DEDICATED TO THE MEMORY OF WOLF STANLEY - OUR PH BROTHER

Wolf Stanley

As many of you will have seen Wolf Stanley is in the last stages of his ph illness.  I believe he said initialy they were stopping treatment today.  What an amazing young man he is.  He wants us all to know how loved he felt by us, how our posts encouraged him and the support meant so much.  He too in turn was supportive to many of us.  He was to write a book telling of our stores about Pulmonary Hypertension.  Some of his last words on his video to us were that he was sorry he never got to write the book.  Today as I write this he is safe in the arms of his family.  His little girl should be with him too so I pray that brings him some peace.  It goes without saying that he is a guy who will be sorely missed.  He said he was shocked at just how much love was sent his way when he posted his video.  He should not have been shocked, when we need somebody we are generally all there for each other.

Since writing that post we have sadly lost Wolf, here is a Video he wanted to share with his PH brothers and sisters.

https://www.facebook.com/wolf.stanley.833/videos/10214340497363459/

I am gutted  that I missed a video call from him.  When I got back to him 18 minutes later there was no reply.  I will always regret that missed call.

Go well now Wolf wherever your journey takes you.  You take with you so much love.  xxx

Remember to cherish the ones you love.  Wolf had been doing really well, even asked us for our stories for the book he hoped to write, and then, almost without warning he was so desperately ill.  We cannot take anything for granted with ph. 

People without ph, except those dealing with us daily have no conception that the ph and/or the meds also can destroy our organs over time, the medicines are so toxic for our bodies, causing liver damage, organ failure and many side effects.  What is the answer!  There isn't one, we have no choice but to take the meds and hope for he best, hope for another day, week, month, year.

Christmas is over for another year.    

My Christmas Eve was so nice spent with lovely friends.  Though it meant an early to bed for me I was blessed to have the time I did with all of these lovely people. 

I know that sadly we lost a ph sister on Christmas Day and that another ph patient was admitted to hospital on that day.  Ph is relentless.  It does not stop and let us have a decent day, it does not think, "oh they deal with so much throughout the year let's give them a day off from ph".  Sadly it just continues its way with us.  Many of us will have been able to enjoy the company of family and friends but will now be paying the price.  It can take us days to recover from activities or just the sheer effort of speaking to our friends for what is actually too long for us. 

I was so spoilt this Christmas and received really thoughtful gifts.  Pyjamas from Ted Baker,  they are gorgeous it seems such a shame to wear them! A lovely new wheat bag for the pain in my shoulder and neck and some beautiful soap, the best I have ever had that made me feel so decadent when using it.  Plus so much more.  I realise how very fortunate I am and that family take into account my needs when buying me gifts.  

Though my Christmas was nice it was marred by an awful event, it ruined part of Christmas day but we do what we know how to do best, we just climb out of the pit and get on with it.  What we need do  now of course is to conserve our strength for the New Year, if you are looking to celebrate it. I personally I do not since my parents both died, I will be in bed, maybe seeing the celebrations going on around Trafalger Square etc.  Wherever you are or whatever you are doing I wish you a great 2018 and I go into it with the hope of more trials and maybe a cure on the horizon.

New Trial ahead

For those of you on treprostinil, a therapy that is painful for you they are now looking at a different form of treatment.  It was in the Pulmonary Hypertension News.  It might be of interest to you though it is in fairness only at the beginning stages of trialling.  I have put the link here so that you can check it out if you have not already done so.

https://pulmonaryhypertensionnews.com/2017/12/22/pulmonary-arterial-hypertension-cam2043-phase-1-clinical-trial-underway/?utm_source=PH&utm_campaign=06f3ad89eb-RSS_MONDAY_EMAIL_CAMPAIGN&utm_medium=email&utm_term=0_fd7f2e1d35-06f3ad89eb-71490805

I am also adding a link from another of the wonderful PH News items we receive from America. This is a ph sister who shares her frustration of people thinking she is well as she looks good.  Maybe you copy  the page and show it to people who firmly believe you are not poorly at all.

https://pulmonaryhypertensionnews.com/2017/12/22/pulmonary-hypertension-physical-appearance-is-an-illusion-normal/?utm_source=PH&utm_campaign=2ac6f3d921-RSS_WEDNESDAY_EMAIL_CAMPAIGN&utm_medium=email&utm_term=0_fd7f2e1d35-2ac6f3d921-71490805

Tooth Extraction and pain injections

The went really well, better than myself or my dentist expected.  My mouth did not feel sore afterwards and all is great.  I can now eat anything I want to do again, oh joy.

My pain injections for my neck did not work at all sadly.  I rang the pain clinic today to report this and they will get back to me next week when my Doctor is back at the hospital.  Hoping I do not have to wait too long to get more and of course hope for a better result.  

Really!  But he got a result. 

We went to the sales yesterday  I looked at the kitchen appliances at John Lewis and though we are not in a position to buy anything yet the guy seemed to take a shine to me.  In fact he actually said he would lower a price cos he liked me!  I think it was a bit of  clever marketing really! However I did come away with an AEG dishwasher at a really good price. This will sit in our neighbours garage now until we are ready for it, fortunately they are family.  

As we head off into 2018, a new year ahead for us all,  quite what it will bring we cannot know.  I know my hope is for a cure, as it always is.  Cures are found for many things and I still firmly believe ours is not far away.  

Enjoy seeing in the New Year if you are able to stay awake.  Take care and take my love with you. 

Carole xxx

PS - A picture of our Park today - Winter Wonderland.

DO NOT D0 THIS WITHOUT YOUR DOCTORS APPROVAL

I read a post from a ph patient who decided to cut her meds in half.  Please do not attempt this without talking to your doctor.   There is a really good reason why.  My doctor told me  coating on the tablets are there for a very good reason.  They help the tablet to release the medicines as we need them, not in one big belt.  if you cut the tablet you are leaving a part of the medicine open to release all the meds in one big hit.  This truly could harm you. Trust your doctors.  They decide how much we need.  If they decide to reduce your meds it will be done in a controlled way and the new lower dose medicine will be delivered to you in the correct dosage. I think the person who was cutting her tablets in half was struggling to afford them. We are fortunate here in the U.K. to not have to deal with this worry..   We are fighting a huge battle as it is, don't make the problem harder by playing Russian roulette with your medicines .  I believe children are given medicines for ph crushed so not sure how that works but for me I am told not to do this. Maybe for those of us on the biggest dose of viagara it can be harmful. 

A really bad day

Yes today I am broken, I do not know why.  I woke to a "normal" day but it was not to be a good day.  I searched the computer for the latest blog I am writing to Izzy.  I knew there were quite a few pages of things we had done together in this latest one.  I appeared to have lost it and as Colin searched for it I broke into a thousand pieces.  I cried and cried.  Colin held me and said all would be well but I knew I was not just crying for the lost blog, though I hated to lose the memories we had shared.  I was being selfish and crying for me.  I try to be strong, I really do and generally I am.  I am positive and even with all this crying I am still am but the damn had broken, the tears would not stop.  

I knew I was crying for my parents and brother who have died, I so need them right now, for their strength to help me cope with pah. I was crying for my family I no longer have, for the friends that stopped being supportive when I had to keep saying no to events I used to manage easily.  I cried for all those we have lost to ph, they are never forgotten.  Oh I just cried and cried.  Ridiculous as I am generally so strong.  Something broke in losing the blog, something crashed into a thousand pieces.  The pieces will mend, of course they will and just as we can fix a special broken cup with glue I too will mend, I do know that  but right now feel I will never be quite the same again.  The cracks will show, no matter how much I try to paste over them. 

