Sunday, 26 November 2017


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. 

Link from my Friend Tess: -

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. 

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.  


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

Sunday, 5 November 2017

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