Tuesday, 25 April 2017

Breakthrough points to CURE for debilitating heart and lung disease.

Could this be an end to my trialling

As most of you will know the last five years of my life has been spent trialling or researching one med after another in the hope to find either a cure or another medicine to help us to cope with this dreadful disease.  Please read this article though to the end, it is the one I am to be trialling either at the end of this year or early next year.  Now in clinical development it is hoped to begin its next phase of trialling on humans.  I know it sounds scary to be trialling meds, there is always the fear of the unknown but we must do this.  I am counting off the months, not wishing them away, just counting them off, to the time when I will once again trial a medicine that is hoped will be the cure.  One of them will be, so I just trial them all in certain knowledge that one will hit the mark and cure me of his terrible pah. 

So here we have a couple of links about this particular hope for a cure.  

If I have put this link up before I offer no apologies, some of the new ones will not have seen them and above all else we must offer them hope  of a future without feeling so terrible poorly all the time.  To live without hope is only half a life so I prefer to live with this hope of a cure.   Yes, yes to you grammar buffs there is a lot of "hopes" in this paragraph but that is deliberate.  Hope hope, hope, we cannot stress enough that we live in hope.  

Our conference looms ever nearer.

In the UK, as I am sure in whichever country you live, this conference is always looked forward to so much by us all.  A chance to meet old and new friends and to also catch up with all the news things opened in the world of ph.  I always believe there surely cannot be anything new I can learn as I do my best to keep up with research into ph but no, once again they will astound me and have a speaker talking about yet another aspect of ph, either with the hope of a new treatment or talking about better ways to manage it.  

When I was first diagnosed for instance we were told to be very careful of exercise, they were so worried that it would make our hearts work too hard and we could go into heart failure.  Research however has now proved otherwise,  all these aspects are talked about at our conference.  I now know that to just be scared of working our hearts too hard or being sedentary is very much the wrong thing to do.  For those in wheelchairs they have now said that some form of exercise is beneficial.  Working the muscles even whilst sitting and watching tv can be done. Move your toes into circles, move your arms, do not just be the blob in the chair, help yourself.  After all it is your body that does the suffering so help it to help itself so to speak.   

At our conference we generally have a class about wellbeing in ph.  I attended one where we were taught to exercise all our body whilst barely moving so really there is no excuse to not do something. Please, I know there is always an exception to the rule and if you are that exception then do not berate me for typing this, I am reaching out to the now over 700 people who read this blog, many of them, though not all are ph sufferers. We have a cd about exercise with ph and if you can get hold of a copy then I urge you to do so.  

Stacie Pridden

My lovely young friend is still blogging, sill fighting and is still telling us not to give in.  She is fighting to live the life she can and is very much supported by Mark, her lovely guy and her family.  Please if you send her message do not expect a reply, write her message of support but not those asking questions as she cannot spend all her precious time responding to all your questions.  Messages of encouragement though are good as there is no need for a response from her.  I have absolutely no idea if she will be at our conference this year, I have not asked her but I know if she is she will be overwhelmed with everyone trying to give her cuddles.  This in itself is such a dangerous thing for Stacie.  A simple bug could kill her as her heart and lungs struggle to cope with everyday living.  Please if she does make it, (though I somehow think she will not) do not try to give her support in this way.  

Busy week

So much to deal with and so much stress regarding the house.  As I always said Mr Sunderland has forgotten anything to do with the trees so there is no argument about this now.  I have bought a bow saw so that my daughter can every now and again as they begin to grow back again remove the height immediately so as not to have to cope with this costly experience again, and of course the stress.  We do have a chain saw but Colin will certainly not allow anyone but himself the use of this.  When we called at our land on Saturday Danielle, Chris and Izzy were all in the plot looking at our now so wonderful view.  I will get sone proper pictures to show later.  They said that as the trees were now lowered on their plot they now have a lovely view of the horizon which is lovely to see in the morning.  Hopefully at some time in the future a new owner of the house in front of theirs will reduce the height of two enormous cypress trees which will give them even more spectacular views of the hills in front of them.  

Time out

With all the stress {yes there is so much to do with the plans now needing more work before going out to tender) we decided to take a little break and we went to Hardcastle Crags.  Many people were walking by the side of the river, up and down over the crags, so very peaceful.. Some were enjoying picnics by the side of the water as it bubbled away making its way downstream.  