Maybe I am tired, we did have a meal out with 14 of our lovely friends last night so hopefully it is just that pushing me over the edge.  I hope so as I would hate to lose the optimism I have for the future, hate to be forever in a dark place.  So to that end I will cry it out and push it away.  Hopefully to not cry again for a long time.  Not that I am ashamed of tears.  I always say we need to use them from time to time.  Why did god give us tears if we were never to be used.    So use them  I will today and then put all of this behind me, hopefully to wake up to a new day dawning where I have all my positivity back, my faith in the future and new beginnings.

Oh and by the way, Colin found the blog and I completed it so it went in her memory box with the other 86!  Result. 

I think my husband is trying to kill me

Colin actually is a super star and does all the cooking.  I always did it until ph raised its ugly head but actually he enjoys it and does a great job.  However one thing I just can't seem to get across to him is I really should not take too much salt.  I can't see me living a life with no salt in my diet at all but we are fortunate that all our meals are home cooked.  We know exactly what goes into them unlike processed foods  which often are loaded with salt.  I am happy for him to put a little salt when cooking veg and for seasoning when cooking BUT I do NOT want salt on my meal when it is cooked and ready for eating.  However I can hear him grinding the salt and pepper over our cooked meals.  I keep telling him and telling him it will kill me.  The following day we go through the same process again!  Should I read something into this. Does he has an ulterior motive?  Anyway I think I have got the message across at last, we will see. 

Do you have one of these

If you have one of those Medic Alert bracelets like or similar to the one I have there is something you should remember to have recorded.  As we were filling in my form for any changes to my condition or meds this past year I remembered this.  People with pah really should be very wary of being given nitroglycerin if taken to the A and E Department.  Our meds already widen all our arteries and nitroglycerin does the same. The result could be an overload.  So be very careful.. We have added it to our list so that should I ever be taken poorly and my medic alert bracelet comes into play they have all the correct details, it just could save your life.  Mine says under no circumstances must I be given nitroglycerin, maybe you should think about adding it to yours.

Transplant News

Steph Ingram had a lung transplant last Saturday.  The news was that all went well.  We have not heard anything since then but we all hope it continues to go well for Steph and she can begin to enjoy a new life.

Lyz Clements - Well what a Topsy Turvey week she has had with three calls for new lungs in a week only to have them show to be of no use to her.  As she is at the top of the transplant list along with about another two I feel sure it will not be long now.  Of course our hearts go out to the ones who donated organs.  Such a hard thing to do.  Everyone who is waiting for organs I hope you hang on, I hope your time comes.  I can't say soon because that of course means another death.  Gosh how awful all of this is.  

So many lovely things

Ahead of us are many delights to look forward to.  Meals out with friends, always lots of fun, a weekend away with Izzy and two of our friends and their two grandchildren, Christmas day with the family and boxing day with family too, though this time at Danielle and Chris's place.  Pantomimes and festive fun, all on the agenda.  { I actually don't see the panto as there are too many steps to deal with but still happy times for family}  For healthy people there is just the nice thoughts of all ahead.  For those of us phighting ph we have to remember to rest, rest and rest between each and every event.    If we overdose on things it can knock us back for days at a time so remember, take it easy and get your feet up whenever you can do. 

House news

Well it is growing for sure, in fact they are beginning to put in the earth wool that is used to insulate the house.  As it is an eco house we of course want to use eco friendly insulation and this is one of them.. Sadly as the weather changes so will the time being spent on our house become less and less.  The builders take two weeks off at Christmas and the New Year so again all things stop.  The hoped for roof on before the end of November won't even happen before the end of December but at least we can see progress each and every time we visit the site.   Cant wait to be in and getting sorted.  Seems such a long way off yet though.

Emphasis Magazine

I received mine yesterday and I just need to say a heads up to all who contributed to this wonderful magazine and to the ones who made it possible.  We always find so many things of interest.  We have articles from our ph family and we always have the latest updates on what is happening with some of our medical professionals.  It is always put together so beautifully and is so looked forward to each and every time a new one is due out.  Well done for everyone connected with it in any way.

I wrote this blog a few days ago. fortunately I am feeling much more upbeat, don't know quite what happened to push me so low.  We are going to see our Izzy in her play this afternoon.  Looking forward to seeing her with a beard!

Go well everyone.  It is so lovely when you take the time to comment or click like, it really is.  Sometimes writing a blog is hard but when I see the response it makes it all so worthwhile. It is a great way to stay connected to you all and that is so important to me.

Oh just an add on.  The Porter Crest of which I had asked for from the family to complete my family tree has turned up and it was left outside my door for me.  I can't tell you how pleased I am to complete the project I worked so very hard on.

Right, so now my day begins.  Hope yours all go well and that in the lead up to the festivities you remember to rest.

Warm love

Carole xxx

  

DIDN"T WE DO WELL TEAM PH!!!!

PH Brothers and Sisters Unite

Many thanks to all of those that contributed to the raising awareness of ph campaign going on right now.  Each time I open up my computer I see posts, blogs, awareness posters etc about our fight to get this seen as the terrible rare illness ph is.    Worldwide they just keep flooding in.  How amazing and it proves we work so well as a team.  Together we went into the month of November with a determination to try to raise awareness for this little known disease and I think we smashed it.  I am so proud to belong to such a group of amazing, resilient people with such a phighting spirit.  

Please ignore the videos and clippings below most of you as I know you have seen my bit of raising awareness. I am putting them in for those that didn't.  I was very fortunate to have been asked by the PHA Association to do my bit and Mary Ferguson was assigned to the case!  She came armed with her video and her writing pad, plus her recorder.  How easy it all was.  It was just like talking to a really good friend, somebody I had known for a long time.  She made it all so easy to talk about ph and afterwards did a really good job of editing it. 

https://youtu.be/37ujQPEmhQo

https://youtu.be/wvizOC0sB4c

Link from my Friend Tess: -

https://l.facebook.com/l.php?u=https%3A%2F%2Fwww.youtube.com%2Fembed%2F-1RvPZhf24I&h=ATN0TwDtq-vfDdAuq91kkcaQYPo1uWXVZQbU7jotTm1AJxFoC00fTy2kvGHqESnGQM_nBJ8y0-U76y6YaVr02kObHGl9b8nmI35zXKZh1K7ZXJS4j5cvWvn6lsssZgDX8rYYE_uujNvQ_Id1

Izzy was thrilled to be in the Courier and the Yorkshire Post, plus Brighouse Echo.  In fact she felt quite the celebrity when she went to school and many of her fellow school mates kept telling her she had been in the paper. 

https://www.halifaxcourier.co.uk/news/living-with-disease-that-leaves-halifax-grandmother-carole-struggling-to-breathe-1-8860762

Her form teacher asked her if she knew she was too.  She came to be in the picture with me as Mary, quite rightly said it was evident that Izzy played a huge part in m determination to push back ph, to keep going for her sake.  Mary was so right, I make goals and milestones to reach with her and then keep pushing back these goal posts, moving ever forward in life with her.

I was pleased though that my illness had been picked up by the school as on the days I just do not want to drive to school and for whatever reason I am taking grandads place in collecting her my mobility scooter comes into play.  I did wonder if people in the school playground just thought 'i was using it as a fun thing for Izzy to ride home on!  Now hopefully they can see that this piece of kit is an essential part of my life.  Though I love it when I do not see it for weeks I also love the fact it sits there, a sturdy, trusty steed to take me where I need to go on the days it is needed. 

