For the newbies, revisiting my past.

Back to the Future

Some of the newbies coming on here have asked me to write a blog about what happened in the time after I reached my A Team at the Royal Hallamshire so here goes. Now before you read it I need to tell you NOT to get scared, it gets better, honest. So here it is.  A catalogue of how I  got from there to here, now living a life as you can see from my blogs.  I am doing things I could not do when first diagnosed,  It took time to get here but step by step, baby steps at first here I am.

Now remember this goes back five YEARS.  It is what most of us go through at the very beginning but it is all very necessary to get us on the right track.  Please leave this page now if you know exactly how it goes, I don't wish to bore you as you too will have been through mostly the same things. 

The three days I spent in my local hospital were pretty tough as I was given no medicine, though they wanted to give me morphine as the pain in my heart was bad.  I refused as I am not good with this medicine, or indeed any of the medicines from this family.  Colin had to come and shower  me each morning as the staff were too busy,  I remained in bed the rest of the time BUT I had my family and friends around me, the staff were marvellous in that they allowed anyone and everyone to visit so in fact it was quite like a party.  Despite the pain I made people laugh at the fact I was dying.  I remember making a joke and my sister in law Linda asked how I could laugh at such a time,  I said I didn't want everybody to say when I died well wasn't she miserable just cos she was dying!  All I wanted though was to get to Hallamshire and the day arrived and I was told the ambulance was on its way for me.  Oh the feeling of relief. 

On my arrival I was immediately given a bed in the room opposite the nurses station so they could keep a close eye on me,  I went through the routine of answering all the questions to a lovely junior doctor, Richard who I remember had his arm in a sling as he had broken his shoulder by being hit by a car whilst on his bike and he was finding it very tricky to write all info down.  Then the professor Iain Sabro came to see me. After an examination and a look at the X days of my heart sent over from Spain and Halifax he sat down, held my hand  and said it was very serious.  I said I knew and begged him to keep me alive longer than the six weeks I had been given in Spain, almost a week ago now.  I told him my daughter was having a baby in six weeks,  I needed to see this baby and see if it was a boy or girl., He promised to do his best but warned me I would be very busy for the next two days as I would be going for so many tests.  I had been put at the top of all the lists to have these done,  meanwhile I must not get  out of bed as he feared a heart attack. I was pushed in my bed or wheelchair to all of these.

I then met the nurses who would be caring for me for the next five weeks and oh what  a joy they were, as  was the sister, how I loved them all.  There were no surnames used on this ward, not from the cleaners to the specialists, first name terms were used  all around. I liked this as it made everyone taking care of me feel like a friend and indeed they did become good friends. Though some have left over the five years we still keep in touch and I love that they follow my progress and I can see into their lives too.  Sister Taylor was kind and loving and wiped my tears when I asked if I would ever hold my grandchild.  She assured me that I would,  Allison was the nurse always singing and must have known every song known to man. Liz had problems with her partner but was always a tower of strength and the list goes on and on,  

I was immediately put on a drip into my arm and given the drug iloprost,  I had to sign a form to agree to it as it was not, at that time, approved by the UK though it was in America.  As ipah is what they call an orphan disease, meaning very rare we could use these drugs whilst awaiting approval from our medical federation.  We quite simply didn't have the time it takes to approve the drugs through normal channels.  

Then began all the tests,  so many and the fear of them.  I know now there was nothing at all to be scared of but I was so poorly and as my body was being pushed from one department to another, one machine  to another it was scary.  I was terrified of the right heart catheter.  I had no idea of where I was being pushed to when I went from the cat scan to the theatre for this. There were so many people in the theatre and three people behind the screen doing what needed to be done with computers,  huge cameras  over my head were moved into place and the procedure began.  My fear quickly turned to a feeling of peace as a doctor I had met on the ward was performing the operation and a nurse from the ward was holding my hand and monitoring my blood pressure.  The atmosphere in the theatre was lovely, calm and dare I say it loving.  I felt very little, just the injection into my neck and around twenty minutes later I was being helped back onto my own bed in the theatre,  it took longer than the twenty minutes performing the right heart catheter to set the theatre and cameras up for it.  I never feared them again and have  had many,  

My next fearful one was the MRI, Having being told I was so poorly the thought of going into that tube was terrifying, really terrifying,  Here again the staff came up trumps,  As I had a line into my arm with the iloprost they needed to extend this line and have  the drip outside of the room as it was, of course  metal,  A huge line, metres long was attached to my drip so I was never without the iloprost and a nurse, Lisa from my ward stayed in the outer room where, as they were setting up the MRI she talked to me and calmed me down,  I knew I would be in this machine for at least,45 minutes. How did I cope, well I closed my eyes and thought about my grandchild about to be born, my husband who I love dearly and is my rock and my children.  I knew I didn't want to leave them, there was so much more to say to them. I also wanted to make peace with special people from my past soI  knew I had to let them do this, to see all there was to see so I was determined to stay strong for this test. I also made sure to listen intently to the instructions given to me, to breathe as and when told to and was determined NOT to press the panic button.  Again I have now had many MRIs and know there is nothing to fear.  It's warm in  there and now my only fear is that I will fall asleep and miss the instructions about when and how to breath.  It is truly not scary.

