CPFE

[Charlene]

Hi Susan,

I’m not sure what CPFE is, but with a quick google search, is it Combined Pulmonary Fibrosis and Emphysema? I’m not sure anyone has shared with me that they have this on our forums, but they might and if so, I hope they respond to your message. I’d be curious to learn more about this myself. Were you living with IPF/PF before they discovered the emphysema, or is CPFE diagnosed initially?

Sorry to hear of your other troubles, this must feel incredibly frustrating and “just” living with a lung disease is exhausting enough! I definitely notice that since my IPF diagnosis, I have a lot more extremes in temperatures – either I am really hot, or very cold. I’m not always hot so I wouldn’t say I have the hot flushes, but I typically am warmer than I ever used to be. I do have lower limb swelling, especially in my feet. This is due to cardiac troubles, or when I’ve been on them too much. I do experience the motivation lack and fatigue as well. Both of these are so tough to deal with on a regular basis! Have you brought up your concerns about these side effects to your doctor?

I hope someone replies with CPFE knowledge for you, but if not please feel free to connect back as I do have some of the same symptoms with IPF. I also have some cardiac and kidney troubles too unfortunately.

Take care,
Charlene.

[Susan Howitt]

Hi Charlene

Been being treated for asthma for 5 years after what I now know was an exacerbation 5 years ago. X rays all clear so just carried on. This Christmas past I had a virus (had flu jabs already) that never seemed to go, another x ray showed nothing, maybe incompetent examiner, don’t know. So just carried on ignoring my growing inability to breathe and the day and night coughing until I collapsed with another exacerbation, then I was sent to the scanner, then to a pulmonologist, then I received a letter marked urgent from the huge hospital 60kms away giving me an appointment with the specialist, to say I was shocked was an understatement. At that appointment, 6 weeks ago, I was told what I had, did lung function tests and today get results etc, holidays of nearly every one in France (August) got in the way of anything quicker, will also be the opportunity to tell him about these side effects, also my fingers and toes are starting to club, more so my toes, so as you see I am VERY new to all this. CPFE is mainly a male disease 90% and a heavy smokers one, I gave up smoking 28 years ago, but it is possibly the fact that I did smoke that gave me the emphysema and the zinc chloride smoke gave me the fibrosis, have always had lung problems from a child, pneumonia almost every winter and sometimes even pleurisy with it.

For my lower leg swelling, was really getting bad to the extent that I had no shoes I could get on so I started to drink a lot more water, really worked now only have mild puffiness, this was thanks to a friends advice as I was restricting fluids in the thought that I wouldn’t swell as much if I had less fluid in my body when in fact the opposite for me was true. Removing all products with yeast in them has totally cured my nasty acid reflux too. Put myself on a reduced carbohydrate diet, boring but effective.

Living in France I have the *technical* language problem, my French is pretty good BUT there are certain things that I don’t really understand, a friend has been with me to my first appointment and is coming again today so between us we can hopefully understand most of what is said or discuss and research together afterwards.

Sorry written a book

A bienôt Sue

[Charlene]

Hi Sue,

Thanks so much for getting back in touch with me. No worries at all about long replies, or writing “a book” when you respond, I do it often too. I always joke with friends and say that I couldn’t write a short email or reply to someone to save my life… it applies to the forum responses too! 🙂

I can’t imagine the frustration you feel with being mistreated for 5 years, especially for something as serious as an exacerbation with a common aliment like asthma. Your second collapse must have been very scary, and I sure hope there weren’t warning signs of it in your imaging and an examiner just missed it. Although I wish you didn’t have to experience this, I’m glad it prompted follow up with a pulmonologist who can hopefully help manage the CPFE as long as possible. Did you feel comfortable with this specialist? That is always important too!

Increasing your water intake was helpful to reduce the swelling in your legs? I get this sometimes too, especially in the heat or if I have been on my feet all day so that is good to know. I am really bad with wearing unsupportive footwear (flip-flops for as long as I can) so I am sure that doesn’t help either. I would have thought that less fluid would have worked like you, but I guess it is the opposite. Thanks for sharing that! I often choose a low carb diet/foods as well, and agree that while it can be a boring diet, it makes me feel better generally.

