Pulmonary fibrosis

I would like to find other people with Pulmonary Fibrosis I feel so alone and would love to hear how other people manage it. I have been diagnosed 10 years ago and started on high dose steroids to get the symptoms under control, that was terrible as my face was so swollen,

I eventually came down after a year to 10mg. This year has been hell due to worsening of the symptoms and I cough all the time sometimes until I am sick. I have a oxygen concentrator which helps a bit, but the thing I would love here about is I only have Ventolin and I seem to cough so much after taking it I'm wondering how if effects other people. The

hospital said steroid inhalers will not help much, so I just plod on struggling through the day.

Any feed back how anyone copes with this awful disease would be so helpful.

Last edited by

13 Replies

oldestnewest
  • Hi Buddygirl

    The BLF recently held an online surgery on IPF which I just uploaded to Youtube

    Maybe that will give you some answers as well as all the members on here who are always so helpful

  • thankyou for this

  • Just watched it, brilliant. I have learned so much and will show my dad tomorrow. How sad though that there simply isn't enough known to answer the simplest questions. We really must do all we can to help fight this.

  • my husband has ipf and copd emphysema. He coughs a lot too, all the time. he has 3 different inhalers, spiriva, ventolin and seretide. plus lots of other drugs. he has an oxygen concentrator and ambulatory oxygen.

  • Hello hun,

    My dad has IPF and Emphysema like amagrans hubby and they are like two peas in a pod! I would like to say how sorry I am to hear you have this condition and how inspiring also that you have lived with it for 10 years. Dad was diagnosed 4 years ago and like you has a concentrator at home.

    I would like to suggest that you speak to your GP and request Mucodyne Syrup. It helps to bring up the mucus that is in your lungs and most people find it really helpful. You can get it in capsule form so upto you to ask for your preference. The generic name is Carbocisteine.

    Spiriva, is a steroid inhaler capsule that dad takes every morning. The generic name is Tiotropium. Serevent diskhaler is another one. Generic name is Salmetarol Xinfonate (or similar!). He takes it twice a day. He hardly ever uses his ventolin now, in fact I don't think he has used it for ages. And this past month or so he has been using Oramorph, a morphine based liquid. It is being used to open up the airways and help with breathing and dad says it is excellent. It also doubles up as a pain relief so you will find it beneficial with the pain from coughing so much.

    Dad is on a maintenance dose like you of prednisolone and next week it will be 10mg a day. If his quality of life suffers when it drops to 10 then he will probably be increased again because that is what we are all aiming for with dad, his quality of life. He has been told he is not suitable for longterm oxygen therapy but while it is doing him some good we are all positive. We laugh all the time, spend most of the time together and tomorrow I am taking him out in a hired wheelchair with mum if the sun comes out. He is very happy, despite the illness and is very much a glass half full kinda guy.

    You are so brave to be fighting this condition and have proved that what you read isn't always true and when I tell my dad about you tomorrow I know how inpsired he will be.

    Thankyou for sharing with us, especially as I know how tough this is for you.

    Take care and I hope you get some help from your GP asap xx

  • Hi Tanyamarie,

    I have just joined today and what a wonderful bunch you are, feed back already l feel

    not so alone any more. I must give the Carbocisteine another go I just found it made my lungs struggle to bring up the mucus. I was offered a morphine cough mixture but my consultant said it does make you feel sick and sleepy so l declined, she has given my Pholocodine which made my head feel so rotten, my system does struggle with meds. It's lovely to hear you love and care for your dad it is a horrible disease

    I am no longer able to walk to the shops and use a scooter but i can walk around it's

    lovely to be able to walk on the flat surface. A stair lift had to be put in this April hard to accept, but you have to get on with it and live your daily life. The video was fantastic because l do get questioned by people because l look well and sitting in a

    chair l look normal although l am coughing all the time, l didn't know the lung formed

    cysts, give my love to your dad and l wish him well.

  • Ah, thankyou. I told my mum an ddad about you today and they were thrilled to bits. Dad was even talking about going on holiday next year and even saying about taking his driving test again when he is 70.....in 4 years time. To hear him talk so positive is amazing and if he feels like that then so do I too.

    I think my dad is lucky because he has the constitution of an ox. He has no ill side effects at all, apart from a little constipation. Im sorry you have tried many meds and they dont agree with you. Thats naff isn't it.

    Well you take care and hope to talk to you again swn xx

  • Hi buddygirl

    Sorry to hear you have this horrible disease. Do you know if it is ideopathic or if it has some specific cause ( e.g. an auto-immune disease?). If you watched the BLF surgery you will have seen that the two conditions are treated rather differently and sometimes have a differing prognosis. Have you been sent to a specialist hospital? I have PF linked with Sjogren's syndrome (an autoimmune disease) and have quite range of medications(including steroids). In the past I have had 6 infusions (chemotherapy)to damp my immune system down to try to stop lung inflammation happening. These worked for a while but now the Royal Brompton hospital are considering rituximab as the next treatment. If you have IPF then as the specialist said in the programme, a new drug pirfenodone is now getting some good results.

