Does anyone know anyone else that suffers from IPPFE?

[dodgylungrunner]

Lots of you have been asking about what IPPFE is which I've previously made a real dog's dinner of explaining in the absence of any real information about the disease online bar very dry research papers that paint a pretty bleak picture.

However the team at the Brompton have now put together some info on the disease that does a pretty good job at explaining what it is and what some of the main symptoms are: rbht.nhs.uk/patients/condit...

However,

the page struggles to explain what causes IPPFE, how it's treated or is likely to develop because they don't know enough about it.

That's not their fault as the condition is really rare. The absence of cases means that they haven't been able to obtain enough data to make generalisations or establish patterns. As a result treatment remains trial and error.

This will remain the case unless we can get more IPPFE patients together to help the doctors establish trends and patterns that will inform future treatment protocol.

With that in mind I'm looking to track down others with IPPFE so that we can help doctors understand more about the disease and it's patterns which maybe, just maybe might give us a fighting chance at stopping the progression of the condition.

If you know anyone that has IPPFE that would be willing to help out then please do share this post with them and encourage them to get in touch. Thanks in advance BLF family!

[sassy59]

They don’t seem to really know what the cause of IPPFE is as it’s so rare. I hope you get some others coming forward so that more can be done.

You take care xxxxx

[dodgylungrunner British Lung Foundation in reply to sassy59]

Absolutely Sassy. It's really hard for them to make any real sense of the treatment options, too, as they've got no other results to compare mine against. As a result it's trial and error really. That's why it would be great to find some others with IPPFE to see if we can help the doctors at all.

[Hidden]

If i was to pin tail on donkey I would ask given what i have read MIGHT be tad personal but did you ever have operation to drop a ball.

I had operation myself and my lungs are bushed AND my brothers kid had opperation and he's lungs are not great

But EVEN if people had ippfe would doctors know what it is from other conditions given its rare let alone dx you with it

[Hidden in reply to Hidden]

Pleural endometriosis. Dendriform calcification.

[dodgylungrunner British Lung Foundation in reply to Hidden]

Hi JeffAjaxSmith. I haven't had an operation to drop a ball but I did have a bone marrow transplant which the doctors think could be partially responsible for my condition. Take your point about diagnosis with IPPFE however I was looking to get people with the condition together more to help doctors establish patterns and possibly establish a better treatment protocol. At the moment it's very much trial and error with very little to compare against.

Sorry to hear about your own condition. How long have you had it and how is everything going?

[Hidden in reply to dodgylungrunner]

Well you bone marrow thingy would defo of done it ... is all very complicated BUT like peter said autoimmunity dieses.

There is consensus about thiroyd or how ever spelt MY next point would been XY chromosome issues guess that’s scope of bone marrow.

Why did you have bone marrow transplant ANYWAY did read quite a lot and some would not like to mention but research in China 🇨🇳 Japan 🇯🇵 is hot on subject.

Only cure I read was lung transplante given marrow stuff had done ... the guy in China had transplant and was cured of he lung issues and no longer needed oxygen.

[dodgylungrunner British Lung Foundation in reply to Hidden]

I had a bone marrow transplant for leukaemia when I was 18 which was a fair few years ago now. The doctors do think that the two are linked but can't be sure.

Lung transplant is certainly the only 'cure' for my condition although there are many risks attached with that as I'm sure you already know. Thanks so much for all support and the research. Encouraging to hear about the guy in China.

[Hidden in reply to dodgylungrunner]

Hi Dodgylungrunner i do admire your spirit AM sure i would not be so composed in my quest.

I think there is conflict with chromasos YA x y stuff that as causes some genetic issue to raise its head that normal would not.

Am sure you know rish of doing nothing I.E steriodes ant inflamatry drugs antibiotcs is not really a proactive treatment.

My understanding is IPPFE is next stage up from PPFE and given that i would ask about bone marrow transplant AS possable cure.

I know risk of doing nothing IS not a risk i would except every option thought would be on the table.

There is other option that would be to try Ginseng its been used for thousand years in medicine also as immunococal propites as well as celuler antiinflamatry.

In japan china ippfe is called Amitani disease

If ya was to give ginseng a waz i would always run it past your doctors

[Trinitee in reply to Hidden]

Hi Jeff

They do not know what causes IPPFE (Idiopathic Pleuroparenchymal Fibroelastosis), Idiopathic means undetermined/unknown. This is the primary form of the disease. The secondary form is known as PPFE (Pleuroparenchymal Fibroelastosis) because the disease is acquired by another process/procedure (i.e. Lung transplant, chemotherapy etc.) or separate ongoing disease (I.e. Lupus, Anylosing Spondyliosis etc.). Hope I explained that so it makes sense.

[Hidden in reply to Trinitee]

Hi yer i know the types BUT can i add most people don't wake up with ippfe its starts like most ild but as advances thats defining feature.

My replies in my view opinion ARE just as credible as anyone elses

And my other replies i think covered what point your making.

