PULMONARY HYPERTENSION?

[momo17]

Hi, I’ve just received results of my echocardiogram. I have interstitial lung disease so knew I was at risk of pulmonary hypertension. The results show my right atrial pressure being 23mmHg. I see perhaps 20/25 is PAH. Just wondering if anyone else diagnosed with this alongside ILD and what treatments you have received? Thanks

[Alice1956]

Hi Momo. I also have diffuse systemic sclerosis, interstitial lung disease and PAH. I had 10 cycles of Cyclophosphamide but my fibrosis continued to progress. I was then switched to Mycophenolate Mofetil which helped greatly with skin softening but not so much with my fibrosis. I am now on a drug called Nintedanib which appears to keep me stable. I also have PAH. My CT scan showed increased pressure in my pulmonary artery but I was sent to the Scottish Pulmonary Vascular unit at the Golden Jubilee Hospital in Glasgow for 4 days of tests. This included a right heart catheterisation which is the only way to accurately measure the pulmonary arterial pressure. I now attend there regularly to review my PAH medication. They started me on Sildenafil and increased the dose at the next review. I also take Macitentan , added at another review. I hope this information helps. Which part of Scotland do you live in? I am on the Black Isle in Highland. There are smaller local support groups too. Our group used to meet in Inverness before the pandemic. We are currently meeting through Facebook video calls. Alice.

[momo17 in reply to Alice1956]

Hi Alice. Thanks so much for your reply. You sound like you have had a terrible time but glad to hear certain meds have seen improvement or at least stabilisation. I am in Elgin but not on Facebook so limited to forums. I’m quite surprised that there are more people in this area with scleroderma than I thought! I am seeing rheumatologist 27th July so hopefully will get a better idea then but you probably know what it’s like. Just kinda panic that things are deteriorating so really appreciate all the info on what’s available. You take care of yourself. Best wishes x

[Coldhands65]

Hi. I too have ILD had fir a few years now. have been doing ok until recently more breathless since June last year. Echo delayed few times due to. COVID, when I did eventually got it done it revealed right sided pressure. The chest doctor I see along with rheumatologist thought possibly Pulmnary Hypertension. Since had lots of tests, currently awaiting right side heart catheter to find out. Obviously hope it’s not. Trying not to worry too much. Hope you get good results when seeing your rheumatologist in July. Tina.x

[momo17 in reply to Coldhands65]

Oh I really hope you get good results too. I haven’t been so much breathless as more heart just pumping out of my chest with the slightest incline or steps. I was nearly a year late with my echo too so it’s an poor time to have anything wrong with you. Have you had recent lung function tests and if so did they show a decline? Mine had went from 63 to 59% for DCLO which they said not to worry about but I wonder if i wasn’t limited with my mobility n I was active at all I would have an issue with breathlessness now. All the best x

[Poppy221]

I have mild ILD and formerly severe pulmonary hypertension - they are uncertain as to whether the PAH caused the ILD or vice versa or they both occurred at once. This was only formally diagnosed with the heart catheter- echocardiogram tends to be a good indicator but not definitive. Over the last two years I have been put on various medications for different problems. For the PAH I was started on tadalafil (vaso dilator) then on macitentan (endothelin antagonist - endothelin is a vaso-constrictor in your body that only affects arteries in the lungs so this dilates lung arteries). Then I was started on mycophenolate for the ILD and then on selexipag which affects prostaglandin so is again a vasodilator especially for the lung arteries. I used to have a dilated right atrium from the pressure but now it is only mildly dilated. Over the last two years, the three vaso drugs (also known as the triple therapy btw) have improved my heath from being class 3 to class 1 - functionally normal. I am awaiting lung function tests and CT scan to find out if my ILD has been halted by the mycophenolate.

[momo17 in reply to Poppy221]

Hi poppy. Thanks very much for your reply. That’s great your PAH has responded to medication. I hope you get the same results with your ILD. Mycophenolate did very little for me pain wise and skin but my lungs did stabilise for a few years. Only now they have gone down 4% which isn’t considered anything to worry about. I am due to see a cardiologist on 27th so I will see what he says. Good luck with your tests

[Poppy221 in reply to momo17]

Thanks. I was also put on hydroxychloroquine starting just as soon as I was stabilisied on mycophenolate. Had a sudden improvement in my hands and feet about 6 weeks after - in terms of tendon pain and upset joints. Then gradual improvement for about a year - including exercising with therapy putty and doing stretching exercises. Turns out my feet had problems not directly due to the scleroderma, but probably made worse by it. Seeing a podiatrist (privately in the end) has helped a lot - manipulation to free up seized joints, orthotics, exercises to stretch bits that were too tight and strengthen other bits that had weakened and now I am having shockwave therapy on neuromas between my foot bones. Big improvement. Need to find additional ways of fixing my hands.

[momo17 in reply to Poppy221]

I also have a neuroma. I didn’t know of that treatment, thanks. My hands are badly curled despite physio. I too have had to go privately for a lot of different treatments but not many have helped. I did try hydroxycloroquine for a few months but I didn’t find a difference in pain. That’s good you had success with it.

[Poppy221 in reply to momo17]

Poor you. Neuromas are horrible. Sorry to hear about your curled hands.

With regard to shockwave therapy, until a few months ago it was not recommended for people with autoimmune disease but the recommendations changed to "use with caution" . It can knock anyone back for a day after - headaches, fatigue for example. I've had three sessions so far and my reaction was less bad after each one - basically tired for about 48 hours after the first one, 36 for the second and not much for the third. Be cautious about who you go to see - as in a registered medical professional like a physiotherapist or podiatrist - as there are warnings about bits of your anatomy not to use it on - especially over air filled parts of your body like guts and lungs and you want to see someone with proper anatomical training. I had mine with a Swiss Dolorclast machine and a podiatrist who'd been on a two week training course in its correct use. Dolorclast were the inventors of the technology. I'd personally be cautious about folks using other brands "just in case" - enough things can go wrong already. With the neuromas, having the manipulation first to make my feet flex properly and then the shockwave to fix the neuromas was the right order of events - because the neuromas can come back if you don't fix the underlying cause first. What the shockwave machine does is stir everything up and take something from chronic to more acute, and then your body has a second run at healing it. Once something is chronic your body isn't actively healing (is my understanding of what I was told). Also helps with blood supply. Shockwave therapy is now also being used for erectile disfunction. Regarding hydroxycholoroquine it takes at least two months to start showing an effect.

[momo17 in reply to Poppy221]

Thanks. That’s great info and always good to hear of new treatments.

[Coldhands65 in reply to Poppy221]

Hi Poppy. Sorry for very late reply. I already have ILD have had for a few years now. On mycophenolate for 5 years so fir me the IKD came along first. I finally have date for right sude heart cath 5th August. Really hope I don’t have it, although will be good to know exactly what’s wrong and get appropriate treatment. Once again sorry for late repky. Ive been having flare ups of all sorts. Keep well.

[momo17 in reply to Coldhands65]

Good luck with your appointment on 5th. Not long to wait now. I’ve had all different flare ups recently too n blaming the vaccination. Settling down a bit now tho few months done the line. I really hope you get some answers