I AM NOTHING MORE THAN A PIECE OF BLOTTING PAPER AFTER ALL

I have long considered myself to be a strong independent person.  My life has not always been easy and I can’t say it has always been happy but i did consider myself to be in a good place right now a happily married woman with kids and grandchildren.  Like everyone there have been pitfalls to climb out of, some of my own making and some through others but I have always seemed to cope with what was thrown at me.  One of my sayings was “down but not out” when going through bad stages in life.  Something happened tonight that knocked the stuffing clean out of me, kicked my legs from under me and left me a pure shrivelling wreck - a person with no voice to answer back or energy to deal with my life threatening  predicament. 

I am in hospital right now due to catching a cold which quickly turned into a cough.  Yes to somebody immunosuppressed or pulmonary arterial hypertension that is all it took a simple  cold for me to face up to the brutal reality that I am not invincible after all.  That I am in fact nothing more than a piece of blotting paper laying on the ground in the rain.  All it takes is for the blotting paper to become too wet before it becomes nothing more than a piece of mush, unable to be used at all.  Try and pick up the paper and it reduces it even further to just a squelchy mess and here is where I found myself in the early hours of this morning.  

I was admitted to my local hospital due to low oxygen levels which had hit me like a sledge hammer.  Only two days before I had been to my specialist centre and had almost the full works, bloods, 6 minute walk test, ecg, echo and the list goes on.  We were all so pleased to see how well I was doing. These were my check ups for a trial am for my illness so were very thorough  For those without any knowledge of pulmonary hypertension please read up on it not forgetting the PULMONARY  hypertension which turns it into another ball game than the hypertension many people may have. I have pulmonary ARTERIAL hypertension . So feeling secure for a while in the knowledge that this heart and lungs so badly damaged when first diagnosed it was deemed ready for the knackers yard had -  due  to the wonders of science, researchers, doctors, nurses and yes you name it the list goes on of people the who have kept me safe -  led me to a safe haven for yet another length of time.  

Moving on then to my admission to my local hospital reluctantly as I did not want to use up the precious time of our overworked NHS staff and resources I had to admit defeat.  Being plugged into an oxygen machine for the first time as an absolute must became something very scary indeed.  I fly with oxygen always but never needed it throughout  my daily life. I have been checked out for this but fortunately was deemed too well for it.  My experience now of being unable to move from the bed unplugged without canisters and nose plugs or face masks has given me yet a greater understanding of how it is to live for those burdened in this way.  There is a way for those who do not have this condition to see how it is to live with the narrowing of lungs in daily life.  Put a straw in your mouth and a peg on your nose and then try to go about your business.  Yes the air you breathe will be sucked into your lungs through the straw, let me tell you it is tough, very tough but that is everyday life for lots with this condition.  Please let’s see this condition for what it is, life limiting, life threatening and even though thanks to research and new meds our status can often now be termed life shortening instead of terminal ( though indeed it can be terminal too) we do lead a more limiting life than most. Yes there are a lot of words with limiting in that sentence but that is the fact, limiting life,   

Now I am rambling.  So here I am in my hospital bed and liaising with my family at home and my wonderful PHA UK association team as my hubby of 24 years and our ph media is putting together a press release  and a JUST GIVING page for PH research  You will read more about Colin’s story on Facebook and media so this is not about his challenge but to ask you though to please if you can and do donate do not leave it there.  Equally as important as donations which go directly to our society I am asking you to share if you will any media about this condition to raise vital awareness about this very rare unknown condition,  It just may save the life of yourself or a loved one someday.  It can be just one of the articles read and almost forgotten that sits at the back of your mind that may resurface and jog a memory and move things on to a quicker diagnosis and better outcome for others.  

Now back to my story,  Despite lots of meds and oxygen plus changes in antibiotics I am still plugged in 24/7 to this life saving oxygen.  Just to go to the loo only feet from my bed means I have to unplug from the wall, plug into a canister which goes with me to the loo and back, unplug said canister, plug back into the wall and stuff mask on face and lay down to recover.  My heart goes out each and every time to those of my friends who do this 24 hours a day whether through ph or transplant,  Life is so tough.  

My experience this morning was one I never wish to repeat.  I had thought maybe life was getting better.  Despite one doctor feeling I may have a blood clot myself and another doctor thought not which has proven to be true, my predicament has proven to be NOT a clot but the after affects of a cold I caught which quickly turned into a chest infection limiting even more the power I had in my lungs.  So I was feeling on the up, yes still poorly for sure but more upbeat about it, less scared.   A good friend had visited and helped me to shower in a calm and peaceful manner ( first shower in a week as too poorly to cope before ) and rubbed moisturiser on my poor cracked and broken skin due to the systemic scleroderma.  Life had to be getting better didn’t it,  I had a couple of nose bleeds so oxygen pressures were taken down a notch and I was still managing as long as I was plugged in so surely things were on the up.  

