I have been diagnosed with this life threatening condition and wanted to learn from others who also have it, particularly how to make life easier.
It's quite a rare lung disease that affects mainly people in their late 60's & 70's. Its even rarer to see in someone of my age ... 44.
In short our lungs become scared over a short time resulting in a change to the Interstitial tissue and therefore a loss of alveoli. There's no cure and sadly the prognosis is poor .. just a few years after diagnosis. I'm in my first year.
I'd be grateful to anyone who could offer any advice or knowledge that would ease this journey for me. Thank you for reading this.
Written by
Oxyjan
To view profiles and participate in discussions please or .
it's another name for idiopathic pulmonary fibrosis, well the old name for it, but exactly the same disease. you are very young to get it, but maybe its not the idiopathic form, maybe you have a known cause for it, for example, working with asbestos or chemicals. You should educate yourself about it and then come on this site for support , they are lovely people on here. You do not have copd , it's a different disease altogether, but there are a few on here who have or are carers for someone who has. hope this is of some help. xx
Thank you for getting in touch. I have read that page before and it frightened the life out of me. I've since become aware that its paints quite a poor picture and one that isn't necessarily that accurate. I wish you well too. Jan x
In my case the cause is unknown. I'm currently on Prednisilone 15mg, Mycophenolate 1500mg, NAcetylcysteine 1800mg per day and go through 5 O2 cylinders every 8 days.. It's a daily struggle .. particularly with breathlessness and coughing.
Recent LFT's, fortunately, have showed little change in the last six months but a CT scan indicated the structure to be more textured in apprearance. This seems at odds with the way I feel. Life is gradually getting harder.
I have a wife and son of 7. This has been devastating for us all.
I hope your husband is coping and his spirits are up. I wish you well too .. it is hard for the partner of someone with this condition and I can well appreciate how much you do in your home.
richard is on prednisolone 10, and the n-acetycysteine 1800, plus 11 other things a day, I have sympathy because I can know exactly how you are feeling and you are so young to have this damn awful disease. if you would like to private message me at any time I can try to offer what ;little help I can. x
one definite I would think at your age is, push for a lung transplant. x
Oxyjan, I know little about your condition but can only reiterate what amagran said, the support on this site is available to you. Please feel free to access the site as often as you wish and don't forget the BLF helpline on 03000 030 555.
So sorry to hear you have this horrible illness Oxyjan. I have Non -specific interstitial pneumonitis and more recently pulmonary hypertension, both caused by an auto-immune disease sjogren's syndrome which I have had since I was about 45(am now 68). The lung problem (then labelled pulmonary fibrosis) was diagnosed in February 2010. Since then I have been given a variety of treatments as the disease progressed in order to try to dampen down my immune system and thus reduce the scarring.
I was referred to the Royal Brompton Hospital in London last January and am now receiving excellent treatment from their specialist units. If you are not being treated in one of the 10 specialist units in the country, then you should probably be asked to be referred. It is worth the effort of getting there in order to be treated by specialists in their field. They can do tests in one day that would take weeks in a general hospital (not the latter's fault - they just don't have the resources to do the same ).
I need ambulatory oxygen now (since I had a major infection and was hospitalised for 10 days about18 months ago). I also have to use oxygen at night for 8 hours - since tests revealed pulmonary hypertension about 6 months ago.
I did find Pulmonary Rehab very helpful - it showed me how to manage my breathlessness better and understand what is happening in my body. It really is important to keep as mobile and fit as you possibly can. I have let the exercise go in recent months when I was feeling more unwell and am really noticing the difference - must get back to it!!
At Pulmonary rehab I met a chap who also had NSIP ( the first ever person I knew with the disease). He was younger than me but further down the road with the disease. He was on the lung transplant list and has since had a double lung transplant and is doing well - working hard to gain his fitness. At your age this is something that you should explore with your doctors.
It is a hard disease to live with - my philosophy is to try to take advantage of every opportunity for treatment that will extend my life and provide the best quality of life possible. It is really hard on our families too. I know it has affected my husband also - it can be frightening to watch someone you care about fight for breath. At first he tried to do everything for me - wanting to stop me getting breathless - now he is learning to let me do as much as I feel I can do, even if it makes me breathless. We are both learning as we go along.
The fact that you do not come across other sufferers can make you feel very isolated - there are some of us on here so keep in touch.
Thank you so much for getting in touch. It was very kind of you to write such a lengthy reply.
I am also at the Royal Brompton and I too was referred in January 2012. I'm pretty sure we're under the same consultant and registrars as our diseases are so similar in nature.
Although I've been diagnosed, by the countries leading authority on the subject as having UIP there are still some question marks around it. The first and most obvious is my age and the second, I'm told, is my excellent response to steroids. I'm hoping the diagnosis is more towards NSIP rather than UIP because life expectancy is slightly more favourable although the journey is just as difficult.
