CPFE

[Gravel]

Hi I have CPFE. Not a lot wrong with my lungs or heart but oxygen transfer rate dropping yearly. Now 2.7, 3.7 last year. No treatment as yet. Anyone have any info on this. I'm very breathless on exertion.. When I move. Can anyone tell me at what level of oxygen transfer a heart ceases to function please? Is it 2, 1.5, 1? I really need to know. Thank you

[Hidden]

Hi

You are the first one I have seen on here with CPFE Sorry I cannot answer your question as newly diagnosed my self with CPFE plus Systematic Scleraderma as yet only on prednisolone to stop the coughing;

[Gravel in reply to Hidden]

Hi I have Ss too. Will let you know how if I get any more info. Although I have just read an article about acid reflux, auto immune and CPFE which I think makes aot of sense. Best wishes

[Hidden in reply to Gravel]

Oh my looks like the three go together !! How long since your diagnosis?, mine is about 5 weeks, though I have had symptoms for about 5 years, my fault nothing was done then, now know I have had 2 exacerbations, You? Another MRI for one was in May when investigations started. Next week plus x rays plus a capilliarascopy,? ( very hard to read the professors writing) very thorough here. Got my acid reflux under control without medications, I don't like talking meds if they are not REALLY necessary. Can you point me to the article please.

[Gravel in reply to Hidden]

Hi Reglois Take a look at American Thoracic Soc 2018 International Conference. A103.ILD Outcomes. Role of Gastroesophageal Refux Disease In progression of CpFe. It’s complicated to understand but seems to make sense. No one has related my breathing probs with this. I’m just being sent to lung and heart people - been waiting since May. But I have been seeing the gastro guy over the years. He recently discharged me from his clinic.

[Hidden in reply to Gravel]

Thanks Gravel will take a look when I come back from the heart specialist today.

I assume you are in GB, everything you read about CPFE says that the acid reflux has to be got under control as it exacerbates the CPFE. I am so glad I am not now in GB (in France and have been for 28 years) as I am getting excellent treatment here and speedy, Phoned Friday to make appointments the proff had given me papers for and this week (actually first one today) I have appointments for ALL three of them, CT scan, heart specialist for the SS plus x rays . Were you discharged because your reflux is under control or because you are too expensive or too old ? I read such dreadful things about the NHS and the older people now. I also read that CPFE is predominately a male disease, 90%, by your moniker I jumped to the conclusion you are male. I am one of the 10% females with it.

[Gravel in reply to Hidden]

Hi I'm female, lol. NE England. No one but myself has associated CPFE with acid reflux. In fact I haven't been able to discuss CPFE with anyone yet. Waiting to see lung man Oct. Waited since May. But there doesn't seem that much wrong with my heart/lungs. But last few years my acid reflux has been very bad. Was discharged before CPFE diagnosis. I have never smoked. Which part of country are you being treated in?Best wishes

[Gravel in reply to Gravel]

My doctor says CPFE is just another acrylic.

[Gravel in reply to Gravel]

Acrynom

[Hidden in reply to Gravel]

I suppose CPFE is another acronym just easier to say than Combined Pulmonary Fibrosis and Emphysema which any one must admit is a bit of a mouthful. lol

[Hidden in reply to Gravel]

Wow everything I have read says only 10% of females get CPFE and the only other person I have found happens to be female. I am so lucky to now be in France. The time from my first CT scan to seeing a lung specialist was 4 days, now seeing the top man in my county Burgundy, really knowledgeable and a really nice man. My CPFE is due to my old job, zinc chloride smoke, inhaled it for 25 years, in those days health and safety was non existent. If you do research, there is not a lot on CPFE but mostly idiopathic Pulmonary fibrosis, is non idiopathic PF the same thing? there is quite a lot that states acid reflux exacerbates PF, maybe I have been lucky in being able to control mine with diet alone before it has gotten really bad, but it was bad enough to make me vomit on several occasions. Anything with yeast in it seems to trigger an attack so I no longer eat that nor pasta or rice in fact most carbohydrates (in the common conception of these) seem to trigger an attack in me. How did they diagnose your SS my pulmonologist ordered blood tests for it unbeknown to me, had so many blood tests I ought to be anemic lol. I have read that people with CPFE have good lung function, (though I struggle to breathe out), but our gas exchange is corrupt. I get very breathless since an exacerbation in May, when I was sent for my first CT scan a week after first seeing a doctor, until then I had tolerated exercise well, even with emphysema could walk the dogs for kilometres and I live in mini mountains so nowhere is flat but what I do have is clean pure air, NO pollution.