Hardcastle Crags was once home to a railways and a cotton mill and is so very interesting.  Right in the middle of the Crags there is a mill called Gibson Mill and this is where weaving was done over 200 years ago.  This mill has now been restored and many enjoy looking into the past as its history unfolds.

This mill was built in around 1800 and was one of the first mills of the industrial Revolution.   Though it only produced cloth for 90 years its time was not yet done,  It then began a life as an entertainment emporium with roller skating and tea dances held here until 1940.  It was then left to fall into a ruin until the National Trust acquired it and the restoration began.  

It has no connection to the outside world for energy, waste and water.  This property is the only one owned by the National Trust that is 100% renewable.  Going to the loo here is quite an experience as the sign above the toilet tells ALL, yes men included to sit to use the loo.  If you want to poo you sit at the back of the toilet, if you want to wee then you sit at the front.  When completing  what nature intended there is a big box of sawdust and you are required to throw a handful when finished!  Yet still this mill has a tea room where all water is brought in from the outside daily in large containers!   

Absolutely all of the energy is generated by water turbines and photovoltaic panels on the roof.  There is no connection to mains electricity at all.  When the water levels are low or there is not enough sunlight then of course there is no energy being stored.  This then brings into use the batteries that have stored the power from when the water was at the correct height or the sunlight was sufficient to store the power.  Of curse at times such as these the usage is monitored very carefully.  

Everything here is recycled, there are no rubbish collections and the waste from the toilets is turned over back to the land once the tiger worms have done their task.  It certainly makes us appreciate how our modern houses work with the flick of a switch.

Who can this possibly be

A picture of Izzy of course on one of her forays with her beloved grandad.  what a great time she had wearing the costumes of bygone days.  She has developed so much since attending school, coming out of her shell and making us laugh so much with her antics.  She will miss us as we go away on one of our small holidays on Thursday.  Our motor home will be loaded up tomorrow and food put in on the morning and away we go.  We are gong down to Wales and then moving into the picture postcard places in middle England.  Both of us can't wait. 

I have a bug, a tummy bug and am ending this blog now as I feel so rotten.  I intend to go and lay on the bed for as long as I like without feeling bad about being lazy and with the sick bowl by the side of me.

Take care everyone  Thank you so much for always being there for me, for bothering to read etc.   must go, bowl awaits.

Warm love

Carole xxx

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

Thursday, 6 April 2017

The saddest week for ph.

This weeks  post is very pulmonary hypertension related and it is terribly sad.  It began with the anniversary of the death of our very lovely Laura Parker.  It barely seems two minutes since I went for my hospital visit and was looking  forward to a catch up with Laura as I generally did.  Sadly this time I had missed her by a day as she had been told there was nothing more that could be done for her,  We lost her a few short weeks later as she left this life surrounded by all her loved ones, so terribly sad and she left behind a husband and young son.

I have no idea how they have been coping but I do know Laura made sure there were cards for birthdays, Christmas, anniversaries etc left behind for them.  Laura was and still is missed terribly not just by her own family and friends but by the ph community who loved her.  I know the staff of M2 where she had the best of care loved her too and will have felt her loss so much.  Awful  that we lost her, ph is a very cruel disease.

Stacie Pridden

Most of you will know Stacie, a bubbly young lady who has been fighting ph for so many years.  Stacie had a transplant a year ago that appeared to be a huge success.  We were all astounded as to how quickly she recovered and she was soon home and making up for lost time,  We saw her on television and in newspapers telling her story and attending Wimbledon ( her dream event which she finally managed to attend) and her holidays away with her guy.

Sadly we heard the news that her lungs are not doing well at all.  The doctors tried so many things to help them but the news now is that they won't recover and that the hoped for second transplant can never take place.  It isn't just a set back, a wait till you are in a better place kind of news, it is a no way for another transplant  now, you would not come out of the theatre alive.  To say we are all reeling at this news just would not cover it,  I am gutted about this BUT I also know Stacie.

Stacie is above all else a fighter.  When others might be down and out Stacie jumps up ready for the fight to continue and I know this is what she will be doing now.  She will be looking to do everything she wants to do and  she will cram into her life so much, all the while grabbing as much enjoyment as she possibly can do and making lovely memories for those that know and love her.   There is still a life to live for Stacie and I am sure you will all be wishing her well and rooting for her to make each second count. Stacie will continue to kick the ass of ph for a long while to come.

