Monday 30 November 2015

So Tell Me What is PVOD?

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.


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 ( '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.

Sunday 29 November 2015

The Start by Stacy's Mum

I am going to be writing this blog to keep everyone updated, that wants to be kept in the loop of how Stacy is doing.

It's been a hard 20 years for her.  When she was a teenager Stacy kept on telling me her stomach hurt and my silly rule was no temperature my kids went to school.  I took her to the Doctor constantly and he kept on saying it was nothing.  

Well it wasn't nothing.  When Stacy was 22 it was discovered that she had Crohns Disease.  So she was never telling me she had stomach ache when she didn't and boy did I ever feel bad.

She spent some time in Watford General Hospital under a Gastro man (who shall remain nameless so I don't get sued or anything and boy would I ever like to sue him).  It turned out that she had the Crohns in the small bowel instead of the large bowel which is another ball game completely.  You see if you have it in the large bowel they can give you an Ileostomy which would give the bowel time to recover.  But that was 20 years ago and things have really moved on from there. 

Her doctor at Watford labelled her a hypochondriac and every time she went into hospital with a stomach ache they just automatically put her on Steroids.  Now steroids are a wonder drug of sorts but like everything else there is a downside as well as an upside to them.  Stacy was given these drugs ad hoc when she wasn't even having a Crohns attack.  

G-d forbid they would have done a blood test before giving them.  But no!  As soon as she went into A&E the steroids were given intravenously and we were never told of the side effects and to be honest I never even looked on here to see what side effects, if any, there were.  Once they are started they have to finish the course or 5 days of intravenous steroids followed by tapering off doses by pills over a three or four week period.

So what did the steroids do, I hear you ask?  Well she now has, via the overuse of them:
  1. Osteoporosis
  2. Fibromyalgia
  3. Losing her teeth because she has no bone density from the Avascular Necrosis
  4. Avascular Necrosis - this is basically the death of the bones in her hips. The surgeon at Watford General has replaced the left hip twice and it still isn't right.  He also left her hipless for 9 months because it got infected and he removed it and didn't put it back until we complained to PAL. 
  5. Rheumatoid Arthritis  
She has other things wrong thanks to the steroids but I just can't remember them, sorry.  I know I'm a rotten mother, what can I say!

I will explain about the PVOD tomorrow because she is really poorly today and I can't write anymore.  But thank you if you are reading this.