SSc ILD & PAH

[waermebaer]

Dear all,

I was wondering who of you have been suffering from ILD and/or PAH and if so for how long ? How has it affected your life and what medications have worked for you ?

Thank you

Kind regards from Germany,

Waermebaer

[trunchalobesity]

I’m not sure what PAH is an abbreviation for?I’ve had IKD for over 20 years.ive had prednisone but the wonder drug for me has been Mycophenolate.

I couldn’t tolerate Azaphiaprene, but I know this works for some.

In the early years I had oral methotrexate and two 6 month rounds of Cyclophosphamide (by monthly infusion), which was the first treat treatment to halt the relentless progress of the disease but these are aggressive drugs.

Good luck x

[waermebaer in reply to trunchalobesity]

Thank you for your valued input. Pulmonary arterial hypertension (PAH) is one form of a broader condition known as pulmonary hypertension, which is high blood pressure in the lungs. In PAH, this increased pressure in the vessels is caused by obstruction in the small arteries in the lung for a variety of reasons.

Kind regards,

Waermebaer

[momo17 in reply to waermebaer]

Hi, I have had ILD for about 4/5 years now. It has stabilised with 3g of mycophenolate daily but this has done nothing for any of my other symptoms. I am fortunately not too aware of my breathlessness as have limited mobility but am aware my heart works overtime to compensate for low oxygen levels n I worry about PAH also. I’m sure if I had a normal lifestyle the ILD would impact more. Have you recently been diagnosed? I was initially panic stricken reading the mortality rate of ILD but I’m still here albeit struggling a bit!

[Tiggywoos in reply to momo17]

Hi mom17🙂Do you mind me asking if you suffer with waking up every morning with headache ?

Thank you

[momo17 in reply to Tiggywoos]

No, headaches is the one thing I don’t have! Last couple of weeks since jab I’ve had one but had it checked out n now I’m fine.

[Tiggywoos in reply to momo17]

Thank you for your reply . Glad no lasting headache from jab

[waermebaer in reply to Tiggywoos]

No Covid jab related headache here either.

[Bkart]

I’m borderline with PAH but have been affected badly with breathlessness to the point whereby I can only walk a short distance before it sets in. It is now happening with just daily activities, climbing stairs, even light housework etc., can set it off. Last year I was being accessed but due to the pandemic the tests were halted but I have just had a CT lung scan. I also have Cardiac issues and the cardiologist was puzzled with my unexplained breathlessness but I now have an appointment at a Specialist Scleroderma unit so I am hoping that the last piece of the jigsaw will explain what is happening to me.I’m desperately hoping that I can then get targeted treatment for this. I feel mine is exercised induced PAH, what is yours? Mine has been getting worse over the last 4 years but previously nobody could give me a definitive answer. I understand the mean pap for diagnosis has now been lowered from 25 down to 20 to reflect borderline cases. I try not to think about it too much but it does have a serious impact on the quality of life for people with Scleroderma and PAH, it must be the same for ILD. Do you have both conditions, if so that must be so difficult for you. Trouble is these things take so long to be diagnosed and the sooner you can start treatment the better the outcome. I understand treatment for these conditions really does work and there is a lot of targeted ongoing research. Best wishes to you.

[momo17 in reply to Bkart]

Hi bkart, did you get your tests at scleroderma centre yet? I’m waiting to see cardio as my ILD slightly worse at last lung function n pressure n right ventricle was 23. I have unexplained racing heart rather than breathlessness. Hope you get some answers.

[Bkart in reply to momo17]

Hi, thank you for asking and sorry to hear that your ILD has worsened with RV pressure at 23.Yes I went to Salford Royal Scleroderma Unit. They were very good, they are seeing me again in 4 months with the hope that I will have stabilised, which unfortunately things seem to be progressing, my face and mouth are now much worse as with other areas. Professor Herrick asked my GP to monitor me but that doesn’t seem to be happening too well. I had to ask for an appointment, and having said that he knew nothing about Systemic Sclerosis he finished by suggesting it was psychological, which didn’t go down well with me and he didn’t refer to monitoring me either!

Have you had a cardio monitor ever, I am asking because I had similar symptoms a few years ago which couldn’t be explained. Eventually I went privately to find that I had Paroxysmal Atrial Fibrillation (heart arrhythmia which comes and goes and can be very difficult to detect). It can’t be cured but can be treated successfully with either medication or if needed a cardiac ablation procedure ( I have had two). I read that AF can go hand in hand with Systemic Sclerosis but please don’t be alarmed by this just a thought.

As I don’t have ANA antibodies etc., it will take time to get a definitive diagnosis but I feel it possibly does look like SSc., time will tell but a diagnosis would certainly help with a PH diagnosis as well. Like joining up the dots isn’t it. Good luck and read up to see if AF could possibly explain your symptoms. Don’t want to sound alarmist but good sound knowledge is empowering at times and for me helps me to stay in control of my situation without going overboard if that makes sense. I suspect it’s the same for many others. Please keep in touch and let me know how you are progressing.

[momo17 in reply to Bkart]

Oh that’s disappointing when doctors don’t know anything about illness. I took over a year to be diagnosed. Sorry to hear you feel things are worsening too. Hopefully now that you are on the radar at the scleroderma unit you will be monitored appropriately. I have had a heart monitor a couple of years back which showed I do have an ectopic heart beat that explains some of my symptoms but things seem to have worsened recently with the racing particularly with any incline. Thanks for the suggestions tho. Like you say any knowledge we can glean ourselves is helpful. Good luck with getting the right care yourself. Take care.

[Bkart in reply to momo17]

Sorry I haven’t replied sooner, like many of us this hot weather is really getting to me. If you feel your heart symptoms are getting worse make sure you get a referral back to cardiology, insist! Take care.