It's not always ph

This occurred quite a while ago as I had my Hickman line in,  I remember this as  if it was yesterday. It was Christmas 2010 and I had not been feeling too good at all.  Still all the family were due round for lunch and then a couple of days later we were going to meet up with friends and also one of my ph nurses down in the Village of Hope ( lovely word hope isn't it! ). Anyway the days leading up to Christmas were not good at all.  As Colin cleaned my line and filled my pump I remember sitting on a chair and silently crying,  He asked  why and I said it was so painful.  He was shocked as he was as  gentle as he could possibly be but I know pain and I knew how bad it was,  A friend called to see us and noticed I was walking all hunched over,  The reason for this was my heart did not hurt as much in this position.  I was sure my heart was giving up the battle but I was also sure I would have my last Christmas surrounded by my family.

I managed it albeit in so much pain and had my first Christmas with my new granddaughter Izzy.  I didn't know it then but times were changing so much for my daughter and she was carrying her own burdens but neither of us spoke of them, we didn't want to spoil the day. Still memories were  made of what I was sure would be my last time with all my family feeling joyful.

Two days later I cancelled my trip to Hope and we instead went to  our A & E as I was so sure I was having a heart attack.  They could not help me so spoke to my A Team at Sheffield who said they were to get me there asap.  I remember throwing up in the ambulance as I had been given morphine at my hospital for the pain.  I do not do well with opiates at all and was now dealing with the consequences.  The ambulance team were so lovely and assured me that I was not having a heart attack as I was wired up to the ecg machine and it would have shown up on it.  I was bewildered, as indeed was Colin.  What was happening to cause such an incredible degree of pain that wasn't related to my heart.  My line came out just above my heart so I was convinced it was heart related.

I know the lovely staff were shocked  by my appearance, grey, shaking, throwing up and in so much pain.  Once again all the wheels were set in motion and tests carried out.  I remember Charlie, my doctor telling me he didn't know what was going on but it was not a heart attack too.  Though I felt relief I was still in incredible pain.  I told Charlie about  my hunching over as it was the best was to ease some of the pain.  He had a light bulb moment and cane to check my chest.  He pressed on the area over my heart and I screamed.  this shocked  Charlie and Colin.  Colin said he had no idea just how much pain I had been in, that even to get washed must have been agony, it was,  Then Charlie pushed again and again I screamed out it was unbearable.  We were then told that I had a condition called muscular  skeletosis.  There are many reasons why this occur's and some do not have it too bad but the muscles, tendons  etc all around my chest wall were so badly inflamed that even the slightest movement caused pain.

Over time with pain killers this went, it sometimes comes back a little to give me a gentle hint of how bad it can get.  For me I was so relieved not to be having a heart attack.  I think sometimes we put every little thing that is wrong with us down to ph, easily done as it does affect our bodies so much and the meds are so powerful they cause problems but we do need to remember not everything has its cause in ph.

Mary, Mary, leave your tights alone!

Our local school where Izzy attends is a church school and we were invited to attend the Easter Show.  Izzy was Mary and though she was on the stage for quite a long time she did not have much to say or do.  However there was a lot of time standing central stage.  In her outfit depicting the clothes of the time she looked so cute but......... her tights must have felt uncomfortable and she kept on pulling them up on top of her clothes.  We tried to catch her eye to tell her not to do this to no avail.  We now have a lovely video of her spoiled by this continues action.  In time we will smile, she will too as she grows older but right now I am just wishing she had taken the darned tights off before going onto the stage.  The play itself was beautifully performed and the singing was outstanding.  This is a lovely school and we are so pleased she managed to get a place here.

New Admins for PAH Group

Once again we felt the need to ask for help with the admin side of the group.  We were quite overwhelmed with responses which was amazing.  We now have two new ones, Lyz Clements here in the UK and Nicole Burish who lives in the USA.  I am sure you will find them both helpful if you need to talk,  As I said before both myself and Paul are still here, we just needed a little help and we asked and we received it.  

Right, just waiting permission from Stacie and I will post this blog.  

Oh on the denervation news.  I am so thrilled to be able to tell you that though no MRI results back yet I certainly continue to feel so much better,  I hope things continue to improve.  My next MRI is in two months time.

Go well everyone, thank you for the likes and the shares.  I love comments  too!..  

Warm love to you all.

Carole xxx