[Jofac O'Handlin]

Hi Jim,

You ask most pertinent questions. It is accepted that there no cure for our complaint, and the extension to our life by taking either of the two treatments against the side effects is questionable.

I started a thread asking for experience of the transfer from OFEV (nintedanib) to Esbriet (Pirfenidone). I received many replies indicating that for some the side effects of Esbriet were small, brain fog, nausea, and sun sensitivity.  I was concerned by the prospects of all three.

I am under the NHS  system in Britain, so do not have the insurance worries experienced by North Americans, but the NHS prescibes!.? I ‘was’ a long term patient, 5 years +, on OFEV at 100 mg x 2 per day, which I survived on. I had for the first 3 months been on the 2 x 150 mg dose but tummy troubles, and was dropped to the lower dose. On the 100’s I had the usual bowel problems at a lesser level, and slight nausea…… but OK!

The alleged reason for the proposed transfer was due to a loss of weight (nominally I’m 6 foot, 11 stone drop to under 10 stone) this over 6 months. However, I had over 2 months or so with dietary advice (chocolates and sweet puddings)  started to correct this and when the intervention came had regained some 6 lbs. I was content to stay with OFEV! But they insisted!? I am suspicious of their motivations. So! At the end of January, first week of February I started the 3 week ‘titration process’ of building up to the full 3 x 3 x 267 mg Esbriet tablets  daily.

Sorry to take so long setting the scene.

But tben, having received a 84 day supply, and having worked up to full dose, all seemed OK. I continued for about 60 days, and contacted the IPF nurses to report all satisfactory and to put in place a repeat prescription.

Can you imagine my dismay, over the weekend following, my exposed skin (ears, neck, backs of hands)  became hypersensitive to light. This was always a concern as I am fair skinned, typically Irish / Scottish Celtic gene pool, with a hint of red in my hair. I had long ago learned not to sun bath, and to cover up. I were wide brimmed hats, even if they do not suit me!

Two things happened, the redness of sun damage which did not fade away. The itchyness which was disturbing over night, and a third item little lumps under the skin around the neck and side of face. I descibed it as Braille, rather like the impressions on our medication boxes.

The advice was to stop for 2 weeks, and was monitored to check my recovery. It was agreed that I could start the titration process again. On day one, after just 3 tablets, I was immediately aware that my skin was reacting. I was keen to continue (what can you do?) and covering up, much as the women in Afghanistan are now required to do, or factor 50 sun screen indoors. Towards the end of the week my local pulmonary nurse checked with me and agreed to continue into a second week rising to the 2 x 3 tablets per day. However, that did not happen, as over the weekend the prescibing IPF nurse called and INSTRUCTED me to discontinue! Bear in mind I now have a further 90 days of Esbriet in hand! Note: monthly liver function tests were normal.

At present I am not taking any medication. I await a further lung function test, in 2 weeks time. I anticpate that my FVC will have dropped and that they will have a reason too discontinue treatment …. but that is me being pessimistic!

As a bye the way, i am suspicious of the reason for the change in meds.

The Esbriet was delivered with an invoice indicating the cost to the NHS, which seems to be something near £ 2,500 per 12 weeks, i.e. about £ 11,000 per year.

The last indication for the costof OFEV to the NHS was about £ 72 per day, i.e. about £ 26,300 per year.

So, Esbriet seems a significant saving!

I have indicated a preference to return to OFEV.

I will say that I am on tne cusp of going onto regular oxygen. I have noted a significant decline in my physical ability over this year, and have doubts about reaching my 81st next year. Cannot complain ….. still vertical!

Sorry about the length of this.

Best wishes and regards to you you all.

Joe

[Jofac O'Handlin]

Dear Jacqui,

I anticipate that you will get lots of support on this site, much of it with better knowledge than I can offer.

First, I would suggest getting medical advice for the pain / discomfort that your Mom is experiencing. It is, I believe, known that IPF causes stress on the right ventricle of the heart.  Does this cause chest pain?

Second, I would agree that body mass should be related to medication levels. Let us face it, small people get drunk a lot quicker than large overweight, And I imagine that anethnetist’s monitor levels related to body weight.

Thirdly, brain fog seems par for the course. In my initial period I did not particularly notice, but today maybe, or was it my disturbed sleep last night.

