Monday, 17 April 2017

I’m aware I am rare

May 5th is marked around as World PH Day.  This is going to be a month long effort around the world to try to get acknowledgment of PH and to try to get others to see the importance of improving the quality of life for people suffering with this condition.  If you see anything about Periwinkle Hearts Around the World then please read it.  We really need to get the message across about this condition we are living with.  It may help people to be diagnosed before their condition gets too bad, it may help people to understand that because we look well on the outside so much bad stuff is happening inside.  I like that the world is going to be united in this effort, particularly as everywhere we look today that are so many dreadful things going on.  
I know that I am rare, I am one in a million.  I did not choose this illness, it chose me.  I can’t walk away from it, I cant leave it behind just for a day, I can’t even, at this moment in time think there is a cure and travel across the world to take a magic pill, it has not arrived yet BUT it will and my aim is to be here when this does. 

 Being rare is not a good thing to be as unlike illnesses where there are many people suffering  from the same disease we have virtually no recognition for ours.  People still do not understand.  They can have no concept of just how cruel this disease is, how it takes away so much of our lives.  It just keeps slamming doors in our faces.  Things we could do all of a sudden we cannot, places we wish to visit are out of bounds as it can be difficult with oxygen, mobility scooters etc to access them so here we go again, another door slammed shut. 

When I am with friends I do my best to act as I used to but sadly it does not take long until the tell tale signs appear and my need to either sit down, or go to bed seems to gallop so quickly towards me I can sometimes despair. Generally though I do not let it worry me too much, I always believe that the next day will be better.  I prefer to be a glass half full person than a glass half empty.


To be quite honest I have done really well these past few weeks.  As it is a trial and research we cannot know if it will quickly go downhill as my lungs will try to repair themselves, I hope they do not in this instance.   Promises have not been made about this procedure so we will wait until the next MRI to see  but even if the worst does happen and my lungs remodel themselves back to how they were I still say it was worth it for the quality of life I have had these last few weeks.  My breathing still feels better than it was and my ability to do things has improved so long may it last.  After saying that Tuesday was a day my legs were not strong at all and I did nothing all day but hey, I used to have many more days under my duvet than I am now so result. 

Sadly number 13, or David as I now know him to be has still not had his done yet,.  There is so much that needs to come together all at once for this to take place.  The ones from Israel need to take a few days out, our specialist Alex needs to be available,  the theatre needs a long time to be booked for, the MRI, the RHC, the 6 minute walk test, the ECG need to be carried out and they must be all done just before the op to satisfy the criteria needed for it to take place as it is research.  All this does not seem to be able to get in place for David so he still waits.  He is trying to put it behind him for the time being and get on with things in his life.  Waiting is so hard, I know and mine was only put off for a very short time.  I hope things can all get sorted so that he can have his done and then live an improved life.  

Stacie Pridden

Well what can you say about Stacie.  A girl now denied her second transplant due to complications but she still manages to be encouraging of others, still is telling us all not to worry, still is determined to live her life to the full.  She is now telling us all to quit complaining about the small stuff and she is so right.  When we look around us and see others that are suffering much more than us we need to take stock of just what we can do, not what we can’t.  Life passes by, we cannot stop the sands of time so we must make the most of what we do have and not think of what we do not.  As I said earlier I am not about to think that this disease will kill me, I aim to make sure that it will not.  We could all take a leaf out of the book of life of Stacie and learn to make the most of each day, live life to the full and most of all to enjoy what we are doing.  Stacie is one hell of a woman and how proud I am that she is my friend.  As I said last week in my blog she will be around for a long time yet.  


Our plans have now gone off again to the council, this time with the building regs to be checked out and then they will go out to tender.  I want to call the house a name, Colin is not so keen but I think I will get my way on this one.  I have no ideas yet but I will keep thinking and I will get the right one in time.

Tomorrow at last the trees will come down. It should have been last week but our tree surgeon had his things stolen so it set him back a week having to go out and buy more.  Thankfully the neighbour of Danielle and Chris has agreed that the height of the ones that obscure their view can be cut.  First he said we could then he said we could not and last week when I gave him a lift to town he said she could.  I am not talking to him again before the operation has been carried out as I cant bear to think he might change his mind again!  We are ok  We do not have neighbours in front of us or to the side except for Danielle and Chris..  There are none behind us either so thank goodness ours is not a problem.  I am so looking forward to be able to see our view in full glory.  

While the cats away

Colin and Izzy are due back tomorrow after spending a few days off in the motor home.  They have done so much it has made me dizzy listening to them.  Nesting box has been built, they made the best sand sculpture of a whale {though this was immediately walked over by a girl after the judging} , she did get told off, I think she was jealous as she did not win.  Swimming was a very important part of this holiday and once again she came out tops.  She can be quite a little show off can this madam and so she was again.  She made friends with an older girl who could not swim so Izzy goes into full show off mode, jumping in, swimming under water and on her back, swimming to the bottom of the pool to pick up objects and oh just everything she can to swank.  It just makes me smile.