Where will I be headed next

It wont be long until my first year post denervation is up and I will be free to begin another trial.  I know that the one I hoped to trial is not yet ready so just what will I do next?  I am looking forward to talking to my specialists and discussing what is in the pipeline.  It may well be that for a few months I just cruise along with my ph until the one I really have been hoping to trial comes to fruition or it may be that I meet the criteria for a different one.  We will see but you can be sure I am ready and waiting to see where my next journey with ph takes me. 

Just love this place

A couple of weeks ago I was at my usual haunt, between the river and the canal.  This tiny village  is called Copley. about half a mile from my home. This is a beautiful place, cottages that look tiny outside but are deceptive and prove to be large inside.  Lovely neat gardens.  The houses there today were built in 1847 when the Akroyd family moved their worsted mill to the site and the model village was built all around it.  The mill is long gone but the cottages remain and outside have not changed their appearance since the day they were built.  

Lying in the valley this place has so much history.  The first Copley Hall was erected as early as 1050 and then was rebuilt by Sir Henry Savile in 1421.  

I know some of you love a good ghost story so look up on google Ghosts and Legends of the Lower Calder Valley, or just look up the history of Copley, Halifax.  it really is fascinating. 

Anyway for two years I had been unable to get across the river due to it being swept away in the floods.  At last it has been reconstructed and I was able to go over it and take pictures of this tiny toll house at the side of the river.  I  was so pleased to see it had remained intact.  I love the sign that still exists about the cost of taking over the bridge sheep, cows etc .  These animals would have crossed the river and clambered up the steep bank to the top of the valley and would then have been free to roam on Norland Moor. { I think the best bilberries ever are to be found on these moors.  I myself spent many hours with my family picking these tiny berries and taking them home to our mom who I knew would turn these delightful fruits into the nicest pies ever. } So I managed to get a picture of the toll house and thought some of you in America would like to see it as I know well how you love history.  

The sad demise of Hammy

Hammy is, or was the hamster of Izzy.  A much loved animal she was treated to a palatial home and many toy and treats.  Nobody could have taken better care of her and she lived a little longer than the expected two years.  We all knew the end could not be far off as the deterioration in her was plain to see.  She was losing her fur and she walked very slowly and, it seemed painfully.  It got to the point where she could not walk to her water and so my daughter put the water right in front of her.  Izzy watched all of this with dread.  She loved her Hammy and was dreading the day she would no longer be part of this world.  We had spoken to Izzy on the telephone the evening before and asked her how Hammy was.  Her answer was that she was clearly getting worse but that she was "all right"  The following day was a different matter.

She turned up here as we were having her for the day with tears in her eyes.  Her mommy had just told her that sadly Hammy had died in the night.  The tears were very real for a beloved pet.  We sat around the table and discussed what was the last thing we could do for Hammy and of course the answer was a funeral.  We talked to Izzy about the inevitable fact that living creatures all die.  So with respect we drew pictures of Hammy living her happy life rolling around the floor in her ball and crawling through her tunnels in her huge cage.  She wrote to her about the pleasure she had every time she saw her and then the last thing  needed was to make a cross ready for her burial today.  I found the perfect twig, long and straight and grandad washed it and broke it in the appropriate place so he had two sicks to fashion into a cross.  Out came the twine and a super cross was formed.  Now all it takes is something that will disintegrate when in the ground quickly to wrap her in and a few soft words spoken over the site where she will be laid to rest later today.    

Its a lesson learnt.  It hurts when somebody or some creature we love dies, how well we know this but at 7 this is the first time she has really experienced a beloved pet leaving this life.  I think between us all we dealt with this pain very well and I hope it helps her in the future when she suffers other inevitable losses.  

Horrible

I had a dream.  We all dream don't we.  I generally remember mine, I always have done even as a small child. in this dream I know I was trying hard to breath, I was looking at a pictures with somebody, I think it was my mom and I just couldn't get my breathe.  Struggling hard I woke up and for just a split second thought "gosh what a horrible dream".  It was then I realised the reality, that my dream was in actual fact my living nightmare.  I DO struggle with my breathing and it is in fact getting harder some days.  I woke in the middle of the night to terrible pains in my right lung, scary.  Other days I don't notice it so much.  It brought back a memory of my dad who woke to a dream in which my elder brother died.  For a split second my dad thought what a horrible dream he had then realised it had in fact been born out of reality as we had lost our brother.  

I know I am pretty fortunate that right now I do not take any supplemental oxygen except for when I am flying but I also realise that my day will come.  Right now I am getting more short of breath very easily.  I do then test my oxygen levels and they are ok, my heart rate rises, as does everyones but it then drops down as it should so it puzzles me.  I tire more easily, Colin sees this so easily in my face and I am afraid my bed calls me so much more than it used to do.  Once I rest on the bed though I  do  not sleep, in fact I sleep for only a short period of time in the night too. So quite what is happening,  just  what is going on inside my body I do not know.  Time will tell, we shall see.  In the meantime I try to continue to be as busy as I can be within my limits.  I refuse to give in to this illness though I do pay the price for being so determined sometimes, as indeed we all do.  

The last two weeks have been taken up with hospital appointments for bloods, physiotherapy for my ankle and the dentist.  Though my dentist and I were hoping to save one of my teeth it has sadly cracked and it needs to come out.  I hate the thought of this.  No I am not bothered about pain etc but just the though of losing a tooth.  All because I bit on some uncooked rice when on holiday in Scotland!  The consequences of that has led  me to an appointment on the 18th December to pull the said tooth.  Three appointments I have had to try to sort this out.  Antibiotics were put into the root of the tooth to try to stop an infecion, this worked but sadly the tooth just kept cracking more and more until we have reached the end.  I can't have it capped due to it being cracked so I have to have it removed and a false one on a plate!  How I hate the thought and for a few days, well only three I will be without a tooth and no plate.  I will not be venturing far from home then I can assure you. 

House news

Despite the weather it is certainly growing!  We now have clear indications of each room and it grows daily.  The dry stone wall we had straightened looks really good, I am so pleased with the end result though disappointed in the guy that did it.  He worked hard and diligently until the final day when he rang and asked to be collected to go home.  We were rather shocked to see that for half the wall he had not put back the coping stones, the vital part that holds the wall together!  He said we needed to buy more though it was obvious to Colin that there were some scattered around the detritus he left behind. A good workman clears up after himself but Mick certainly did not.  however we let him go and Colin went to work.  Moving and changing the positions of the coping stones on the top of the wall and putting them in different positions he figured out how to get the best result.   He picked up the discarded ones and placed them on the top of the wall.  The result I have to put on here as I am so proud of how he completed it.  We have so much of this wall, in fact it surrounds ours and our daughters house.  Fortunately all the rest is now and straight so this is a job that will never need to be done again for hundreds of years.  

Joining the 21st century

As we begin from scratch with our house we have decided to go with all the new gadgets that are around now to see who is at your door, to talk to them even when we are miles away etc.  To that end we have also decided to buy Alexi so that it will make it easier for me to play music throughout the house just by a simple voice to Alexi.  I love music but struggle to find the piece I want to play so how wonderful at the sound of my voice and an instruction given she will heed my command and play exactly what I want to hear when  want to hear it.  I will tell her to turn off the lights etc or turn them on at will.  I am quite excited to be joining in with the youngsters with these new gadgets.  Cannot wait to try them.

Well another day begins and I must get washed and dressed and get on with it!  I hope you are all doing ok but remember there is a big celebration of Christmas coming up so keep some energy in reserve.