Finally, is your Mom taking the tablet with a meal? This seems to be recommended for minimum side effects. I have just restarted Esbriet, literally yesterday, after a 3 week rest, due to skin issues. After only 2 tablets my skin has become sensitive again, itching overnight, but that is a different issue. Starting the titration process again. Last night on our evening walk I noted some chest discomfort, chose not to mension it! If it is heart, so be it!

After over 8 years, I now carry a DNR (Do not resuscitate notice), as recommended by my G.P., sorry to admit that this is the situation. I try to stay active, and recommended this if possible.

Kind Regards, Joe

[Jofac O'Handlin]

Hello Charlene,

A quick note, I was diagnosed with IPF August 2013, my sister in 2015. But our daughter diagnosed with sarcoidosis, following the birth of her two children, about 2012, aged 37.

As I have said on previous threads, I would like to know more about familial / gene links. My daughter ‘is my daughter’, she inherited my curly ears (as has one of the grand kids) and my very pale / fair skin. Sunburn very easily.

If I make August, it will be 9 years, can I make 10 mmmm! Not sure.

Regards to all contributors.

Joe

[Jofac O'Handlin]

Hello all,

Well just to update you all on my progress on my transition to Esbriet. There was a week or so as I ran out of OFEV, and delivery of the Esbriet. Then the two week build up to the full dose. I noted a reduction in my physical ability over the period, as monitored by our 30 – 45 minute evening walks. Not scientific, but aware that I am obliged to stop on the hills that I could get over a month or two back.

So, up to full dose by mid-February, and then over a month in which all seemed OK, with a little ‘brain fog, and improvements in bowel / GI tract control. Feeling confident about continuing!’

Then ! Last week,  I had just emailed the nursing team regarding the repeat prescription, and commenting on how well the transfer had gone. Reduced GI problems, occasional rectal discomfort. WHEN, I started with skin discomfort, is the way I put it. It started with a low level irritation on my cheeks and under my jaw. Initially I thought it was a shaving issue, still a wet shaver. But over this week the irritation and itching has spread, Cheeks, neck, on and behind ears, top of head, and backs of hands. There has appeared small lumps / bobbles on either side of my neck below my ears.

I wear hats ( as have little hair on top), and have had virtually no sun exposure, except for our evening walks. The instructions do say to apply factor 50 sun screen even indoors. Started yesterday. I do not like the geasiness and am investigating ski / cycling face and neck tube type covering …yashmak?

I am itching even as I type this missive!  Covered with my sun-block! And trying NOT to scratch.

Good luck to all, thank you for your support and interest.

Kind Regards,

Joe

 

[Jofac O'Handlin]

Hi All,

Especially, Patrick, Linda, and Margaret, and your conversation on ‘silent reflux / GERDS’, barium swallow tests, and hiatus hernia.

When we are diagnosed it is natural to wonder what caused this disease. I have said it before, I blamed my time working in small Diesel driven Power Stations. Much vibration, creating dust, probably laden with asbestos! However, my sister did not work in any industrial situation, yet she died from IPF  at the age of 85.

Now, she was diagnosed with refux some years (5 to 10) before her diagnosis with IPF. As a late diagnosis of her IPF she only survived 18 months.

She was taking proton pump inhibtors (PPI) (Lansoprazole or Omoprazol), which I understand inhibit the uptake of calcium and thus likely to accelerate osteoporosis. She, in her late 70’s / early 80’s had a couple of minor falls which resulted in broken bones. At the time of my diagnosis I was also prescribed a PPI but found that they caused me GI problems, especially when eventually prescribed OFEV.

Looking back on my history.  Like Patrick and Linda, I had a barium swallow test about 20 years ago. The why was that done? Well, the year before, when I’was 59, I had a bicycle accident which rendered me unconcious. The ambulance crew fitted a tongue restraint / breathing tube. When I came too in the hospital my throat was my main discomfort. Very sore,  About a year later I followed up with the barium swallow test, which proved that there was some small leakage into the wind pipe. This was regarded as minor and left to self heal.

A few years later, in lifting a motor scooter into a motor home cradle, I suspect that I caused a ‘slight’ hiatus hernia.  About the same time I started with a regular runny nose, rhinitis. All this in the 10 years leading up to my diagnosis of IPF.

So here I am nearly 9 years on from my preliminary diagnosis, 7 years from my lung wedge section biopsy that confirmed ‘the worst’! Now almost 7 years on OFEV, and a few months on Esbriet.

