Hi my mother in law was diagnosed with IPF 10 years ago. In the space of 5 months she's gone from walking around to bedridden is this normal

[Kt73]

As I said she's gone from a 'normalish' life,walking round the shops etc. She couldn't run up a flight of stairs but she could walk up them, now she can barely walk to the bathroom and is confined to bed. She's been for an oxygen assessment twice but been told it won't help. She's very depressed at the moment as you would expect and we are all feeling that she's getting no help from the hospital. I believe she's also developed pulmonary hypertension. Has anyone else experienced any thing like this. The speed of the decline in health has been shocking. Many thanks.

[Hidden]

Hello I care for my husband who suffers with severe Copd so cannot offer you advise but would suggest you ring the helpline on Monday - click on the red balloon I am sure they will be able to help you Loved TAD xx

[kevinrobinson in reply to Hidden]

You can call the BLF Helpline on 03000 030 555. Or email [e-mail] and we’ll call you back. Ringing the helpline never costs more than a local call and is usually free, even from a mobile. Lines are open from 9am to 5pm, Monday to Friday.

[Kt73]

Thank you TADAW, I really wish she could use a computer and see how great everyone is on here. I will ring on her behalf as she's a stubborn thing and doesn't want to acknowledge how bad she has become, but reading peoples stories on here has given me hope. X

[Catiosha]

I have IPF myself, I was diagnosed about 2 years ago. Unfortunately, I have learned that with IPF/PF, one can be stable for quite some time, just to deteriorate quite rapidly and suddenly. Is she going regularly to her lung consultant?

Are you on Facebook? If you are, there are some great groups for people with IPF/PF, there are also a group for carers, which might benefit yourself.

[Kt73 in reply to Catiosha]

Thanks Catiosha I'll look for them. Best of luck and I hope you are well at the moment. X

[Lotsofquestions]

Totally empathise with you. My dad was driving in September to being almost bed ridden in 4 months. Bathroom was an ordeal and independence at home led to carers four times a day. Like you we were told he wasn't suitable for oxygen, if he needs oxygen then he needs to be in hospital they told us. You need to be firm with all the medical staff. Good luck x

[Kt73 in reply to Lotsofquestions]

Thank you lotsofquestions, it's so difficult to see it happen to the people you love. I think we do need to be firm and find out what our options are. I'm tempted to take her to A&E at least she would get a full assessment but I know she won't go, she's an ex nurse and a little knowledge is a dangerous thing. please let me know how you get on with your dad. X

[cms_torq]

Hello Kt73, I was diagnosed with PF in 2008, caused by Rheumatoid Arthritis. I was stable for a few years then it deteriorated very quickly. 6 weeks ago I was breathing fresh air now I'm on Oxygen 24/7 but it really helps me. They do say PF/IPF is different for everyone. I hope you can get some support locally. Chris.

[Kt73]

Thank you everyone, she's having a tough time, she knew it was going to happen but the speed of it has taken us all by surprise. She went to see her consultant in Feb and he didn't want to see her until July! She's said herself the way she feels at the moment she won't make until then. I think I or my husband will be ringing the clinic at the QE on Monday. She's also going to ring the number here. Thanks for your help and good luck each and everyone of you on your journey. I'm off to find the Facebook groups as well. X

[maggie44 in reply to Kt73]

Does your mother-in-law go to a specialist hospital for pulmonary fibrosis and pulmonary hypertension? I have got both of above (caused by Sjogren's- an autoimmune disease)and have got medication for both. I think it is not unusual for PF or ILD patients to develop PH. I go to the Royal Brompton in London to both departments. I take sildenafil for the PH and a whole range of medication for the ILD including oxygen 15/24 hours at 2 lpm and ambulatory at 6 lpm. There are only 8 hospitals in England who have been identified by NICE as Centres of Excellence to treat PH patients with the new drugs. Many respiratory staff in general hospitals have very little experience of treating ILD/PF patients whose needs are quite different to those with COPD. Once I was diagnosed with PH (after ILD-PF) I was taken into RB for more tests and kept in for a few days to monitor my response to the new drug. I saw the PH consultant every 3 months and in between I saw my Rheumatologist every 3 months to monitor my progress, so really saw a consultant every 6-8 weeks at this time. They wrote letters to each other, my GP and myself to keep everyone in the loop. I am going to see my PH consultant at Brompton on Thursday - this time he has let me go 5 months between appts as my PH seemed stable with the proviso that I rang them if my condition deteriorated. Saw my rheumatologist about 6 weeks ago - had lung function tests and range of blood tests in order to monitor my condition.

