Pulmonary Arterial Hypertension diagnosis

Hello all.

I have just had my diagnosis of Pulmonary Arterial Hypertension confirmed and feel a bit overwhelmed. We had a talk with a Specialist Nurse who stressed that PAH is a lifelong degenerative condition that I won't recover from and that we have to take it very seriously as it will be life changing.

I have been put on Sildenifil for it, and have also recently started taking Methotrexate for my Mixed Connective Tissue Disease ( I do have Scleroderma in my hands, and also Raynauds, but not an overall Scleroderma diagnosis) I was hoping that the combination of meds would maybe improve the PAH and that I would be able to increase my mobility from the rather sad and limiting amount I am able to do at the moment.

Anyone with more experience than me I would love to hear your thoughts!

21 Replies

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  • Hi Runalong,

    How do you find Methotrexate for the Scleroderma? does it help with skin tightening?

    Have you tried the wax baths for your hands?

  • Hello, Thanks for replying.

    I haven't been on the Methotrexate long enough for it to have had an effect yet (i'm told it takes 3 - 4 months and i've only taken it for 2 weeks!).

    I'm also on some steroids at the moment and they have helped my hands quite alot, but i won't be on them for much longer i don't think.

    I haven't heard of wax baths - what are they? and what are they for?

  • Hi Runalong,

    If you dont mind me asking which steriod drug are you taking.

    Wax baths help soften your hands :

    thh.nhs.uk/documents/_Patie...

  • Thanks for this. I had awful Raynauds ulcers this winter and am very keen to avoid them again, so will have a good look at this.

    And, i'm on prednisolone - 40mg at the moment but hoping this will be reduced once the methotrexate starts working.

  • Hi Im sorry to hear of your diagnosis. I have PAH which has been caused by systemic sclerosis (scleroderma) and was diagnosed with both conditions in Dec 2015. I'm taking sildenafil and ambrisentan for the PH. For me the diagnosis has been life changing particularly in terms of mobility but everyone's situation is different. I've ended up having to give up my career but I don't need the stress and exhaustion of work any longer. Give the medication time to take effect and take things a day at a time. Which specialist centres are you under for your scleroderma and PH ? I'm under Salford Royal and Royal Hallamshire in Sheffield. X

  • Hi Bagpuss,

    Am under Salford Royal and MRI.

    do you have skin involvment too?

  • Hi Naila_007 yes I have tightening of the skin particularly on my feet and have been suffering with calcinosis quite a lot lately.

  • Hi, thanks for replying.

    I'm at Papworth. I am currently off work, and will be for another few months. I have been wondering if I will manage to go back or not, but as you say I need to see what the medication can do and am trying to take things a day at a time.

    We would be in a difficult place financially if I can't work at all....

    How much mobility do you have now? Has the Sildenafil helped do you think?

  • I hope that you will be able to continue working. I worked as an exec PA so no longer a viable option and we have a five year old son so it takes all my energy to be a mum. Unfortunately my mobility has been severely impacted - combined impact of the PH and scleroderma so I need to use a mobility scooter outside of home. The sildenafil has definitely helped but I get terrible pain in my feet and legs due to skin tightening. X

  • Sorry to hear that your mobility is so impaired - but good that you can still get out with the help of the scooter. You're doing great if you're managing with a 5 year old anyway - that's no mean feat!

    Thanks x

  • Hello. I'm sorry to learn what you're dealing with and just wanted to say that I have nothing this serious to deal with myself but I do sympathise.

    I am just starting Sildenafil. I have primary Sjögren's with Raynauds, hypertension and subclinical atherosclerosis and am hypothyroid. I take Mycophenolate for my connective tissue disease. I'm prone to side effects so have started Sildenafil at the very lowest dose of one 25mg tablet at night. I'm hoping to introduce another tablet in the mornings as soon as I'm ready.

    I'm only on day 2 so it's too soon to say, but so far the terrible toothache-like pain in my feet and legs including knees has eased up. My rheumatologist thinks this pain is vascular and is related to my secondary Raynauds. So we are trying Sildenafil to confirm her theory.

    It is giving me a headache and some facial pain but then I already have sinusitis an ear infection (on second course of antibiotics) plus TMD/ inflamed parotids so this might just be me.

    I'm hoping it will settle down as my body acclimatises to it. But am cautiously excited by the diminishing of my intense feet and leg pain and have had fewer Raynauds attacks so far. Hoping it works really well for you as you adjust to this life changing diagnosis.

  • Hello - thanks for replying.

    It's nice to meet another mctd sufferer - but obviously I'm sorry you have it too.

    I have had a couple of mild headaches since starting the Sildenafil as well, but am trying not to dwell on the side effects too much, but i'm sorry that they affect you badly, that's tough.

    I am also taking Nifidipene for my Raynauds which I think has helped quite alot, although obviously it's the Spring time so the real test will come when it's colder. Maybe you could try this too?