My present situation. I do not take PPI’s, but do take standard anti acid tablets. Recently,, I found that I was waking in the morning with a sore throat. My routine was evening meal at 8 / 8:30. No wine, no sweet desert. Then at bedtime 11:00 ish, an anti-acid. It was on this routine that I in the last 3 months started with a morning sore thoat which wore off as the day progressed. Initially thinking that it was a cold or other infection. I then wondered if it might be reflux? So over the last 3 weeks I have been taking my regular anti- acid as I get into bed, also setting my alarm for 3 am and taking a double action (Gaviscon) type anti acid tablet at that time.

This routine seems to have stopped the sore throat, and reduced the runny nose. By the way I have used wedge pillows to raise my shoulders, but, one inevitably slides down though the night!

Margaret, you mension Barrett’s Esophagus, possible result of silent GERDS. It does make me wonder, and interested in having this checked. The reason being that a male cousin, on the paternal side of the family, meaning that we should share the Y chromosome, (unless a milk man got involved) suffered from cancer of the esophagus. With his problem, and my sister, I wonder about familial connections.

As a friend of the Jewish faith used to say, ‘you cannot beat your genes’. Or in the 10 commandments, ‘even unto the fourth generation’. All this from an atheist!!!.

Kind regards to the community. Joe

 

 

[Jofac O'Handlin]

Hi Cecil,

Like you I started Esbriet (pirfenidone) 2 weeks ago Monday, so have just had my 3rd day on the full 3 x 3 – 267 mg dose. At present I feel lack lustre but not dizzy or nauseous. GI problems much better, but not quite back to normal.

A quick summary of my position. I am the person transitioning from OFEV to Esbriet due to weight loss. My history is preliminary diagnosis Aug 2013. Confirmed by lung biopsy Dec 2014. Started OFEV May 2015.

In the discussions with the Respiratory Consultant prior to the biopsy it was indicated that there was a ‘family’ of lung problems which wlll show on scans. Some less serious than outright IPF. In my case, the biopsy revealed the worst of the options. I am fortunate that over 9 years on I am still ‘vertical’.

On the forum I found: http://www.atsjournals.org   A Novel Antifibrotic Mechanism of Nintedanib and Pirfenidone. Inhibition of Collogen Fibril Assembly.

This is a big read, using a lot of medical language that I, as an electrical engineer, have difficulty with. The main thing is that it indicates the effect of the treatments are dose dependant.

Due to GI troubles I couldn’t take the recommended dose, 1 x 150 mg twice per day of OFFEV (nintedanib). After 1 year I was reduced to the 100 mg capsules. So I’ve had almost 6 years on that treatment at the reduced dose.

The mechanism of PF is highly complex, and variable from person to person. My observation is to keep as active as possible, eat healthily,  and keep moving as far as ones declining abilities allow.

My evening walk of 2 miles (+ if poss) is essential, my speed is not what it was, and hills harder, but still making it without O2 …. just about!

I wish you well, and hope that you are able to adjust to the medication.

Best wishes, Joe

[Jofac O'Handlin]

Hi Sybil,

Most interesting to hear from you and your experience on the nebulizer trial. It sounds rather like the two approved treatments for IPF, it is difficult to know how effective they are, or have been.

I have been on OFEV for almost 7 years (living with side effects) and suffering a gradual continuous decline in my physical abilities. How quickly would I have declined without it? I am in the process of transferring to Esbriet (pirfenidone). Will updated this thread soon, when I have reached full dose.

I wonder if your trial is a blind trial, with a proportion receiving  a placebo. Which trial are you on?

We all wish you well on this, hopefully successfull, trial.

Kind Regards, Joe

[Jofac O'Handlin]

Dear Julie,

We offer our sympathy at the loss of your father. Our thoughts and prayers are with you at this time.

Kind regards, Joe

[Jofac O'Handlin]

Dear Contributors,

My observations.

I have survived over eight & half years since diagnosis in August 2013. Pre the antifibrotic medications. The consultant said about 6 years, a GP in our medical centre gave me the standard 3 year prognosis.

My sister, 11 years my senior,  was diagnosed (or we learned about it) about 9 months after me. She survived only 18 months.

As this disease is progressive, I suspect that it may depend on what triggers the investigation. I had suffered a bout of pleurisy some years earlier(2010?) and had a heightened awareness of lung health also due to my time working in power stations with much asbestos.

In February 2013 both my wife and I got pneumonia as a spin off a viral infection. My wife recovered reasonably quickly. I did not. This triggered further medical investigation. I was also feeling it, knew something was wrong by my declining athletic ability.