Warm love to you all and again many many thanks for the comments, the likes and the shares.  It makes my day to see you have read my simple blog from wherever you are in the world.  

Carole xxx

HUMPTY DUMPTY Get off the fence NOW if you are able.

Talking to my consultant the other day he has reassured me that there has never been such a rich pipeline of drugs to be trialled.  We often see that people believe there is not much work going into finding a cure and this is so very wrong.  As I have stated before though we need participants in this, they can't work without us.  

How would you feel about trialling if you did not need to go through the endless rounds of right heart caths, MRI's etc.  Would it make you feel more inclined to trial drugs?  Well I know that there is a germ of an idea being worked on that may help to eliminate them so just keep that in mind and just maybe we can get more people onto these trials.  It could well be that you have told your ph specialist that you have no interest in trialling BEFORE but are now coming around to the idea.  If everyone able to trial registered their wish with their specialist the list of participants would grow and grow.  Just think about it as you swallow your next meds.  

I know I take a tablet every day.  A very expensive one called Ambrisenten,  This bright reddish oval shaped teeny tiny thing is helping me to still be here.  My friend Mandy Leonard was one of only six people that trialled this drug in the UK.  Not sure of elsewhere in the world but what a brave thing to do.  Many of us now are taking this medicine daily as part of our drug regime.  I thank you Mandy as it certainly got me off the hickman line and onto the oral meds I have been taking for the last six years. Many on this site will be aware of this particular drug and we take it thanks to Mandy Leonard and the others who took the bull by the horns for us and did their bit in the developing of this drug for ph. 

I also take viagara, huge doses and I know somebody within the group trialled it.  Sadly I did not take the name so if you see this post please give me a heads up.  Many of us are dependant on this particular drug or its generic make.  I know I would not be here without either of the above drugs so many thanks for trialling them for us.

James A Hazlett is on a trial which appears to be working so well for him  This particular trial is called Reata's "Lariat" trial for Bardoxolone Methyl.   In December he will transition to the longer range portion of the same drug/dosage (10mg) titled "Ranger"  He has not experienced any side effects from this drug but his RHC went down from 26mm to an amazing 17mm because of trial med and 10 mgs of opsumit.  His six minute walk test rose from 500 metres to 600 metres.  No change in shortage of breath.  

So we are moving forward, steadily steadily trial by trial to the day when we can hopefully eliminate the need for all these meds.  

Some friends you would be better off without.

I was so sorry to read about my lovely friend Tess who heard her friends had been talking about her behind her back, thinking she was not nearly so poorly as she told them.  Yes she was so hurt and angry and posted that it took time and effort to get dressed, slap on the make up and go out to be with them.  They had no idea of the impact this had on her body, of the crying she does at home in the quiet of her own space.  Why, well as we said before we look good dead!  Helen Akers had it right when she said we live too long!  Though she does not wish for us to die of course, she herself suffers from ph, I get exactly what she meant.  If we are diagnosed with an illness that we are told will kill us then yes, for sure friends rally around.  It doesn't take too much time though for us to suss out the ones who will stay when the going gets tough and those that will fall along the wayside because this illness is  "going on too long to be as bad as we say surely!"  

Let me assure you that this illness is as bad as it can get, that it is devastating to us but we have learned to fight it, we have learned to try to live as normal a life as we can to the outside world, at home is where it can all fall apart.  Personally I have learnt to take no prisoners  I can't be bothered with a "friend" I let go but it  still makes me smile today as to how it came about.   On the death of my mother she did the right thing"and called at my door with a bunch of carnations.  She said she had not got the time to come in as she had a very important appointment.  I was just so pleased at her thoughtfulness until  found out later in the day that the very  important appointment was a cup of tea over the road with another friend!"  So people like this I prefer to let  go and concentrate on the real friends we have, the ones we know will help us when we call, as indeed we have helped them in the past. Life is too short to cry over the ones that are not real friends Tess, concentrate on the ones who are always there for you and like HUMPTY DUMPTY who is sitting on the wall give your "not so real friends" a big kick in the pants off that darned wall!!!!!!! let them fall, you are better off without them.  

To the ones whose family and friends have stayed faithful and true then I congratulate you on having a really good supportive network, long may it continue, I am sure it is well deserved.  

For myself my "real" friends are still my friends, it is some of the ones I inherited when I met my husband that have proved to be fair weather ones so it is very easy to let go.  Some of these inherited ones though have turned out to be good true ones and I am forever grateful to you, you know who you are an the help you have given me.  

 Last weekend we enjoyed a lovely afternoon with friends at one of our local restaurants to celebrate an 80th birthday!  I think I did too much talking around our particular table and paid the price when we left but it was a small price to pay for spending time with such wonderful people in such beautiful surroundings.  So yes, we push ourselves to enjoy life but we are always aware of the consequences.  If you think the pleasure you will get for pushing yourself hard is worth the effort then go for it, if not just say no and forget about it.  

Other news for my family and friends.

As this blog is not just dedicated to ph I make no apologies that I post what is happening in my daily life of living with ph.  Well I can happily tell you the Izzy has learned to ride her bike!!!!!!  I never thought I would live to see the day but grandad worked with her in the park then invited me up to see the progress.  I was delighted to see her riding her bike, ok it was wibbly wobbly but now she will grow in confidence of course.  I was so pleased I actually bought her an LOL.  For those uninitiated it is a tiny doll that comes with accessories all fitted into a ball.  A huge money maker for the ones that created this doll but a big pleasure to see her open it.  Once opened she decided she wanted her grandad to video her playing with it and then put it on utube!  All went well for a while, she is a great narrater and not shy of the camera.  She then decided she needed the spotlight moving a little higher so indicated this to grandad.  To our horror the spotlight actually then fell onto the hair of a Barbie Doll that was to be part of this little production and burned it off!!!!!  There was much hilarity but it does show the danger of certain dolls if not being taken care of in the correct way.  Anyway the video was abandoned and then they moved on to making a safari scene in a shoe box, way to go!!!!!    

PH Awareness month

As most of you know this is a special month in raising awareness for invisible illnesses.  So it was with a happy heart I participated in an interview to talk about ph, research, awareness etc that I told you about in my last blog, and then this was followed up with a video.  I am waiting to hear when these will be seen but I don't think it can be too far ahead.  There is so much going on right now it is so hard to keep up!  An article will be appearing in the Yorkshire Post at some time so again not sure when.  Mary who took the interview asked if she could get a picture of Izzy and I together as she is a huge part in my fighting ph, I need to make many memories.  Whether this particular picture will be included who knows but once again  I feel I have done my bit towards raising awareness of ph and the need for all who can to get on board the trialling train. 

Shocked at my pain clinic appointment

As I went for my injections into my neck I was pleased to be one of the "early doors" patients as I always prefer to get things done in the morning.   All went well until the nurse took my form with my medicines and logged them into the computer.  She came back into the room and told me I would have to be last on the mornings list.  When  asked why she handed me a paper that she had taken from the file.  Now this paperwork I had never seen before, never been told about so I was horrified to learn that I am now insensitive to most antibiotics.  Terrible news for a patient with ph to hear, particularly as this sentence was directly under a line that said I was in chronic heart failure.  We know a simple cold can turn in an instant and kill us so I was  shocked at this news.  This meant that I had to be last on the list and that the operating room, all the equipment used etc had to be deep cleaned before use after lunch.  It clearly states on my form with a big red triangle that says confidential ISOLATE is an ESBL producer.  So just another thing to add to my list of problems!!  Ah well, we do what we do best and just get on with it.