Fast forward then to 5 am this morning.  I woke with my whole  body screaming out for breath but none would come, Never do I recall fearing death was so close.  Not only was it snapping at my heels but it’s teeth were snarling into my face as it tried to take me out from this world and into its dark and dismal own.  I was on the road to hell.  I could feel nothing but terror when I realised I had lost my oxygen, I was no longer attached to this life saving pump which had been keeping me alive.  I was on my own and it showed me how absolutely necessary this pump was to me at this time.  My body was screaming out for air but I had no strength to shout out for help, no strength to scramble to find my call button, no strength to do anything.  My desperate gargles because that is all they were and feeble gasps bought two nurses running.  Fortunately my bed is the closest it could be to their station and they quickly took charge.  One found the oxygen and attached me again whilst the other pumped up the flow to get my pressures under control.  The enormity of what could have happened and the realisation of how fragile this life is hit me once again so giving me the heading for this blog about blotting paper.  I am not so tough as I believed I was and like blotting paper which has its uses that can be gone in an instant if faced with a deluge of water so too could I have been without the oxygen and the quick thinking actions of the wonderful nurses who tore into action and saved the day. 

After recovering from being a blubbering, shaking quivering wreck I felt the need to write this blog and hope that there are some who will when they find the Just Giving page please donate.  ALL MONIES go directly to the cause,  Colin has funded this whole journey himself and was happy to do so in the hopes of raising of course money but also awareness of this shocking disease.  The link to the page is below and you will be helping such a wonderful charity.  By the way if you are a taxpayer there is a button to click which gives even more to the cause through the government.  You will be able to see each day Colin’s journey on Le Jog ( Lands End to John o Groats ) unaided, no support, all under his own steam, a one man band indeed.  For those on twitter etc if you would be so kind as to share as and when this story to whoever you have my heartfelt thanks, we need to share it far and wide.  It could be your loved one one day dealing with such things. Let’s work as a whole and do all we can to spread awareness for diseases that are so rare they are little understood,  All it takes is a click of the share button but it would mean so much to the cause of awareness of pulmonary hypertension. 

So another day looms for me attached to my pumps, right now I am also attached to my transfusion of antibiotics The doctors and nurses are calmly going about their business taking care of us all in such a wonderful way and thanks to their quick action I am able to write this blog.  Yes the blotting paper is drying out thanks to them and though I will never ever forget this experience and hope it is one I never endure again it has given me a greater understanding of lots of my ph friends worldwide.  

So as you go about your day I wish you a peaceful one my friends, I wish for you calm and good health.  

The link to the page is below and I leave it to you if you feel you can donate but if you do not then at least by reading this a tiny seed has been planted that may help you to one day bring about the much needed awareness of such a worthy cause.  

Warm love to each and every one of you from this partially dried out blotting paper.

The link - https://bit.ly/LeJogForPHAUK

Carole 

This is a Test

Just testing to see if my blog uploader is working.

UPDATES!!

It has been a while for sure since I sat at this commuter to write a blog and I thought a quick update first on my systemic sclerosis, the medicine I am taking seems to be doing a great  job on me.  When I met with my specialist three weeks ago he wrote on my notes “astonishing”  this was in relation to the tightness that was in my arms legs and shoulders.  My skin was stretched so very tightly that of course it hurt a lot.  As it slackened off I found myself being able to get up from the floor without  help which is great, I can wring out a dish cloth instead of leaving a soggy messy one in the bowl and I can also open a can on my own.  Whilst these things do not seem high on the can do list believe me they impacted a great deal on my life and so he relief is beyond words.  On the downside I have my bingo wings back!  As my skin slackened off gradually they returned.    Ah well we can’t have it all.

NEW TRIAL

I cannot mention the name of the trial I am hoping to go on but will update later in the blog if and when I am informed  I may do so.  That is if I actually get on it.  My recent echo showed such good results that I was told by the doctor who did it that if he had not known I had ph he would not have realised from the results.  Now as more tests are carried out we will wait to see if I am bad enough to go on this.  I have divided opinions, sad if I cannot as this will likely be my last trial, I have done so many but pleased that my condition seems to be stable. I sill put this down to the denervation which seems to have stopped my pressures rising.  It was a huge thing to undertake but is amazing the difference it makes.  I am told it has been approved in China, I may have the wrong country but anyway approved somewhere but not here yet  so I wait with bated breath for it to be  offered. Though they had hoped one hundred would trial this it sadly turned out to be just 17.  I do understand people being nervous about it but  now the proof is out there maybe others will feel they can do it when and if offered.  

Now I must get on and go next door to see Izzy.  She is growing so quickly and it makes me smile when I look out of the window in the morning to see her walking down the road with her backpack on to catch her bus to school.  Now more independent each day I feel pride but also sadness that she has left behind all those childhood ways that pleased us so much.  We did our best helping Danielle and later Chris raising her and feel we did a really good job.  Our role now is not so great but we are still an integral part of the family and always will be.  It always reminds me of the Abba song “Schoolbag in hand she leaves home in the early morning”  The song is about regrets that you did not do all you hoped to do with them and they keep sort of growing into the next phase of your life without your realising it.  I always think of it with my daughter too.  Time moves so quickly and things take over and before we know it they have grown and gone on heir own sweet way.  ……. ah well such is life.

