I CERTAINLY OWE THIS LADY MY STABILITY - DO YOU?

As most of you know we have been away again in our motor home.  One lovely consequence of moving around means that I just might be fortunate enough to be in the vicinity of another ph patient.  This time we were in Norwich.  Beautiful place this certainly is and we spent a night on the drive of our niece and her hubby.  This couple certainly have the green finger touch and I was so shocked as to how their garden had come on since the last time we saw it.  Veg were growing in abundance as was fruit, the chickens were laying well and I can testify the fennel was certainly showing who was boss as it kept catching around me as I went from motor home and into their house.

Our welcome was a very warm one and made all the better as they had no idea we were going to park on their drive until the evening before. Whilst I was there I wrote a post asking if anyone lived close by. Mandy Leonard wrote that she did, in fact only around 7 miles from where we were. Without ado off we went  to park on her drive and have my first ever look at this lady that I owe so much to.

Now yes I know about my A Team, I mention them so much but I knew just what a huge debt I owed to Mandy.  You see Mandy was diagnosed with PAH around 2005 after having symptoms for years before that. Now those of you with PAH know that the new meds are slow to come onto our space, slow to be allowed etc although as we are are a rare breed we do get special allowance from the government to say that ours can be given to us without all the delay needed for most meds.  That is because without our meds we absolutely will die.  

So think on this -  here we have a lady who had never heard of PAH, scared for sure and not really much out there other than channel blockers, bosenten and iloprost etc.  The specialists got their heads together and after talking to Glaxo Klyne who was hoping to trial a new drug they believed would help they asked Mandy if she would be willing to trial it.  There were just 6 people trialling this med.  Generally when I trial there are around 20 so only a small number for ambrisenten indeed.  Well long story short really as those of you on it know the success of this particular drug and that also unlike Bosenten which has a greater danger of the two drugs to cause liver and kidney damage this med appears to be a good one for PAH.  I myself switched to it after coming off the hickman line and have been so grateful ever since.  

After the trial the drug was expensive and the government just could not, or were not willing to pay for such an expensive medicine right there and then. It all needed to go before boards and talked about at length. The drug company though saw just how well Mandy for sure was doing on it, I cannot say about the others as I have no knowledge. They allowed Mandy to continue to take it without charging the NHS who would not then have allowed it and always asked how she was doing whenever her packet of this life saving drug was ordered. In time of course the drug was approved and the money found to allow the drug to be available for the many of us.  Thank goodness for that.  

Mandy looked great, here she is 15 years after diagnosis keeping a beautiful home for herself and her hubby and son. By the way her son is really gorgeous if there are any young single girls around that area!!!!!!  He just might kill me for that. Anyway the crux of the matter is without this drug I just might not have remained as stable as I am. I might have liver damage or kidney damage etc.  So yes to me Mandy is top of my list for helping me to remain alive after my initial diagnosis.  It is thanks to people like her that I decided to trial as I knew of the benefit I was getting from drugs trialled by those before me.  So yes  - Mandy a HUGE thank you to you and I hope you continue to do as well as you are doing. I will not give up trying to persuade her to attend our conference.  A lot of the newbies can see how well she looks 15 years after her initial diagnosis.  It will certainly be a huge boost to their minds.  

Home again

My family next door are home again after their holiday.  It sort of felt lonely for the few days we were home and they were not.  Nobody running around the garden, no rabbit hopping merrily around in the sunshine and no faces at the window.  To welcome them back I took them flowers from our garden for their family room and a few sweet peas for Izzy as she loves the smell of them in her bedroom.  They asked me to get them bread and bacon as they were craving a bacon butty!  Turkey of course does not serve pig and though they rarely have it here I think it is what you cannot have you miss. So up got Chris early this morning and made bacon sandwiches for breakfast for himself and Danielle.  Happy faces for sure.  