House News

Now this is moving on a storm, particularly as my family next door have allowed us to use their electricity so the builders can make a cup of tea!!!!     Electric is due to be connected in four weeks time,  For now very little is used.  The big usage is when the house is built and the joiners are in doing all their work, quite a long way down the line for that so we will have our own supply way before then.  The water is used in small amounts to mix the concrete, this is a petrol concrete mixer so no electricity used for this.  I am pleased we don't need to use their supply so much but will still feel better when we have all our own.  The walls are beginning to go up and we see progress daily which is so lovely at last. 

Looking inwards, being reflective

Do you ever have days or hours when you do not want to talk to anyone, you just needlessly watch rubbish tv to take you mind off things.  Well that was my day yesterday.  Colin had lots of work to do organising bathrooms etc but it just went over my head.  I wrongly thought in my head we had already decided on the tiles for the bathroom.  I was wrong of course so this led to a bit of an atmosphere as I "never listen".  I do of course but my brain seems to be mush from time to time and I try to make myself out of the world of ph and put my brain into low gear.  I need to get back on track I know but I still think it was the shock of the recent report from the hospital, my local one not my ph one.  

So I leave you with another blog, I hope there is something of interest in it for you to read.  My last blog saw me receive 1027 reads so far which is amazing for little old me sitting here in the study trying to connect with you all.  I thank you from the bottom of my heart for any comment or a like or share.  

Warm love to all

Carole xxx

ANGRY, ANGRY, ANGRY

DCA Trial

This was the first trial I went on.  I read about it on google when i was so very poorly and spent most of my time in bed.  I was on the hickman line and we all believed death was knocking at my door.  I saw a research facility in Atlanta were going to trial a drug already in use but now with the hope of a cure for us.  Indeed this DCA did cure rats of pah.  I knew I was not in a position to trial it, I barely knew anything about ph at all and had no clue how to get onto the trialling part anyway.  Like a bug on an apple that worms its way into the fruit so did this idea worm its way into my brain.   How could I begin to trial, who would I speak to.  It would be a year before I found my way into the trial and research programmes.  A year it took to get off the line and onto oral medication.  Then it took six months to prove stability in my pah.

Here is a link outlining in depth findings of DCA trial results: - 

https://www.google.co.uk/url?sa=t&rct=j&q=&esrc=s&source=web&cd=1&ved=0ahUKEwi7l5a1j5PXAhUNb1AKHb9rDdgQFggoMAA&url=https%3A%2F%2Fmedicalxpress.com%2Fnews%2F2017-10-mitochondria-pulmonary-hypertension.html&usg=AOvVaw2xgycuvR3dvYe4bYUH91He

A throw away remark to my specialist about how I would have loved to trial DCA led me to begin my journey of trialling medicines for pah.  My specialist spoke to Martin Wilkins at the Imperial College and asked if I could join in the study.  I was thrilled to be accepted as there were only 20 worldwide that participated in this particular one.  At the end of the trial, Martin said he would have paid money out of his own pocket for all the help  my blood was doing in research labs worldwide to find a cure for pah.  Yes there were complications for me as I was given the higher dose, I developed neuropathy really badly and very shaky hands but I still wanted to continue when these side effects hit me.  My own specialist however took me off the trial so I quickly got in touch with Martin and begged him to ask my specialists to let me continue.  The result was that I did, though on a lower dose.  Though  I now take gabaphentin as a result of this trial I am still pleased I did it.  

The joy Colin and I had when we were sat in my specialists office, talking about denervation, and the email came up that informed David Kiely that DCA has proven to be another tablet to add onto the ones we already have in the world of ph.  The three of us were thrilled and though I wrote about it in my blog I said I could not tell you the name of the drug then as it was not my place to say.  The jubilation then when I read the posts about DCA and its proven benefits to us with pah were so lovely.

It is no secret then that since then my life has been all about trialling, one new drug follows another in my world.  I have my own trialling passport as it would be easy to forget which one we have been on!   

In view of this I feel so hurt and ANGRY when I read posts that say I am forever "banging on about a cure" or that the cure will not be here for a long time yet as there is not enough research being done. I then click the like button to see the ones who actually agree.  All these people are on meds that were TRIALLED and RESEARCHED for PAH.  If I, and indeed all those that trial for pah were to believe that we would stop trialling.  Why put our bodies through so much when the cure is so far away in the future.  Well let me tell you if we all believed this, if we all took away our bodies and said no then research would flatline.  For every drug keeping us alive today people have been happy to trial.  So to the ones sitting there in their chairs spouting this I have to say be thankful for the ones that trialled the medicines you pump into your bodies today.  Without their belief and the belief of the researchers you would not be having that medicine, yes the very ones that have kept you alive.  It is a good job they did not all take their bat and ball home and think "well they are so far away from a cure why would we put our bodies into research."  Indeed some of the medicines today came about in the belief that it would be the cure.  Further testings showed that though it wasn't it did actually help our pah.   So though you THINK you know more about pah than the specialists and researchers believe me you do not.  Yes the answer may not come in my time but I will still put my body out there to be used to try to get a cure for US ALL, yes even you doubters.  I will also continue to think positive thoughts, not negative ones and dismiss your comments as absolute garbage.  I also hope that the next time you take your drugs you actually give thanks to the ones that have made them possible, without them you would be dead.  

I make no apologies for my rant, it makes my blood boil when I see such posts and negativity.

House news

So what I have been up to these last three weeks.  Well it is fair to say I have been very busy and so I have had to push myself harder than I should. I paid the price though with three duvet days, not consecutive fortunately, I did get a break between each one.  This was great as now we really must push forward with sorting things for the house.

The concrete has been poured for the foundations so I am excited at that. Tim, our builder has put a ton of men on this job as he really wants to get our house done as soon as possible,  He knows about my condition and wants me next door to my daughter and her family as soon as possible.

I had a “do” whilst looking at doors.  I am never at my best in the afternoons so generally get done what  needs to be done in the morning, This was mid afternoon  and I had barely looked at the doors when I knew - I felt the room spin and a sickness and hurting in my belly.  I told Colin we needed to go whist at the same time  sitting on a pile of wood.  When I go “off” I sit, I don’t care where it is.  I have sat on a wet pavement in the rain before today.  I can’t bear to think of the damage done if I faint in hard places so despite the looks I sit.  Colin helped me back to the car and home to the safety of my bed.  My temerarure  dropped initially to 33.4 and then fluctuated up to 35.7 so I decided to get in touch with Alex, the specialist dealing with me since  my denervation.  He said it was bizarre and to keep an eye on it. Anyway the following day was a duvet day and I have come out of it fortunately.

I always try to think if I am feeling down and really poorly with my ph that tomorrow will hopefully be a better day and it was.  So off we went to choose tiles.  This is a tough job when we have not chosen kitchen units and cloakroom and bathroom etc but after a lot of thought I think we have found our perfect one.  When laid they will look like wood, the pattern is super with the wood knots in etc.  We did not want wood as we are having under floor heating and we know that porcelain holds the heat  really well and needs no maintenance apart from cleaning .  As our house is going to be an eco house we feel we got the best of all options available.

We think we have found the cloakroom furniture, this wasn’t too hard but our own en suite is proving to be rather difficult.  There are so many lovely options out now and we keep changing our minds as to which to go for,  We can’t leave it much longer as the builders need to know where to put pipes and outlets etc.  I am not worried about the bathroom upstairs, we have a little longer to deal with this one fortunately but we really need to shift on ours. 