How did the second appointment go? I hope it went well, and your English is great on the forums. Not too worry about that!

Take care,
Charlene.

[Susan Howitt]

Hi Charlene

I live in France but am English, been here for 28 years but all this technical French is hard, some times get a little *lost* when it gets complicated, that is why I do a LOT of research to make sure I am properly informed.

The first pulmonologist was a nightmare, hated him, luckily when he got the results of the broncoscopy back he sent me straight to the big hospital here and a *real* specialist who is a sweetie, does his very best not to be too technical but as in all French do not explain much as the population here is not apt to ask questions of qualified people believing they know best. Had a right run in, years ago, with a doctor when trying to get my deceased husband diagnosed, he very obviously hadn’t looked at the x rays I supplied as he told me that my husband had a stone in his right kidney. I challenged him because it was impossible as my husband, like me, didn’t have a right kidney !!!!!!! he then said that he will send him into the gastro section, challenged that too as I just knew it wasn’t that, don’t know why but just knew. Took me three more hospitals before some one would listen to ME (a woman and foreign to boot) by then my husband was paralysed except for one arm, dear doctor actually listened to what I had been saying all along, knew it was an important detail, lumber puncture proved I had been right all along, my husband had Lymes disease but it had gone straight into his spinal fluid and not the blood (when it was tested) he was life flighted to the hospital where I am now being treated and a day later he was on his feet after intravenous antibiotics!! so yes I question or research everything now.

Second appointment has found I also have Systematic Scleraderma !!! 99% positive test, so yes I have a right fight on my hands More MRI scans next week plus x rays plus a cappi something test, looked it up and it a test done on my hands, predominately fingernails to see if I have internal or external scleraderma (researched) I think. all three being done next week, must say very fast appointments here. Next appointment with the proffeseur Boniaud is in November, plus lung function tests.

Temperatures cooling down here so may be capable of walking the dogs, got to get myself as fit as possible again, not easy walking here as I live in the mini mountains. Little dog to vet on Monday as he has a cold due to the sudden decrease in temp, from 37° to 12° in a few days.
Sorry I keep writing books, I live alone and much as I love my rescue dogs, they are not brilliant conversationalists, good listners though.

A bientôt Sue

[Charlene]

Hey Susan,

Great to hear from you! Yes, I can imagine digesting and learning information about a lung condition in another language would be tough, so kudos to you for doing your research to ensure it is well understood.

Glad to hear you also have a new specialist that you like, I think that is so important as we embark on such a journey. Hope he elaborates on information for you and answers all your questions thoroughly! I don’t blame you at all for questioning / challenging doctors given your experience with your husband. I’m glad you were able to be his advocate for him when he had Lyme disease, but so sorry you went through so many hoops and difficulties getting medical professionals to believe you. That must have been so frustrating!

I hope your appointments go well this week. I know it can be scary when they happen so quickly, can leave you wondering why you’re such a priority with concerns that something is really bad news. Hope they go alright, and that the cappi test produces good results for you.

Isn’t it so nice when the weather cools off enough that we can walk our dogs again? I have to admit, that is what I am looking most forward to in the next few weeks as autumn arrives. Today it is 41 degrees celsius again… mother nature has not been nice to us this summer. The humidity has been really wild here! Glad the forums have been helpful for you in terms of getting out an opportunity to talk to others also effected by this terrible illness 🙂

Take care,
Charlene.

[Susan Howitt]

Update

 

Had another exacerbation  beginning of May and ended up in hospital this time, result now on O2 for 18 hours a day house one is at 2ltrs and my back pack at 5ltrs.

Has helped enormously with the swelling in my legs and I can walk well on the flat but very disappointed that I still cannot walk up a slope without being severely puffed and the alarm going off in the back pack because I am *mouth breathing* in an unconscious attempt to get more air and my lungs get extremely uncomfortable like some one is sticking a knife in. Cannot understand how O2 can help so well if I stick to the car and anywhere flat but be of no help at all where I need it, stairs, slopes etc.  One bad side effect is that my acid reflux is back for some unknown reason, is it connected to the O2, don’t know, could just be a co-incidence.

