This is my last blog from this side of the Equator. 

Update on our fundraising for Benjamin Revell;

We raised $520 for Benjamin and Malia’s daughter.  And, it is with great joy I can say that “Luisa Rose Revell” was born this week :)  Congratulations to the new parents.  Exciting times!

Sophia has gone back to the shelter, but she is to be adopted by friends of mine in Wisconsin.  I’m so happy about that!  I know that Sophia will have a wonderful home with Debbie and Cliff.  I met this lovely couple when I went to San Antonio for the Pompe conference last year.  I think Sophia will be perfect for them!  Don't foget to send piccies to Aunty Allyson :) 

This week I had my final infusion in Florida.  What a sad time it was to say goodbye to the wonderful people who have taken such good care of me during my time here.  We had a pizza party in my room while I was having my last infusion.  They bought me a lovely cake with a gator on it, and some lovely gifts to take back to NZ with me, including a breeding pair of squirrels.  Now I just have to work out how to sneak those through customs… maybe one down each…hmmm nah…

George brought along some wine which Russ hooked into.  George wrapped the wine himself in a classy brown paper bag!  Apparently it's also classy to drink it from the bottle while it is still in the paper bag.  Thanks for the heads up George, I'll give that a crack!  Notice the name of the wine?  *sniggers*  Thank you George, that's going back to NZ with me!  I've also got some Cheer Wine to take home.  No it's not an alcoholic beverage!

I’m really going to miss “all ya’all”!  Did I say that right Betsy??  The girls have been trying to give me lessons on how to speak with a southern accent.  I don’t think I ever really got the hang of it!  I have made so many good friends here.  I really never expected that to happen at all.  I guess I don’t really know what my expectations were, if any.  I was excited to be accepted to come over for the trial, and that was pretty much all I had in mind.  To have met so many people who were so good to me, was the icing on the cake.  I have enjoyed my time here immensely.  To say the American people are awesome is an understatement.  Kind, warm, caring, polite, fun and truly giving are just some of the things that come to mind.  Thank you all, thank you.  I really hope we meet again.

Tomorrow (Sunday Florida time) I leave Gainesville and head to Orlando with Katie.  I have an early morning flight Monday to LA so it’s easier to stay in a hotel at the airport.  I fly out to LA and get to spend the day there with those crazy California girls before I ship out to NZ later in the evening.  Then I have a 13 hour flight to Auckland where my son James and his girlfriend Rochelle will meet me for breakfast.  Following that I hop a flight to Palmerston North, where Steve and the boys and Pops will pick me up and take me home.

I’ll do another blog from NZ.  Thank you to my avid reader for reading this!

See you on the other side...

                                              Fundraising for Benjamin Revell

I’ve put an auction up on my Face Book page for a service to each of my miniature stallions.  Another 3 friends have also offered the same.  Isn’t it wonderful how people come together at a time of need?  We hear so many negatives in the news and various other outlets.  I’ve often thought it would be great to have a “Good News” channel on TV where you could see wonderful things, rather than the horrible things we are subjected to on a daily basis.  Anyone wanting to bid on these boys please do.  It’s for a great cause!

http://www.facebook.com/allyson.lock?sk=notes

Thinking about wonderful things...  I have to share with you one of my favourite web sites.  Not only because it is MEGA CUTE, but because it is an absolutely wonderful display of beautiful people, working with beautiful animals, to help those in special need. 

http://www.horse-therapy.org/

And if you need extra cuteness overload, check out Baxter the cat! 

Baxter works with the horses at Gentle Carousel's Reading Is Magic literacy program.

He shops at “Build-A-Bear” for his snazzy outfits.  One person on facebook said, when looking at Baxter’s photo in his fur ensemble, “He looks like my Great Nan”!  LOL

Actually if he was wearing pearls and red lipstick he would look just like my Nana too!

I think I need to make a trip to “Build-A-Bear” before I go home.  We have 4 cats…  :)

Which leads me to Sophia.  She is still with me, but going back to the shelter on Wednesday.  

I'm trying to get her to Wisconsin.  Is there anyone reading this who is heading to Wisconsin from Florida soon??  Some friends up there are keen to take her. 

It would be fabulous to know she’s gone to someone whom I know.  She’s a really awesome cat.