We are seeing a kitchen fitter this weekend, a family friend, and see what he comes up with.  Our local kitchen showroom place had no idea of design and just threw down on a bench pieces of paper.  With kitchens you need to see the whole affect at once, not piecemeal so we have sacked him. So on we go, much to do so I am hoping my ph behaves itself for a time at least.

Plants to help us

I was reading about 3 good plants to help us in the bedroom as they take away pollution in the air and aid sleep.  Aloe Vera, English Ivy and Madagascan Areca palm.  I have not had a chance to buy one yet but I certainly will do and see how I go on.  

National Invisible Awareness month 

This is for most of November so if you have your chance to make people more aware of these invisible illnesses please do so.  I have been asked to do something about research {won't know quite what until next Tuesday} and to talk about PAH on our regional news channel, Calendar,  so that should hopefully go some way to bringing awareness.  

One thing that made me smile in all of this ph business was my local GP.  He asked if people thought I was doing ok as I looked so well.  When I responded yes, some of them  even think  I am putting on being poorly he chuckled and said I should tell them the joke about how we people wth PH are the healthiest looking corpses in the morgue!  I have always said  that was the only upside of our disease, we look good dead!  The reason we look so well is we retain quite a lot of carbon monoxide as our lungs do not expel it all.  Carbon monoxide causes us to have a healthy glow.  So what in fact is making us look good is in fact doing us harm inside.  

Izzy

She is doing so well with her piano lessons and enjoying them  She even got an award last week as she could play a tune, albeit small with two hands at the same time.  I am so proud of her.  She still continues to be a wimp on her bike though.  We may be getting a breakthrough as she did ride around 20 metres this week.  Slowly slowly we just might beat her fear one day.  Her swimming though continues to amaze me, her stroke is lovely and she can do everything asked of her by her swimming instructor, often getting praise.  I am so pleased she is a capable swimmer now.  Though we could stop her lessons we see no need to.  As in anything just because you can do something doesn't mean we stop doing it, there is always room for improvement.  

It is school holidays here next week so there will be busy times ahead.  I also need to see my dentist as I have an abscess and as I have had to come off warfarin for a few days it is the perfect time to see him.  My pain injections will be given to me on Wednesday morning so yet another thing to be grateful for. I am starting phsio on my ankle as the specialist said I have damaged my ligament badly so as always busy busy busy with all these hospital things on the horizon.

Well I have been wittering on long enough for one blog I think.  Sorry if you disagree with my comments re the trials etc but there it is, I have my own personal dislike of people saying we are way off a cure which in my book means they are knocking the trials and the research which in turn belittles all of us out there trialling.

So, all being well its out for lunch now and then onto my daughters to discuss kitchens with their friend who just happens to be a kitchen designer.   I hope we get a good result. 

Warm love to all and again a huge thank you for clicking like and commenting on this blog.

Carole xxx 

From "One Bloody Mess" to "One Muddy Mess'

Well it has been a busy week here with one thing and another.  First of all we have had Izzy spending her days with us as she had a bout of croup.  Silly me did not realise we can catch it so blithely said "of course" when asked if she could come here instead of school.  It was only when it was too late to bother that we discovered that of course we can catch it too.  As the days have passed it seems I have been spared fortunately as I do not think I would have fared well had I come down with this just after my chest infection.  Izzy is well now and back at school, suffering no long term worries after this horrendous illness and I am happy to report neither of us came down with this so all is well.  

As I wrote in a post I have had not one really bad nose bleeds but two, both resulting in the bed having to be stripped right down to the mattress.  How lovely it was then to receive a private message from a ph brother who suggested that I always kept some Tampax ready for such an emergency.  Of course, why didn't I think of this as before whilst in hospital I did have a similar thing inserted in my nose to stop such a bad bleed.  These items are  in fact even handed out to soldiers to use in times of being shot as they will plug a hole to a degree.  Thank you Joseph, I have now added some to my bedside drawer.  Though they can't stop a nose bleed they can save our bedroom looking as if carnage has occurred.

Here is a video of my friend Sarah Marshall as ever raising the awareness for pulmonary hypertension and a really good job she did of it too.

https://www.facebook.com/sarah.marshall.56/videos/10154638778911511/

Her hubby Phil does so much to raise money for our small charity too so this young couple deserve our thanks very much.  A follow on piece on the same tape comes from our own head of the PH Association Iain Armstrong who I really like and hold him in high regard.  Not so much this time though as he went on to say that nowadays with the new meds and better understanding of pulmonary hypertension we could in fact live for eight years after diagnosis.  As I am coming up for my 8th year it was hardly reassuring to either myself or the many others in this position.  I just wish he had chosen his words a little more carefully.  Nevertheless his pride in the ph association as a whole is so obvious to hear and so rightly so.  It is an amazing organisation and we all owe so much to it for all the things they do for us and all the info for us just a phone call away.  Well done Iain.

We went down to our land to visit with our builder who has all the land pegged out now ready for the build to begin.  Talk about a quagmire, there was so much mud even my daughter has stopped foraging on it for the dandelions that fed her rabbits a large part of their diets.   (Of course as these dandelions are dying off I don't feel so guilty we are at last getting our house built at last.}  The digger is going in next week and so it will begin in earnest.  I am so excited that at last we will begin to see this longed for house begin to take shape.  Lets hope the weather is not too bad until the roof is on.  Time will tell.

Above is a picture of just a small muddy section of it that has been dug out ready for the cabin they intend to put on the site.  

We both decided we could do with some time out for good behaviour from our routine everyday stuff so off we went to Liverpool for a coupe of nights.  The object was threefold.  We knew there was an amazing music shop and we needed to discuss certain things that Izzy needed to complement her new piano so she gets the best out of her lessons.  What an amazing shop it was and the gentleman we spoke at length to was so very knowledgable on all aspects of music ( as we would only have expected) that we came away feeling so pleased we had made the journey.  Whilst I was there I also purchased a ukulele for my son's girl Anna as I know she does play a few instruments,  I hope she likes it and gets a lot of enjoyment out of it.

The second reason was to buy some new outfits.  I hate shopping for me and so does my hubby, he hates clothes shopping too but needs must.  Off we went.  He was quite easy to deal with and we were both delighted with his choices.  I on the other hand was getting despondent and also very tired as I tried on outfits.  My legs were getting wobbly and my face was so very red and I was so hot.  Just as I declared NO MORE we hit upon two pairs of trousers I really loved and so quickly following on from that we added four blouses to the pile.  A new pair of boots completed the ensembles and so we could at last make our way back to the hotel for a well deserved rest.

The third reason was something we had meant to do for a long time and never got around to it.  As you know we are members of the National Trust,  an amazing trust that does such a wonderful job taking care of our heritage, all the old houses, castles lands etc.  We must preserve our heritage at all costs so we have had annual subscriptions since we were both quite young.  We had said many times that we really must visit Speke Hall. This hall is not too far from us so quite why we never got around to visiting it is beyond me.  Built in around 1530 on the site of an old farmhouse this magnificent Manor House is such a pleasure to walk around.  With a fascinating history we were blessed to be shown around by our guide Vera who brought the house and previous occupants alive.

We were captivated as she told us of each and every generation, the good, the bad and the downright awful.  The last occupant Abigail sounded to be delightful in that she took so much care of the surrounding schools, hospitals and homeless etc that I wished I could have shook her hand.  A fair but strict employer she was one that could be talked to and reasoned with.  A very astute lady she was not for letting go all her worldly goods as happened in those days when a woman married.  No way would she hand over all her chattels so she remained unmarried.  Quite a lady before her time though as when a servant got pregnant before wedlock  she allowed them both to marry and stay in her employment.  Not too many would have done that in those days.