    I am hypothyroid too and have been for 20 odd years - at the moment that seems to be the least of my worries!!

    May the cautious excitement continue :-)

  • Thanks. I have tried Nifedipine already but am terribly allergic. Re MCTD - I've not got the specific antibodies for this. I was first diagnosed with RA but this changed after my ANA showed clear positive last year so had a lip biopsy which was 100% positive for Sjögren's. So my diagnosis has been changed to primary Sjögren's with overlap CTDs/ conditions inc RA and Raynaud's.

    The headaches are defintely Sildenafil related for me but I have just had a really bad ear infection preceded by sinusitis and throats infections so I'm sure these aren't helping and my dose of Losartan has just gone up the week before I saw my rheum - could be contributing to facial pain I'm 🤔

    I am still awaiting tests for PH but hey - at least like you my thyroid has been well controlled for years so something is working for us! Best of luck with the Sildenafil. X

  • Goodness it's all so complicated isn't it?

    Sorry you were allergic to the Nifidipene. I got very dizzy when I first started taking it, but seem to have pushed through this. I got really awful raynauds ulcers this winter and i really don't want to go through that again, so fingers crossed the meds will help.

    Sounds like i've got lots to learn still, thanks.

  • Hello Runalong.

    Sorry to hear your diagnosis and I am also feeling very unsettled today...

    I am probably a couple of months behind you on the 'roadmap'. I was diagnosed with limited systemic sclerosis 2 years ago (but have probably had it for a lot longer). I am still active - I am a farmer - but I saw my rheumy today for routine apt and she reported back that on my annual echo a few weeks ago it showed signs pointing to mild PAH. She is referring me to Sheffield for further investigation and a probable right heart catheter. Given that I myself have noticed a very slight breathlessness (only when exerting myself but my job is quite physical) and have recently noticed palpitations as well then it is not looking good. I am already on 3 x 25mg sildenafil/day as well as Losartan potassium for my Raynauds as well as mycophenolate (and iron and vit D).

    I was going to post on here today anyway... as I have a recent echo and I also did my annual lung function tests last week, does anyone know if I am likely to go to Sheffield for more 'tests' first, or given my diagnosis, history and the evidence that is already there, almost straight to a catheter? How long are you admitted for to do a right heart catheter?

    I am going to see my GP and request a 24 hr + ECG anyway to investigate the palpitations (I have had a standard ECG when I first had a 'long one' and that was normal) which may also provide more info.

    I see a general rheumy at Leicester not a scleroderma specialist so I was relieved that she is referring me straight to Sheffield which I know is one of the PH specialist centres.

  • Hiya,

    At Papworth I had a day assessment first, where they did lots of test (Ecg, echocardiogram, walking tests, breathing tests etc) and then decided they wanted to the the RHC. Ithink that they know you have PH or not by the time they decide to do the RHC but it's the only way to measure the pressures accurately.

    For my RHC I was admitted the night before and after the procedure they said I could go home if I was okay on the meds (the Sildenafil), so I only had to stay in one night.

    Everyone at Papworth is great - i'm sure it's the same at Sheffield so you'll be in good hands. but it's a tough diagnosis to take in. I hope you can carry on working and they've caught it nice and early - you must be nice and fit which i'm sure can only work in your favour.

  • Hello Cowhide, You will be well looked after by Sheffield, I've been under their care since January 2016. My local cardiologist arranged for me to have RHC prior to being seen at Sheffield but after my initial appointment when I had various non Invasive tests they would have done RHC but for it already being done. I was in Wythenshawe as a day case for the RHC. At Sheffield I also needed To have perfusion scanto check for any blood clots in lungs and an MRI. Do you an appointment date when you will be in Sheffield yet ? X

  • Thanks Bagpuss, I had read some of your posts on this topic. I don't have an appointment yet but my rheumy said it's likely to be at least a couple of months away.

    I had a change of GP 8 months ago - my new one is only a semi-permanent locum though - and I decided it was time I went to see him. If I need to see him because of a crisis I'd rather we had been through my situation first so he has my background rather than starting from scratch. I got a 'routine' appointment in less than 24 hrs which totally floored me for starters!

    I was very pleasantly surprised - pity he's a locum!. I expressed concerns that I was having more frequent palpitations and explained about my SSc and the echo and the possible PAH. Given the length of time it will probably be before I go to Sheffield, he checked my heart and pulse all OK but has referred me to local cardio unit at Glenfield for a 24 hr ECG which is rather what I wanted - another 'test' to get out of the way. He also asked for bloods to check my thyroid function in case that's the problem (never been checked before).

    Trouble is we can blame everything on our scleroderma and alternative causes can get overlooked!

    He has also upped my Vit D above what my rheumy has me on, just to get my levels back up as they seem to have got reversed again whilst still in the deficient category despite 2000 iu/day.