Danielle does say that without us and our help she would not have been able to progress in her career as much as she has done.  Colin has taught her much about management and people! and we are a phone call away to offer any help we can do I do feel pleased of the difference we have made to their lives.  More importantly though we feel so blessed to have been able to guide and help Izzy on her way, from a new born to the 12 year old young lady she is today.  

We are meeting friends for lunch today and hope when we return to get a picture of Colin and his bike to put as a profile one on the sponsorship page.  Thank goodness for our family next door who have set this up for us.

LEJOG

Colin is intending to ride from Lands End to John o Groats, the whole length of Great Britain for US.  World awareness day for ph  is on May 5th so he will begin his mammoth ride a few days earlier so that he is actually riding on that specific day.   

Colin likes solitude fortunately and he has chosen to do this on his own.  Of course you will need to see where he is each day, that is a given but I am hoping to have a word with our very own Sarah Marshall whose hubby Phil also did a mammoth fund raising.  How many of us woke in the night just so we could follow his blue blob on the map.  We have no clue of how to go about doing that but hoping they can give us some pointers.  

Our own ph society which amongst the trillion other things they do for us publish our Emphasis magazine and we are hoping that a feature will be done in that to raise more awareness.  Mary who does the features will be in touch with us at a later date re this.  Sean is helping us too as he is talking us through the various aspects of fundraising and I know he will only be a phone call away.  (He did actually ask if Colin knew what he was taking on as they had looked at the map and went WOW) Yes he does.  

Let me tell you a little about Colin.  He has always been active and threw himself into teaching children at Sea Scouts how to canoe safely.  He led many walks with them over the Yorkshire Dales and our beautiful Lake District.  Trying to make sure our Izzy enjoyed his outdoors activities he has taken her up mountains and made sure she grew to love the country as he did.  When we lived in Spain he joined the local Mountain Walking group and it was not long before he was leading walks in the mountains of the Costa Blanca and ultimately he was invited to join the groups A Team.  One had to be invited to join this group and there were not many asked to do so.  They were and still are considered to be the elite of mountain walkers that venture out to do amazing walks over almost impossible terrain and abseiling down places etc.  In fact the  Guardia {Spanish Police} called upon this team to help them in the search for a missing walker.  When the A Team arrived one could hear an intake of breath as these mostly retired walkers turned up.  However they quickly went on to realise they could not cover the terrain as these more experienced walkers and decided to set up camp lower down the mountainand let the A Team carry on!  Respect for this team grew ever stronger.  

Colin appreciates all that has been done for me and all of us inflicted with this terrible disease.  We need to raise awareness yes of course but we also need more funds so in appreciation of all that has been and is still being done for us all  he has decided to do this challenge.  Not bad for an old guy of 74 years.  

He is in training now, this cannot begin on the day the challenge begins.  Fitness has to be built up gradually and he goes for many a 70 mile ride on a Sunday or takes the motor home to different places that are challenging for bike riders and throws himself into each day with gusto.

We are planning to spend time in Spain on the lead up to this challenge as it is very taxing for bike riders and where we used to live teams from all over Europe do their training there.   Colin will be doing a lot of riding over there to make sure he is fit for the job.

He has already booked accommodation for most his of the journey so he is aware that he cannot slack at all,, the miles must be done but he has a purpose and that is to do all he can too give back to the A Teams that keep us all alive.  To say thank you to the ones that have gone before us, to try to help the ones that will come after us and  to honour all inflicted with ph and just generally do all he can for our society.   

I could go on but he has just shouted up the stairs “don’t be saying too much! “  Those that know him know he is a private person and so to put himself out there is a huge step for him believe me.  

I can only hope that all those that are able will go onto the JUST GIVING page when it is organised and show your appreciation for everything that is ph.  

I will from time to time send snippets of him training and tell you where he is up to in his planning to keep it in your minds,  I feel sure you will appreciate what he intends to do!

Well enough for now  I will write a normal blog later but this needs to be out there now, so I am told by those in the know!  

Love as ever

Carole xxx

PAYBACK !!

It has been a while for sure since I sat at this commuter to write a blog.  This one begins with my telling you what Colin is up to as we hope to raise money for ph.  He is intending to ride from Lands End to John o Groats, the whole length of Great Britain for US.  World awareness day for ph  is on May 5th so he will begin his mammoth ride on the 3rd May so that he is actually riding on that specific day.   

Colin likes solitude fortunately and he has chosen to do this on his own.  Of course you will need to see where he is each day, that is a given but I am hoping to have a word with our very own Sarah Marshall whose hubby Phil also did a mammoth fund raising.  How many of us woke in the night just so we could follow his blue blob on the map.  We have no clue of how to go about doing that but hoping they can give us some pointers.  

Our own ph society which amongst the trillion other things they do for us publish our Emphasis magazine and we are hoping that a feature will be done in that to raise more awareness.  Mary who does the features will be in touch with us at a later date re this.  Sean is helping us too as he is talking us through the various aspects of fundraising and I know he will only be a phone call away.  {he did actually ask if Colin knew shat he was taking on as they had looked at the map and went WOW}  Yes he does.  