My sister led a sedentary lifestyle, so the lung vital capacity was not tested, until it affected her pesdestrian lifestyle.

So, how long from prognosis? This depends on many factors, but also how far the IPF has progressed at the moment it is discovered.

I think mine was found early, sister found late!

Regards to all.

Joe

[Jofac O'Handlin]

Hi to all participants on this thread.

I am in the process of transition from OFEV to Esbriet (when I receive them!). This propossed due to progressive weight loss. A loss that I now have under control, Having re-gained 10 lbs over 2 months!

On another thread, I had asked about side effects of Esbriet and had a lot of positive responses. This thread has got me somewhat concerned. Nausia and tiredness are not desirable, I do not handle them well.

We will see!

Thanks to all, Joe

[Jofac O'Handlin]

Hi Christie,

I have just spotted your thread on ‘IPF Awareness’. So will follow you there. Jeff remarked that he is a, “research champion for the APF here in the Wessex region”.

Jeff, what does that mean? Is it a mechanism that raises awareness, and publicity for IPF sufferers?

I got annoyed with the BBC radil the other day as it had a 3p minute programme on a rare (male) affliction, whilst the increasing number of IPF sufferers seem to go unheeded. I suspect that it is the imagined link to cigarette smoking.

Something that I would like to follow up is the ‘familial’ side of IPF. My sister died from the same at 85, hope to make 80, but doubt 81! I wonder about gene testing ot establish if I have any of the mutations that increase our risk.

Kind regards, Joe

 

[Jofac O'Handlin]

Thank you Terrance for writing about your experience on Perfenidone.  I really do not want to feel ill, or debilitated. I did ‘off’ at times on OFEV (ninredanib) but now, other than bowel problems and weight loss. I have adapted, and tolerate it well now, admittedly on the lower 100 mg  dose.  It has been the weight loss that has triggered the proposed change, despite the fact that I have regained about 7 lbs over the last 2 months or so.

As a northern man , with avery pale skin, exposure to sun will be a concern, I will take your advice on protecting my lips.

I await my replacement medication, and will update the site with my ongoing experience.

Regards to all, Joe.

P.S. Christie, and Jeff, I will follow up, and ask about raising awareness of our afliction.

[Jofac O'Handlin]

Hi Tim,

Thank you for your very informative email. I will take your advice on taking the medication mid meal. I am very concerned about the prospect of nausea. This was also suggested by other participants. I feel easier about the sun burn risks. Living at 54 degrees north means a weak sun strength most of the year, with longer days in summer. Also the fact that I have been avoiding direct sun for the last 55 years. Like you I am surviving with O2 levels at the low 90’s / high 80’s, and also trying to stay active. The ‘walk & talk’ groups give me the option, walk and listen, or slow down and say something!

I am trying to maintain weight despite OFEV, and snacking with ‘rocket fuel’, ie chocolare bars are back on my menu! My sugar levels being OK.

Regards, Joe

 

[Jofac O'Handlin]

Dear Friends,
Thank you all for sharing your experiences of Esbriet with me. It has certainly reduced my fears of the side effects.
Dave, Brian, Malcolm and Russell, your positive comments regarding sun burn risk and the lack of nausea give me confidence to follow the recommendation of the Aintree IPF clinic in Liverpool. They offer me the option to revert to OFEV should I not tolerate Esbriet well.
I am less concerned about the sun risk, as I gave up attempting to get a ‘tan’ in my 20’s. I now wear hats (even when they don’t suit me!), long sleeve shirts and long trousers, only backs of hands and face exposed. Russell the idea that you can survive sun exposure on Esbriet in the sunshine of Australia gives me most confidence.
I accept the caveats that we are all different, and may react differently.
Bernard, I note that you are also transferring from OFEV to Esbriet. As I, are you in the British NHS system? Your comments on the nebulised treatment trials are most interesting. The logic of feeding the treatment as near as possible to the site of injury has merit. IPF News has published a number of hopeful therapies, and should I say that we all wait with bated breath. Dear, Dear, Sorry. All I know is that they will be too late for many of us long term sufferers. I failed to pass the entry requirements for the Galapagos trial at the end of 2019. Also, your comment regarding Lansoprazole. I was also prescribed that or a similar protein pump inhibitor. It was to reduce the risk of reflux by passing the sphincter and entering the airway, a suggested cause or irritant to the lungs. I found this combined with OFEV seemed to make the diarrhoea worse. I simply take a proprietary anti-acid on retiring to bed, hopefully to give me protection overnight.
Russell, do you associate the insomnia with Esbriet? My sleep patterns are a bit erratic, but assume that this is due to my advancing years, a couple behind you!
Mike, I have friends living in Fort Collins, a mile high. Once I was diagnosed with IPF I knew that I would not likely accept their long standing invitation to visit. Your average air pressure at 7500 feet is about 11.12 psi, at 4500 feet about 12.45 psi. So, if you moved down, I imagine that you will get a boost of about 12% more air and therefore oxygen. I live at sea level, well under 200 feet, so we here are at a nominal 14.6 psi, so do I get about 33% more per breath? Or is it not that simple?
I am typing this as the New Year bells are ringing. With the advances in medicine that have been demonstrated over the past 2 years, we can hope that an accelerated effective therapy for IPF appears this year.
My good wishes to you all.
Joe

