Paris

Just back from Paris!   We had amazing weather - although seem to have missed the great weather in Scotland while we were away.  Never mind - can't complain because we got lots of sunshine and had a lovely time.   Our hotel was in the latin quarter about 10 mins from Notre-Dame.  We did a lot of wandering just taking in the sights and crammed a lot into our time.  


This is The Louvre entrance.  It is so quiet because they had cordoned it off as we arrived and it was surrounded by police.  Then a little robot came trundling out of the entrance and we were allowed to start queuing - guessing it was checking out a bomb alert!    The Louvre is massive and you would need days to go round it all, so although we spent a few hours, we still only got the high lights.

The Mona Lisa - the tiny painting on the far wall!  It is surrounded by crowds although you do get a chance to get to the front and have a good look....through all the bullet proof glass.  It has to be done though!

This picture of the entrance was taken from the cafe inside the Louvre, where you can sit out on the terrace.  It was about a fiver for a coffee but the view was nice!

This was from the tiny balcony of our hotel room - 6th floor in the roof!  It was very parisian (e.g small rooms haha) but very clean and friendly. and a decent location.

Stairs in the hotel.  The lift was off one morning and we had to walk back up after breakfast.  Thank goodness for working lungs!

The Notre-Dame  - very impressive.  We walked past most days as it was near the hotel, so saw it from every angle by the end of the trip.  We went inside to see the lovely stain glass windows.  The gardens around it were also nice and there was a brass band playing one day we were there.

The arc de triomphe!  I have climbed to the top of this before with my friend Gillian so we gave it a miss this time -especially because it was about 29c that day!

We didn't go up the Eiffel tower either - one of the lifts was broken and the queues were ridiculous.  I have been to the top before and  although its a good view, it's kind of busy and hard to move up there!  We got lots of pictures of it from various points in the city, but I liked this one.

My favourite trip to the menagerie of course!  I remember going to this 13 years ago but don't remember it being as well laid out back then.  The statues were there and, in fact, I have since discovered I have pictures of me hugging/sitting on them 13 years ago as well haha.  However the zoo part did seem much cleaner and nicer this time round.

Red Panda

 Hungry hippo!

 

 

We spent a day in the Monmartre area.  At the bottom of the hill is the famous Moulin Rouge, which still holds shows.  I stopped myself from doing a can can dance here, but had to at least pose.  

Lots of lovely streets winding up towards the Sacre Coeur.  We walked up the street where the film Amelie was shot (if you haven't seen that film, you must!)  Cobbled roads and old buildings, then the square where the artists used to gather.  Now it is packed with artists who will paint your picture or sell you some art, although it was really too busy for us to look properly that day.  

The Sacre-Coeur, standing at the top of the hill, with a view from the steps below.

We went on a few wanders round the streets and would stop in the many parks and squares to watch the world go by and eat our lunch. The building behind is the Palais de Justice

Andrew reading his kindle..

This is from the hotel balcony one evening as the sun was setting.

On one of the bridges there is a tradition for couples to write their names on a padlock, put it on the bridge and throw the key into the Seine.  We decided to put one on - here I am with it!  I decorated it with our names, the date and pictures of my favourite animals (that would be a bird, panda, owl, hedgehog, tiny seb....!!)   Maybe it will still be there in 10 years and we can go and find it...

Paris at night - the Eiffel tower is lit up, with a search light sweeping around the top.  Every hour the lights all flash and do crazy wee patterns.  You really need a tripod for these kind of photos but this gives you an idea.

This was the musee d'Orsay, which is a converted railway station.  It had a lot of the Impressionists paintings that Paris is so famous for. We also went to the Salvador Dali museum, who I find fascinating with his surrealist work.   There are hundreds of museums in Paris but maybe those are best saved for a trip when the weather isn't quite so nice!

 Although we decided not to go up the Eiffel tower, we did go up the Montparnasse tower which has 360o viewing platform with amazing views over the city.   Much shorter queue and a better view!

Back to reality again and today has been rather damp and grey.  I do feel like I have been away for ages though because we fitted so much in!  Work tomorrow but then it's the public holiday on monday and tuesday so I can wait until next week to wind back up properly.

 À bientôt!

Memories Day

This weekend we were at the Day of Memories in Perth.  This is organised by the Butterfly Trust as a day to remember all those we have lost to CF.  Families are invited to attend and there is a service with some music and readings, followed by lunch to allow people to chat and mingle.  The Butterfly Trust has a book where you can write your memories  - they have started a new one this year for their 10th anniversary but the old messages will still be kept in the first book.  Looking back it was strange to see a page mum and dad had written in memory of Claire and both myself and Frances Ann had signed it - really strange looking at Fran's best writing in the book (very different from her usual writing!)  Then there was an entry from a few years back to Frances Ann that we had all left messages on, which was quite difficult to read but sentiments that still ring true.