Looking back over this year I can see how much my life has changed. Yes .. the medication is slowing the process for which I'm extremely grateful for but it's sad when I realise for the first time what I can no longer do, like walk around my local park, climb a flight of stairs without extreme exhaustion, go to my attic to get a toy for my son .. and so on. I'm loosing ability and it seems to be getting faster than ever.
I do have oxygen of cause and its helping enormously. I've got over the novelty of having it the house, fought against using it for as long as possible ( because it means I really am poorly ) and now have become accepting of it as a friend. I don't use it in public yet but I'm sure that's coming soon.
I go to the gym twice a week for an hour each time. I'm in the first month of a twelve week course. It is difficult. My blood / oxygen levels dip into the late 70's early 80's almost straight away so I have to struggle on through the exercise knowing that it could be dangerous for me. If I feel a bit dizzy I simply stop. With this said I always feel great the following day. My lungs and general well being seems virtually normal.
You're absolute right .. it is a hard disease to live with and the impact on the people around you can't be underestimated. My beautiful wife has so much on her shoulders not just in the home but also as our sole breadwinner. Our son is fully aware of my health apart from the prognosis. We tell him as much as possible. I want him to be informed and that there are teams of scientists around the world working on cures for the many lung diseases. I feel informing him empowers him rather than be a shock if this all goes the wrong way.
I must clock off for now. Thank you again for your lovely reply. I'll be thinking of you and hoping each day is an easy and comfortable one.
Nice to hear from you. Obviously I am not medically qualified but it doesn't seem quite right to me that you should be regularly exercising with your SATs in the 70s - sounds as if you need to be using oxygen while you exercise. Have you told any doctor or respiratory nurse about this. While I understand the embarrassment of using oxygen in public at first, you nevertheless must look after all your other organs. They are put under considerable stress at these levels. I'm not suggesting that you don't exercise but that you do it as safely as you can.
I'm off to the Royal Brompton again on Thursday- whole load of blood tests, lung function tests and then seeing my pulmonary hypertension consultant in the afternoon. I recently started on a new drug (sildenafil) for the PH so it is a follow up to see if there is any improvement.
How old is your son? I spent last week at Centre Parc with my husband, both of our sons, their wives and our two grandsons. My sons are a similar age to you- grandsons are 7 and 10. It was lovely to be altogether as we live a fair distance apart. I wasn't able to do much but hired a motobility scooter and got around to some extent.
Take care - keep up the exercise but think about oxygen levels!!
I do use oxygen during exercise. I've been advised to put it up to the maximum level of 15. My SATs drop according to the exercise. Most are above 80 % but walking and doing squats causes me to dip into the 70's. I have discussed this my my trainer but wasn't given a deffinative answer as to weather I should or shouldn't pursue at those levels. I need to discuss this with my to my respiratory nurse.
I have a very minor indication that my heart is working harder and as such I've also seen the PH consultant. The advise was to use my oxygen more to avoid developing Pulmonary Hypertension. I actually get an ache in my left lung and minor heart pains too. Nothing I can't cope with but it does seem to surprise the consultants when I tell them .. almost as though they've not known of this before. So sorry to hear you have Pulmonary Hypertension too.
Hope all went well at the Royal Brompton. It's a fantastic place. Do you have any thoughts as to how your disease might have started ? I have a few myself but its just guesswork. I'd love to know but I don't think I ever will.
Hello, Maggie, we just found out and my husband is very depressed, we live in Toronto,and I wonder how can we get to this hospital in London; any more advice?, please let me know, thanks.
I have only just seen your message and so sorry to hear about your diagnosis. I expect that I am under the same team of consultants at the Royal Brompton as I too have IPF. It sounds like you are doing a fantastic job by going to the gym and keeping as fit as you can. I have just started going again after quite a break and I must say that I am feeling better.
I was officially diagnosed in 2006 whilst in the later stages of pregnancy with my son. I have had rheumatoid arthritis since the age of 7 and had no idea that it can cause complications in the lungs as well. I had a major flare up after my son was born and was prescribed a new anti TNF treatment which they now think caused further fibrosis of the lungs. I had to start using oxygen from Feb 2007 and whilst my son was still a baby. I use liquid oxygen and it enables me to get out and about with a bit more ease. I do get frustrated when even doing the littlest things makes me breathless.
I do so feel for you having a young son and trying to say the right things. I suppose my son has only known me with the oxygen cannula attached to my face. I am also under the Harefield with a possibility of going on the lung transplant list. I am still trying to decide what to do having been given the ok to go on the list should I wish. My biggest fear is what on earth do I tell my son and how can I explain it.
I am so glad I joined this site. There are many times when I feel so alone as I never come across anyone in the street like me so to know there are others who can understand gives me great comfort. I wanted to drop a quick note this evening but would be happy to chat some more if you would like to message me directly.