I presume she has been given blood tests to ensure she is not suffering from an infection/pneumonia. In PF/PH you can have these without any more obvious signs such as COPD patients get.. I have twice had this happen and ended in hospital for 2 weeks. I always retain more water (wt goes up 5-7 lbs in few days and feel rubbish. I know it is time to take rescue antibiotics.

Are you confident that your M-in-L's consultant understands her condition? If not ask to be referred to a specialist hospital like RB or Papworth. You can find a list online or ring Helpline at BLF.

Hope something in this long post might help.

Maggie

[Kt73]

Thanks Maggie, that was helpful, I don't think she's seeing a specialist as such just a lung consultant who has said that basically there is nothing they can do for her. She did try a new drug ( i need to find out which it was ) but she had awful side effects and wouldn't have tolerated the next step which was to double the dose.

Neither of us can wait for the phone lines to open on Monday morning, it says something when I've learnt more about the disease and treatment options in 24 hrs on this site than I have from the medical professionals. Only wish we lived down South instead of in the Midlands but I feel much more informed for the fight ahead.

Maggie, if I may ask, are you able to get around okay? My MInL can talk for England whilst sitting down but even just standing up has her breathless. I wish you well and many thanks for taking the time in replying to me. She feels very alone and abandoned at the moment and being an ex nurse she doesn't often feel sorry for herself or defeated but I know this is getting to her.

Good luck and I hope you stay relatively well.

Kristy

[maggie44 in reply to Kt73]

Hi Kristy

Hospitals currently permitted to prescribe Pirfenidone (as of May 2013) Centres for ILD

Senate Areas

Manchester & North West UH of South Manchester NHS Foundation Trust

Cheshire and Mersey Aintree NHS Foundation Trust

East of England Papworth Hospital NHS Foundation Trust

London South Guys, King’s, St Thomas’ Hospital NHS Foundation Trust

London North East

London North West Royal Brompton NHS Foundation Trust

Imperial Healthcare NHS Foundation Trust

North East Newcastle NHS FT

East Midlands UH Leicester NHS Trust

Nottingham UH NHS Trust

South East Coast Southampton General Hospital

South West North Bristol NHS Trust

Royal Devon and Exeter NHS Foundation Trust

Thames Valley Oxford UH NHS Trust

Yorkshire and Humber Leeds Teaching Hospital NHS Trust

Sheffield UH NHS Trust

Hull & East Yorkshire Hospitals NHS Trust

Wessex UH Southampton NHS Foundation Trust

West Midlands UH Birmingham NHS Foundation Trust

The situation is different in Scotland and Wales but it is likely that Glasgow, Edinburgh and Cardiff will be regarded as ILD Centres on the basis of current NICE Criteria.

Hope this helps

Sorry about two posts but lost part of the other one so thought I had better post what I had and then come back with this info.

Magge

[maggie44]

Hi Kristy

It is typical of PF/ILD to feel reasonably ok when sitting and to become very breathless with movement. Pulmonary hypertension adds to that breathlessness. Unlike COPD our .tubes are not blocked, it is our lungs which are scarred and become rather leathery, often the alveoli ( which pass oxygen into the blood via the lungs) are badly damaged. As you move your body demands more oxygen to make your muscles work - this is when SATs drop. Pulmonary hypertension affects the artery between lung and heart - usually restricted, so again blood cannot get sufficient oxygen to support activity. I think I have explained that more or less right, even if not quite technically correct.

There are a couple of different PF's - one is called Idiopathic pulmonary fibrosis (no known cause) Pirfendone is the latest drug used for this. If like me your PF is caused by your own immune system (Rheumatoid Arthritis, Lupus, Sjogren's syndrome et al) then pirfenidone is not used. The medication we have is mostly aimed at damping down our over-active immune systems- often the type of drugs used by someone to stop rejection after a transplant.

