Has anyone with scleroderma in the lungs, actually had a straight answer regarding the prognosis.

[SandraMarie]

Consultants seem half afraid to let me know. Obviously I know it's different for everyone,I just want worse or best case scenario's. Can anyone tell me please?

[Carolanne]

I was diagnosed with Scleroderma/Lung Fibrosis Aug 2011, since then I have had regular Breathing tests, CT Scans and echo cardiogram. I am bing monitored by The Royal Free Hospital London and also my Hospital specialist. I have had 6 Cyclophosphomide infusions and am now on mycophemolate tablets. I have had a recent Ct Scan of my lungs which showed the lungs were no worse and a little better. I was told to aim for the Fibrosis not getting worse but they cannot cure it. I accept this and try to enjoy my life as much as I can

[SandraMarie]

Thankyou for your reply, I feel none the wiser, but I knew that might be the case. It's interesting to hear that there is some treatment available, as I keep being told there's nothing. I know there is no cure, I just want them to tell me examples of duration of living with it. I have a CT scan on Tuesday but no follow up appointment until September, that's a long time to wonder what the scan showed up. May I ask at what point does one get referred to The Royal Free, as I keep hearing it mentioned, or is it to do with where you live? Very best wishes to you. x

[lakemon]

I too have Scleroderma/Lung Fibrosis diagnosed in 2008. I am from Virginia in the US. I am also taking mycophenolate (2000 mg day). I have a lung specialist and also see the Scleroderma Director at Johns Hopkins in Baltimore, MD. I can seem to pin anyone down on the final progression of this disease. I would just like to know what to espect so I cam prepare my family. I have also developed pulmonary arterial hypertension so am taking Letairis to help open the artery from lung to heart. I guess we just have to keep pressing for answers. I can't even find anything on the internet. Good luck with all of your treatment Lake Mom

[AnnieMc]

Hi there, i was diagnosed with scleroderma in 2005, along with lung fibrosis. I am under the Royal Free specialist clinic for Scleroderma, but have my lung function tests at Brompton . My lung function tests which are every six months have been stable, and i was told thats how they would probably stay after this amount of time. However, since my last lung function i am certainly more out of breath than usual, and have a permanent ache in my chest. This might of course be due to other health problems i have at the moment, but never the less if your lungs are involved it is debilitating to some extent, you certainly want to do more than you probably can. Be positive as it's something you have to learn to live with .

[VanessaJoanne]

I don't think it makes sense to sit and wait for a referral to Royal Free. Tell your GP you want one. And as soon as possible. With this disease we have to be proactive and really hone up on symptoms and treatments. I don't have lung involvement but I have twice yearly lung function and echocardiogram.

Others with lung problems beem to be treated with cyclophosphamide, methotrexate and/or Cellcept. These are all very heavy duty drugs obviously with their own accompanying side effects but are generally preferable to death.

I think you said on an earlier post that you did not take nifedipine because it has side effects. Did you replace it with another drug for reflux?,

Untreated reflux results in stomach acid going in to the lungs which causes lung fibrosis.

[VanessaJoanne]

So sorry. I confused nifedipine with lanzoprazole. My brain is fuddled. Please ignore the comment about lung fibrosis. Also mycophenalate is the generic of cellcept.

[SandraMarie]

I don't take any medication, although I do have Lanzoprazole on hand in case I have a very severe acid attack. I was also prescribed an Atrovent inhaler supposedly to help with unexplained breathlessness and panting??

[stevefull2 in reply to SandraMarie]

hi, just read your message .talk to your g. p and ask for omeprazole this will control the acid i have had this now for 12 years just gone on two a day at 40 m g a day.please dont leave this to get worse good LUCK.

[SandraMarie in reply to stevefull2]

If you read my responses regarding this you'll see I have in the past had this (or Lanzoprosole same thing different make) and why I don't ...too many side-efects etc. etc. Be careful! x

[stevefull2 in reply to SandraMarie]

sorry to hear that this did no good. I have systemic scloroses and related problems and can understand what you go through. I will ask my g.p if there is anything else he can come up with if this is o k as he is very good with me and things like this.x

[SandraMarie in reply to stevefull2]

I prefer not to take pharmaceuticals if I can help it, thanks anyway. I was really originally just wanting some answers on long or short-term prognosis once Scleroderma had reached the lungs, I've been living with and coping with all the other aspects of this condition for decades now, but just wondering roughly how long one can go on with it in the lungs??? x

