Idiopathic Pulmonary Fibrosis

[tuky]

My Father has just been diagnosed with Idiopathic Pulmonary Fibrosis, he hasn't told me that himself but he told my sister and she told me. I think he doesn't want me to worry about him. My sister says he is on the lung transplant register.

I have tried looking around for info but I can't make head nor tails of what I have read.

Does anybody know what this is in laymans terms?

Is he going to die? if so how long has he got left?

A little background...I was working with him about 4 years ago in Azerbaijan, which the CIA Factbook lists as the most polluted place in the world for Air, Land and Sea. Whilst we were there (he doesn't smoke) he contracted pnuemonia in both lungs and this pnuemonia has recurred nearly monthly since. So I guess damage has been taking place for a long time.

[Mossfinn]

Here is a link but if you have been looking it up already, you may have already seen it.

Is he going to die? if so how long has he got left?

There are currently no effective treatments or a cure for Pulmonary Fibrosis. The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress.

Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients after diagnosis vary greatly.

as the most polluted place in the world for Air, Land and Sea.

While the exact cause remains unknown, associations have been made with the following:

Inhaled environmental and occupational pollutants

Hope the link helps, I have never heard of it before.

Good Luck

Moss

[zzdocxx]

That is really tough, sorry.

Idiopathic just means that the cause of it is unknown.

Pulmonary -- Lungs

Fibrosis -- the tissue is being invaded and replaced with fibrous tissue (diffuse scarring), this is generally the body's response to an inflammatory process. Obviously this will progressively compromise lung function over time.

Lung transplant -- the risks and benefits have got to be weighed, never mind the availability. The doctors taking care of your dad have the benefit of first hand information of his condition and clinical expertise.

Are you able to go and visit?

Good luck.

[tuky]

I am hoping to get there to visit him in the next 12 months sometime. It is not easy for me to get away from work at the moment and apparently he is unable to fly anymore.

This sounds interesting, I have sent the link to my Dad with the hopes he may look into it.

Aussie drug may prevent kidney disease

The drug prevents fibrosis, which irreversibly scars organs and is the underlying killer in ailments such as kidney disease, diabetes, heart failure and lung and liver problems, the report said.

Initial trials on rats have had a 100 per cent success rate.

Human trials on about 30 patients will begin soon.

The drug, known as FT-11, inhibits the production of a protein that causes fibrosis but will not inhibit healthy scarring such as that which forms after surgery.

[Nordlys]

I have fibrosis in my lungs caused by very rare disease called LCH (Langerhans Cell Histiocytosis).

In my case the disease that caused it has long been in remission but the irreversible damage caused to the lungs can one day trigger respiratory faiure or pulmonary hypertension that might kill me, and I expect myself to have shorter-than-average life expectancy. If your dad has cysts in his lungs he should anticipate pneumothorax too (puncture of lung). I have numerous cysts in my lungs but I haven't developed pneumothorax yet.

His prognosis depends of course on the extent of the damaged to the lungs. I was told by a pulmonologist at Bumrungrad that if I lose more than 50 - 60% lung function then I should anticipate pulmonary hypertension and not-so-long life expectancy. But then I was also told he knows many patients who live on only one lung or less lung capacity than me and lead a normal, healthy life. And that might be the case for your dad if he hasn't lost more than half his lung function (although he may not be able to do diving or climbing). Pulmonologist can perform pulmonary function test on your dad (a bit arduous test) which should reveal how much lung function he has left (not necessarily capacity). Is his lungs bad enough he has to breath bottled oxyen or oxygen concentrator at home? If not I wonder if really has to be put on lung transplant register.

What he might want to know is what triggered his pulmonary fibrosis. I've gone through numerous testing that included broncoscopic examination (really unpleasant test). Doctors narrowed it down to LCH and Sarcoidosis but in the end I had to take open-lung biopsy for concrete diagnosis which took me 3 weeks hospitalization for recovery (during which time I was put on oxygen). Pathologist has determined it was LCH. Without it, not only can they treat you or to determine if it's an active disease or if it's already in remission. And if it's determined to be active diease ('positive' diagnosis in pathological test) then he will need extensive medication attention or it might progress irreversibly.

Edited August 30, 2008 by Nordlys

[tuky]

Nordlys, according to my sister he has only 50% lung capacity now. She says they determined this through an MRI. I am not sure how it all works to be honest.

Anyway, he has another appointment with a specialist on the 18th of next month. Hopefully we will know more then and hopefully it will be good news.

He says he thinks the Docs (3 of them so far) have made a mistake because he feels fine.

Thanks for the information and I hope things go well, not only for him but for you too.