    Also confirmed that in our area he is not able to do the shared care for mycophenolate which will annoy my rheumy as she keeps trying to move me off 8 week appts but if she has to prescribe it then she'll have to keep seeing me which is better for me!

    And - another reason for going to see him, I explained that with the myco, the Raynauds, working out of doors and surrounded by muck and bugs on our dairy farm, infection is a very real risk for me and I wanted to check that this was highlighted on my records. Without me even asking, he immediately wrote me out a prescription for flucloxacillin to keep on standby which is better than a trip to hospital if I can't get an emergency Dr appt when I next get an infection!

    Luckily for me he said when he was mentored at Uni he was attached to a Rheumy and there was one patient in particular he saw a lot of who had the whole CREST set (like me) so he knew the score!

    Not bad for a 10 minute appointment with a new GP in a practice which has just been rated 'inadequate!

  • Hi Cowhide, what are the odds of two dairy farmers getting this awful disease! I was diagnosed with cutaneous scleroderma this year and am on methatroxate. The swollen limbs, tight skin and shrinking tendons are making my life a challange. Only a fellow farmer can relate to not being able to help a cow calve or bottle feed calves. The shrinking tendons in my arms means that I now only have a maximum 40 degree bend in my wrists and the constant challange to infection from bugs that you just can't avoid on a farm is a constant source of worry. You sound like you have a good team to call upon!

  • Hello there Milkmaid (are we the only ones who define ourselves by our livelihood!). At present I am fortunate in that I'm am not being too seriously debilitated but we are normally hardy souls so perhaps others would just have given up!. The Raynauds of course is a killer in winter,and probably my biggest issue, trying to juggle gloves (warm and or waterproof) in winter when feeding calves, tying baler twine, milking, helping a calving, opening gates, doing injections, is a nightmare. The compromise between dexterity and keeping fingers warm and clean is a very difficult one! At least on our own farm I can go to the farmhouse for a quick warm and sort my hands out if I have a bad Raynauds attack unless in the middle of something critical. I have one index finger ulcer that is about permanent and chucks out calcinosis at regular intervals - it was crushed by a heifer 20 odd years ago and has always had dreadful circulation since. When a calf butts that and knocks it against the calf pens, or against the calcinosis in my knee caps I'm screaming in agony. I've had a couple of finger infections in the last couple of years, one was dirt in the skin cracks on my finger joints and another when a thistle from some hay went into my thumb. Being immunosuppressed is a worry.

    I get bad corns and callouses on my finger tips where I am picking up straw, hay etc or trying to stand a new born calf.up or move it. So far I do not have sclerodactyly thank goodness. The reflux is the same for me as for anyone but inevitably I am bending over a lot which does not help.

    And as for saying to a farmer... 'avoid stress'.... We had a horrendous TB breakdown, our first ever, (we are in the Edge area), 2 years ago and lost nearly all our in calf heifers and we had to go for TB testing ourselves, as there was a high level of visible lesions and they were worried we might have got it either from the bulk tank or cattle breathing on us. We have a small 70 cow closed herd so lost a lot of our bloodlines (including sexed semen in the 19 heifers and 11 cows that went), and it certainly didn't arrive here from other cattle......... say no more on that point.

    Then after losing so many (every cow that went was 5-6 months pregnant)we had to break the closed herd to keep our milk income up, a worry in itself, thank goodness we were meticulous on biosecurity, some of the heifers we looked to buy came back positive on bloods for IBR (we rejected them needless to say).

    We are TB free at present, but testing, RPA, cross compliance, farm assurance, brexit, current drought, and in our case, a farm partnership being split up, a wind turbine shut down for 10 months waiting repair and the insurers quibbling over payout, a new bypass set to come right through the middle of the farm in a few years time, no holidays for years ...and now possible PAH

    stress???? (laughs manically)

    #keepbritainfarming #lovecows. My ladies keep me sane (I think).

  • I understand completely, and I agee that perhaps we persevere when others would just give up, after all if we don't do it nobody else will! Luckily I don't have (at the moment) many Raynaulds attacks and because I have probably had a mild form of it all my life I have learnt to cope/live with cold hands & feet. The main thing for me is loss of movement and muscle mass, dexterity is a thing of the past trying to draw up a syringe to dose a cow becomes a major event! The chance to avoid stress would be great!

    Sorry to hear about the Tb breakdown, we too are on the "edge" (nervous breakdown or badger related I am not sure) and have and a inconclusive this year the first ever, fortunately we have gone clear since but it is a constant worry

    Perhaps we could form a "midlands Scleroderma group" - not just for dairy farmers of course. I am fortunate that my dermatologist is only based at Nottingham as some specialists seem to be miles away, trying to run a business and fit in hospital appointments, physio sessions, milking,calving etc can be challenging.

    I have found this group to be a great help, it's nice to talk to people who understand what you are going though.

    Keep smiling !!!

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