[SandraMarie]

Thankyou for all your answers. I shall just have to hope I can persuade my consultant to be brutally honest even if that does still leave us all in the dark. Best wishes to all.xx

[CWats]

I too have limited Sceroderma with Pulmonary Arterial Hypertension. I am on Remodulin to soften the arteries to my lungs. When I was first diagnosed with Scleroderma I was given 7 years to live, that was 22 years ago. In March of 2011 I was given 6 months to a year to live, I'm still here! I am on 4-5 liters of oxygen 24 hours a day, I am now being evaluated to be included on a lung transplant list for a double transplant, as it is my final option.

[SandraMarie]

Thankyou for sharing this with me, it's a great help. I wish you very well in your continued battle. You sound like a positive person, and that really does help. <3

[VanessaJoanne]

Why arent you taking any medication? Particularly with lung involvement. Where do you live?

[SandraMarie]

I haven't actually been offered any for my lungs, as the Scleroderma hasn't been confirmed in the lungs as yet (I had a CT scan today) For Bronchiectasis that was noticed about 4 years ago they said there's no treatment other than to be on anti biotics everyday. I refused this as I don't wish to become immune to them. I have discovered herbal treatment for this though, so just about keep infection out of the copious amounts of phlegm that come up every day.

[SandraMarie]

Sorry! I live in Norfolk

[VanessaJoanne]

If you only take lanzoprazole when you have a very severe attack of acid reflux what do you do when you have a less severe attack of acid reflux?

Any acid reflux is a VERY bad idea. It can enter the lungs and cause lung fibrosis. You also put yourself at risk of Barrets oesophagus which is cancer. I think you need to think really carefully about taking a very low drug approach with this illness. Also I say again, you cannot wait for your GP or even an inexperienced rheumatologist for very few are familiar with scleroderma to have a dawning idea that maybe they should refer you to a specialist hospital like Royal Free. YOU need to tell them you want a referral.

Don't waste your energy sitting worrying about when you are going to die take action to live!

[Tullos12 in reply to VanessaJoanne]

How do you get a referral to royal free is it on nhs

[SandraMarie]

It's so easy to say don't waste energy worrying, I'm actually not doing so particularly, don't actually have the energy in the first place! As for the acid reflux, I just make sure I am well wedged upright for sleeping, with umpteen cushions, pillows etc. Don't bend over, try to avoid foods that aggravate and so on... I was originally prescribed Lanzoprozole because the acid had burnt the opening to my stomach so badly that the swelling was visable and I thought it was a tumour.12 months later nothing had changed, as regards the reflux, and I was suddenly aware that this medication was not to be taken long-term. This is when I went back to GP who referred me to our local hospital, and it was one of their wonderful consultants that diagnosed CREST syndrome. I am surprised that so many people on here take so much medication, which in turn leads to other problems and severe side-effects? I think after my follow-up appointment in September, I will make an appointment to see my local herbalist again??

[Juniebug in reply to SandraMarie]

I just lost my BFF of 30 years to scleroderma - we didn't know how bad the disease really is, we didn't take it as seriously as we should have. Don't take this lightly PLEASE!

[Angela-Lee]

A lot of people on this site, like me, take prescribed medicine because without it our conditions would be considerably worse. I was advised on one of my visits to The Royal Free Hospital in London, a specialist Scleroderma clinic, that taking large doses of Lansoprazole long-term is safe and a lot less harmful than the effects of stomach acid on the oesophagus and lungs. I also try to go the 'natural' route - but not at the expense of my health!

[SandraMarie]

There are so many conflicting views, I'd rather sleep upright anyway, thanks! I originally asked about how long one was expected to live once the Scleroderma was in the lungs and all I'm getting is people telling me how to try avoiding it???

[BarbJ in reply to SandraMarie]

I don't know the answer SandraMarie. I have some lung fibrosis (diagnosed with that about 5 years ago) but have never been offered medication. I understand that it is true that if it is stable for quite a long time then, statistically, it is likely that it will remain so. But autoimmune diseases are unpredictable so there can be no accurate answer. I also understand (from reading on the internet) that those of us with limited scleroderma have a greater risk of developing pulmonary hypertension which is more likely to be life threatening more quickly.