Next week is my final Florida infusion.  I have 3 days where I have to be at the hospital for testing.  There are 2 walk tests this week!  The one I am most keen on doing is my breathing test.  I had increased my lung function by 5% at my last test.  That might not sound much right?  But if you think about it for a minute…I have a progressive illness, my lung function was decreasing at a rapid rate before I got here.  And now, not only has it stopped decreasing, not only has it stabilised, it has actually improved.  5%, it’s absolutely tremendous!

On my way home I will be laying over in LA for the day.  I’m meeting up with some friends whom I met in San Antonio, Sabrina and Alicia.  I’m a little worried those girls will lead me astray.  They’re wild!  And me being so quiet and all, well, let me just reiterate, I’m a little worried.  I hope I make my flight back to NZ!

I have my hotel and flights booked for Australia.  Someone asked me the other day if anyone was going with me for support…I thought that was kind of funny actually.  I said I’m in the US by myself lol.  Ozzie will be a doddle!  Hope I didn’t just jinx that!

Have a great week everybody!

Well that week went by rather fast!  Only 15 more days before I depart for home. 

This week I did my part in an exercise video.  Yes I can hear you sniggering!  Believe me no one is laughing more than me! 

This video is for those of us with Pompe disease, showing exercises which will benefit us in particular.  I’m not sure when it will be ready for the “In cinemas near you” announcement, but probably about the middle of this year.  Just kidding about the cinemas bit!  Although I see James Cameron of “Titanic” fame has bought a property near ours back in New Zealand.  Maybe he will have heard of me by the time I get back and offer me a part in the new Avatar movie.  I could be one of those blue people.  I haven’t seen the movie, so I hope the blue people are goodies. 

Actually that reminds me, years ago I was asked if one of my horses could be used in the Marlborough Man ads.  They liked his Appaloosa colouring.  I declined because I was worried about him being hurt accidentally.  I’m sure the Marlborough Man went on to sell many more smokes regardless!

A lot of people have used the word “lucky” when they refer to me being over here and seeing the wonderful things that I’ve seen.  I admit to being a tad miffed when I hear that word.  Then I contemplate it. 

I think, if I didn’t have this stupid disease I wouldn’t be here, so I don’t feel particularly lucky in that respect!  Wouldn’t I be more “lucky” if I was here because I decided I wanted a vacation?  Then I think, would that be luck?  No, that would be good management.    

Then I think, I really am lucky that I am here, that I have the opportunity to be here doing this medical trial, that there is a medical trial, and that there is hope for my future.  Although, we make our own "good" luck.  I went out there and grasped hold of it, so, it’s not luck that I am here. 

But, I am lucky, or should the word really be “fortunate”, to have had so many people who have helped make all of this possible.  Is it luck that I have such good family and friends, good neighbours, people who care and have helped us in so many ways?  I would say blessed, fortunate…

I just looked up the word “lucky ” in a thesaurus.  The words that came up are;

Fortunate, blessed, jammy, fluky, auspicious, propitious, providential, timely;

I guess that blows my argument out of the water!  I am LUCKY!!!

Here’s something I’m excited about.  This week I bought a battery for my bipap (breathing machine) so that I can now go on a plane without being at the mercy of airlines who won’t accommodate my need to breathe! 

Most chicks would be excited about shoes, I’m excited about a bipap battery.  Sad but true! 

Oh, I also got an “inverter” with it.  Whatever that is…  But all of this does come in a snazzy black bag.  I’m sure I can dangle something blingy from it to make it look even more snazzy.

This week I have “met” a young man who is serving in the Royal New Zealand Navy.

Benjamin Revell has been diagnosed with Motor Neurone Disease.  First and foremost Benjamin is a loving husband, and very soon to be Dad, to a new baby daughter. 

I would like you to have a look at Benjamin's blog (soon to be published) and try to gain an understanding into what life is like living with MND.  My husband’s grandfather had it, and my father was diagnosed with it (fortunately incorrectly). 

Benjamin you are already making a huge impact on those around you with your guts and determination, your spirit and your kindness toward others.  I hope to meet you some time!  I really like your style xo

Here's the boys out boogie boarding back in NZ yesterday.

See the lighthouse in the background?  This place is called Castle Point.

Sophia is still with me.  I’m so surprised she has not been adopted yet.  Is there anyone reading this who would like a beautiful black kitty cat that is full of love and purrs?  I said to my husband that I wanted to bring her back to New Zealand.  He said “no”!  What?  Sorry…I couldn’t quite make out what you said…

I have my first appointments already set up in Brisbane, Australia.  First infusion is the beginning of March, and every fortnight thereafter.  It will probably involve an overnight stay in Brisbane each time due to flight times and available flights in and out of where I live.