 

 

[Jofac O'Handlin]

Hello to all you kind folks in this IPF community.

I thought that I had ticked the ‘Notify Me’ box, but seemingly not. If I had done so I would have replied to each of you as the emails were received.

I am putting this quick response on, to say a grateful ‘Thank you’ to all your very helpful replies showing the positve side of Esbriet. I think that I will take your corporate advice and accept the medics proposal for the change.

I will be having a blood test on Thursday for liver function values, this will be for a follow up consultation in the first week of the new year when a decision needs to be made. Your answers are most timely.

Thank you, thank you, thank you,

Will reply soon, Joe

[Jofac O'Handlin]

Hi Martha,

Like you, I had a preliminary diagnosis of IPF by X-ray and CT scan. That was in  August 2013. Under the NHS system in tne UK, referrals and consultatios tend to proceed at a pedestrian pace. I was offered a biopsy about a year later.

I was, as you are, faced with the choice of  leaving well alone (acceting the diagnosis) or going for an invasive procedure with the attendant risks. It was put to me that there is a family of lung issues under the expression IPF. As I like ‘to know’, and on the retional that I would add a miniscule amount to the knowledge of the disease. It would also confirm if the the IPF was associated with exposure to asbestos, having worked as a young man in power stations. I decided to have the procedure,  a so called wedge section. This took place in December 2014. My results confirmed in March 2015 indicated the worst of the apparant several options, but no asbestosis.

I will also say that I may have a genetic propensity for IPF, as an older sister died of the complaint at the age of 85.

I was then prescribed OFEV (Nintedanib), which I have taken for over six and a half years. My physical abilities have slowly declined over the years, and whilst I have oxygen prescribed I am able to proceed with only occasional O2 use.

This may not be helpful, but it is my story, so far!

All the best,

Joe

[Jofac O'Handlin]

Hello everyone,

A couple of notes:- my local care team do not seam to understand that the bowel troubles on OFEV are variable. Periods of relative stability ( Oh! Joy) interspersed with periods of discomfort & diarrhia. Also weight loss.

Their latest proposal is to swap me onto Esbriete (Perfedinone). I feel a reluctance as nausea seems to be a side effect, one that I would not react well to. Am I being suspicious in the motivation for the proposed transfer, as the UK approval authority, NICE, has recently approved OFEV for non IPF use and they need the resources?

Also, on Thursday, I see a dietician to check my eating habits, and to see if diet change may help me maintain or increase my weight. I am 5ft 11 ins and 140 lbs, having lost 10 lbs this year, and about 10 in the preceding 4 years. I am taking photo’s of my meals to demonstrate my present diet!

Good luck all, when is a new better control going to appear?

Joe

[Jofac O'Handlin]

Hi Brian,

In principle I agree with you, but when you feel that time is running out, straws start to look very attractive!  There have been so many miricals in the treatment of cancer in the last decade, one feels that there will be a breakthough in the treatment of IPF soon. But how soon?

Cheers (keep smiling), Joe

[Jofac O'Handlin]

Hello contributors,

A hospital (Ashford and St Peters NHS ?) in the south of England is apparantly involved in a trial of N115 for COVID 19 patients. I made an enquiry from the lead doctor, but as of the present I have not received a reply.

My situation is 8 years from diagnosis, 6 years on OFEV and realise that it may be too late for new therapies to be helpful, but one lives in hope.

Regards all,

Joe

[Jofac O'Handlin]

Hello John and Dennis,

Thank you for your replies.