[Nordlys]

tuky, I don't think pulmonologists can tell what percent lung capacity a patient lives on just by looking at the radiographic image of the lungs (and it usually takes CT scan, not MRI). They can only tell that from lung function test. And if he's not even tested on that chances are he may not have seen a specialist (pulmonologist) yet. And if that's the case I seriously doubt if his condition was determined to be in advanced stage that he has to be put on waiting for lung transplant, let alone if he says he's feeling good and not receiving supplemental oxygen at home.

Anyway, here's an article on Idiopathic Pulmonary Fibrosis and Pulmonary Fibrosis on Wiki.

Remember "ILD (pulmonary fibrosis) is not a single disease, but encompasses many different pathological processes. Hence treatment is different for each disease." Why I told you it's important your dad needs conclusive diagnosis as to what's causing fiborsis and whether or not it's progressive (or in remission).

Edited August 30, 2008 by Nordlys

[tuky]

As an update, seeing as it has been 3 years since I raised this topic.

Dad is still kicking, he still goes to work each day.

He is however getting worse, a short conversation will leave him breathless.

He uses oxygen at home and whilst sleeping, I bought him a portable oxygen generator that has wheels and fits into a check-in luggage sized bag, he uses that when he wants to get around.

He lives in fear of catching a cold, as that would tip him over the edge. Recently he has been hinting quite brazenly that I should visit him so I have made the arrangements and will see him next week as I am sure he would not hint for no reason.

One thing he told me about this problem that really struck me was this...He says most mornings when he wakes up he has forgotten he has IPF and he spends the first minutes thinking he is drowning, he then remembers he is sick and that is why he can't breathe and settles down again. That must be terrible for him.

Anyway, I am glad he is still around and I look forward to seeing him next week. I am sure then I will have a better idea of his condition. I miss the old bugger

[tuky]

Well, just another update.

Dad is still alive.

He is on morphine 24/7 to help keep him calm and get rid of any pain.

He also takes a lot of prednisone, ativan and a whole load of other drugs.

He doesn't move unless he has to, even the need to urinate is taken care of with a special container so he doesn't have to get up as he cannot.

he is in hospital at the moment as he had a bad turn and panicked which dropped his oxygen saturation point too low and he lost consciousness.

He has done well, he was told he had about 12 months to live and that was 4 years ago now.

I have been reading up on this condition and everyone agrees that it is a very cruel way to go.

I guess the next time I post in this thread will be soon and it will be a lot sadder than this post.

[Sheryl]

Sorry to hear this Tuky.

From what you have said the situation is now terminal and if it has not already been done, he should be formally enrolled in hospice care and a "do not resucitate" order given (he would have to agree to this, of course).

I would also advise discontinuing all teatments other than those needed for comfort, including antibiotics. At this stage it will only prolong suffering to do otherwise. If he is in/gets into a hospice program hospice staff will probably advise the same.

[tuky]

Hi Sheryl,

He is staying at a nice place now. http://www.glengarryprivate.com.au/Our-Services/palliative-care.aspx

I have decided to get some help myself in order to deal with what is going on from an emotional point of view.

I think that is a wise move on my part.

I have moved back to my home country in order to spend time with him but to be honest it is not a nice thing to be watching.

Do you remember the other health issue concerning my sister in law we have discussed? I brought her with me and have had her seeing a Renal specialist here, expensive but worth it.

She is doing really well. The specialist has her on Cyclosporine and a heap of other meds. Her urine initially had around 1200 parts per whatever protein but is now at around 400 which the doc is really encouraged about. He told me he had never seen an albimun level so low in any patient, if I am correct it was at 4 and the lowest he had seen before was 12. I can't quite remember what the normal level is but it was quite scary.

As her liver wasn't producing enough her body was taking the protein from elsewhere, which meant she is now a stick. Her muslces have been eaten away. We didn't notice this as the fluid build up was such that she had to sleep sitting up in order to breathe, we upped her diuretics and she shed 12 kilo of fluid in a week to reveal a skeleton of a girl.

Anyway, she will continue with the Cyclosporine for probably another 12 months. Thanks so much for all your advice and understanding on both these issues. You are a true blessing.

[Sheryl]

Looks like an excellent facility.

Gla dto hear your sister in law is doing well and getting proper care.

And very glad yo uare getting help, it is indeed not an easy thing to watch, it creates problems on many different levels and is a lot to process.

Goodl uck,

[tuky]

Dad finally succumbed to IPF last night.

He put up a good fight, four years.

His last few days were spent sedated as the Drs in the hospice said it wasn't fair on him being awake.

It is family time now, time to start life again.

Thanks to those that responded.

[Buchholz]

Dad finally succumbed to IPF last night.

He put up a good fight, four years.

His last few days were spent sedated as the Drs in the hospice said it wasn't fair on him being awake.

It is family time now, time to start life again.

Thanks to those that responded.

Condolences to you and your family.

Your Dad raised a good son.

.

[Sheryl]

Our condolences. Your father was fortunate to have such a loving son.

[harrry]

I am sorry for your loss. Take care. DO not forget to look after yourself now.