And for today's cuteness injection...

tadaaaaaaa!

I know!!!

Until next time, over and under.

Happy New Year to you all

My avid reader put in a complaint that I needed to update my blog.  So here it is.  There are only a few more blogs to go.  I leave the US on the 20th of February, arriving back in New Zealand on the 22nd.

Such a lot has happened since my last blog before Christmas!

Visits from Father Christmas, my oldest son James and his girlfriend Rochelle, my husband, children and father-in-law.  2 more infusions have gone by…

Where to start…

Christmas! 

I was invited to the Bedford house for Christmas dinner.  Such an awesome family!  They love Christmas as much as I do.  Thank you for having me over to your lovely house Bedford Clan, wonderful memories to take home with me!  Love you guys!

My son James and his lovely girlfriend Rochelle came to visit. 

It was fun spending time with them and showing them around where I have been living.  James is a sports nut so of course he enjoyed meeting Tim Tebow, well the statue of Tim Tebow! 

This was taken outside Ben Hill Griffen Stadium, affectionately known as "The Swamp".

Here we are in The Swamp.  Would love to see a game there one day!

Pretty cool for a college football stadium huh?

This family trip was given to us by some extremely kind people back in New Zealand.  I am going to write a thank you letter and send them some of these photos so they will know how much fun, and how many wonderful memories they have given to us. 

How can you even thank people enough for all of the kindnesses that have been given to us over the last year...hundreds of people whom we don't even know.  Pretty amazing!

Legoland 

What a great place!  So many things to see, and all so well put together...literally!  It took 2 years go construct all of this stuff.

The kids loved it.  They even got their driving licences.  Look out for them driving Lego cars around Masterton lol.

Tom and Jerry's Airboat Tours

Of course a trip to Florida would not be complete without seeing some 'gators out in the wild!



When I was a youngster I used to enjoy watching a program on TV called Gentle Ben (I know some of you will remember this!).  On this program they they used to cruise around the Florida Everglades in an airboat.  Since I first saw that program, I have wanted to do the same.  So, finally, I did it!  My husband, the 2 boys, Pops and myself went out on Lake Panasoffkee and did a tour of the headwaters of the Florida Everglades.

I can cross that off my bucket list.  Although I would love to do it again :)

More on Lake Panasoffkee here

Pompe peeps

I’ve met a couple more Pompe peeps this year.  Kerri and Addy.  Addy is affectionately known as “Addytude” and I am “Allytude”.  We need matching t-shirts! 

This is Addy on the left with her buddy Donald. 

It was also my pleasure to meet Addy's Mamaw (Grandma), and her Mum and Dad.  Addy's Mamaw and I share a love of horses.  It was wonderful to meet such a lovely family :) 

Here is the lovely Kerri, along with myself and the ever incredibly good looking and handsome Dr Barry Byrne (he does read this, right?) 

Kerri has introduced me to the delights of the Waffle House.  Thank you Kerri, I wish you had done this at the beginning of my trip!  lol

Anyway, Dr Byrne doesn't like us being called "lab rats" cos he said we don't look like rats.  I agree!  So i'm thinking "lab hamsters" or "lab squirrels"...they're cute!...see pic below for confirmation!

Anyway, enough of the fun and games and frivolity!! ... Just kidding :)

But, on a more seriousererer note...

Facebook seems to be the vehicle for allowing us to form wonderful friendships among the Pompe community and many of us have met in person.  Only another person dealing with this disease can ever really “get it”.  So to have this online community and to be able to share our lives with them is a wonderful thing.  It not only helps the patients themselves, but also the families who are also going through the difficulties of what this disease heaps on us all day every day. 

I LOVE YOU FACEBOOK!!!   mwa!!!!

I had my #10 infusion yesterday.  NUMBER 10!!!  Cor blimey!  It’s the last one before Christmas, and the last one for this year.  Only 4 more left to go!

As Sandra pointed out, I forgot to mention what happens after the last of those 4 treatments.  Thank you Sandra :)  Nice to know I have another reader lol.  xo

After my last infusion here in Florida, I will move onto the extension part of the study and have my infusions in Brisbane, Australia every 2 weeks, forever and ever Amen.  Or until there's a site in New Zealand, or a different treatment/cure is found.  But I don't mind at all having to do that, it's just great that I am able to keep on being treated!  And no I will not buy smokes for anyone duty free each time I cross the Tasman!  Just sayin'! 