John, I did not drink a lot of coffee, but enjoyed an occassional Latte. Like you, I eventually, after a lot of years, identified a link with coffee and a reaction in my bowels. So,  I have also stopped.

Dennis, Thank you for your suggestions, also identified coffee as a problem. I have taken up your apple cider vinegar and honey drink. It is most pleasant. I was looking for something, other than the usual beverages, to maintain hydration. A friend suggested a slice of cucumber in a litre of water, but it was not to my liking.

Thank you for you support, anf of all on this site.

Regards, Joe.

[Jofac O'Handlin]

Hello Girls & Boys,

I had a call this morning from one of the IPF nursing team to check my level of suffering on OFEV, …. loss of weight, feeling off, and loose stools. I try to get an evening walk around our village taking about 3/4 hour. Frequently it is a struggle to get back, straight into the toilet. Try to control it with Loeramide capsules.

After 6 years on OFEV my weight has declined from a lean 162 lbs to an almost anorexic 140 lbs but presently stable.

The enquiry lead up to the suggestion of a change from OFEV to Esbriet (Perfedinone). Having read some of this rather aging thread, it would appear that it may be a jump ‘from the frying pan into the fire’. Has anone experience of this?

Can anyone give me suggestions, or is it an individual case. …. We all react a bit differntly?

The group thoughts would be appreciated.

Thanks, Joe

[Jofac O'Handlin]

Hi Charlene and other participants,

I seem to have an answer!

This thread is appropriate to my situation, if rather dated. As noted in my following enquiry of yesterday. The suggestions of an OFEV holiday seems appropriate for me. So propose to take a week off.

This was my enquiry:-

Hello All,

I have been on OFEV now since May 2015. Initially on the usual 2 x 150 mg for about 9 months to a year.

The usual gastric / intestinal problems resulted in a reduction of the dose to the 100 mg tablets. This has been now my regular dose for over 5 years. During that time my intestinal problems have generally been under control with occasional upsets.

I have recently been having an ongoing upset which makes me feel generally unwell.

I would like to ask the question; has anyone taken a rest from OFEV, maybe a week or two allowing the bowels to settle / recover?

Any suggestions?

Thanks,

Joe

 

 

[Jofac O'Handlin]

Hello Marj, You pose an interesting question. My blood pressure has always been in the healthy range, 125 / 80 occasionally 135 / 80 ish which I attributed to having been a lifelong runner/jogger. Recently, despite being restricted to a much more sedentary lifestyle my blood pressure numbers have fallen into the 110 / 70 range, which does not make sense unless other factors are involved. Your observation about pulmonary loop pressure does need investigating. A casual remark by a consultant some years ago referred to right ventricle stress and distortion indicating that the side of the heart was working harder, thus higher pressure,  to circulate blood through the pulmonary side. I have a series of appointments over the next few weeks, starting tomorrow, so shall make some inquiries. ….however, … you are dependent on my fragile memory! How often do we get back in the car and remember the vital question that you had intended to ask? Kind Regards, Joe

[Jofac O'Handlin]

Hi Jeff,

Thank you for your contribution, it did make me laugh, ‘engineers small brain’. I was / am an electrical engineer, now retired, the years of study and training does give us a wide understanding of mechanisms, perhaps even biological! Like you, when diagnosed I was as fit as a flea. Never smoked, and at the age of 70 was getting out for about one hour running / jogging / shambling (down grading with advancing years) twice per week. Resting rate at that time 48 bpm or lower depending on fitness level. I was going to try for a local 200 metres record for the veteran’s 70 – 75 age group record, but!

I  was lucky in so far that I was prescribed OFEV prior to its approval by NICE. The consultant said that it would be provided on a ‘compationate basis’. I took this to mean, that as I was (had been) in good physical condition they would let me have it. My presumption being that it was being supplied directly by Bohringer Ingleheim’s on their research budget! That was in May 2015, so now 6.5 years down the track, now almost 8.5 years since preliminary diagnosis.

I agree with you that any treatment (engineers brain) should be targeted at the goal, not spread around the field,  our body in this case. So, it is logical that a nebulised form would seem to make sense. PF News has had many articles on prospective promising treatments. This is where I get upset, the time to get through trials! Covid demonstrated what is possible when there is an economic driver. That is what we, with our disease, appear to lack. Yet I am surprised by the number of people in our small community in the Isle of Man that I know with IPF.

Let us hope for a break through this year.

Regards, Joe