Let me tell you a little about Colin.  He has always been active and threw himself into teaching children at Sea Scouts how to canoe safely.  He walked with the children following him over the 3 Peaks and took many children, along with other adults of course tracking and camping in our beautiful Lake District.  Trying to make sure our Izzy enjoyed his outdoors activities he has taken her up mountains and made sure she grew to love the country as he did.  When we lived in Spain he was a member of the A Team walkers.  One had to be invited to join this group and there were not many asked to do so.   They were and still are considered to be the elite of mountain walkers that venture out to do amazing walks over almost impossible terrain and abseiling down places etc.  In fact the  Guardia {Spanish Police} called upon this team to help them in the search for a missing walker.  When the A Team arrived one could hear an intake of breath as these mostly retired walkers turned up.  However they quickly went on to realise they could not cover the terrain as these more experienced walkers and decided to set up camp lower down and let the A Team carry on!  Respect for this team grew ever stronger.  

Colin appreciates all that has been done for me and all of us inflicted with this terrible disease.  We need to raise awareness yes of course but we also need more funds so in appreciation of all that has been and is still being done for us all  he has decided to do this challenge.  Not bad for an old guy of 74 years.  

He is in training now, this cannot begin on the day the challenge begins.  Fitness has to be built up gradually and he goes for many a 70 mile ride on a Sunday or takes the motor home to different places that are challenging for bike riders and throws himself into each day with gusto.

We are planning to spend time in Spain on the lead up to this challenge as it is very taxing for bike riders and where we used to live teams do their training there from all over the world.   Colin will be doing a lot of work over there to make sure he is fit for the job.

He has already booked accommodation for most his of the journey so he is aware that he cannot slack at all,, the miles must be done but he has a purpose and that is to do all he can too give back to the A Teams that keep us all alive.  To say thank you to the ones that have gone before us, to try to help the ones that will come after us and  to honour all inflicted with ph and just generally do all he can for our society.   

I could go on but he has just shouted up the stairs “don’t be saying too much! “  Those that know him know he is a private person and so to put himself out there is a huge step for him believe me.  

I can only hope that all those that are able will go onto the JUST GIVING page and show your appreciation for everything that is ph.  

I will from time to time send snippets of him training and tell you where he is up to in his planning.  I need to keep this story active so none of you forget!  I feel sure you will appreciate what he intends to do!

I do not know how many people who have their names on the T-shirt are still alive.  I would hope all but truly do not know.  If you zoom in and see your name or know of somebody still with us please let me know.  

Well enough for now  I will write a normal blog later but this needs to be out there now I am told by those in the know!  Please be as generous as you can.  PH is such a great cause. I will speak again soon.

Love as ever

Carole xxx

Well that sat on the computer for a while so just a general update of us here today.

It seems the medicine I am taking for my systemic sclerosis is doing a good job on me.  When I met with my specialist three weeks ago he wrote on my notes “astonishing”  this was in relation to the tightness that was in my arms legs and shoulders.  My skin was stretched so very tightly that of course it hurt a lot.  As it slackened off I found myself being able to get up from the floor without  help which is great, I can wring out a dish cloth instead of leaving a soggy messy one in the bowl and I can also open a can on my own.  Whilst these things do not seem high on the can do list believe me they impacted a great deal on my life and so he relief is beyond words.  On the downside I have my bingo wings back!  As my skin slackened off gradually they returned.    Ah well we can’t have it all.

I cannot mention the name of the trial I am hoping to go on but will update later in the blog if and when I am informed  I may do.  That is if I actually get on it.  My echo shows such great results that I was told if the doctor that did it did not know I had ph he would not have realised I did.  Now as more tests are carried out we will wait to see if I am bad enough to go on this.  I have divided opinions, sad if I cannot as this will likely be my last trial, I have done so many but pleased that my condition seems to be stable. I sill put this down to the denervation which seems to have stopped my pressures rising.  It was a huge thing to undertake but is amazing the difference it makes.  I am told it has been approved in China, I may have the wrong country but anyway approved somewhere but not here yet  so I wait with bated breath for it to be  offered. Though they had hoped one hundred would trial this it sadly turned out to be just 17.  I do understand people being nervous about it but  now the proof is out there maybe others will feel they can do it when and if offered.  

Now I must get on and go next door to see Izzy.  She is growing so quickly and it makes me smile when I look out of the window in the morning to see her walking down the road with her backpack on to catch her bus to school.  Now more independent each day I feel pride but also sadness that she has left behind all those childhood ways that pleased us so much.  We did our best helping Danielle and later Chris raising her and feel we did a really good job.  Our role now is not so great but we are still an integral part of the family and always will be.  It always reminds me of the Abba song “Schoolbag in hand she leaves home in the early morning”  The song is about regrets that you did not do all you hoped to do with them and they keep sort of growing into the next phase of your life without your realising it.  I always think of it with my daughter too.  Time moves so quickly and things take over and before we know it they have grown and gone on heir own sweet way.  ……. ah well such is life.