Mum was heavily involved in the organisation and I was so proud of the work she has done!  She had chosen some beautiful music and readings, everything being very appropriate for the day.  During the service we also lit a candle for each month of the year, where we could remember a birthday or anniversary and mum read out some lovely words about each month.  Both myself and Andrew read, dad looked after the slide show and music and Carol Ann sung the psalm.  We like to get the whole family involved :-)  I had the idea of making some butterfly charms from fimo so that we could give one to each family in memory of the person they had lost.  They were red and yellow to match the butterfly trust logo and I mounted them on card to make them look more professional.   I think people liked having a small token to take home.

Yesterday we had a Sunday brunch with friends who are moving away from Glasgow,which was a nice way to spend the morning.  Then I was organising wedding favours for Sarah's wedding but wont say anything about that yet!!  I have got shoes to go with the bridesmaid dress - they ended up being too loose around the strap so I have made some adjustments with velcro - haha - and it actually works really well.  I am getting my hair cut tomorrow so will ask the hair dresser (who will be doing the wedding hair anyway) about some ideas for the wedding - I will definitely have my hair up as I think this would suit the dress best.  Only about 5 weeks until the big day!  My friend Nicola is also getting married later in the year and I had the excitement of going to pick up her dress with her last week...my lips are sealed!

Cystic Fibrosis Week

This is national CF week so you may see more in the media this week and the CF Trust have a web page with information and stories here.  They have a good video talking to some people with CF (with the lovely Kerry, who is an ambassador and poster girl for LLTGL!)

I have previously written a post about CF so will shamelessly reuse this - read here - the only change is a good one because the statistic for life expectancy is now 50% of people living beyond 41.   Sadly that does still mean that 50% are still dying in their teens and as young adults, but the treatments are ever progressing.

Gene therapy is something that always seemed to be just around the corner - when I was born we were told it was 5-10 years away.  That was 33 years ago.  However there have been many advances in this area since the CF gene was identified in 1989.    The basic premise is to replace the faulty gene in the lungs with a working copy.   The studies so far have looked at inhaling treatments which carry the correct gene but it has proved difficult to find the safest more effective way of transporting this into the lungs.  Studies so far have focuses on identifying this and ensuring there are no serious side effects when patients are given a single dose.  The current study is for more than 100 people with CF, some taking the active gene therapy product, some taking a placebo, once a month for a year. The results will then be analysed to see if patients show improvements in their lung function. If the trial is successful the aim is to then find a pharmaceutical company to take the product into a phase 3 clinical trial from 2015 onwards.  If effective, this could help limit any damage to the lungs caused by the gene defect and increase life expectancy to near normal, although other aspects of CF would still remain.

Alongside this, many new treatments are in the pipeline.  Various studies looking at ways of making the faulty CF gene work more effectively and another focus is on medications that might treat the basic defects e.g in CF this is related to salt and water movement across cells.   In the past the focus has been on treating infection, then a move to more preventative measures  - the focus now is on trying to correct some of the cellular changes that cause the sticky mucus in the first place.  These treatments could make vast differences to those born with CF today and help to prevent progression in those already living with this condition.

Sometimes I wonder how on earth I fitted in all those treatments before my transplant, but I guess you just do.  You just learn to cope as best you can and get on with it!  I don't think people realise that CF is a multi organ disease, as the focus does tend to be on the lungs primarily.  Despite my fantastic lungs now I still suffer from the other effects - diabetes and the need to take insulin and digestive enzymes with everything I eat, because my pancreas is damaged,  the CF arthritis where joints become acutely painful and stiff, the chronic sinusitis/pain because the CF mucosa still lines my sinuses, the osteoporosis because CF causes malabsorption of the necessary vitamins for bone strength,  the horrendous stomach cramps when I don't get my enzymes quite right and just the tiredness of your body working hard to overcome all these difficulties. 

I do however count myself so so lucky - I have many friends still fighting ALL these issues and at the same time struggling to take a breath.   They still face the prospect of transplant and the fear that it might not be an option.   I can cope with all the add-on's of CF because I can breath and laugh and live.  I know how hard it can be thought because I have been there.. so for those not so lucky please remember how devastating this illness is and spread the word, raise awareness and ensure that CF becomes a treatable illness, not a life threatening one.