Yesterday I met another lady who also has Pompe.  She is from Canada and is unable to get treatment funded in her province.  It’s always great to meet other people with this stupid disease.

I went out for dinner with her and her husband last night to a great Mexican restaurant.  It was a lovely evening talking about kids and their lifestyle in Canada.

This week I went to the store to top up my mobile phone.  I use one because it costs a fortune to phone from the hotel phone.  Mobile phone plans are very cheap here in the U.S.  Anyway, I digress.  The lovely man who served me asked where my accent was from.  I still always think that question is hilarious; I don’t even have an accent!!  Right?? 

I said “Take a guess, bet you can’t guess it”.

He guessed Australia, my reply “NO”!!…

”UK”?...

”Nope”…

“You’re right, I can’t guess”.

“New Zealand”…

“Oh you’re a KIWI”!

“YES!  How did you know about Kiwis”??

He said he was in the Olympics in 2000 and another one and met many Kiwis and they were “beautiful people”.  I asked what he did at the Olympics.  Turns out he lost a leg in a car accident when he was younger, and he was running in the 100 and 200 metres in the Paralympics…

Can you imagine that?  The guts it would take to do that?  In the face of total adversity, he kicked it up the backside and did it anyway.  Maybe I will meet him in a race someday...

 

On Saturday night I was invited to the staff Christmas Party at Dr Byrne’s house.  Who gets to go to their doctor's Christmas parties???  The gorgeous Katie and husband Dean picked me up and dropped me home again.  We had a great time.  I would love to have been able to dance along with the rest of the crazies! 

Some people also took part in the Xbox Kinects Sports games.  WOW!  That is so cool, and watching the people is very funny! 

There was a Secret Santa.  Very different than I have seen it done before, but totally fun!  If you bring a gift, you get a number.  At the beginning of the Secret Santa #1 goes first, picks a gift from the pile, opens it and sits down.  #2 then gets called and if they like the look of #1’s gift they can steal it.  If they steal it, #1 gets to choose again.  This goes on until the last person gets a gift.  Gifts can only be stolen twice.  The bottles of alcohol were quite popular where stealing was concerned lol. 

I think this will have to be employed at our Christmas get togethers back in New Zealand.  What do you Willow Parkers think???

I still have Sophia.  She will be with me throughout the holidays unless someone specifically phones up to adopt her.  I’m starting to get attached to her since she has now been with me for a while.  So if she doesn’t get a forever home soon, she may like New Zealand!  I love her, she has spunk and she is also SO loving! 

Actually she could be described as “awesomesauce”.  This is a new word that the gorgeous Lindsay has tuned me in to!  You know I’m coming back to New Zealand with all of this new stuff!  Including fried turkey, turducken, and a BEAUTIFUL marinade courtesy of the lovely Mrs Byrne, oyster sauce and garlic, this is for pork.  SO YUMMY!! 

More on turducken and fried turkey below!

Fried Turkey

Wash bird inside and out, and allow to drain. Rub turkey all over with House Seasoning. Coat turkey with dry rub. Allow the bird to sit until it reaches room temperature.

Heat peanut oil in a turkey fryer or a very, very large stockpot to 350 degrees F. Lower turkey into hot oil, very carefully, making sure it is fully submerged. Fry turkey for 3 minutes per pound plus 5 minutes per bird. Remove turkey from oil and drain on paper towels.

Full recipe instructions here

Since the next blog is due on Christmas Day, I will either have to be a day early, or a day later...maybe I will skip a week.  Sorry in advance to my avid reader ;)

It has crept up on me so quickly.  Christmas Eve here will be Christmas Day in New Zealand, I wonder if Father Christmas will know where I am.  I’ve been quite good all year…

1 more sleep until my big boy gets here, and 8 more sleeps until my little boys get here!!  I can’t tell you how much I am jumping out of my skin to see them!! 

To all of my family, who sometimes I don’t know how they put up with me!  To those friends and strangers who have supported us throughout this strange yet wonderful year and to all of the new friends I have made along the way.  To all of the people whom a year ago I didn’t even know existed, and have now had a huge impact on my life… thank you, thank you thank you.  Merry Christmas everybody, and blessings to you all.

To my fellow Kiwis who are still fighting for treatment in New Zealand for Pompe, let’s make 2012 the year in which we ALL get treatment.

 Much Love, Allyson xo

It’s blog day again!

