My Ph was diagnosed two days after giving birth to my daughter Paige (Paige was born 22 December 2007) I was told that I had a 5 cm hole in my heart which caused high pressure in the right side of my heart and lungs. I was seen by many Doctors and surgeons all of which i never gave me straight answer. First I was told I needed a transplant, then it was surgery, then it was they were going to send me home on tablets for the next 20 years. I was in hospital for 3 weeks on 24/7 oxygen. I couldnt even get out of bed to go to the toilet. At one point I think on the way back from the toilet my O2 sats dropped to around 30 thats when i was surrounded by about 50 people.
When I eventually got home I had an oxygen consentrator that was on 24/7. 3 weeks after I got home I was started on Bosentan. On my first check up I was told I only have 15 to 20 years to live and maybe the possibility of a heart/lung transplant but he was positive I wouldn't live past 60! By this time I was shaking and crying and all I could think was I have this new born baby girl at home who I wasn't going to see grow up. And the worst though was that she wasn't going to have a mummy who was going to show her how to brush her hair and clean her teeth and love her unconditionally. That's when I dug my heels in and said to my self lets show these people they're wrong!!!
On my 6 month check up my 6 minute walk test had improved and my pressures where down by about 20 points (from 100 to 80) I was off my oxygen, my sats where 94% at rest and my aterial blood gas was about 60%. My Cardiologist was very impressed with me. Shocked actually. He then said that if the medication continues doing its job and my pressures keep dropping then they will look at closing my hole in the hope that my PH will come down to a minimum. But I am constantly reminded that I am a very rare case and there isn't a 100% guarantee that I will get better with the closure of my hole.
I am not in any rush to get my hole fixed as my quality of life is pretty good. I work 5 days a week, 5 hours a day, I manage a house, a baby and a boyfriend (who I class as another child ha-ha) I only get short of breath if I climb a hill or a set of stairs but who climbs stairs these days any way???
I am on Bosentan and warfarin (8-9-mg) and I am pretty stable which is amazing as I have only been diagnosed with Eisenmengers (PH due to a hole in the heart) for 6 months. If any one has any questions feel free to ask any time as i am happy to answer. I am very interested in hearing from other Eisenmengers patients to see how they are travelling along.