We appear to have got rid of Mr Fox as he has not been seen for over two weeks now!  I hope it continues.  I am giving the box of bone and fish meal we bought for the garden to a friend as studies show that foxes are attracted to it, anything to stop it coming.  So there you have it, it appears that male urine, lots of it, in particular the first morning wee and my smelly dousing of all the plants with boiled up garlic and scotch bonnet chilli seems to have worked.  I am very relieved.  I used to see it in the driveway at 4.10 am each morning. Strange how it always came at the same time and I always seemed to be up making a call of nature. Anyway I saw it three times in the same place at the same time.  It must then have run around and came back to attack our plants from a different angle.  Well no longer does our drive smell of fox pee and poo.  

Today I woke up feeling good, no PHAT day for me {pulmonary hypertension arterial torment} or at least very little.  Would that all days were the same. So as we had just had more indian paving stones put down at the back of the house I decided to jet wash all the ones that were already down.  They were certainly grubby compared to the new ones.  I ended up with sand in my hair, face and I will not tell you how bad my clothes were as they were unbelievably bad.  Chris said, and he was correct, I should have worn my outdoor wet weather gear but the sun was blistering hot and I think I would have died!   The problem is that doing one job sets up another.  The sun terrace threw all its sand onto the bi-fold windows and so another job to do, plus all the glass surrounding it needed to be sorted out.  Fortunately I did manage to complete the job and did not have to hand it over to Colin who himself was working very hard cutting all the hedges.  So I am rather proud of my accomplishment today and thank you pressures for staying good whilst this was going on.

Pressures

Talking about pressures as you know I have the cardioMEMS machine.  I thought I was doing pretty well, I really did so it came as a shock whilst we were on holiday to be rung by a member of the team and be asked to make an appointment to see Alex.  It appears my pressures have been steadily rising for some time.  Strange how today must have been good for me to do the job that I did.  Yes I do get  tired a little earlier, I do puff a little more than I did and yes that annoying cough is showing its ugly head a little more but generally I have no complaints.  Anyway the top and bottom of it is that I have an appointment at the Northern General in Sheffield on the 12th September.  

At the same time I attend this appointment  I believe we are talking about two trials and hopefully one that just might be ok for me to join.  I know many of you  - like myself - are still waiting for Nick Morells trial from Papworth but I have heard nothing about it being that one though I will of course post if it is.  Well of course I will post no matter what trial so watch this space.  

Looking ahead

Loads of lovely things to look forward to which I believe gives me a huge boost.  Our niece is coming to stay, though just for a couple of days. I have a lovely ph friend called Paula Joanne Smitham and her fab hubby coming to stay for a few days in September too and just maybe, in the offing a friend who lives in America might be able to come over and stay with us for a while.  We also have a holiday booked a little later back in Javea and have already pencilled in lots of diary dates to catch up with our many friends who live in this beautiful town.  So I will certainly do my best to keep on top of things, to stay healthy and well.

Ah there goes the bell.  I am hoping it is a small package containing the sheets that are now used on top of the barbecue grill. Chris uses them next door.  The food cooks well with the lovely lines on that come through from the grill and no need for a messy clean up.  You just wash the sheet or sheets out with hot water and put it away until the next time.  We were amazed when we saw him using them and for sure the worst part of any barbecue is the clean up.  Now it will be made so easy we might use ours much more than we do.  

Izzy

Well now she is home tomorrow I will have her pick her first apple off our tree, pick her first strawberry and cut green beans for tea. I want her to learn to enjoy the garden and I believe if there are nice things growing it just might encourage her more.  Colin is still to make me my raised bed to grow more but I have done ok.  When the bed is in I will ask her to help me to choose which fruits or veg we plant.  She loves her veg so we should get a good variety.

Right all for now, I made a curry for tea yesterday, yes even in the blistering heat and now we are taking it down to our friends house and will all enjoy it whilst sitting in his beautiful garden. As we are still without a lawn we cannot do that here yet.  I am so hungry as I have put myself on a diet since hearing my pressures have risen, maybe that might be the reason, who knows. Anyway we have also just been out to buy a water butt which seems to fascinate Izzy, I do not quite know why and that needs to be fitted today. We also purchased lots of crocus bulbs to plant as it is a perfect time once the top soil has been laid and she can do all the bending down to plant! Slave labour for sure.  Just maybe I might buy her an LOL if she does well.  

Take care, enjoy the sunshine and I will speak again after my appointment at Sheffield and let you know about new trials and my pressures etc.  