My goodness, you're going through so much and you seem so young too. Having fibrosis in itself is tough enough but to have rheumatoid arthritis as well must be extremely difficult. On top of that you have a young son to care for. My heart goes out to you.
My point of view regarding what to say to a child about such health issues depends on the age of the child. I'm guessing your son is around 6 possibly 7.
For me, with a 7 year old boy I realised that as my condition was being discussed by friends and family the one person who was kept in the dark was our son and I thought if this was to continue he would be the one most shocked and affected if I was to loose the fight against this disease. My wife and I want him to know about my health along the way as it changes in ways a 7 year old can understand. Above all we've decided we're not going to leave him out. To do so, we feel, would cause heartache and pain as well as the overwhelming shock of having lost a parent.
Its just our point of view. A lot depends on the personality, maturity and age of the child and as we know every child is different. This is the route we've decided to take with ours. It may not be right for everyone but for us as a family it dose. I hope you find a way that is right for you.
It seems I may have the same as you. I have Rheumatoid Arthritis and have been told that I have Rheumatoid nodules on my lungs and UID as well - which is the scarring on my lungs. I live in Australia and would love some advice or positive thoughts.
I was diagnosed with it almost two years ago. Not really that rare but the treatment and prognosis was a game changer for me when I first looked it up on line. So far, I only use oxygen when I exercise at rehab center and take the N-Acetyl supplement daily. It's been a rough winter in the USA and I just finished taking a round of prednisone to get over a round of the viral flu. Eventually I'll be put on lung transplant list but, hopefully not for a year or more. I'm 65 and I've met people who have had successful lung transplants; some still going strong after 10 years out. Wishing you all the best.
I too was diagnosed with this in November 2013, so you are not alone. I am on N-AcetylCysteine 1800mg per day as well as Lansaprazole 20mg. The consultant at my hospital says that the N-AcetylCysteine slows the progress but there is no cure. I am 55 years old and have a family who are supportive. I apologise for the delay in responding to your message.
Take care and I am here if you need a chat at any time.
Hello...I live in the United States and was diagnosed with U.I.P. in August 2010 via lung biopsy,,,the advice from my Pulmonologist at the time was: avoid getting sick,,,use a mask when out in public, use hand sanitizer after touching things in public, avoid using antibiotics and prednisone unless absolutely necessary, avoid inhalers and use of oxygen as long as possible, I am a smoker I asked should I quit smoking his response was it is up to myself as in studies those who quit have a life expectancy that is 7-8 months less than those who continue to smoke. My story is: my first symptoms began in July of 2008, shortness of breath and a rapid heart rate (120 sustained at rest). First i saw a Cardiologist who said my heart is in good shape except the tachycardia which is easily treated with medication, however that there is some thing wrong with my lungs...so off to the Pulmonologist where I had a PFT's, CT scan high resolution, broncoscopy, and finally a VAT. Going through all those test and getting to the lung biopsy took two years and one month, I'm in year 3 almost 4 since diagnosis and year 5 almost 6 since my first symptoms began. I just started using oxygen with exertion, so when I do dishes, laundry, shopping, walking my dogs, and I am having a hard time getting use to using the oxygen. My advice to you is listen to your body and give your body what it needs. I have had days when I;m so exhausted and tired that I just sleep, and days that I have the energy to get many things done. I do as much as I can do and I bought my own pulse oximeter and kept walking and doing the things I normally would but paid attention to keeping my oxygen saturation at or above 93% since my biopsy. I slowed down and paced myself. This lung disease no matter what you call it affects every area of your life. So live, love, laugh, and enjoy all that you can for as long as you can.
Hy Jan I have and rea wikki page and whilst It frightened the life out of me I decided to push ahead as if it didn't exist decided to walk my way out of it Apart from the obvious, ie my general health has greatly improved but I now blow 400 on the wrights flow meter and just had my six monthly spyrometry and the specialist saw an improvement Its a hard pill to swallow at any age but if those who compiled the prognosis on wiki are right we had better make the time the best ever
My dad was told yesterday he has iup, can anyone please give me more info on this? My dad is 78 and has vascular dementia and Alzheimer's, also he has bladder cancer and non Hodgkin's lymphoma thank you in advance
My name is Anne brockmann I live in Guadalajara in was diagnosed with usual interstitial nemonia 2 years ago and my disese has not progresses I have found many things to keep me happy prays friends family movies books I have totally expected my limitations which are many if I can help you in any way please contact me.
Content on HealthUnlocked does not replace the relationship between you and doctors or other healthcare professionals nor the advice you receive from them.
Never delay seeking advice or dialling emergency services because of something that you have read on HealthUnlocked.
Does anyons has experience if Steroids make worst Bronchiectasis?
does anyone know about pleurisy 
Quite worried there's something seriously wrong.
Does anyone get dizzy when coughing up phlegm? 
Anyone else have Eosinophilic Asthma with chronic rhinosinusitis?