As I was having a good day my good friend Barbara and I decided to head off to Leeds for a bit of retail therapy.  Now the good thing about this friend is that I met her at my pulmonary exercise class so this is somebody who can relate to how I can feel from minute to minute, suffering herself from a lung problem since birth.  I was so pleased to find a super pair of trousers and a lovely shirt ready for the conference.  I was also able to bag a summer coat I had seen before but thought it was too pricy down to half price, I bagged it this time.   My friend bought a few things and then off we went to an Italian place for lunch and very nice it was too.  I had such a lovely day and am blessed that we met at our classes.  Being with somebody that you have so much in common with is truly a bonus.  

4 days later - We have our view and our walls are safe

Well the tree felling did not go quite to plan!  As the tree surgeon was actually up the tree with his chain saw the neighbour of my daughter came out saying nothing was to be cut, he would be seeing the council etc,  I was shocked and reminded him of the conversation we had had but to no avail.  Anyway as most of the work being done was to remove all of the branches etc that hung so far into my daughters garden was absolutely allowed I told the men to carry on and if any consequences I would take the blame.  I also asked the guys to lower the height of the trees, these trees could not even be seen by the guy saying no as there are even more trees, higher ones too being around 45 feet high!  

The work took two days and both I and Chris' mom were taking out cups of coffee and tea and cake etc.  The two trees on our land have been felled and the children were thrilled to see them being pulled down once all was in place.  There is so much wood we can't cope with it all so a friend who helped is taking some of it too.  A neighbour from down the road asked for some so that he can whittle little sculptures, I hope to see one eventually.  Fortunately the worry  we had about our dry stone walls falling down when the trees were felled were unfounded, they are steady and strong so that they can once again stand the test of time. 

Something a ph person really should know

There are many complications for somebody with ph and one of them is our suppressed immune system.  It brought it home to me again this weekend on the return of our granddaughter and Colin,    I noticed a rash on the back of Colin and knew immediately it was shingles,  I also know that if this is caught and treatment begun within 72 hours the condition can be stabilised quickly.  After 72 hours no anti viral meds are given.  Typically when I saw it on the day he returned the time was 9.00 in the evening and the doctor was closed until Tuesday!  I decided to ring 111 for advice and was told he should go to A & E.  Of course he rightly refused saying it was neither an accident or an emergency.  No wonder our services are overflowing with people when anything from a stubbed toe and other minor things are being sent to A & E.  On his refusal he was told they would talk to a triage nurse.  Another phone call later and after explaining my condition and susceptibility to diseases we were told a doctor would come out,  He consequently arrived at 12.30 in the morning and agreed it was indeed shingles and gave a prescription for an anti viral drug.  Were I to contact the shingles it would mean a hospital stay and my anti viral drugs would. be delivered via an I.V line, something I would rather not happen.  So here we are being careful not to make direct contact with the rash, his towels going on a very hot wash and certainly not touching mine!  Fortunately as this is all on his trunk he can wear a t shirt at all times and so hopefully I will not get shingles,  This cannot be caught by touching hands, kissing etc but by actually touching the rash when the blisters have broken I don't aim to be doing that so I should dodge this bullet at least, 

It is always best when a member of your family is poorly to remember our suppressed immune system and to take steps to try to avoid catching whatever illness they have,  This is easier said than done I know but remember what may not seem to be a problematic illness for a healthy person for us it can become serious in an instant.  The doctor that came out to see Colin said calling 111 was exactly the right thing to do given my illness.  On top of the shingles he now has a cold, first one in around three years, oh joy! 

Ph patch now delayed

Sadly the much longed for patch to deliver meds needed for patients with pah has now been postponed until 2018.  The longed for patch to take the place in certain instances of the dreaded I.V line was considered safe to use and those of us that were either using the line or heading that way have now had hopes dashed for another year. I am hoping that before that time to be trialling a drug hoped to be the cure for ph, I am always optimistic that the cure will be in my lifetime, whether I will still be of an age to enjoy good health will be another thing!

So here we are, once more playing the waiting game.  This is a game we are getting good at  playing for sure. 

Ok, another blog going out and then to get on with our day, not decided what to do with it yet but it won't be spent as a blob in the chair!  I am always aiming to make the most of each day so will talk to Colin and make our plans. 

Thank you so much for reading, liking, sharing, commentating etc,  I have been shocked and pleased to see how many are now reading my blogs.  I realised a few months ago that one ph page was not having them posted!  Horrified this was quickly rectified and now my blog is read by over 600 people, the last few over 700 so of course I am so thrilled!  

Have a lovely week everyone and I send warm hugs to you all.

Carole xxx

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