11 December, the time is speeding by until my family arrives.  I have one more infusion scheduled between now and then, so I should be in good form to keep up with them all!  My breathing is feeling pretty good, I’m excited about that!  I'm so lucky to be here, I can't even tell you how much.

Sophia the foster kitty is still with me.  She’s so cute and friendly.  It must surely be only that she is a black cat that she hasn’t been adopted, because her nature is beautiful.  If she hasn’t been adopted by the time I go home, I think she may come with me.  Wait, does my husband read this??...ah well, he knows what I’m like lol.

Sophia getting into the Christmas decorations... I mean Christmas spirit!

This week I went to Disney World in Orlando with Monique who is another Pompe patient.  Monique was recently in New Zealand to help with the campaign for getting medicine funded for Pompe disease. 

Monique invited me to Disney to go to Mickey’s Very Merry Christmas at Magic Kingdom.  We stayed there overnight and went to Animal Kingdom the next day.  The Christmas party was lovely.  The decorations and the fireworks were just beautiful.  I felt lucky to be there and witness it all, such beautiful memories to last a lifetime. 

One of the things I love about being in America is the way they honour their troops.  Everywhere you see "Thank You" messages and other messages of support to the men and women who are in the military.  TV, the theme parks like Disney and Sea World.  Disney chooses a veteran to help take the flag down and when you see the proud face on that veteran, it really brings tears to your eyes.  At Sea World during some of the shows they ask the service people to stand up, then they put them on the big screen and everyone gives them a huge round of applause.  We don’t see this sort of thing in New Zealand unless it is a day like ANZAC Day. 

Animal Kingdom is fantastic!  We went on a safari ride in a truck.  Our driver had just the best sense of humour!  We went tearing around through rivers and dirt tracks, past cheetahs and crocodiles, rhinos and hippos.  So much fun! 

The Tree of Life is beautiful!  It has a whole range of animals carved into it.  See how many you can pick out of this photo! 

There are no prizes for guessing, because I don't know how many there are, so...

Monique and I at the Tree of Life

These naughty squirrels raid the baby strollers for food while the occupants are away on rides. 

How can you mind when they are so cute?!

I hope you've enjoyed this exciting installment of Allyson's Florida Blog :)
 Stay tuned for more next week! 
And if you haven't checked out the below link yet...you're missing out!

Norad Santa Tracker

It’s blog day again.  I’m sure I just did one yesterday!

I had my 8th infusion on Friday.  All went well as per usual and I slept through it as I normally do.  I could do with some of that IV Benadryl for sleeping at night time, it puts you out like a light!  A couple of people have mentioned my breathing looks a lot better, and I have been thinking that myself.  Sometimes it’s not so good, but I think it is improving.  Hope so anyway!  Breathing is a good thing for “aliveness”!!

Foster Kitties

In my last blog I introduced Blossom the lovely foster cat.  She has now been adopted to a forever loving home and I now have “Sophia”.  Sophia is just gorgeous and a total love schmuck!  She enjoys sleeping in sinks and detests having her claws clipped.  So if you’re looking for a gorgeous black cat who will smooch you until you can be smooched no more, try Sophia on for size! 

And for anyone who is superstitious about black cats…A friend sent me this saying;

"A black cat crossing your path signifies that the animal is going somewhere" (Groucho Marx)

See, that’s all it means!  It’s just going somewhere, just like the chicken which crossed the road, it was only going to the other side, simple as that!  Cast your superstitions aside and get a black cat, they're awesome! :)

Critter of the week

Iguana

I saw one of these sitting on the grass verge on the side of the road driving out to the Florida Keys.  We don’t get these in New Zealand, so to see one of these just sitting there on the side of the road was very cool!

...Ok I just did a google search on Iguanas, apparently they are not very cool!

Adult iguanas are large powerful animals that can bite, cause severe scratch wounds with their extremely sharp claws, and deliver a painful slap with their powerful tail. Iguanas normally avoid people but will defend themselves against pets and people that try to catch them or corner them.

Due to a combination of events, the Green Iguana is considered an invasive species in South Florida and is found along the gulf coast of Florida from Key West to Pinellas County.  The original small populations in the Florida Keys were animals that were stowaways on ships carrying fruit from South America.  Over the years, other iguanas were introduced into the wild mostly originating through the pet trade. Some were escapees and some were intentionally released by their owners; these iguanas survived and then thrived in their new habitat.  They commonly hide in the attics of houses and on beaches. They often destroy gardens and landscaping.  They seem to be fond of eating a native endangered plant, Cordia globosa and feeding on Nickernut, a primary food plant of the endangered Miami Blue Butterfly; additionally on Marco Island, Green Iguanas have been observed using the burrows of the Florida Burrowing Owl, a species of special concern, all of which can make them more of a serious threat to Florida's ecosystem than originally believed.