[Charlene]

Hi Susan,

Thanks for writing us, and sharing this update, although so sorry you experienced an exacerbation and hospitalization. That really sucks, and I hope you’re slowly on the mend. Sadly, when I had my first real bad exacerbation in May 2017, it is what caused things to move a bit quicker for me too… sad that it took that event, but what I needed seemed to come sooner as a result of having it. I know it is a pain, but I am glad you now have supplemental oxygen. It will help your other organs not over stress due to not getting enough oxygen…

I’m not sure about the acid reflux actually; I don’t experience this much so I’ve not been able to link it to my oxygen use or any other triggers. Someone might be able to connect them though, so hopefully they see this post. Glad your walking on flat ground is better, any small improvement is good right? 🙂

Take care and thanks again for writing.
Charlene.

[Susan Howitt]

Has any one had to reduce their oxygen intake?  My portable oxygen concentrator was set by my doctors orders at 5lts (pulsed) the max for my machine,  tolerated that for a month but felt *blown up,* is the only way I can describe it, so turned down to three, not enough, went back up a bit so am now at 4lts. Oxygen people came the other day to test and maintain the machines and wanted to put it back up to the prescribed setting, I said no. Here the doctors word is law but surely if I cannot tolerate what they have prescribed it must be wrong.

[Charlene]

Hi Susan,

Thanks for writing and good question! I can’t say I have a for sure answer, but I’ve often adjusted my oxygen needs to how I feel when using. I know for patients with PF/IPF, getting too much oxygen isn’t possible but I don’t know if that is the same for what you have. There is a risk for some people (mostly those with healthy lungs) to get too much oxygen, causing C02 build up or damage to the lungs after prolonged use. Can you call your doctor and confirm that you feel better at 4LPM as opposed to the prescribed 5? They may change it, but they may have set it higher so that your other organs are getting enough oxygen too. Not sure, but maybe worth a conversation?

Talk to you soon,
Charlene

[Charlene]

Hi Susan,

Thanks for writing and good question! I can’t say I have a for sure answer, but I’ve often adjusted my oxygen needs to how I feel when using. I know for patients with PF/IPF, getting too much oxygen isn’t possible but I don’t know if that is the same for what you have. There is a risk for some people (mostly those with healthy lungs) to get too much oxygen, causing C02 build up or damage to the lungs after prolonged use. Can you call your doctor and confirm that you feel better at 4LPM as opposed to the prescribed 5? They may change it, but they may have set it higher so that your other organs are getting enough oxygen too. Not sure, but maybe worth a conversation?

Talk to you soon,
Charlene

[Susan Howitt]

Hi Charlene

One of my conditions causes a CO2 build up so have to do a fair bit of pursed lip breathing out for 7. Haven’t got a lot of lung capacity left, think I am now on 30/40% .  I do have an oximeter and used it fairly regularly to check myself after I turned down the pulsed machine, most of the time I am on the static machine with nose hose and have no problems there, stay around 90/92 about the house and after an evening of TV even go up to 97 on occasions !!

 

Just catching up on here.  So sorry to read about your friend may she rest in peace now.

 

 

[Charlene]

Hi Susan,

Thanks for sharing – glad you’re able to hold off the C02 retention with pursed lip breathing. I have to get better at doing that on a regular, I’ve heard it is very beneficial for patients. Sorry to hear about your lung function, 30/40% capacity must be tough. It is nice to hear from you, hope you’re able to keep pushing through and thanks for your kind words about Kim. Hoping she is at peace.

Regards,
Charlene.

[Mike Ktori]

I have been recently diagnosed with CPFE and my pulmonologist speaks as though I’m dead already. I’m 78 and on oxygen 24/7 up to about 3.5 Ltrs/hour. I’m not able to do much but I don’t feel terribly unwell for 95% of the day. I’m told that I don’t have long to go but I just don’t feel that ill. It’s confusing. I’m in Queensland Australia