On then to another machine that was to find out, through radiation, where the blood was going to from the artery leading from my heart to my lungs.  So large was the right side of my heart that it had actually pulled and stretched this artery and torn a hole into it as it was so stretched,  I was  told later it was ready to sever!  That would have  been certain death!  As I lay quiet and still in this machine I was waiting so long for them to start I was getting fed up.  After around half an hour one of the team came into the room to do some adjustments to the machine and I asked him how long it would be before it began.  He smiled as he told me I was half an hour into it already,  it was unlike any other machine so quiet was it.

Then onto the breathing test, or the lung function to give it its correct name,  Now this was a bit traumatic as they were running late and my iloprost was almost finished,  I knew it was supposed to be continuous and I was terrified it would run out, as indeed was the nurse that was with me,  Here is where my amazing hospital showed how they made sure I was kept safe at all times,  I went into the room for the test and was horrified to see a bike, I truly believed they expected me to ride it and I begged them not to make  me get on it as I could barely sit up by this time,  they smiled  and assured me I was not doing that but I was to breath into the machines that tested my lung function. I did so poorly at these tests that they told my doctor it was impossible to measure my function at that time,  The  nurse who was with me was horrified to see just how little iloprost was left in my pump and rang ahead to the ward so they could prepare me another syringe to be ready when I arrived back.   A porter went ahead opening doors and another held the lift as I was wheeled back to the safety of my room and the new syringe applied.  Then came the nights and the machine beeping all the time.

The problem was the machine was very sensitive and every time I moved my arm the alarm went off and the nurses came running.  My lovely friend Maggie, who has sadly since  died, would be scared to go to sleep at night so worried was she that the nurses didn't hear the beeping so she was always pressing her buzzer to attract the nurses attention.  Her husband Bill Hamilton is still is on our site and I want him to know I loved her and cherish the times we spent together.  

Having to be hoisted into the bath and being physically washed was a tough one for me.  Care had to be taken not to wet the line and as I was in the hoist movement was not easy,  the nurse bathed me and washed my hair, I found this hard and oh so tiring. One would think that having everything done for me in the bath would be relaxing and not strenuous, how wrong was I.   On being taken exhausted back to bed I had no strength to brush my teeth and I cried.  When I was asked what was wrong and I told the nurse she replied '"well I can brush them for you" and so she did. 

Hickman Line.

Lisa my ph nurse arrived and pulled the curtains around my bed.  Colin was with me, as indeed he was every day, being allowed in all day long. The look on my face was a terrified one as she asked if I knew  what she was coming to talk to me about,  I said yes I did, it was about my dying.  She said oh no I am coming to talk to you about living!  She then showed me a piece of felt made to look like a human diaphragm and explained where the Hickman line would be put into my body, where it would come out and where the pump would be, around my waist,  This line was to be with me forever and pump the iloprost into me through the line every two minutes to the end nestled close to my heart.  It was to allow all my blood vessels  in my body to dilate  and so help the blood to flow better into my lungs, in this way it slows and can even stop the worsening of the ph.  We were to be taught how to fill the pump daily. We were told that infection was a very real danger and we must not get blasé when doing the procedure, cleanliness was soooo important, 

I won't go into all we needed to do to change the pump daily but it would take around twenty minutes daily and twice a week half an hour as we needed to change some more of the line,  It was hard to learn but a hugely important lesson, that once you began this routine you must not scratch your nose if it tickles or put your hand, even when gloved anywhere you had not  disinfected. If you did you began again as just by carrying on the procedure with hands that were not now clean could introduce an infection which would travel down the line to my heart, 

I found it hard when they increased the iloprost,daily,  it hurt all my body badly and my head ached so bad,  painkillers that others could take I could not, I had once been hospitalised after being given codeine so anything of that group was a no no for me.  Richard my junior doctor worked hard to find a painkiller I could take and it took him four hours to come up with one and what with the pain killer, two paracetamols and an anti sickness tablet we got there and after five weeks I was allowed home!  

Before going home though David, my doctor wanted me to leave the safety of the ward and have a couple of hours out.  So it was then that Colin arrived with my clothes and a picnic and we left the ward.  How I hated it.  My body hurt, walking was hard, I didn't want to eat but most of all I was terrified. I had left my warm, safe cocoon.  How could I ever get any semblance of a life back outside of my hospital, away from my nurses and doctors.  I despaired and if truth were told if I was told I could have stayed in the hospital forever at that stage I happily would have.

