Idiopathic Pulmonary Fibrosis with Scleroderma

[marilynmcl]

Have been diagnosed this by my respiratory consultant due to decrease in my lung function tests. I don't have much information on this but have been put onto new medication after being taken off of mycophenalate as it had stopped working and put onto Ofev. Have been told that it won't stop further damage to my lungs, just slow it down. Trouble is, the breathlessness and fatigue are not good. I also have severe osteoarthritis in both knees, no knee replacement ops as won't be able to have anaesthetic, survival of op or recovery is not a good prognosis. So, have a lot of pain ...no pain relief is recommended for me due to other problems in my stomach and am a bit fed up with all of it to be honest. Am sure there are others worse off than myself but I am really down just now, the covid virus is just the icing on the cake ! Not been outside for a few months now so depression is pretty well set in. Just wanted to ask if anyone else has been diagnosed with this IPF etc. and can offer some advice on carrying on.

[Sosie]

Hi, so sorry to hear you are having such a bad time. I am curious as to why your pulmonary fibrosis has been labelled as idiopathic. Lung fibrosis is very commonly caused by scleroderma. Idiopathic means of unknown cause. Why would they not put the lung fibrosis down to your scleroderma? Does your respiratory doctor know anything much about scleroderma? Sorry, I know this is not helpful directly but I feel like the two conditions should be recognised as connected for you to receive the best possible treatment. Xx

[marilynmcl in reply to Sosie]

Hi Sosie,thank you so much for replying and caring.

I am under very good consultants for both Sys. sclerosis and for respiratory. When I was told about the IPF I was given a leaflet to help me understand what it was all about as well as explanation on how it was going to worsen, also, no cure. The Nintedanib medication I was prescribed is to slow down the progression of the IPF ...it gets delivered to my home. Mr. Tan, my resp. consult. has been great and knows I have Sys. Sclerosis - I have three different types of lung damage through it. The mycophenalate I was on originally was not working to stop further damage to my lungs and this is why my medication was changed.

I am really happy with my treatment by my consultants who have always done their utmost to look after me. Am going to a connective tissue consultant ...well, once this virus is allowing us to go back to some kind of normal life...as my rheumatologist consultant thinks this would help me better.

I believe that associated with the Sys. Sclerosis is Interstitial lung diseases which has many conditions and symptoms. They can be caused by both autoimmune [Sys. Sclerosis] or rheumatologic diseases, and can also have no known cause so.. as it is a group of lung diseases which includes IPF this maybe why I was given this diagnosis? Anyway, am so glad you took time to reply, it means a lot. Take care xxxxx

[Sosie in reply to marilynmcl]

It’s my pleasure! Sounds like you are in good hands, so I am glad about that part, at least. I see a scleroderma specialist team and I must say it really reassures me as they really understand the disease and all the complications that go along with it. I hope you get on well with your new medication and that is slows down progression for you. Xxx

[marilynmcl in reply to Sosie]

Hi again Sosie....where do you attend for your scleroderma appointments? I am in South Lanarkshire and go to Wishaw university hospital. xxxx

[Sosie in reply to marilynmcl]

I see Dr John Pauling at the Royal National Hospital for Rheumatic Diseases in Bath. They have a great team of specialist nurses too. Xxx

[marilynmcl in reply to Sosie]

My rheumatologist consult. was Dr. Anna Ciechomska and Respiratory consult Dr. Lynas....my new consult. will be Dr. Murphy for connective tissue disease. But, as all appointments are cancelled until who knows when I haven't seen this new consult. yet. How are you with the scleroderma? xxx

[Sosie in reply to marilynmcl]

Yes, I am due all sorts of tests and appointments but most are on hold for now. I have been diagnosed nearly ten years. Very little skin involvement but quite a lot of internal organ stuff - especially digestive system and heart. Also very severe Raynaud’s. I still manage to work full-time (usually) and am studying for a law degree part-time too. I won’t let it beat me! Xx

[marilynmcl in reply to Sosie]

Hi, am same with the skin..not a problem but swollen joints and along with the severe osteo arthritis I need a wheelchair to get around, problem is my house is not adapted to suit my wheelchair so can only use it outdoors with help from my husband. No heart problem, thank goodness, but digestion...not able to eat well at all, have lost a lot of weight, no appetite to blame. Breathing is very poor, not on oxygen but am sure it will be the next thing. Have been told I can't get pain relief because of problems with my stomach. Have had my diagnosis about 7 yrs now.

Have had the cyclophosphomide infusions at the beginning then the mycoph. tablets. Only been on the Ofev for the last few months but not many bad effects from it...touch wood! Latest worry is the anaemia which is not too good. It just seems that every time I go to the hospital they tell me something else is wrong with me...most discouraging!

Am so glad that you are still working and managing with your symptoms. Many thanks for replying too...it is good to relate to someone who understands what this is like.

I am an old lady now...70 this year...so I suppose I have had a good innings so far..except for the last few years that is! Anyway, you look after yourself and hope good things for you going forward. xxx

[Lucinda227loz]

Hi sorry you are going though this it’s not good but you are been look after I’ve had scleroderma for too years got lots more now with it I am in house been shield so l been doing all the jobs that I’ve trying to do it take my mind off the pain until you stop lol. That’s if you can I get a lot of pain in my fingers and now my big toe can’t put my shoes on my face has got thin and every morning I have lots of Whit spots not nice so not much help to you only reading this takes your mind off you’re lol hope you feel better soon x

[marilynmcl in reply to Lucinda227loz]

Hi Lucinda...sorry you too are having not such a good time of it. The spots you are getting...I also had problems with spots on my face...but not the usual type..I think it was an infection that was causing mine so got onto doctor and she prescribed me antibiotics ...Lymecycline... and advised me that I may have to keep taking them for a long time.My spots have all gone and my skin is a lot better now. Might be worth having a word with your doc. The joint pain is very bad just now for me, but I put it down to the osteoarthritis, so I hope you are getting some pain relief for yours. I know how debilitating it can be.

You take care and hope things improve for you going forward. I too am in the shielding from this virus. So fed up with being stuck indoors! xxx

[Lucinda227loz in reply to marilynmcl]

Thank you I’ll ask my Doctor for lymecycline as it’s not good the spots are very white and so many I am Flucloxacillin for my toe not doing much at all I’ll have them time and time lots of painkillers lol anyway people are a lot Worse off them me thanks again take care x