In January 2008, large numbers of feral iguanas in Florida dropped from the trees in which they lived, due to uncommonly cold nights causing them to go into brumation in which they lost their grips on the tree branches. Though no specific numbers were provided by local wildlife officials, local media described the phenomenon as a "frozen iguana shower" in which dozens "littered" local bike paths. Upon the return of daytime warmth many (but not all) of the iguanas "woke up" and resumed their normal activities.  This occurred again in January 2010 after a prolonged cold front once again hit southern Florida.

That’s a word i've never heard before…”brumation”.  Although i've also never heard of a "frozen iguana shower"!! 

The Painter

When I was in South Beach there was a man on the sidewalk painting tiny little paintings through a magnifying glass.  The paintings were of local buildings and people.  I bought one for only $20!  It was of a church in the same street, so I took a photo of the man doing the paintings, and a photo of the church itself.  It is a great memento of my trip to the USA.

Here is the website for the man who was painting.  If you’re interested in miniature art, this is well worth checking out.  The detail in the paintings needs to be seen to be believed, exquisite!

http://www.houseofcharm.com/

This is the church i got the painting of.  It has a beautiful stained glass window in the front there, but it doesn't show up well in this photo.

I love the architecture in South Beach!

And the countdown is on for Christmas. 

I LOVE Christmas!!

My husband is probably glad that I am not in town at the moment because the house would be filled with all of those wonderful Christmas songs being played from my countless Christmas CDs. 



I'm looking forward to seeing my family soon, James and Rochelle on the 20th and Steve, Ben, Josh and Pops on the 28th.  I still can't believe how lucky we are that wonderful people have helped bring us all together for the holidays :)  Thank you!!  I don't even know their names to be able to thank them personally.

Until next time... xo

Ok so last week I didn't do a blog and thought no one would notice.  But someone said "hey you didn't do a blog this week"!!!  I was worried people might have been getting bored with them and no one would notice if I missed one!  Sorry to my avid reader lol.

Last week was my halfway point in this clinical trial.  I did loads of tests, the same as I did when I arrived.  Pulmonary function, quantitative muscle testing, 6 minute walk test, chest x-ray, ECG, MRI.  We don't get to know what the results are.  So, neither do you lol.

Ok if you really are wondering what "quantitative muscle testing" is...

http://www.qmasystem.com/testing_photographs.htm

On Thursday I celebrated my first ever Thanksgiving.  I was fortunate to be invited to Katie and Dean's home to share Thanksgiving with their wonderful family and friends. 

We had turkey with stuffing and cranberry sauce, broccoli, beans, mashed potato, sweet potato (or kumara as we know it) which was mashed and had a yummy topping on it of pecan nuts and something sweet.  I will have to find out what that was!  I also need Dean's recipe for stuffing!

For dessert there was apple pie, pumpkin pie, chocolate puddings...I had pumpkin pie for the very first time!  It was yummy!

There were about 20 people there from all over the world.  America, Canada, Alaska, New Zealand, Germany...it was fabulous!  Lovely memories to take home to New Zealand.  Thank you Bedford family, you guys rock!! :)

Gobble Gobble!

Yesterday marked the last tailgate of the season.  I was going to go but then I had a phonecall saying that a special guest was coming to stay with me. 

"Blossom" is my new foster cat from the rescue centre.  I wish I had thought of doing this when I got here!  I have missed my cats at home, so I have foster cats now.  Yaaaay!  As you can see, Blossom has made herself quite at home :)

If you're thinking of something nice to get yourself for Christmas, how about a rescue cat? 

They come in all shapes and sizes, colours and fluff length.  There is a purrrrrsonality to suit everyone.  They're all "fixed" up so no problem with any unexpected population explosions, and they have a lot of love to share :)

Coral Castle

Now this is a very cool place!  On a trip to Miami and the Florida Keys a visit to the Coral Castle is a must!  

"Built by one man, Edward Leedskalnin. From 1923 to 1951, Ed single-handedly and secretly carved over 1,100 tons of coral rock, and his unknown process has created one of the world's most mysterious accomplishments. Open every day, the Coral Castle Museum welcomes visitors from around the world to explore this enchanting South Florida destination".