I used to live in the south of England in Wiltshire moved here 28 years ago

[Gravel in reply to Hidden]

Hi So glad you are getting info and good attention. My lung volumes are good too. Although I have scarring and emphysema, my health conditions are generally through Lupus. My gas exchange is the problem down from 5 to 2.7. No apparent reason. Hoping having discovered about acid reflux this will help. Be careful about controlling it with diet. I thought I was at one stage but sometimes there is no burning or soreness. Over the years have had my gullet stretched twice through narrowing. At no time has my lungs been discussed. The stress of not being able to discuss this with a specialist is very bad for me. Don't have a lung appointment till October, so being able to talk to you - another patient - is really helping. Best wishes

[Hidden in reply to Gravel]

Glad talking helps, it does for me too as nowhere have I found any one with CPFE plus SS now got another as shown up on CT scan. Luckily for me I managed to stop my acid reflux with diet alone only a couple of months after it started sounds like your really played hell with your throat. I am really surprised that no one investigated further as to why you had such acid reflux cos that is basically the second thing I read when researching. and set about finding out what I could and could eat, my favourite, hot buttered toast, is now out of the question as is anything else made with yeast and most things made with flour and with raising agent. Put myself on a low carb diet and that seems to have worked. Only draw back is that Prednisolone makes me sooooooooooo hungry, even on the tiny dose I am on.

My CT scan results were not too bad, found something else but hey ho have so much what does one more problem matter. Most of the growths have calcified, or have they been got at by the fibrosis, don't know but now only one growing bigger, 1mm per month, in a very awkward place though, I can feel it. Doctor that wrote up the results for the specialist told me that the images were not *beautiful* lol, so very French.

Stress in NOT good for us so please try not to as really there is no point in stressing as it doesn't help matters, in fact hinders. I suppose I am lucky in that I am not a worrier, my daughter isn't either, she is being tested for Multiple Sclerosis and Reynauds, .

Trying to get my fitness back now it is a little cooler, took my dogs for a walk tonight, they were delighted, mini mountains here so it was quite a work out even if I only did two kilometres, one of them was steeply UP of course the rest was downhill !! my poor tiny three legged dog nearly went a over t she was going down so fast. My other little dog, I have three all rescues, goes for an operation tomorrow, his remaining tooth is communicating with his nose so has to come out, he's a rescue and suffers badly with PTSD, yes dogs get it too.

[Hidden in reply to Gravel]

You can e mail me if that will help you and of course if it is allowed here, maybe you can ask the admin to give you my e mail address if you want it.

[Gravel in reply to Hidden]

Hi Sorry to have taken so long to reply to your kind email. I have just read on my report from the Dr that my levels fluctuate due to smoking and they are going to give me an inhaler. I have never smoked in my life! I give up. Best wishes

[Hidden in reply to Gravel]

Hi Sorry to hear that the doctors do not seem to be taking you that seriously, does depend what inhaler they are going to give you though. I was given Symbicort, a lot of people with lung problems use them, I was highly allergic to it, my whole body cramped up including my lungs so it is out for me. Luckily you have an appointment soon with a lung specialist so will hopefully have more appropriate care, trouble is it seems there is so little know about CPFE + SS. Re-iterate to your doctor that you have never, ever smoked, you don't want that on your records, your emphysema must come from somewhere else. Is your PF idiopathic? Mines not, comes from my old job, exposure to Acid chloride smoke, they use that now in chemical war fare, so I consider my self lucky to have survived 25 years of severe exposure to it . Please don't give up I am finding that a positive attitude really does help, even getting angry at the situation and people jumping to conclusions keeps me going.