If that makes sence

[Trinitee in reply to Hidden]

I was not implying your view or opinion was not credible and I apologize if my comment came across that way. I also have narcolepsy, on top of PPFE, and at times the extreme fatigue is affecting me cognitively.

[Hidden in reply to Trinitee]

Hi sorry to read you also have ppfe ME personally I don’t think this much diffrance in ippfe or ppfe.

Guess it’s good people are talking about it AND don’t worry about cognitively lung disease defo effects all of ours.

Do you know why ppfe is not called it’s proper name Amitani disease.

[PeterG_123]

Hi dodgy lung runner, I think my diagnosis is very similar, but described as fibrosing alveoli. Its linked to my SLE - autoimmune disease. It’s complex, and in late stages with oxygen on exertion but I’m happy for my consultant QEH to share info with Brompton so will ask them to make contact. I initially had lung issues from 8 yrs old but not properly diagnosed until much later. I have no definitive known cause, but family history now indicates it may be hereditary linked. I wish you well buddy

[dodgylungrunner British Lung Foundation]

Thanks so much for getting in touch Peter. Your condition sounds complicated and the diagnosis sounds like it was also a long time in coming. How have they been treating it? I guess for you it's even more complicated by your SLE.

None of this makes sense and while I've accepted that the doctors don't know enough to be able to stop progression, I refuse to give up on the search for greater understanding and improved treatments.

[PeterG_123]

Hi, I have started the 100,000 GNOME project as my 5 year old daughter is showing symptoms. It has taken me a long time to realise I have been an experiment when it comes to treatment. I have no doubt they have tried their best. However, the reality it is rare, lots of confident speak but the consultants have to use suppression to treat it as there is no cure yet. The rarity means the drug companies won’t invest. I am determined to find a treatment for my daughter and anyone that is affected to switch it off though.

[dodgylungrunner British Lung Foundation in reply to PeterG_123]

Hi Peter. As you say it's very rare and the consultants can't really do anything other than suppression with steroids. I completely understand that there are other more common diseases that need research which are more of a priority but what's frustrating is that there doesn't seem to be much in the way of attempts to get together cases and try and establish trends. Hope your daughter is ok and never stop trying to raise awareness.

[TwinklingStar]

I have Non-specific Interstitial Pneumonitis with groundglass affect. My report stated something about parenchymal banding?? I think but I will have to get my report out to be sure. I was told I have hardening of the lungs and that this would be permanent and terminal. There are so many different types that I am not sure if this is at all relevant to your post. If it is you can contact me if you require any further information.

Thank you.

Best wishes from

Twinkling Star

[dodgylungrunner British Lung Foundation in reply to TwinklingStar]

Thanks so much for getting in touch TwinklingStar and sorry to hear about your own lung condition. There are so many different types of lung condition which makes things so confusing. In all honesty I have no idea whether non-specific Interstitial Pneumonitis is related at all but will definitely speak to my consultant about it.

Wishing you all the best going forwards - how is your treatment going?

[TwinklingStar in reply to dodgylungrunner]

Hi Dodgy

I don't have any treatment at all other than using Oxygen for most of the day and night. The only thing I was told about my condition is that it has caused hardening of the lungs which reduces my lung capacity as it prevents me from breathing in fully and breathing out fully. As far as I know there is no treatment although I was given Prednisolone at first just to reduce the inflammation. I understood that the disease I have is an auto-immune type condition of unknown cause where inflammation has caused permanent scarring and hardening of the lungs. The report stated widespread groundglass and air trapping and a lot of other stuff too.

I suppose if I start to suddenly deteriorate from acute inflammation that I would have to take the Prednisolone again. I hope I don't have to because when I was put on it I developed Secondary Cushing's Syndrome and the Consultant stated that I should not ever take the drug again. I put 5 stone of weight on over just four weeks and a large amount of this was round my neck. It was like having a large snake round my neck - it was massive. I kept waking up gasping for breath because the massive amount of fat round my neck caused difficulty breathing. I also developed many other severe side effects too. I had been on 50 mg for over 4 weeks so I could not just stop the drug. I reduced it really slowly because I needed to make sure my adrenal glands kicked in otherwise I may have had to take a very similar low dose of the drug forever. As I had such a bad reaction that would have been extremely dangerous for me.

I also have Hepatitis C (Hep C) from a six pint blood transfusion. I was infected at age 18 over 43 years ago in September 1974. I cannot help thinking that my lung condition has been caused by the Hepatitis C. It was strange that when I first developed Cirrhosis of the liver in 2012/2013 as a result of the Hep C that I developed the lung condition at the same time. Cirrhosis is inflammation of the liver causing scarring and permanent hardening of the liver. Basically the same process which has affected my lungs.

I think the inflammation caused by the Hep C has caused the scarring and hardening of my lungs which causes severe Hypoxaemia (low oxygen levels) especially when moving. The research I did showed that over 48% of people who are on the liver transplant list suffer from severe Hypoxaemia when active.