Not the same for all the previous occupants who would not have dealt so kindly.  I was pleased though to see that one of its former owners,  a Richard 1V actually married his servant girl and though  his father would not have been too happy they did continue to live in the house until their deaths.

Reputed to be one of the most haunted houses in the UK it has appeared on quite a few television shows and is said to be haunted by different ghostly figures. One of these being the real wife of one of its occupants.  On being told that they were ruined as he had gambled his money away she was reputed to have thrown their infant child out of the window into the moat to meets its grisly death and she threw herself in after said child.  Not a happy ending at all.

We have been incredibly busy, what with the house, Izzy bathrooms and kitchen hunting, hospital visits and much more.  I actually love weeks like this as being the blob in the chair is never my idea of a good time.  Today we had a super visit from our friends that live in Canada.  Friends of over 30 years we quickly picked up from where we left off and there is never an award moment between any of us.  Sadly the visit was not a long one due to commitments on either side but they have promised to come and stay with us when our new house is built.  

As the build is upon us we also decided to get in a dry stone waller to straighten up one of our walls that has, over the course of the hundreds of years got bowed.  Though it will likely stand the test of time of many more years we decided we would get a better edge to our garden if we did this job now.  Living in a county where lots of our walls in the fields and surrounding district are dry stone we were fortunate to find a man who comes highly recommended.  He has said he will do the wall, though not this week.  How fortunate that he can get on with this swiftly due to being retired.  I can't wait to see him in action.

My denervation appointment is due on the 12th of this month, Thursday.  Despite my asking it seems that for the very first time EVER there are none of my ph friends going to be either as a resident or for an appointment.  I needed to ring the hospital today and was alarmed as I could not get a call through, all the lines were blank!  anyway hours later is was resolved so the taxi will arrive around 9.00 and testing here we go.  I shall post how  get on with this, though there is no right heart cath this time or MRI, just the usual ecg, bloods walk test etc.  

So all is well here, though busy.  I trust this finds you all as well as can be.

Much love to you all and again thank you so much for just clicking the like button, comments are super too.

Carole xxx

How can they fail to respond!

Gosh how I just loved this post that was shared by Ashlee Hutchins .  Its made me smile whilst also making my heart break for what is happening here.  Read the words that are written under the illustration.  See how poignant they are and how true that it is what many of us would love to happen to us.  How could anyone not see the sense in this post.  This is one post that blew me away.  I shared it with one group but want it to reach others in the hope that it makes people more aware.

Give my sight to the man who has never seen a sunrise, a baby's face, or love in the eyes of a woman.

Give my heart to a person whose own heart has caused nothing but endless days of pain.

Give my blood to the teenager who was pulled from the wreckage of his car, so that he might live to see his grandchildren play.

Give my kidneys to one who depends on a machine to exist from week to week.

Take my bones, every muscle, every fiber and nerve in my body and find a way to make a crippled child walk.

If you must bury something, let it be my faults, my weaknesses, and all prejudice against my fellow man.

If, by chance, you wish to remember me, do it with a kind deed or word to someone who needs you. If you do all I have asked, I will live forever. -- Robert N. Test

Anthony Nolan

Bone marrow:- It  brought it home to me that if you are so generous as to sign for organ donation  then why not sign  the Anthony Nolan one.  This is where you send a sample of your blood off to this trust and all details of this are kept on file.  It is worldwide.  If you match somebody who needs a bone marrow transplant you could be invited to donate your blood marrow.  Over 1.000.000 lives have been saved since this trust began back in 1974.  I remember well the parents of Anthony Nolan begging on the tv for people to be generous and see if their blood marrow matched that of their little boy.  Sadly no match was found and he died aged just eight.  Shirley, his mom, campaigned so hard to make his happen as she did not wish for another family to suffer the heartbreak her family did. 

In 2008 this trust set up the first dedicated cord blood bank.  This allows mothers to safely donate blood from their umbilical cord cord and placenta after they give birth, this is rich in stem cells and it is used for lifesaving transplants.  

Knowing how some people are needle phobic they have now achieved another goal.  By sending out "spit" kits it means that you do not even have to have blood taken if you don't wish to.  The kit is sent back and all details are stored in their files, what a great idea.  Just something to think about for those that like to think they can go some way to helping others.  

Sad really

When we lived in Spain and as my condition, though not diagnosed deteriorated I spent rather a lot of time researching my family tree.  nice and sedentary though the stress did get to me I must admit.  One thing was missing when I finished it was our family crest.  There are many family crests for just one name and it takes lots of research to find the one that really is yours.  In this regard my brother, sadly deceased, did the hard work and bought one for my mom and dad.  Framed and on the wall it was proudly looked at often by all of us, in particular as it said my grandfather 17 times removed was "a good friend to Charles 1st"  Quite why none of us asked to take a copy I just don't know but we didn't.  

After the death of my mom and before I was invited to the house, I did not have a key, this crest disappeared.  I asked a family member who knew who had it if I could just borrow it to take a copy.  Well how pathetic is it that this has not materialised.  All I wanted was a copy.  I took years researching the family tree and some of the family benefited by using my site to move on with their own research.  As I am trying to add photographs and places etc to the tree I feel it is sad that I am to be denied just a copy of what was not even theirs in the first place.  Pathetic really.  

Piano time

As Izzy has begun to learn to play the piano we were asked to provide her with one for home.  A rather expensive piece of kit  this, she should have taken up the triangle, three of us shared the cost.  With bated breath we awaited its arrival.  The stand was bought and ready, the seat in place and all that was missing was the keyboard.  It arrived and my did it look big!  All unpacked and placed on its stand and plugged in Colin began to test the keys.  He did learn piano as a teenager so has way more idea than I do about these things.  Though we did have a piano as a child my sister dropped some money down the keys so she destroyed the whole key board getting out her money!  That was the end of any hopes I might have had for learning to play the piano!

It was discovered that the last four keys on the key board did not play correctly so it has to go back.  What a huge disappointment, in particular as this was part of her birthday present too.  We are hoping it gets collected today and another is sent out to us quickly.  I know Izzy and her attention span.  She really needs this to push her on further. One hour lesson at school is not enough if she is really interested in mastering the art of piano. 

Well you could have told us.

In Leeds on Saturday with Izzy buying her a  new winter school coat we discussed the state of the new house, or lack of it with my son.  Though we were reliably informed that the build would take place at the end of this month or beginning of next we had no firm date.  Then Izzy piped up that they had "put some wood in".  On talking to my daughter it transpires that indeed yes, a start, albeit a small one has been made.  The site is all staked out now so we can see exactly where our house will sit on the land.  I was shocked as to how much of the land is taken up with the house, though we always knew it would but I really can't see" things in my head, I need to physically see them.  It is not a particularly big house rather the land is rather small.  Now I need to write a letter to all residents telling them where we are up to and giving our telephone number if there are any issues they wish for us to resolve whilst the building work is going on.    

Autumn

I love this time of the year.  The weather is crisp and I love the nights coming in earlier.  I can't cope with the heat since I got ph so it suits me right down to the ground.  We have already begun to get things booked into our calendars that really work the best in the autumn.  One of these is being a National Trust cooking on a camp fire where we will take Izzy.  This is being held at one of our favourite places,  Hardcastle Crags.  Two of our friends will also take their grandchildren and we should have so much fun.  It is no secret that we love open fires outside, the best place for them when it is chilly all around  and you get toasted as you sit close enough to stay warm but not too close you scorch.   Yes we have had many fires ourselves outside but as this is being held in such a wonderland and it will be lit up with fairy lights for the kids etc it should be a great evening out.   

