 3 I am allergic to and can only be desensitized to 2 of them. The one I am intermediate to is there go to one that I am not getting resistant to. My concern is becoming to resistant that by the time a transplant happens, there won't be anything they can use or I will be at a higher risk of complications.

My question is how many of you have a lung function between 20-25% but don't require oxygen? That seems to be the biggest factor on keeping me inactive. I am getting worried that it may be awhile where I am placed on the active list. I know the longer I have my lungs the better, but I also feel like I am going to be at a higher risk for complications when the time comes if this lingers on for a lot longer.

Sorry this is so long! Thanks for any input!

Julie

Edited to add: I just want to state that just because my experience with Orkambi wasn't benefiting me personally, that is not the case with everyone. There are many, many people who have benefited from it.

 

[stylecomfy]

Hi julie61554, it sounds like you have been having a really hard time. I can't give any insight to your questions, however I think these are fair questions to ask answers for from your CF team...20-25% FEV1 does seem rather low not to require oxygen, but perhaps this is just how your body works. Do you have a home pulse oximeter with which you can measure your sats at different stages of the day to get a better picture of your levels?

Either way it would be best to ask your team for an explanation that satisfies you, e.g. of the risks of using O2 during the day vs not using O2 and how to know if you do need it, as they know all the factors (88% is not exactly high, perhaps they would be open to you using it during the day if you feel you need it).

Good luck and hope you get things sorted out

 

[julie61554]

Thanks reading my long post and for your reply. I actually have asked my doctors regarding the oxygen use and they are a bit perplexed themselves that I don't require oxygen at this level. They don't really have an answer as to why I don't. That's why I am curious how many CF'rs have and FEV1 in this range and don't require oxygen. It seems that I am a bit of an oddball.

I do have a pulse oximeter and use it frequently. For the most part I am always in the 90's. First thing in the morning before my breathing treatment is when it is the lowest and that is usually 92% at rest. The only other time I use oxygen is when I am exercising but even the lowest it has ever fallen is 88%. They also have done 3 arterial blood draws to check oxygen which have all been normal as well as having me do the 6 minute walk test 3 times. During the 6 minute walk tests, my oxygen only drops to 92%.

Don't get me wrong, I know it's a good thing to not need oxygen. I am just concerned that by the time I may require enough oxygen to re-activate me that I will then be more resistant to the antibiotics and will be at a higher risk of complications.

Thanks again for your input!

Julie

 

[scanboyd]

Hello Julie61554
Your and my status sound some what alike. My FeV1 is between 23-25, resting sats 97-98. With 6 min walk sats drops to 96 at 3 min. I use O2 at night none during the day. I have been worked up for transplant but yet to be listed because of no O2 requirement during a normal day. Sometimes during the day I feel tight chested and O2 deprived but when I ck my sats it will be 91-92. I go back in 3 months for follow up 6 min. walk. Doing a flight of stairs will drop my sats to 80-82 in a heart beat.
Good luck hope you do well. As you already know things with CF change quickly. My sister went from requiring no O2 to 24-7 O2. She has since had a double lung transplant this was17 yrs. ago and is doing great!
Take care!

 

[LittleLab4CF]

Julie61554

I'm amazed that you are able to function without oxygen. I'm very curious why you don't need it. In my working years it was common to have business guests from other countries, most of them living near sea level. As a good host, I took nearly all of them up into the mountains. Their stays weren't long enough to get over jet lag but every single one fell asleep by the time we hit 9,000 feet. A paved road going to 14,000+ is within an hour from the business. Some of my guests were definitely hypoxic. I can't argue with your oxygenation readings, but something has the hairs on my neck up.

The first real experience I had with low oxygen was a friend of my mother's who had the blood pressure reduced to the brain to prevent rupture of an aneurysm using shunts in the brain's blood supply. The reduced blood flow starved the brain of oxygen and saved his life, for what it was worth. His personality changed, became angry and violent and ended up in a nursing home at way too young an age. It was so sad and to that end I urge you to consider supplemental​ oxygen. In a way I am surprised that your CF team hasn't been more aggressive about O2. It would be great if you could have a live and vibrant brain to appreciate your new lungs when you need them. It may be a flair, a mucus plug or whatever, but if by chance your oxygenation reflects your low FEV1, the consequences could be horrible. Maybe oxygen at night?

What really caught my attention is your "concern". Are you a gardener by chance? Burkholderia gladioli is a soil bug like most Pseudomonous genus are. I was about to suggest rose dust but this is not funny, you have a rare bug that's usually attacking plants. I spent some time studying mold infections in animals and we aren't equipped to make designer antifungal drugs, and antibiotics for a disease that belongs on a plant. Something is out there and it appears to work better than the best antibiotic. Called "Anti-infectives", they are living organisms. This isn't as creepy as it sounds, rumor has it that we are invaded by about 2 pounds of microbes, mites and worms. Our eyelashes would be crusted and disgusting if it wasn't for the hundred or so eye lash mites at work daily, removing dried eye mucus, dead skin cells and the gunk our eyelashes were really meant to catch.

