Pulmonary Arterial Hypertension -

I have just seen my rheumatology specialist and after a lung function test, he has said he wants to refer me to Sheffield, as he thinks that I may have PAH. I am very frightened as everything I have read starts by saying it is a lethal part of systemic sclerosis of which I have been diagnosed with many years ago. I am now anxiously waiting an appointment from the Royal Hallamshire and don't know what to expect. Can anyone out there reassure me?

10 Replies

  • I went for this test at Freeman Road Hospital, Newcastle. They repeated the Eccocardiogramme, did an ECG and did a chest xray. They then assessed the pressures in my Pulmonary circulation. It was decided that my results were adequately treated with my cyclophosfamide so I had no more intervention. I hope this helps

  • Yes PAH can be nasty BUT there are treatments available now and don't let anybody tell you otherwise. I was suspected of having it a few years ago but it turned out that I had suffered Rheumatic Fever as a a child. I had Moderate Mitral Stenosis which was treatable with a minor surgical procedure and at the same time a Right Heart Catheterisation Test was done to confirm that there was no Scleroderma involvement. I was very shocked to be told at first that it was untreatable and to go home and accept my fate!!!!! Fortunately I took myself off to a Cardiologist and was referred to the right hospital treatment and now I am AOK. Hope all goes well for you.

  • Thank you.

  • Thank you. I appreciate your reply and it has helped.

  • My doctor just wanted to exclude this possibility.. the PFT is sometimes ambiguous.. I do not have PAH and I hope you do not.. it is difficult not to worry, but it is not proven till you had a catheterization. It is wise of your doc to make sure .... fingers crossed..

  • Thank you. I hope that I am as lucky.

  • It sounds as though you have been referred to the right specialist & PAH can certainly be treated to reduce the inflammation which causes the fibrosis. Medication may have to be taken for some time. Check out the leaflet on Lungs on the website. Hope it's not long before your appointment in Sheffield comes through. Good luck.

  • Thank you. I have spoken to a nurse at Sheffield and I know they are keeping an eye out for the referral but knowing my local hospital, the secretary won't be seeing this as urgent so it may be sometime before an appointment comes through.

  • I have PAH along with pulmonary fibrosis (Scleroderma). I had a right heart cat in 2012 (easy procedure) and was put on meds. The lung involement is a major issue with PAH but the docs are quick to act on both. The medication I am on is expensive (I live in US) but I was able to get a grant to pay for it. The earlier you are diagnosed the better. There is a lot of treatment for PAH now. I would recomment a saying we use "The squeekey wheel get oiled" meaning call often about your referral and appointment frequently. They just need to know you are concerned.. Good luck and just keep pushing the docs.

  • Thanks for your help. I am planning to phone my specialist's secretary on Monday to see where she is up to but as she only works mornings, I think I will have to keep her on her toes.

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