By the way Barret's oesophagus is not cancer, but it puts you at greater risk of getting lung cancer. I'm on Lansoprazole and couldn't survive without it actually, I can still get reflux if I eat too late at night - 4 hours before going to bed the gastroenterologist advised me. Also, elevating your bed head 6 to 8 inches (the latter preferably) helps a great deal, though it's a bit odd to begin with. You need to be careful about relying on sleeping propped up - if you are bent at or near the middle of your body you might not actually be getting much, if any, benefit as your internal organs push your stomach upwards and the contents, of course, flow back into your oesophagus.

[BarbJ in reply to BarbJ]

Sorry - have just noticed I've said Barret's puts you at more risk of getting lung cancer, I meant stomach cancer!!

[Tullos12 in reply to BarbJ]

What is pulmonary hypertension

[Juniebug in reply to SandraMarie]

We always wondered why her specialist didn't tell her more about her condition. Now I'm wondering if we should have searched harder for someone more experienced. She has multi connective tissue disease. Lupus, RA, Scleroderma and Raynauds. Her specialist was from Mayo, but practices in OKC. I am so mad we didn't go further than him. He's the best here we were told but I'm not sure now. God I miss her so much! Please don't take this lightly!!!!

[SandraMarie]

Thankyou, Barbj for your reply, I do know I have to re-evaluate my lifestyle again. Ive just been reading up a bit on natural remedies for lung fibrosis and am going to try breathing in apple cidervinegar vapours and eating loads of licorice to start with, but I will take heed of advice about the reflux.I also have a problem with Hyatus Hernia, and tablets have a habit of getting stuck, which can be very scary, when you find you can't even drink for a couple of days.To be honest though the omezoprasole etc. tend to go down quite successfully. We must all just battle on I suppose. Today is a new day! x

[thyroidlady]

Dear S,

Scleroderma is different in each individual case. I joined this group because my sister has it and I was looking for ways to help her. You don't say what sort of lung involvement it is. If it is partial or if it is something called glass lung syndrome. So many advances have been made in the last few years that there is no way of knowing what your prognosis is.

My sister has outlived every prediction by years and is really going strong despite having the worst prognosis of a year and that was six years ago. She is still going strong.

She did take the most cutting edge route with chemotherapy and it has helped not only to stop the disease in its tracks but also to increase her lung strength a little.

So there is really no way of knowing what will happen, her doctor told her to take it one day at a time. She is on oxygen 24/7 now and doing as well as can be expected. Avoid crowds and colds and flu by taking all necessary precautions by washing your hands regularly and avoiding situations where you might catch something like sitting in the doctor's surgery with everyone coughing and sneezing around you.

Best wishes

Maria

[SandraMarie]

I have Bronchiectasis in the lower lobes of my lungs, diagnosed 4 years ago. CREST and diffuse Scleroderma diagnosed 10 years ago (althugh it would seem it had gradually been creeping through my digestive system since childhood .(My consultant said way back in about 2006 that he had never seen anyone walking about with it in such extreme state, so I'm not one for giving in to it easily,I have somehow managed to deal with symptoms as they arrive, but the breathing has very much worsened in the last year and this isn't something there seems to be any help with??) Obviously I do all the usual things to stave off infections, but with Bronchiectasis you have to treat them daily.

[thyroidlady in reply to SandraMarie]

Hi SandraMarie,

Are you on oxygen, full or partial? You have lasted 10 years since diagnosis which used to be amazing but is now quite common place. If you aren't on oxygen now, does your lung function get tested regularly? There are some new treatments for Scleroderma make sure you are getting the most up to date advice, but I also guess it is also a case of how aggressive you want to be.

Regards

Maria

[SandraMarie]

I'm not on anything other than an Atrovent Inhaler which can only be used a few times a day. I have regular appointments at the hospital to see if there are any changes in lungs, heart etc. but they don't seem able to suggest any treatment. I think I'm better off sticking to the herbalist, at least she makes sense and isn't living under the strong arm of pharmaceutical companies!!! (I may well be more determined for some answers when I go again in October) Maybe the mention of referral to Royal Free wlii shake them up, although they seemed relieved when I told them I was holding off chest infections with leaves from the garden, rather than pumping myself full of antibiotics daily, and actually said there was nothing they could offer.

[SherrySanford65]

I was diagnosed with pulmonary hypertension as a side effect of systemic scleroderma. There are medicines that treat it though that improvesoverall function and life expectancy.