[Gravel in reply to Hidden]

Hi Sorry to have taken so long to reply. Lost my account somehow. Hope things are improving. Sorry to hear about your daughter. I have had mctd for 30 years. Take a look. Basically auto immune mix. Mine includes Reynaulds, me, Ss, sclederma, sticky blood, arthritis, lupus. Ms is also auto immune. Don't worry it's not obligatory to get all of them! Lol I'm just greedy. 😁 But thought it might help you to know, especially as you have Ss.

Saw Prof. last week. He says my lungs are beautiful. I have no desernable disease, even though I have the same symptoms. I asked if I would still be alive if I dropped to 1.7 and he said yes I would although poorly. That is as long as I didnt get flu or chest infections. But he didn't want to give me heavy duty drugs that may or may not help. I go back to see him in four months. He took me for a "run" along the corridor to check my oxygen and recovery rate. He is a very lovely man and I am sure he would help me if he could. But the are not worried about me because my lungs are great.

I'm feeling not so great.

Long letter lol Take care.

[Hidden in reply to Gravel]

Hi Gravel

Sorry to read you are not feeling too great, me not too bad at the moment, tried to take a little holiday with my daughter and my dogs, catastrophy, tiniest dog put his foot down a mouse hole, hit his head hard and dislodged a bone in his neck, by the time we got back to the rental place, carrying him of course, he was completely paralysed, phoned a good vet there and luckily he was an osteopathe for animals too and the fact I had got there within an hour of the dog being injured he could put the bone back in place and he could walk again but the dog collapsed again two days later, on a bank holiday so no vet there, rang my vet and came back home to see her, not good news he has done more damage than first thought and will be on tablets for the rest of his very shortened life. Fell SO guilty.

[Hidden in reply to Hidden]

I have an appointment with the lung specialist next week, I have decided I am not going to go on Esbriet , no idea what he will say, French doctors really don't seem to like the patient making the decisions, pff, as I have PF not Idiopathic PF I am opting for quality rather than quantity as every single thing I have read is not overly confident that Esbriet can help slow down the diseases (I have 4 in my lungs, greedy I know) and could actually damage the kidneys, I only have one and need to take good care of it as it can also be attacked by the Systemic Scleraderma. I also have an appointment with a specialist re the SSl. at the end of the month. This damp foggy weather is really catching me, chest tight, back hurts and I can't breathe and so so tired, but I don't want to be taking too many chemicals, only taking prednisolone at the lowest level I can to stop/limit the coughing, so on 1/4 of a 20mg tablet, my choice, doc says I can go up if I have to but he doesn't want me over 20mgs as that is counter productive, it's true, my local doc put me on 80mgs for an infection but it made the cough so much worse.

[Gravel in reply to Hidden]

Hi Sorry that you are not so well. I think that having chronic conditions certainly builds up our stamina because sometimes that's all we have to keep going on. But things can change and keeping active is the best. Mind sorry about your little dog X. had bleed due to acid reflux and dropped 20 on my blood count. Iron tablets were prescribed but gave me acid! lol Looking at Infusion. Low count not good for oxygen transfer. I haven't the energy to turn around but trying to have a little walk when I can. It's all a lot to cope with isn't it. But we won't take it lying down. All best wishes Xx

[Hidden in reply to Gravel]

Have you tried a wedge pillow for the acid reflux? I bought two at rather an expense £60 for a memory foam topped one and a side sleeper one, double the price, I alternate depending on back pain or shoulder pain, can't do without them now, no reflux at all, I also cut out anything made from flour, although rice or almond flour seem ok. No won't take this lying down far too uncomfortable lol.

My little dog had to be put to sleep as his accident had caused a rupture of the cords that hold the heart valve and his lungs filled with fluid, not compatible with life, poor little soul, just hope he was happy with me as he had had an awful life for 8 years stuck in a tiny cage and badly treated he had severe PTSD because of this and was making enormous progress after 20 months with me, his little girlfriend, a 3 legged mini Yorkie is heart broken too.