Danielle does say that without us and our help she would not have been able to progress in her career as much as she has done.  Colin has taught her much about management and people! and we are a phone call away to offer any help we can do I do feel pleased of the difference we have made to their lives.  More importantly though we feel so blessed to have been able to guide and help Izzy on her way, from a new born to the 12 year old young lady she is today.  

We are meeting friends for lunch today and hope when we return to get a picture of Colin and his bike to put as a profile one on the sponsorship page.  Thank goodness for our family next door who have set this up for us.

Have a great day and take care each and every one of you.

Carole xxx

SEEMS THIS IS A POSITIVE FOR PH

HOME AGAIN

We were delighted on our return from holiday to see the letter below from the lovely team in Israel who developed the machine to enable denervation to be carried out.  On reading it there seems to be a positive for this procedure to be continued so hopefully in the future we will see more having this op done.  The op itself has proven to be safe ( something I know as I came through it unscathed) so praying for all to continue down this path

It is very rare indeed to receive a letter such as this and I have done endless studies and trials so it was very touching.  I count myself fortunate to have been given the opportunity for this so no thanks were needed but nice anyway to let us know where we were up to with it.

Firstly, Sonivie, the Sponsor of this study would like to sincerely thank you for your participation in the very first clinical trial evaluating the Therapeutic Intra-vascular Ultrasound (TIVUS") System for pulmonary artery denervation in patients with pulmonary hypertension. It is because of the involvement of participants like you that we are able to take the first steps to evaluate this new potential treatment for pulmonary hypertension. Without you, this would not be possible.

We would like to share the main results that you and the other participants have allowed the Sponsor to generate. In total,14 participants were enrolled in the CLNS01-001 study for an initial one-year hospital visit follow-up which was then followed by a long-term surveillance at 36 months after the pulmonary artery denervation procedure date.

Pulmonary Arterial Hypertension occurs when the blood pressure in your pulmonary arteries (thevessels that bring blood to your lungs) is increased due to narrowing of the small arteries of the lungs.  In this study, the TIVUS" System was used to carry out a new procedure called pulmonary artery denervation. By deactivating some of the nerves around the pulmonary arteries, pulmonary artery denervation was hoped to lead to reduced blood pressure in the lungs, improved heart function, exercise capacity, activity levels and wellbeing. The pulmonary artery denervation procedure used ultrasonic (high frequency sound) energy during a right heart catheterization procedure.

The main aim of this study was to collect information about the safety of the denervation treatment of the TIVUS System in adult patients with pulmonary hypertension. We are happy to let you know that the results of the Trophy-1 Study demonstrated initial safety of the TIVUS" System when used for the treatment of pulmonary arterial hypertensive patients.  The treatment was well tolerated by most of the patients, with pain well managed when present during the treatment and during the study no device-related or procedure-related serious adverse events were reported which demonstrated the safety of the procedure.

The pre-specified effectiveness endpoints noted in the Patient Information Sheet were for haemodynamic evaluation, quality of life score and the 6 Minute Walk Distance. At the 4-month follow-up a statistically significant reduction was identified in the haemodynamic measurements for the mean pulmonary artery pressure, pulmonary vascular resistance and the right atrial pressure. Improvement was also identified in the patient cardiac output, activity levels using the wrist actigraphy data collection device, the 6-minute walking distance completed and the quality of life questionnaire score. In addition, the procedure was well tolerated by the patients, the catheter was easy to use, and the pulmonary artery denervation procedure was around 30 minutes in duration

long-term follow-up of the participants indicated a potential benefit of the TIVUS" System treatment up to 18 months following treatment. These very first results are promising and support future clinical studies to the confirm the good safety profile previously seen and to see if a repeat treatment from18-24 months could be beneficial to patients.

Thank you again for your valuable help with the first steps of the development of the TIVUS" System for pulmonary artery denervation in patients with pulmonary hypertension.

SIX WEEKS ENDED TOO QUICKLY

Our holiday in Spain went exceedingly well and I was fortunate enough to enjoy reasonable health throughout.  It was such a delight to see all of our friends made since we lived in Spain and indeed some we made since leaving Spain.  We had good weather except for one day so cannot complain.  Each day we walked to the Port for coffee and later walked along the Arenal so I could get my steps in.  I did wish I had had the opportunity to have my ferrinject infusion before I went but we coped.  However had I had this I know I would have had more stamina. 

I received lots of e mails giving me dates to see various specialists when we were on the ferry travelling over to Spain.  Although we had been told we would see a specialist urgently we were assured that it would be at least two months so decided to just go for it and have our holiday.  We were shocked when we could have them so early and had to ring each and every one and explain that this holiday was important and we could not make them.  New appointments were made and we knew there was such much ahead for us once back in the UK so we tried to put it all to the back of our minds and enjoy each and every day of our precious time in Spain.

Long leisurely lunches were the order of the day with me then going back to our apartment to rest. Tiredness would just become overwhelming and no matter where we were I would have no option but to go home.  Lack of ferritin is awful and the tiredness we get is so unlike a normal type.  We with pah suffer so much from it as for lots of us our bodies do not absorb iron  and these infusions are a very necessary part of our treatment.  Our friends are all so lovely and would be so understanding so when I had so rest I had no worries around them at all.  They totally understood. 