I'm going to give you the link to an article that specifically addresses the treatment of Burkholderia gladioli. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5133244/ It isn't the first microbe addressed so be patient, it's in the article, as are the special antibiotics they compared the results to. Anti-infectives are a very real possibility when the problem is impossible for the current antibiotics. The title "Beyond Traditional Antimicrobials" and the term "anti-infectives" will yield a good library of both plant and animal anti-infectives in a Google search or maybe a Google Scholar search.

Good luck you have, I wish you continued good luck,

LL

 

[julie61554]

Scanboyd.....thank you for your response. Yes your situation sounds very similar to mine. However, my oxygen (to my knowledge) doesn't drop that low when going up stairs. I can walk on the treadmill without my oxygen and usually about 15 minutes in, I am down to about 91%. I have only done that a couple times to see how low it would go and how quickly. Otherwise, when I walk on the treadmill I always use 2 liters of oxygen as it does seem to help me even though it doesn't drop too terribly low.

Do you mind if I ask if your lung function has declined very gradually? Mine has been a very very slow decline. I ranged between 28-32% for about 15 years and it wasn't until the last 3 years or so I have declined to where I am now. I wonder if the rate of decline has caused my body to "adjust" and that is why I don't need oxygen?

That is awesome to hear about your sister's transplant and how well she has done! That is very encouraging.

 

[julie61554]

LittleLab4CF....Thank you for all the information! To answer your question.....yes I did use to garden. My husband still does, but I don't. We started gardening about 20 years ago. I use to can. And it was after I started gardening that I started culturing the burkholderia gladioli. I was always curious if that is how I became colonized with it. As of late, I have been culturing a lot of different things. I have cultured the mold fusarium 3 times in the past 9 months. I have cultured Mycobacterium Fortuitum 3 times as well. I have also cultured several other "odd" bugs but they all have only shown up once and then they are gone by the next culture.

I do use oxygen at night while sleeping and while exercising. It's just at rest and at regular activity, my oxygen levels are normal. Like I said, the doctors don't aren't even able to tell me why and are a bit perplexed by it.

Thanks for the link to the article. It was a very interesting read. I will have to do some more research on this.

Julie

 

[mom2two]

Hi Julie. My FEV1 ranges from 18% on a very bad day to 27% on a great day. It can make that jump quickly in my case because I have a strong asthmatic component. When it is below about 24% I am on 2-3L at rest, 3L at night, and 6-8 for exercise. When I am closer to 27% I can get away with occasional usage at 2L, maybe 1-3 hours a day depending on what I am doing, 2L at night, and about 4L for exercise.

With respect to the Orkambi, the stuff took my FEV1 from roughly 40% to 20%. I never recovered higher than about 30% after I stopped it. (I was on it for about 6 weeks only). I know the drug has helped some folks, but for me it was only misery and sped up my timeline to transplant. Did the exact same thing to another friend of mine, but she recovered more than I did.

I used to garden a lot too. Now have several resident fungi species along with MSSA, PA,and Achromabacter.

 

[julie61554]

mom2two....I didn't do well with the Orkambi either. I was on it a little over a year. My lung function declined on it a bit but I had more shortness of breath the entire time I was on it. It was really bad the first 8-10 weeks and I thought it got better (which I am sure it did somewhat) but 2 days after they took me off of it I could tell a big difference in how much better I could breathe. They my lung function did improve by 4% after stopping. The main problem I noticed is the number of exacerbations I had while on it. I It could have been coincidental but after stopping it, I seem to have done better in that area as well. From June of last year through December I was on I.V. antibiotics almost continuously. I would be off for at most 5 days and be right back on them again. And for me, I usually only get exacerbations when I get a cold. Last year when it all started, it just came on....no cold or anything. This year after stopping the orkambi, I have had 2 exacerbations requiring I.V. antibiotics. I have been off of I.V. antibiotics now for 5 weeks and feeling really good.

My transplant team told me that they aren't very happy with Orkambi. They said it isn't the miracle drug they were hoping for and that it actually has hurt people. Especially those with the lower lung functions.

Edited to add: Edited to add: I just want to state that just because my experience with Orkambi wasn't benefiting me personally, that is not the case with everyone. There are many, many people who have benefited from it.

 

[mom2two]

Julie, Yes, I have heard several CF express their displeasure with Orkambi. But *shrug* for some it works well. I really think that they should have a lot more warnings on information page. I was also totally pissed that when I started having issues with the drug, Vertex stopped all their follow up phone calls like magic. Poof!!! I no longer existed because my results didnt fit their happy profile. Did anyone else experience this?