Ed built his creations for his lost love who left him the night before their wedding.  She was 16, he was 26.  Interestingly, and probably not coincidentally, there are 16 steps leading up to his bedroom atop his castle.  Rock star Billy Idol wrote his hit song, “Sweet Sixteen” about Ed’s lost love.  There are photos of Billy Idol taken right here sitting on the various coral "chairs" and "beds".

It was a real pleasure to visit such a place which was obviously built with so much passion.  I would have loved to have met Ed.

Daytona

Actually Daytona was a dive. 

The large sign says it is the "World's Most Famous Beach".  One really has to ask the question "WHY"??? 

One of the biggest things that spoil it is that all of the hotels are on the beach side of the road.  So as you drive along you only get a glimpse of the sea every now and again.  You are able to drive ONTO the beach, but not on this particular day because there was a very high tide. 

The upside to Daytona was seeing the racecar track.  E-NOR-MOUS!

Kennedy Space Centre

GO THERE!  Spent a whole day there.  Plenty to see, all of the history of the space flights.  Very very interesting!

They provide tour buses from the main tourist part of the complex out to where all of the launch pads and buildings are.  The picture above is where the shuttles launch from.  There is a building there which is so huge that it takes 45 minutes for the door to open! 

Seeing Saturn V there in all its glory was magnificent!

I remember when my brother and I were very small, Dad took us to see one of the modules that the astronauts landed back to earth in.  Well they used to land in the sea and then get picked up.  I just wish I could remember which one it was.  It was probably one of the ones I was looking at the other day! 

South Beach, Miami

GORGEOUS!  Didn't see Horatio, but I had the feeling he was going to pop out from behind a palm tree at any moment.  And there are a LOT of palm trees!!

I've never been a fan of art deco, which I will be lambasted for since I come from a city called "Napier" which is known as the "art deco capital of the world".  I have to say though that the art deco in Miami is so much nicer!  The city is very clean, the people polite, shopping is as cheap or as expensive as you like and there is something there for everybody. 

The mannequins have "fake boob" sized chests and extremely skinny waists lol.  Which is kind of indicative of a lot of the ladies you see around there.  Just not me!

There were suprisingly cheap hotels there too.  My room was in a very old hotel but it was cutely quaint, cheap, clean and the service was excellent.  I think it was around $60 for the night and included breakfast.  It was central to everything and in walking/scootering distance to the nightlife, shopping, beach, bars and eateries.  I had my first ever Cuban meal.  Did you know you can eat those yucca plants that are growing your yard?!  I didn't!  I think it was boiled, and then sliced and coated in lemon butter with parsely.  It looked a little like sliced up apple and tasted a little like boiled potato. 

Thinking about cheap hotels...This is my new friend from Titusville. 

He was there to greet me when I woke up after my first and LAST night at that hotel.  When I say "cheap", I mean CHEAP and NASTY! 

"Days Inn" Titusville, give it a MISS!



Florida Keys

I'm not sure I got the best out of this place.  I think I need to go back, but do research on it beforehand so I know what is there.

Sunset over the Florida Keys.  Just gorgeous :)

And another week gone!

This week I want to include some information from the conference in Texas.  It won’t be too scientific...I hope!

There were about a dozen topics covered, but I will just go into a few here.  The following contains some extracts from the conference program and also some of my own thoughts and observations.

History of Pompe

Pompe was discovered in 1932 by J C Pompe.  Improved understanding of the nature of Pompe disease has led to the search for treatments, which so far has led us to enzyme replacement therapy (ERT).  The scientific and industrial development of ERT is intertwined with the growth of an international Pompe community.  We now have patients, scientists and industry working together towards a common goal.

What is Pompe?

Pompe disease is a rare, inherited and often fatal disorder that disables the heart and muscles.  It is caused by mutations in a gene that makes an enzyme called alpha-glucosidase (GAA).  Normally, the body uses GAA to break down glycogen, a stored form of sugar used for energy.  But in Pompe disease, mutations in the GAA gene reduce or completely eliminate this essential enzyme.  Excessive amounts of glycogen accumulate everywhere in the body, but the cells of the heart and skeletal muscles are the most seriously affected. 

Newborn screening

There was a large-scale newborn screening pilot program conducted in Taiwan from October 2005 to March 2008.  The screening involved measuring GAA activity in dried blood spots (This is how I was diagnosed last year).  6 newborns were classified as having infantile-onset Pompe disease and were treated within a week after the diagnoses.  They all demonstrated normalisation of the cardiac size and muscle pathology with normal physical growth and age-appropriate gains in motor development.    13 newborns were also assumed to have later-onset Pompe disease and are being monitored. 

