What can you tell me about treatment for lung fibrosis

[huddy]

[tanyamarie]

Hi huddy?

Do you mean, IPF?

[huddy]

Hi, thanks for your reply.

Not sure, the Dr just said it was lung fibrosis

[huddy]

Hi, thanks for your reply.

Not sure, the Dr just said it was lung fibrosis

[amagran]

my husband has idiopathic lung fibrosis, the idiopathic means unknown cause. treatment depends on a ot of different things. in my husbands case, oxygen i

[tanyamarie]

Fibrosis means, scarring of the lungs. best advice I can give is to read what the BLF website says about it. The more you read up on it the better you can manage it.

My dad has IPF like amagran's hubby, and he was diagnosed back in 2008. Only recently has he started to use oxygen and he also has other medication. There is no cure for IPF, only a way of stalling the progression and the sooner it is detected the better so treatment can start.

Life is not all doom and gloom and it is down to you how you live with it. My dad is very positive and so are we and live life to the full as best as we can. We remain optimistic and I hope you do too x

[amagran]

good answer tanyamarie

[maggie44]

As others have said pulmonary fibrosis is scarrinng of the lungs. If inflammation in the lungs is mot treated fairly quickly,scarring follows. The scarring makes the lungs stiff and thus hinders the flow of oxygen into the blood.

Idiopathic pulmonary fibrosis means that there is no known reason for it occuring. However some people have PF as a result of autoimmune diseases such as rheumatoid arthritis, lupus etc and rarely sjogren's syndrome -which is what I have.

The treatment for the two types of PF can be different, so may be important for you to know which you have.

As others have said - there is no cure but there are a variety of treatments which can halt or slow down the progress of the disease, Again as has been said, the earlier you get treatment, the better - at the moment scarring which has already occured cannot be erased. There are specilist hospitals that are up to the mark with the latest teasts and treatments so you may want to explore that option. I wish I had done so earlier.

Hope this helps. It is useful to find out all you can about PF (you will find that many GPs and even respiratory specialists in a lot of local hospitals do not always know a great deal about the illness.

Maggie

[Posative in reply to maggie44]

hi maggie i have also resently been diagnosed with IPF what is the difference between the 2 types and how can they tell this i have been asked to have a biopsy but have put this on hold untill i can find out more,do you know what treatments are available as no one at the hospital has said anything to me about treatments.

I would apreciate any info you can give me.

regards julia x

[tanyamarie in reply to Posative]

Hi condemned,

IPF is where you don't know the cause and the other is where the cause is known, like from taking certain meds for example. Mostly it is unknown. Alot have a biopsy, that is the most definitive way of confirming a diagnosis.

You have the same problem as my dad, no-one told him the seriousness of the illness and no treatment plans were ever discussed. We are only now getting somewhere because he chose to go private but we are fully supported by our GP. There are a very few consultants who specialise in PF and the best way I have found is to ask your GP for the name and hospital location of a private specialist in PF. They shuld have all the details like our GP but if not look at the nearest private hospital website to you and they should give all the info about the docs specialties. Onc you have located one, you should be able to find out where thy work when they are working for the NHS and you can ask your GP for a referral. We found it makes a massive difference if you are being tretaed by someone who actually knows the disease.

Good luck.

[Posative in reply to tanyamarie]

thanks i think this will be a good starting point

julia x

[maggie44 in reply to Posative]

Hi

As far as I can understand, if the PF is caused by an autoimmune disease, the treatment will aim to damp down your immune system, since it is this that sets off the inflammation in your lungs and then causes further scarring. So like the othres here that have PF caused by our own immune systems I take prednisolone, azathioprine and N-Acetylcysteine(NAC). About a year ago I also had a series of 6 X 4 weekly infusions of cyclophosphamide. I use 6 lpm oxygen for ambulatory purposes and more recently 2lpm oxygen for 8 hours at night.

I think that those who have IPF would not necessarily undergo the same treatment, since presumably it is not their immune system causing the problem. I do know that there is a trial going on at the Royal Brompton using the drug pirfenidone for people with IPF. I was not suitable for the trial because my PF is not idiopathic(incidently they now give my disease a different label 'Sjogren's related non-specific interstitial pneumonitis'. I think this is to differentiate it from the Ideopathic type of fibrosis.

Perhaps they have not suggested any treatment yet because they need the test results back first in order to recommend the appropriate medication. All of these drugs have side effects, so caution is needed before decisions about using them are taken.

Pulmonary Rehab is certainly something to access - it is brilliant - helps you understand your illness and how to manage your breathlessness. It also helps you to get some appropriate exercise with confidence and meet others with similar conditions and limitations.