Warm love to all.

Carole xxx

STILL HERE - STILL PHIGHTING THE PH

Yes I have had another anniversary since being diagnosed, 9 years on!  To think I am still here, still phighting is rather incredible seeing as the specialist who diagnosed me could not promise six weeks!  Thanks to my amazing A Team here I am, nine beautiful years later.  Sure it has sometimes been hard, a tough road to follow or a high hill to climb but with their help I have done it and continue to do it.  I sometimes wonder quite why some of us make it so far along and others do not.  My heart grieves with every one lost to this incredibly horrible illness, I hate to see the pics of the new babies born with it, seems incredibly unfair. I am always so pleased and thankful we have such amazing specialists worldwide who go all out to give us a life. No we know the cure is not here yet but I always have the hope, if not for me then for those that will come afterwards - one day!!!!!  So 9 years on and I am so blessed to still be here, still making memories.  

Busy busy busy

Not all bad kind of busy though for sure some of it. The dratted fox still comes, though it did stay away for a while when I laid down a cocktail of hot peppers and garlic.  Sadly the smell wears off and back the thing comes to burrow under my well tended plants.  I have had Colin peeing into containers and putting it around the plants as it does say it can put off a fox as they are territorial, not this one though.  It appears we could put up a huge fence to keep it out and block all holes etc.  Hate the thought of this though as it will also spoil the views and we have incredible ones from everywhere in the house.  I could not bear to look out and see fences all around me.  Well we shall see what we do next when we have had time to look online again and make decisions.  I have read that Lion Poo is a great deterrent but sadly not too many lions around our parts!  

We have just been on two holidays which has taken up much of our summer so far.   Our first one was to Cambridge where we stayed on a site in Houghton, owned now by the National Trust.  What a fab place this turned out to be with a beautiful river flowing through the grounds.  There is still the old flour mill and it is put to use on certain days of the week. It was very interesting to look around it.  Thickets abound this place and lovely shaded walks are the order of the day.  A really nice one takes you into St Ives, no not the one in Cornwall but a beautiful small town and other small villages are very definitely worth seeing. Houghton itself has  much to offer, with two great pubs and beautiful thatched cottages that really do deserve to be on the front of chocolate boxes.  We thoroughly enjoyed our stay here and intend to go again when we have the time.  I love Cambridge as my family from the year dot were all from here and there is much for me still to find out about my family tree.  

Yesterday we returned from a cruise around the Med, we took Izzy with us as she loves cruising.  On our way down to catch the ship we called in at Poole for a day to see family, it was the first time Izzy had met her two cousins and they had a great time playing in the garden while we all caught up on events.  The weather was very good to us, in fact too hot even out on our balcony so often you would find me in our air conditioned room after we arrived back from our trips to the places we stayed at.

Our first Port of call was Gibraltar and we managed to get a picture of a family of Apes up on the rock.

Izzy was reluctant to get off the ship and would have preferred to stay on it at all times but we did manage to persuade her by offering her many games of golf once we got back on board.  She did get very proficient at this and often beat the pair of us hands down.  She refused to go to the children's club other than for the 20 minutes trial we said she must do. She hated it. So it was all granddad until typically the last few days where she made a lovely friend of a girl called Olivia.  This was thanks to Olivia having a ton of L.O.Ls so they spent time playing with them and touring the ship, playing games etc.  

My amazing cardioMEMS machine travelled well and each day I plugged it in and did my bit so that my specialist Alex  and or team could read my pressures. Some days the results would send easily, others not but they did all go off as soon as we reached a port with a strong enough signal.  As I never heard anything from my medical team I of course assumed all was well and so I hope this pattern continues.  I do wonder if one of the reason I remain fairly stable is because of the denervation I had. Maybe microwaving part of our small bronchi is the answer for stability. Only time will tell but Alex did say that there were a couple of trials I might be interested in so I am looking forward to getting his call. As you all must know by now I am always open to trials or studies.  One day the breakthrough will come.  