PH - the drug I am taking is sildenafil ( basically Viagra). This opens out the artery between heart and lungs and improves the flow and thus the oxygen. Again it is obviously a lot more complex than I have just described but the basics are about right I think. Each time I go to RB to see the consultant I have an echocardiogram before I see him and they can measure the flow.

I live in Northamptonshire (East Midlands) and go the the Brompton - now have transport arranged (ambulance car) because I can no longer manage the car-train-taxi journey that I used to do. I am fairly sure Leicester is a specialist Centre - I will look up and see where you can find the rest.

Best wishes

Maggie

[peege in reply to maggie44]

Hi Maggie, apologies for my ignorance - how does the RA scar & damage the lungs? I ask because my best friends son, age 30, has RA, takes methixotrate?, is very healthy and fit. His mum said he's had very few lung infections in his life. Thank you, P

[maggie44 in reply to peege]

Hi Peeg

Not all people with these autoimmune diseases develop lung problems. The majority will not be affected.My understanding is that it is when the white blood cells in the body(those that fight infection) suddenly go out of control for some reason and 'turn' against itself. Eg Sjogren's syndrome which I have, first attacked my moisture producing glands in my eyes, causing extremely dry eyes and other problems. At the same time they attacked the saliva glands in my mouth -again causing various problems. It seems very rare that they go on to affect your lungs, or other internal organs and systems.

Hope this clarifies the situation.

Maggie

[peege in reply to maggie44]

Ah thank you very much Maggie. That really does help. He's a lovely young man and I'd hate to think it's inevitable for him.

[Kt73]

Thank Maggie for all the information I will make sure my mother in law gets to see it all. It's amazing what you can find out on here. I'll let you know how we get on. Good luck with your journey, I am glad that you are getting the best care and treatment.

Best wishes Kristy

X

[maggie44]

Hi Kristy

Just a thought- both times I ended up in hospital (for 2 weeks) each time, I had suddenly gone down healthwise, was unable to get up and get dressed and didn't want to eat etc. Breathing was worse than usual, was coughing more than usual. I had an infection-pneumonia. The problem is that it only seems to be blood tests that confirm the infection. Unlike COPD sufferers, PF patients have a dry cough, do not produce obviously infected sputum. My GP didn't recognise how poorly I was the second time- RB told me to come in because they thought it was possibly a response to my recently strengthened dose of Sildenafil for PH. Blood tests soon identified the pneumonia problem and gave me three different antibiotics. During the first week in hospital, the pneumonia got worse for several days - then began to improve slowly. All through that time I had to be taken to the loo in a wheelchair - no way I could walk further than around my bed and that exhausted me, even with oxygen. Second week very slight improvement - managed to get to bathroom slowly.

Do you think your MinL could be suffering from an infection? I can usually tell now, if I am starting- feel more breathless, cough more, v tired etc. But also my weight increases over a couple days by 5-7 lbs. Infection makes heart overworked and I retain more water and feel bloated. I start my emergency antibiotics and after about 5-6 days my weight starts to fall and selling on ankles-legs - midriff reduces. I take antibiotics for 2 weeks and get slowly back to normal.

Possibly something to consider.

Best wishes

Maggie

[montymillie]

hi so sorry about your mum, is she really breathless and that's why she stays in bed cos its to difficult to get her breath back after moving around. is she not on oxygen at all. is she under an ILD unit/hospital, who said she dident need oxygen. I was diagnosed 17 months ago, I;ve had it a lot longer and since November I have been out of the house proberly 8 times I now sit in bed all the time only get up to get food drink and use the bathroom. this disease can get bad very quickly as I have found out. I have been on oxy 24/7 since diagnoses and now am on 10 litres per minuit at rest and sleep and 12 litres per minuit on movement, and can walk about10 12 feet and am really breathless and in pain the lungs and got to sit down. I don't understand if she cant get out of bed why they wont give her oxygen,maybe a second opion is needed. it sounds like she really needs ILD hospital help. I really hope you can get the help for her, I send her my best wishes and to you as well love. take care. xxx