Maggie

[Posative in reply to maggie44]

maggie thanks for info very helpfull julia x

[BugsBunny]

I have pulmonary fibrosis due to rheumatoid arthritis. The treatments I have received are immune suppressants and oxygen therapy. However, you will need to see a respiratory specialist as soon as you can to get a treatment plan. There are 2 places you can go to meet people with the same or similar diseases to you. The first is your local Breathe Easy support group which will have speakers, activities and outings. The second is Pulmonary Rehab at your local hospital where you will learn to exercise and be given useful information. Your lifestyle will change but you can still have a jolly good time living with a respiratory disease. Good luck!

[sadie28]

Hi huddy

I have PF due to sarcoidosis. I have steroids, immunosuppressants and oxygen. I know IPF is named that because there is no known cause for it occurring but wonder if anyone can tell me if all PF is the same whether the cause is known or not.

[pechinawa]

Hi huddy

I also have scarring of the lungs and was told i had IPF but after a biopsy was told it was Pheumonitis which is a type of Fibrosis which also has no cure.

The Doc put me on a course of steroids which i must say have horrendous side effects after four months and gaining four stone in weight the steroids were of no help to my breathing and will take a year to come off them.

As this is the only treatment there is where do i go from here

If you are offered steroids i would read the side effects before making a decision.

[tanyamarie in reply to pechinawa]

My dad started an extremely high dose of prednisolone a month ago and has another month to go. What a difference! He hasn't gained weight as the Doc warned him it will not make him put on weight, it will give him a better appetite so he made it very clear that it was down to him to monitor it. And the people he met at the breathe easy meeting said the same.

My mum noticed that he was eating everything on his plate, whereas pre-steroids he would leave most of it. So my mum gave him smaller portions to maintain it and dad knows that weight gain would be detriment to him and he has a belly to lose anyway.

His breathing is a lot better and funnily, he had a really bad problem with his hands, skin peeling etc and very sore and no creams or ointments worked. It has now cleared up. He doesn't have any stomach upset which is common place with steroids which I think is cos he has a strong constitution.

I am only speaking from my dads perspective, I do know others who have had a rough time on them. There are other treatments, azithromycin I think is one. Again, not nice side effects. The main treatment is a transplant.

[pechinawa in reply to tanyamarie]

Hi tanymarie

Wow fantastic that your dad has a new lease of life hope that it continues ,

My experience of being on steroids is only what i have been going through ,my wife kathy has had me on salads and low cal on everything i eat and drink since i went on steroids as i say its just my own experience except from my dad who had a heart valve replacement and was on steroids long term,

He had complications with steroids ,weight gain but also thining of the bones which i have to have a test for,after long term on steroids my dad ended up with bone cancer.

as i have said my experience of steroids is a no go area,

Asi am on the the same medication as your dad,maybe longer i am truely elated to hear your dad is doing so well,

Just dont let him run any charity marathons.

But i am happy for you and your family that your dad is felling the benifits of the steroids

[tanyamarie in reply to pechinawa]

Well, funnily enough, a couple of days after he started on them, he got out of bed and felt like he could run a marathon, hence came downstairs without using his oxygen. My mum and brother told me later than he almost collapsed, he went soo blue and could not breathe. Thankfully they were there to help him. That frightened him as you can imagine and he understood then what the doc said about having false energy.

[pechinawa in reply to tanyamarie]

thats the first time i have heard the term false energy which i think sums up the use of steroids they can as you have said they give a boost but cover up the underlying problems.

Hope your dad enjoys his new lease of life

[amagran]

when my husband was diagnosed with ipf the consultant said there wasn't enough proof that steroids made any difference to ipf and that the side effects were terrible, so he has never been on them long term, he has them in hospital when he's admitted but only for 10 days. The dr said that no treatment would be better in his case as he also has emphysema. He does have oxygen and spiriva, plus several other inhalers and pills and potions that he was taking before the ipf was diagnosed.

[huddy]

Thank you everyone for your replies. I can see I am not alone having IPF and take great comfort from your comments. Once again thank you

[maggie44 in reply to huddy]

Hi Huddy

If you are not under a doctor or centre who specialise in the various types of pulmonary fibrosis I would suggest you enquire with you home hospital/Gp/consultant about it. I wish I had done so earlier - although there is no cure available, there certainly are centres of excellence which have tests,specialists and the latest treatments more readily available. My local hospital has been very good - the rheumatologist who oversees my treatment(because of the autoimmune disease) has been excellent and very happy to collaborate with the Royal Brompton, now they are involved. I had more tests done in one morning at the RB than I had over two years locally and all the results were there to be seen by the RB consultant on her laptop by the time saw her at 12.30pm!

I am at the stage where I am conscious that my breathing is deteriorating month on month. I need any treatment that can halt the progress of the disease once more now!!!

Maggie