Almost all patients with Pompe disease have been misdiagnosed for years and years because even though the clinical presentation of Pompe disease is well described despite the disease being so rare, most doctors are unaware of the disease because of its rarity.  Misdiagnoses are also often caused by the use of sub-optimal diagnostic materials and by misinterpretation of the diagnostic results. 

Newborn screening reduces the role of physicians in the diagnostic process as the screening activity identifies the disease before the onset of clinical symptoms.  It is well established that the earlier treatment begins, the better the outcome for the patient. 

With misdiagnoses happening most often, and lack of treatment available in New Zealand, we are clearly in need of catching up with the rest of the world in the way we approach this disease and spreading the awareness of it.

Gene therapy (my favourite topic!)

Some very clever people have been doing research into the potential to treat Pompe patients via gene transfer based therapeutics.  This research is ongoing and looking extremely promising for those of us with Pompe disease.  Gene transfer has been done directly into affected muscles.  However muscles more distant from those were not corrected.  So, in come the Gene Transfer Technologies which can allow correction for potentially ALL the affected muscles in a Pompe patient.  In fact, it has been confirmed that a simple intravenous delivery of gene transfer vectors resulted in high level transfer of the gene into the liver.  The liver sustained protein secretion into the blood stream over long periods of time.  This resulted in long term correction of glycogen storage in multiple muscle groups.  Furthermore, studies have verified for the first time that muscle strength could be improved rapidly as well as preserved long after the initial injection of the gene transfer vector occurred.

At the end of the conference we had a round table patient discussion led by Maryze Schoneveld van der Linde, a patient from The Netherlands.  During this discussion we were able to talk about anything we wanted to regarding how Pompe effects our lives.  It was a wonderful opportunity to hear other people’s perspectives on how they deal with this disease.  Doctors also remained present and were interested to hear the patients’ views.

Meep Meep

Last week the OZ and I went to a rib joint for tea and look what we discovered!  Of course we just had to have him!  To win him we had to name 5 body parts above the neck with 3 letters.  We could only come up with 3 off the top of our heads and under time pressure.  But “Magic Mike” gave him to us anyway!  And no I’m not telling you what the 5 are, you work it out.  There are also 5 below the neck, and no I’m not telling you what they are either.  Actually there are 7, but 2 are rude and you don’t get any bonus points for those!

Thinking about body parts, I got my nose pierced!  If you’re thinking about doing that, and think it will be painless, think again!  I got a little tiny pink bling stud in the side of my nose.  Donations were being made to breast cancer awarenes.  I thought it was a great cause as my Mum and my Mother-in-law both died of that terrible disease.  I bet they heard me swear from up there in Heaven too!  Sorry ladies! 

These guys are EVERYWHERE!  They're very cute too.  Now that the weather is cooling off they aren't quite as prolific.

Brown Anole

A long, slender, brown lizard, males reach 20 cm (8 in) long, females are smaller.  Males have yellowish spots on the back, a ridge down the center of its back, and an orange to pale-yellow, white-edged dewlap or throat flap.

Brown anoles were introduced to Florida from Cuban and the Bahamas and are now found throughout the state. They thrive in disturbed habitats, among ornamental plants and are common around buildings.  Where brown anoles coexist with the native green anoles, you will usually see the brown anoles on the ground and the green anoles on the upper trunk and in the canopy of trees.

Green Anole

Green anoles are medium-sized lizards with long tails. They are the only anole native to the US.  Sometimes called chameleons because of their color-changing ability, they can be anywhere from emerald green to brown or gray.  When stressed, they turn dark brown. Males have a pink or red extendable dewlap or throat fan. Males often engage in lateral displays that includes head bobbing and pushups. The toes have adhesive pads on the undersides.

Green anoles are easily tamed and are common in the pet trade. They are active during the day, often around human habituation, and regularly bask head down on tree trunks, fence posts, decks or walls.

This Tuesday marks my half way point in the clinical trial.  I will go to the hospital at 8am, do all of the testing including MRI, muscle strength, pulmonary function test, 6 minute walk test.  After all of that I will get my infusion and stay overnight for testing.

I just can't believe how fast my time here is going.

I have had no bad side effects at all.  Although I feel like I'm getting a lot shorter.  It could just be my imagination.

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