They would not refer me for a lung transplant because I am not a suitable candidate. I was born with a rare genetic condition called Ehlers-Danlos Syndrome (EDS). This combined with the Hepatitis C and related cirrhosis means that it would be very unlikely for me to survive a double lung transplant. Like you, the double lung transplant and the steroid treatment seemed to be the only options to help this condition and both turned out to be not available. I think they would have to consider putting me back on a very low dosage of the steroids if I was to suddenly deteriorate as there are no other options for me.

I think all people who have this type of lung condition should be tested for all the different types of Hepatitis because there are so many people who are infected. People think they will have been tested but the tests are not automatically included when you have blood tests because you are really supposed to have counselling prior to having the tests. There are MANY different types of Hepatitis that could potentially cause lung problems. Hepatitis B, C, Delta, E, and I think there are even more now.

If you have previously had any surgery, dental treatment, blood transfusions the risks of being infected before 1991 are very high. Even after 1991 the risk is high because there were no tests for some types. Even now they don't have tests for the newer types. Of course, all blood products are heat treated now but there are plenty of other ways of contracting Hepatitis as Hepatitis C in particular requires extremely hot temperatures to kill the virus so is difficult to remove from surgical instruments. There are treatments for Hepatitis B and Hepatitis C but I am unable to risk these because of my other health problems.

Sorry for the very long post.

Keep Smiling or in my case I will Keep Twinkling

from

Twinkling Star.

[dodgylungrunner British Lung Foundation in reply to TwinklingStar]

My goodness Twinkling Star, you've been through and continue to go through so much!

The fact that the steroid and lung transplant treatment options aren't available to you must be difficult - has your treatment team offered/tried any alternatives with you? Do alternatives even exist?

Wishing you all the very best going forwards and definitely keep twinkling!

[TwinklingStar in reply to dodgylungrunner]

There don't seem to be any other options for me other than to just use the oxygen. Although even with the oxygen I am still very breathless when I start moving. I think nothing much can be done because the damage to my lungs is irreversible damage. The main thing for me is to keep away from people who are ill so I don't pick up a cold. I very rarely see anyone other than my Carer to not much risk there.

I hope you can find some more people with the same condition as you have. It would be very helpful to you to know what treatment has been given to others with the same condition.

[Trinitee]

I was diagnosed in May 2017. I live in the US and have found there is little to no support for this disease.

[dodgylungrunner British Lung Foundation in reply to Trinitee]

Same here Trinitee! I don't know anyone else at all with IPPFE and my hospital only recently put up some information about it on their website. The rest of the info that I found on Google is all scientific research papers with case studies that don't make for great reading. The BLF have been a God send. Seen your private message and will look to reply ASAP.

[TwinklingStar]

I must try to get my report out so I can actually quote what the report states. I want to particularly look up what was stated which included the word "parenchymal" or something like that.

Did you all have lung biopsies to diagnose your condition or just the scans? I did not have a biopsy done as with all my other health problems it would have been far too dangerous.

Does anyone know which would be the safest: CAT Scan, MRI Scan or xray? They usually only do an xray. Should I be insisting on having a scan every year or can that cause deterioration for people with Interstitial Lung Diseases?

Thank you.

Love from

Twinkling Star.

[dodgylungrunner British Lung Foundation in reply to TwinklingStar]

They discussed biopsies with me but felt it wasn't an option that was worth pursuing as it's an invasive procedure which runs the risk of infection which they felt wasn't worth taking for any information they could glean from the biopsy. I've had 3 CT scans in recent times which isn't ideal but does give a far better picture than the X-rays when you're trying to look at things like pneumothoraxes. It's also the only way to really review the progress of my disease.

Without knowing your case I couldn't really comment on whether you should insist on a scan every year or not but clearly any exposure to radiation is best avoided. I think the best thing to do would be talk it through with your consultant to find out the risks of more regular exposure and balance them against the benefit of having more regular updates on your condition. Prior to my pneumothorax I went 4 years between CT scans.

Hope this helps and all the best going forwards.

[TwinklingStar in reply to dodgylungrunner]

That is very helpful information Dodgy. They seem to think that an xray is enough. I will ask about a CT scan as my annual appointment is in May. I go once a year to see the respiratory nurse and once a year to see the consultant. I can ask for an appointment at any time - for instance if I feel worse or if I get an infection. They would see me more frequently if I wanted but I don't think it is necessary. I will just ring if my condition becomes worse. I really don't want to be mixing with all those other patients who are often coughing and clearly a risk to me. I go to as few appointments as I can and as there is no alternative treatment available it doesn't seem worth attending more often.

[TwinklingStar]

I don't even know anyone else except for you and Trinitee. I may be wrong but it sounds to me as if all three of us have one of the very many types of Insterstitial Lung Disease (ILD) but I am not sure whether I am correct. Mine is classed as being of unknown origin (Non-specific Insterstitial Pneumonitis or sometimes called Non-Specific Interstitial Lung Disease).

I think I read that there are many types of ILD's but that they were not able to find a cause for what I had. I strongly believe that mine is being caused by the Hepatitis C. ILD's are classed as restrictive lung diseases. Things like COPD are classed as obstructive lung diseases. That is about all I know about it and I am sure you both already knew that! lol