    During the day I have ambulatory oxygen at 6lm ; at night I use a concentrator and have oxygen at 2 lm.

    If you are not at a specialist hospital I would have thought it worth your while to ask to be referred to one.

    best wishes Maggie

  • Hi Maggie,

    Yes l do have Idiophathic Pulmonary Fibrosis l have had a lung biopsy at Guys Hospital 10 years ago. It's been a year since l have got slowly got worse l started off with Azathioprine but had to stop because l had jaundice then l went onto cyclosporine which l managed to take for 18 months when that turned on me and l had to stop work because l was heavy lifting in our local supermarket but l'm sure that gave me extra years of life. l have a very good consultant who has tried to keep me going but my system does struggle taking any form of strong drugs and all are very powerful. This year l can no longer walk to the shops and use a Luggie scooter l live 2 minuets from our local village shops. I see a new drug is out pirenodone but like most of them l expect l won't be able to tolerate the side effects. Cyclosporine had side effect but l did manage to put up with it. I am on 2 lm of oxygen when needed usually about twice a day, and have been given some oxygen to take out trying to get nasal rather than masks is like getting blood out of a stone What does ambulatory mean l'm not really up on oxygen. I had a heart operation in the Royal Brompton at the age of 5 and l was the first little girl to have this operation but it is thought that has caused the fibrosis because l was cut from the shoulder to just under the left breast and that lung has the most scarring. Thank you for replying Maggie any feed back with fibrosis from any one helps me to know how they cope on a daily basis.

    Best Wishes.

    Buddygirl

  • Hi Buddygirl

    It such a shame when your body cannot tolerate the only drugs that seem to hold out some hope of putting off the inevitable.I read about the pirfenidone trial and asked my rheumatologist (the consultant who has been overseeing my care in relation to Sjogren's symdrome for some years) if he thought I could try it. He wrote to Professor DeBois at RB who was heading the trial to ask if it could be arranged. I went in January to RB, but because my fibrosis is caused by the auto-immune condition and therefore not ideopathic, the pirfenidone is not suitable for me. However they did take me on as a patient and I have had excellent treatment so far. Unfortunately they discovered in the course of the tests carried out that I have pulmonary hypertension as well -another life threatening condition! Still RB has one of only 9 specialist teams in the UK dealing with this and I have been passed over to them. Tests have been done and I started on a treatment plan for PH in July (taking sildenafil). Unfortunately in the last couple of weeks I have become even more breathless -am wondering if it a side effect to the drug (which has many and various). I will contact the PH nurse at RB on Monday to talk it through. Still I am glad that the docs have not given up on me and quite honestly I will try anything that is likely to give me more time in a reasonble state of health.

    As far as oxygen goes ambulatory oxygen means when you are walking about (outside as far as I am concerned)- my sats plummett when I exercise -. I manage around the house without oygen and am ok sitting, but am finding I am getting more and more breathless doing things that I managed reasonbly well a couple of months ago. Worrying!! I have 3l

    cylinders for outside use . Since the discovery of PH I have to use 2l of oxygen at night (8 hrs min). Although I protested when it was suggested, I must admit that I get very tired as the evening progresses and I am relieved to hook up to the oxygen when I get upstairs. That last voyage upstairs is very hard - am gasping for breath when I get there and it takes some time to recover.

    I am going to Centre Parc with my family in a few weeks and I must admit I think I may have to hire a motor scooter if I am to get around. I know it is sensible to do it but it feels like an admission of weakness. Why can't you get nasal cannulas for your oxygen - I have never been offered anything else and could not tolerate a mask - they make me feel claustrophobic.

    Sorry if the typing of this is a bit off - have just put some drops in my eyes and it makes vision a bit blurry for a while.

    Take care

    regards Maggie

  • Hi Maggie,

    I suffer with IPF and it can be a real pain when you are doing sometimes little things but end up out of breath. I use to walk to my local shop taking several breaks to do it. This got too much for me, the answer was to buy a used mobility scooter. It gives so much more freedom.Transported in the back of the car and used it on holiday as well. The stairs at home were becoming another major bother for me, the answer bought a used stairlift for £400, fully fitted which has made life more easy. As time goes by I know everyday things will be harder to do. There are solutions out there, take care, Cheers..Kevin

  • Hi Kevin,

    Do you mind me asking, how do you find putting the scooter in and out of your car? Is it heavy and do you struggle?

    Dad is thinking of getting one but I am concerned that he will find it too much to lift and my mum simply isn't well enough.

    Thanks

    Tanya

  • Hi Tanymarie

    I bought a used small scooter. The handle bars fold flat,seat unbolts and battery can be detached. The battery is proberly the heaviest part. The scooter body has two hand grips to lift it. At home the scooter can be stored upright to save room.My wife is 5 foot 1 inch and tells me to get out of her way and managers ok. My scooter is a Padmore wizzard other brands are similar.I would suggest a visit to a mobility store and see what might suit your needs. Make sure it is suitable for your car.My car is Nissan Qashqai so the scooter is quite easy to get in.Some scooter models break down into several bits for ease of lifting. Have a look around for what suits your dad....Kevin

You may also like...