I did do my best to not overeat and we were both pleasantly surprised to see our weight has remained the same as when we boarded the ship.  We both exercised daily with Colin heading off to the gym first thing before breakfast, well he does it at home so it must continue whilst on holiday.  I know that for me I need to walk.  If I do not I feel terrible with this ph so I would go up on the top deck and walk the laps - 9 laps equalled one mile.  I would always have the goal of 40 laps.  Sometimes I walked with people but mostly on my own as I prefer it that way.  

Izzy ended up on TV!  Well only the ships television as on day two of our holiday her new swimming top that protects fro UV rays went missing. Colin had taken it off her and put it to drip dry on the shelf in front of our seat.  We bundled up our things to return to get ready for our evening meal.  As soon as we got in Colin realised he had left the top and went back for it.  It had gone.  Though we tried daily to see if it had been  handed in it sadly was not.  Chlo the entertainment host did a small piece with her in front of the cameras.   

Izzy was dressed in all her Spanish gear we had just bought her and looked so cute.  Chlo had her talking about her holiday,  and so it went out on the tv.   This is a channel where all the news of what is happening the following day are shown.  Her top was never handed in.  It did seem strange to me that it was taken and left a bad taste in our mouths for the rest of the holiday.  

We watched a show each night with the Ice Skating one being the most spectacular.  I must say the shows on board this particular ship were not the best, I have seen some that would eclipse broadway on other ships but non the less they were entertaining and only lasted 50 minutes, long enough to sit still in one place.  

When we go away I do stay off social media mostly as it is nice not to bother with the computer.  I did wonder though how people were faring and I do hope everyone is doing ok.  

Once again we returned home to a spic and span home but not sadly outside due not only to the fox but also the weather!!!  My goodness our garden was hit badly with it and despite a friend coming up to check it over it has taken all morning to get things back in order and a trip to the tip with all the rubbish from the dead heading I had to do. Colin dug up two potato plants that I had simply planted and did not bother to hill as I just wanted to see if they grew.  My word just the two measly plants, not tended or cared for, not hilled etc came up trumps.  I do dread though just how many we have in the potato sack that I lovingly tended.  We do not eat many spuds so I do believe I will be giving quite a lot away.  I am just leaving these out for a day to begin to settle and then they will go into a dark place until we require them.   

It was lovely when we were at sea not to bother with the papers and not to see all the terrible things happening in the world.. Back to reality to read that a 17 year old boy threw a 6 year old boy, (unknown to him) over the rails at the Tate Gallery where he landed 5 floors below on the roof of a building. Had he fallen the whole ten floors he would have been dead for sure. This poor boy was being held in his mothers arms when this young man came and pulled him away and hurled him over the rails. What on earth is happening in the world today.  What with that and shooting going on and knife crime it is sad that we have to live like this. We had two weeks shutting out all of this and it was bliss - now we are back to reality.

Izzy is spending the week at Stage School to give us a much needed break. As she has never attended  one before it should be interesting. We are invited to a show at the end of the week so will see how that goes. It does surprise me when I watch her now as gone is the timid little girl we once had and now she is a confidant girl and happy to put forward her own ideas and put her own stamp on things.  

(An add on to that paragraph as I have since been round to see her and Danielle told me the head of the school took her on one side and said very quietly that she does not normally offer places to children so quickly but she was so impressed with Izzy she has said she can join if she chooses, not just holiday time.  Izzy is a bit wary as she said they are going on holiday, - the school  - next year to Disney and she does not want to go.  Of course she loved Disney when she went with her mom but away from her mom is different.  I think she might change her mind in due course. 

Anyway we are back to reality now and instead of the sound of the waves lapping against the ship we have the cows lowing in the field opposite. Sometimes it can be sheep and sometimes cows. I did miss this sound when we were away.  Now back to hospital visits, blood tests almost weekly etc but also the fun singing group I have joined and the Pilates class that so far is helping with the pain in my neck really well.  I do believe that both these groups have benefitted me, expanding the lungs with the singing and the exercises in the Pilates to stretch the muscles and bones.  

I am a great believer in exercise and these two classes suit me so well, plus all the gardening which has now produced our first crop of potatoes.

Well, all for now, much to catch up on.  I shall try to read what is happening on the ph sites now I am back.  

Much love to all, stay well and stay strong.

Carole xxx