Stacy did not have any of the symptoms for Pulmonary Hypertension, as far as us laymen knew, except she was swelling up like she'd been given huge doses of steroids and couldn't even open her eyes because her face was that swollen. When Watford General discharged her in June they said they were referring her to Hammersmith Hospital. Much later on, after she eventually went to Hammersmith I found I had her discharge papers from Watford General and on them it said Pulmonary Hypertension? So I guess they knew, or guessed, but never referred her and then in August her face blew up again and our GP (General Practitioner) was called and he referred her immediately to Hammersmith Hospital and the rest is history.
NOW FOR THE FACTS
There is a disease called Pulmonary Hypertension. It is rare, but not nearly as rare as what Stacy has. What Stacy has is a different genre of this rare disease.
Pulmonary hypertension is raised blood pressure within the pulmonary arteries, which are the blood vessels that supply the lungs. So you can have really low blood pressure (like Stacy has) and yet still have Pulmonary Hypertension which is really high blood pressure in your lungs.
PH is a serious medical condition that can damage the right side of the heart making the heart less efficient at pumping blood around the body and getting oxygen to the muscles. Stacy, at this point in time (last August), had a litre of liquid between the lining of her lung and her the outside wall of her lung.
(If you click on the link above you can read all about Pulmonary Hypertension)
The doctor we saw at Hammersmith Hospital, didn't tell us outright what was wrong with Stacy but just said it was incurable. My reaction was 'big deal neither is MS and I live with it just fine'. That was on the Wednesday and on the Thursday the head of the Pulmonary Hypertension unit came and spoke to us. He said that there were only about 0.1 cases of PVOD in Europe so that makes Stacy one in ten million, and that what she has is rare even in the realm of rare diseases and that it is called, by the acronym, PVOD.
PVOD stands for Pulmonary Veno-occlusive disease and it is difficult to diagnose and is the rarest form of PH. Diagrams were drawn and we sort of understood, to a degree, because we were trying to take this all in, which was difficult to say the least.
So I asked him some questions, but not the right ones I guess because I really didn't want to know. But that the only real 'cure' for this rare disease is a Heart/Lung transplant but together with everything else that Stacy has wrong with her, this was doubtful plus she is still smoking and they definitely won't do a lung transplant if you smoke.
I went home and went on the internet and typed in PVOD and was given all the information Dr. Gibbs had already given me but told that the median life expectancy was 2 years from diagnosis. So when I saw him the next day I asked him and he said yes that was about it. I also asked him if she would suffer and he said no, so that at least is good.
So that was where we stood. Definitely not great, in fact far from it and I really don't know how any of us will cope with this dire prediction. All I can hope is that Dr. Gibbs is wrong but somehow I don't think so as he is where people with PH and PVOD come to from all over England, Scotland, Wales and N. Ireland because he is the best.
But this so isn't the way it's supposed to be. She should be burying us and sitting Shiva not the other way around. I can remember months ago, after I had been following a blog about a young mother fighting breast cancer and losing the battle and thinking, and writing to her young widower Elliot (https://fightinggenghis.wordpress.com/) 'I feel really sorry for you and the children, a daughter and son, about Rosie's passing but how on earth are her parents coping. Well now I think I'm in the same boat and can't stand it and don't really want to know. I don't want to be where I am and neither does Larry, her dad. Nor Stacy's brother and sister.
Stacy is my normal lovely daughter who wouldn't hurt a fly and would give anyone, if they asked, her last penny.