Why Why Why !!!!!!!

I watched the news with horror today to see that though there is a government ban on children under 18 being allowed to be sold knives etc there are still many shops selling knives, cleavers, daggers etc. to just such people.  Whilst the news was saying this was happening in London it will be in many places.  When we look around at the heartbreak that is caused many times by children fighting, maiming and killing with these implements why are these shopkeepers doing it, and as if is known that they are being sold illegally what are they doing to the people selling them these weapons.  To be honest I think we are never going to get on top of all the hatred out there now.  Though I hoped for a safer place for Izzy to grow up in I fear this will not happen in my time for sure.  It seems to me the world has gone mad.  

It is a lovely day outside today, a day when we would have gone searching for conkers when we were young and when my children were young too.  Conkers have been banned now in schools and I don't mind that but I do recall all the pleasure we had with them when we were young.  Yes we might have had a banged knuckle or two when having conker fights but for sure there were no deaths that I recall or no stitches needed to stop guts spilling out when we "fought" our opponent.  Oh happy bygone days!!!

Flu

Am I scared about the flu heading our way from Australia, you can bet your bottom dollar I am.  I had flu once in my life and the memory is still strong in me today.  The experience was so dark and horrible and yes, scary even though I was a healthy girl in my twenties.  How much more worried am I now I am older and severely compromised.  I will definitely be using my antibacterial spray more than ever when leaving the house and asking those that come to our home to use it on our doorstep.  Do I think I am taking it too far, no I don't so please, anyone reading my blog take extra special care, remember a dose of flue can kill even the hardiest person.  My friend died from flu when he was 32 and a vey nice strapping lad he was.  Sadly he died over the Christmas period as his body just could not cope, his organs shut down one by one.  He left behind two young children.  There is no flu jab for this one at all and they are anticipating many deaths.  Just take extra special care please.    

All for now.  I need to go and see my butcher.  We are having friends for dinner on Friday and I need to order my meat.  We are cooking a Jamie Oliver main course, one that looks really nice.  Colin will deal with that whilst I shall be in charge of the starter and the dessert. I always look forward to seeing theses particular friends so I know we will have a lovely evening.  

Go well everyone.  Thank you once again for reading my blog.  Thank you for liking it or for any comments, these are so gratefully received!!!

Warm love  Carole xxx

We can't cut and run

Seeing a post a few days ago by somebody who is so fed up she has even contemplated suicide made me think of just how far we have come.  You wouldn't give in if you were in a race and we cannot either.  Most of use are doing way better than we ever expected since we have been on our meds.  For a lot of us our pressures have reduced and kept us here whilst waiting the cure.  We just can't give in to it.  Have your bad days, have your weepy days and duvet days ( I had a duvet day yesterday ) but then pick yourself up and do the best that you can.  Enjoy the good times.  See a doctor if you feel you might have depression but please, please never give in to ph.  Don't let it beat us.

Jane Jellyfish Davis

The funeral was held earlier this week for this amazing woman.  Sadly due to different circumstances most of us did not attend.  We were so fortunate then that Victoria Mayhew was able to go.  She had a brainwave of an idea and bought a card.  She then posted it on Facebook and asked if any of us would like to put something on this.  Once we had posted back to Victoria just what we wished to say she wrote it down for us and put our names with it.  She was then able to hand the card over at the funeral.  Such a beautiful thing to do.

It looked like her funeral was a really lovely one with a beautiful carriage to carry Jane in.  Many many balloons were released to fly away in memory of her.   I wonder what passers by thought was happening when they saw so many floating merrily across the sky. Fly high Jane.   Our thoughts are with the family.

YES at last a medicine dedicated for children!

How long have people been waiting for this medicine.  There has never been one just for children and I believe they had to take the ones for us, though in a lesser portion.  I am sure many parents will be so pleased.  This goes to prove that once again we are moving on with ph.  Once again the researchers keep working away for each and everyone of us.  Their goal is to cure but in the meantime to keep us as well as can be until that momentous day arrives.  I was so pleased to read this.

Since writing this it appears all is not how it appeared. It seems that the FDA have approved bosentan for children and not a dedicated one.  So sad about this but nonetheless another therapy to help.  

It breaks my heart when I see children carrying their backpacks to school with either their oxygen  or their pumps if on a hickman line.  Nevertheless these children just get on with it.  To them for many it is just the norm, the good days and the bad days.  I can't wait for the cure so that these children grow up in a world where ph means nothing to them.

How very bizarre

When we were in Scotland we bought two pirates for the memory cabinet that is on the wall in the study.  Now this display cabinet is rarely opened,  We open it if we have a new memory and occasionally we do allow Izzy to climb up on the step ladder and rummage around with the things.  A few days ago she asked us what had happened to the girl pirates arm and sword.  How aware are children as we had not noticed at all.  When we looked sure enough her arm had dropped off.  We assumed that it would be nestled on the base of another shelf but we looked and looked to no avail.  It has vanished.  Nobody else even really sees this cabinet of memories as it is in the study.  certainly we have not had anyone in the house who would even be bothered so where is it?  It remains a mystery but we decided, with the blessing of Izzy to take out the third pirate we got from her own memory box and replace it with the broken one, along with an explanation for future reference and we put the whole one  one in  our cabinet to sit proudly side by side with her mate.  If anyone has an explanation as to how an arm and a sword could just disappear from a closed display cabinet with a catch hard to open please do tell.  Until then it will remain a mystery.  

Just too much

Stress that is.  How many times are we told that we need to try not to stress out, stress can be a killer for us.  Some people I know do not give a fig and have caused a great deal of stress.  The person who began the whole thing who used to reckon she loved me showed her true colours.  Mind I really should have known as through all the months in total I have been in hospital they only ever bothered to come and visit once and that was at my local hospital whilst waiting to be transferred to Sheffield.  I suppose I should have taken it as a measure of how they felt about me then but I didn't.  Anyway through a silly thing that happened, a joke thing said about a recipe of all things she shut me off, taking with her my brother.  This caused endless stress though I tried to reconnect with them they have refused to meet with me halfway.  So the end of that as I decided to shut them out of my head and move on.  

I do wonder sometimes that as I am still alive they do not realise quite how poorly we are with ph.  Maybe they think I am partly cured!!!!  Until then we go along with all the pain, the lethargy, the sickness that comes with ph.  I will not give up the fight though,  I will just do what it takes to keep strong and stress free.  

Sign the form please!

This week is the one where we try our best to make people aware of how important it is for them to sign the organ donation forms.  Not only that but to tell your family as we know there are times when families in their understandable grief just won't allow this to happen despite the wish of their loved ones.  Remember one day it could be you or a family member that needs this gift of an organ.  Please be aware of this and take a loving step to sign the organ donation form if you have not done so.  The only cure for us with ph is organ donation and I have friends waiting for this gift of life and all the while organs that could have saved them are burnt or  buried.  Many illnesses mean organ donation is the only cure for them.  Please please sign the forms.  

So all for now, I have written this blog twice as I lost the first one!  We are going down to the land to try to tidy it up a bit as hedges have grown a lot recently.

Try to stay as well as can be.  Again thank you so much for clicking the like button on my blog and I absolutely love a comment or two.  

Warm love to you all

Carole xxx