Colin is planning on returning in September to lead a few walks but I do not wish to fly so will be happy to stay here safely at home.  

AN ABSOLUTE TSUNAMI OF COMPEX ISSUES

Not for me a nice gentle pattering of rain that gives you time to get a raincoat on before the heavens open. No my tsunami of issues rained down on me so hard and fast I barely had time to take a breath, pelting me with one thing and then another.   Since our return home we have had endless rounds of hospital visits, x rays, blood tests, echos etc we have barely drawn breath.  It seems that on top of needing oxygen at night and my carpel tunnel in both hands I now have been diagnosed with systemic scleroderma.  My specialists says this a rare form that attacks my internal organs.  Oh whoopy do indeed.  On top of that I am told there is something else showing in my blood that needs to be diagnosed yet.  They have not got to the bottom of it.  What joy lays ahead.  I am shortly to get my muscle conduction tests too to see why my muscles are wasting away.  It appears my weight  loss is due to my muscles disappearing.   No  wonder I cannot lift my leg onto a buffet to tie shoe laces or indeed even get up if laying on the floor after doing my denervation test.  It takes me so long as I do not even have much use of my arms as my carpel tunnel prevents me from putting too much weight on them.   On the plus side my infusion is working and I have much more energy now three weeks after it took place.  

Next step for me is to see my ph specialist at the end of this month and to have an MRI plus the barrage of other tests, walking, breathing etc that most of us know so well is part and parcel of having ph.  Before then I am to have my injections for shingles, pneumonia top up and my fourth covid one.  These need to be in place before the new tablets I have begun to suppress my immune system take full effect.  I have only been on them a week and I was told it takes four months to work efficiently.  Apparently the "norm" if there is such a thing would be to develop the systemic sclerosis  first and later develop ph but I did it the other way round 12 yers after diagnosis.  She says it is all very puzzling.  When I asked the question would this kill me she was very diplomatic and said "we will talk about that later"  Quite why I asked the question when I am already suffering a life shortening illness I don't know.  On my second visit I forgot to ask her so still the question  sits silently in the background.

None of these will spoil my zest for living.  I will not let it leave me in a heap on the floor.  We have to make the most of our lives and make the most of every precious moment and I intend to do just that.  Covid has helped somewhat with this as we have all got used to wearing masks, something I will need to continue as my immune system gets less and less.  Our friends already are good at letting us know if they are feeling unwell so we stay away from them but apart from that we must live our lives.  So much to see and do yet.

JUNE CELEBRATIONS

Danielle and Chris are having a garden party for the Jubilee.  I will do my bit re cooking so I am making pulled pork, Colin is making a good coleslaw to go with it and we will provide the bread buns for this.   He will also do a huge fruit salad and we will supply endless ice cream to go with it.  I will make blueberry muffins and something else I forgot right now what I said I would do!!!!  The ones invited are mostly family from both sides so know about my condition so will understand my mask.  I do hope we have good weather for this.  We have been busy planting out plenty of flowers in the bank leading to the gate and their house as we want a nice display. 

We decided this year due to so many hospital visits etc to not do much about veg.  We have planted potatoes in the raised bed and onions in the other and also dotted onions all around the garden in the hope they keep the aphids at bay.  Maybe next year we will grow more but this will suffice,  We still have two apples trees, a pear tree and blueberry bushes so it just must do us for now.  I have bought tomato plats though as I love tomatoes.  

I am still walking daily.  Today I got Izzy to school for 7.45 and parked up and walked into town to collect something I needed.  I then walked back to the car and by then I had done 12200 steps so was pleased with that.  I may go out for a small walk this afternoon.  We shall see as my son is supposed to be coming but if he leaves early I will go for Izzy early and walk around the park.  I still remain very positive.

Izzy continues to be a delight but to my dismay she is growing up so fast she is almost galloping.  I bemoan the fact I have lost my baby but she just says "well grandma that is just how it is, we must grow up" and of course she is right but it pains me anyway.  Somewhere in the back of my mind is the saying that I blinked and there before me stood a man,  the same has happened with her.  We just turned around and our curly haired little dot that absolutely adored us is now very capable and does to need us quite the same.  Such is life.  

Though it has been a long time in coming this blog I cannot think of anything else to tell you so I will love you and leave you and of course thank you for reading this.  Take the best care.

Warm love

Carole xxx

 

IT’S BEEN A WHILE

I know I promised a blog “soon” to some of you asking where I was but so sorry, things have been pretty hectic around here.  The last few months seem to  have been spent running backwards and forwards either to my hospital in Sheffield  or Calderdale Royal or Huddersfield Acre Mills. To be honest we have had a nightmare time trying to get to the bottom of my “complex issues” as one specialist said and though not quite there at least we are some way to getting answers.  One thing has been confirmed is that I have severe carpal tunnel syndrome in both wrists, eventually I will be sent to have one of them done with hopefully the second one further down the line.  Brain issues have been ruled out after an MRI which is a plus but though my back is really bad he won’t operate cos of the pah and the dangers that may occur.  I am waiting now to see the ‘rheumatologist to either rule in or rule out rheumatoid arthritis or scleroderma.  Oh what fun we have.  We know the appointment will not come through in the next few weeks so we are taking the opportunity to travel to Spain, Javea via ferry and drive down.  So many hoops to jump through.   