Life at the beginning was tough, Days were spent just lying on the bed or settee.  I wrote out my funeral wishes, my husband hated me doing that but I needed to get it done as I knew he would be distraught and would find it difficult if the worse happened and I died, I spent a lot of time in the hospital as every time I had a cold I was admitted and given huge doses  of antibiotics through a cannula.  If my skin looked red or broken where the line came out it was back to the hospital and more antibiotics.  The microbore that covered my chest where the line came out of my body was irritating my skin so I would scratch it and hence back to the hospital as they did not want  an infection to grow. I  truly believed I would never get a life back. I stared out of my window and looked with envy of people walking by, just walking without giving it a thought. How envious was I whose every move was difficult.  My feet felt as though I had big chunks of wood attached to the bottom of them, a weird feeling that was for sure. Steadily though over time the medicines worked on my poor broken heart and after twelve months I was changed over to oral medication, something nobody thought would happen. The  doctors, nurses and Ian my pharmacist were jubilant when they saw the scans of my heart as the  right side had reduced to almost normal and so began the gradual change over.  How lovely it was to not have achy limbs!   The iloprost made my joints hurt and I had to learn that when eating it was important that with your first mouthful of food move it round all your mouth. It hurts but after that I could eat all my meal with no pain,  Getting the line out was simple, though it was not taken out immediately but had  sat closed off hanging from my chest for three months just in case the oral meds didn't take.

At first I hated my heart and lungs, feeling they had let me down.  Now I actually love them because they are working so hard, despite my condition to keep me here and stable. It was a terrible struggle for my heart to pump blood and my lungs to receive it but they valiantly worked away when they could so easily have failed.  It's not their fault I  got ipah and it has made life so difficult for them but they struggle on breathing and beating for me so I owe them so much.

Gradually I began to do things again.  Go out for meals, walk, shop, all the things I believed I would never do again.  I don't lift my granddaughter, lifting is a no go area for our hearts.  It doesn't stop me cuddling her though and she knows I have a poorly heart.  She once got a tissue and put it  over my heart and told me it was a plaster and that tomorrow I would be better, oh how I wished! When she was a baby and I would give her a bottle she would twiddle my line around her fingers! When I have been stupid enough to carry heavy bags of shopping in from the car I pay the price and end up in bed so most of the time I have somebody else come down to the car to do the lifting. 

When I was first diagnosed there were very few medicines for this disease, I believe there were three.  Now there are many more and all in the short space of five years!  It is an exciting time for development into drugs for our condition,  More and more is being learnt. And so much more research is now being done.  Now if one medicine does not work for one person they can be switched over to try another.  Trials are going on all the time both for cures and for medicines to hold back the ph whilst waiting for the cure.  Try to be positive.   I do believe this has helped me to come so far.  Allow yourself to grieve for the life you had but embrace the life you have. 

Five years on after diagnosis here I am living a life, not the one I would have wished for, and waiting for a cure  but a life non the less. I have learnt to listen to my body, not to push beyond when it tells me to slow down. There are things I can't do now but know  I will when the cure comes and I have learnt to take each day as it comes, sometimes good, sometimes bad. When first diagnosed it was all doom and gloom.  I now know that there is a life to be lived with ph.  I know this ph won't beat me and I'm having a ball watching my grandchild, a girl, my darling Izzy,  now five grow up!  

I believe we in the ph world are very fortunate with all our A Teams who work so hard for us to keep us as well as can be.  I believe they really care about us as people and not just a number.  I know I am fortunate to be cared for, and have been cared for, by my amazing team.  I owe them so much more than a few words can say, I know I owe them my life.  I love them all.  

I am thankful daily for my husband who loves me so deeply and hurts for me when I hurt.  I have a strong marriage to a really lovely good man who would do anything to help me and make my life easier whilst living with ph.  It was more than I could have ever dreamt of when I met him and we married.  I don't quite know where I would be without him and I don't want to, he is my rock and one I know I can always depend on.  An amazing step dad to my children and the very best granddad to his darling Izzy.  I say his cos of course she is my grandchild too but honestly if you saw the two together.  The bond they share is soooooo strong and he really throws himself into playing with her, it's so lovely to see. He totally understands ph as much as anyone not living with it themselves can do and does so much for me.  

So there you have it, I hope it helps and for you to see that the procedures are nothing to be afraid of and that we have new medicines coming out all the time, If you are put on a Hickman line it will certainly help your heart to recover. If I were told tomorrow I needed another I would embrace it as I know it gave me a chance to rest my broken heart . 

Live the life you have, enjoy each day and know that there is a future with ph.  I never could have believed I could have climbed out of the abys I was thrown into with this ph but fortunately I am a fighter and with all the help I received from everyone I began the steady climb back.  Yes there are times when I may slip a little back into the hole but changing meds etc helps me then to climb back out. 

If I achieve nothing else other than encourage people to stay strong, be as positive as you can and enjoy life then I have done my very best.  Take care everyone and remember to always raise awareness.  If we can catch anyone else with this disease at the beginning instead of when all the damage is done then we will have earned our stripes!     

Carole  xxxxxx