Typically when i really needed my cleaner to arrive so we could have a good talk about what I needed her to do over the next few weeks she sadly got a migraine so I have written huge lists for her to follow.  She does take good care of our place and enjoys sleeping here so that is a comfort.

I badly needed a pain killer I was given when I had my hickman line fitted so I wrote a nice note to the doctor asking for it explaining that I had just been in touch with Sheffield and they approved it.  Of course the girl on the desk refused to give it to the doctor and told me I could have an appointment for one to ring  me in two weeks time!!!!  I was livid as I was in so much pain. I told her to forget it I would go to Sheffield and ask them if they would hand me the prescription  as they had approved it.  Sure enough they came up trumps and I now have better pain relief, not the best but as I cannot take anything with opiates in it is a tough call for the doctors when t comes to pain relief it is the best I can hope for.    

WILL I EVER TRIAL AGAIN

With all of the above it did make me wonder if I would ever be allowed on a new trial.  I was going to begin one this month but so far it has not come my way.  Though i continue with studies I must admit to liking trialling new drugs to see if they would be of benefit to those of us suffering with pah.  I shall have to sit and see and continue to hope that I can.  I know I owe my life to those that trialled before I received this god awful illness and I am so thankful  but I did love sort of paying back if you know what I mean.  if I cannot at least I gave ten years to trialling them so it will give me some comfort.  

THE WEDDING AT LAST

I was so looking forward to this wedding which was held at the prestigious Holdsworth House and i must say it did not disappoint.  Both Chris and Danelle put so much work into it, the venue the outfits, the favours etc were beyond anything I could have imagined  I was so proud of Danelle and Izzy who looked so stunning.  Chris looked a picture too in his wedding suit as did Harry,, our handsome grandson who looks just like his dad.  At this wedding every single aspect had been organised down to the last detail.  The food was excellent from the canapés brought around while we were drinking before the meal to the wedding breakfast itself.   

My word the room we ate in was truly magnificent, Danielle wanted it to be filled with flowers and filed it was.  White roses and a few pale pink ones adorned every space.  The smell was fabulous.  But I digress.

Before the actual wedding we were in rooms that were used to do hair and make up etc.  This was a lovely place to natter and catch up with those we had not seen in a while.  The photographers were taking a great deal of pictures but oddly enough it did not feel at all invasive.  The atmosphere was lovely but as time went by I could see Danielle getting a little nervous. Izzy too seemed to be a bit worried about walking down the aisle.  Once the ceremony started however it seemed all nerves went and they spoke their vows very clearly and precisely. I was so proud of all that played their part.  Danielle had a wonderful friend Abi keeping her calm and Chris had his best friend Jamie so that quelled their nerves.   

Plenty of pictures were taken both in and out of the venue and outside they had drones flying and taking pics too.  We can’t wait for the  pictures to arrive. At the dinner table there were  tiny almost hidden microphones in the vases of flowers close to where five of us made our speeches.  There were wedding favours for every guest in the shape of a beautiful box tied up with white ribbon.  On opening them we were all given 15 years old single malt for the men along with the stones that you put in the freezer and then when cold drop into the whisky to cool the drink but it does not get tainted with water or any other liquid.  This would be a sin in Chris” eyes.  Along with this were hand made chocolates too in a beautiful box.  The ladies instead of whisky were given a particular gin that my daughter likes with a small mixer and again the chocolates. 

 

Quite a few of us received huge bunches of flowers, amazing and Colin received a delightful gift of not one but two paintings of two people walking along with backpacks on enjoying the mountain scenery.  ( perfect) .  Izzy and Harry received gifts of inscribed jewellery as a thank you for their part in this wedding.  Harry was the ring bearer and Izzy was matron of honour.  

Sadly after the meal I had to go to bed and was fearful I would never to return but I did manage to!  I arrived just as Danielle and Chris were taking their first dance so how pleased I was that I made it.  Though not doing much except for sitting I did manage to stay most of the evening so I was so pleased I dd.  It was wonderful to meet with our now bonded family and thermosphere was so good and loving and yes lively.

My speech went well I think.  Even one of the photographers came over to me to tell me how good it was.  Many told me how very beautiful my daughter looked and though biassed I had to agree.  She looked amazing, we were so proud of them both.  

So the deed is done  My daughter is now Mrs Williams and it was announced at the wedding that Izzy too had now taken this name, we were so very pleased.  

We are fortunate too in that we genuinely like and respect all Chris’ family so look forward to seeing them at more and more family occasions.  

Right well my hands say enough typing and get back to wearing my splints.  

I pray you are all still safe and stay that way.

Warm love to you all.

Carole

HERE WE GO AGAIN

I must confess that this joke did make me smile,  I hope it does you too.  Once again here we all are facing shortages of one thing and another with worse yet to come we are told.  The petrol one was ok for us, we have to be honest been very fortunate.  Plenty of petrol, no queues and plenty of fruit and veg at our stores.   We are not sure if we will continue in this way or if we eventually will get hit as other parts of the country have been.  I just hope we can ride all of this out but not so sure with France threatening to blockade the ports.  Time will tell.  Anyway I thought it would be good to begin this blog with something that made me chuckle.

DENERVATION

Once again after speaking to my specialist I have been informed the my ph levels remain between 30 and 35 so not bad for somebody who should have died 11 year ago.  I do hope that when everything is collated re this trial that this is offered to more and more patients.  Though quite a complex procedure and yes painful I have never once regretted it so I do hope more of my ph family  benefit from this and remain in a stable condition too.  All in all my ph is well controlled which is for me as good as it gets. 

TIME AWAY

We did manage to get away for three absolutely splendid nights at a first rate hotel with the most fabulous food last week.  We went with a couple of good friends and did not seem to come up for air whilst we were away.  I must confess that once we got home I had a much deserved resting day.  We walked around Tarn Hows, went on ferries, checked out once again Beatrix Potters place and took a boat trip around Lake Windermere.  We shopped in Bowness and went to look around  Wray Castle.  We are still very cautious and stayed as far away from people as and when we could and wore masks most of the time.  

Just before we left I realise that my walking trainers needed replacing.  There was very little sole to protect my feet from the cobbles on the old roman roads around here and I did not wish to walk in my walking boots.  Clearly a new pair were needed.  I cannot begin to think about how many miles those trusty walking trainers had taken me but they had done their job well.  A walk around some of the many outdoor pursuit places in Ambleside and I came across the perfect pair.   Gortex lined -  a must, a good sole to cushion my feet and the type of fastening where I could easily lock in the laces half way down the shoe.  Merrell was the one I opted for and they were immediately comfy so I was set to go on my walks again without hurting my feet. 

FACING UP TO REALITY

Yet once again on our return we had to deal with the old problem of the guy from the apartment block where we used to live, he was to stand in court to answer charges for his harassment towards Colin and to some degree to myself.  As we always thought he would, he pleaded guilty at the final hour so really we were not needed in court.  We did however want him to see us there to let him know that we were not intimidated with his threats and to see him get his just punishment.  The judge was rightly hard on him when talking to him and left him in no doubt that should he go before that court again with similar charges his punishment would be very severe as this was his second appearance on harassment charges.

He did hand a letter of apology to the judge and said he wanted to give it to Colin but was not allowed to because of his bail conditions.  We did not see the letter as the trial has not finished yet but Colin said he does not wish to see it anyway.  Generally we are people who can have differences with people but once it is over it is over, such as the last time he was in court and sentenced we shook his hand and said it was over and we should all get on with life, but not this time.   Enough is enough and we want nothing more to do with him, although in four weeks time we will meet him in court once again when he is sentenced.  He should have let sleeping dogs lie after last time but no, he is one of those bullies who when he thinks nobody else can see what he is doing keeps coming up for another nip.  I know one or two others like him and generally once I find out what they are, steer clear of them.  

One sad fact of this is that a lovely lady who held the chair on the committee has had enough and sold up and is ready now for moving out.  All because of this person.  

GENERAL

Izzy is away on a trip with school.  Got loads of pics of her abseiling and making canoes etc.  We will collect her when the coach drops her off today and are looking forward to hearing her tales.

All still going good with the wedding plans.  Colin needs to get his suit fitted at some stage but I also want him to get off for a few days before 18th November when the court date is set.  Still nobody has seen my outfit for the wedding except for the friend that went with me to choose something.  It is sitting nicely in the spare room next to my daughters wedding dress.   

I have two dates this month to see orthopaedic specialists.  One is here where I live and the day after one is in Sheffield where I attend my ph clinics.  This one is to talk me through quite what will happen if surgery is indeed deemed absolutely necessary.  I already know there is a high risk of death, stroke etc or not regaining the use of whatever pulmonary pressure I have now but we will need to weigh up the quality of life and make that decision together.  It is just a bummer that not only do I get such a devastating disease as the rare pah but also issues with my bones too!  

We both have colds and did do the covid tests just to make sure nothing sinister was lurking but no, all clear for covid which is a relief.  We still wear our masks and still do our best to use the two metre rule but this is quite difficult as others do not seem to be bothered.  Me and Colin attended a dinner for a village somewhere in the third world where nobody wore masks either to enter or leave.  I won two raffle prizes which I was shocked at as I rarely win anything.  The prizes were both bottles of wine and wether good or bad wine I have no idea as I do not drink but a nice thing to win anyway.  

Right time to get off this machine as I know Colin is itching to use it.  I have now got a mini iPad as it is so light to hold but for things such as this blog I need the big machine to type on.

Thank you to those that messaged me to see how I am as not much input fro me on facebook for a while.  All to do with technical issues I can assure you, I am not doing too badly at all.

Tons of love to you and please write me a line or two if you can.

Carole xxxx