There goes another week!  Time flies! 

I still have more on Texas to talk about :)

One of my favourite things about visiting San Antonio was going to the Alamo. 

What a great place to see.  The thought of all of those famous names standing, and falling, right there where we were, all those years ago, was pretty awesome. 

Two of the most recognised names of course;

David "Davy" Crockett (i'm going to have that song in my head for the rest of the night now...king of the wild frontieeeeeer...) was one of the most famous figures of his day. Born in Tennessee in 1786, Crockett had many adventures in his youth as a frontiersman and military scout. In the 1820s, he entered Tennessee politics and eventually served two terms in Congress. His reputation as a sharpshooter, hunter, and storyteller grew with his success, and many fanciful accounts of his life were published, both by Crockett and by those seeking to capitalize on his fame.

By 1835, Crockett had become disillusioned with politics and set off to explore Texas, departing Tennessee with the famous quote: "You may all go to hell and I will go to Texas." Crockett fell in love with Texas and joined the volunteers in the fight for Texas independence. He died at the Battle of the Alamo on March 6, 1836.

James "Jim" Bowie, a 19th-century American pioneer and soldier, played a prominent role in the Texas Revolution, culminating in his death at the Battle of the Alamo. Stories of him as a fighter and frontiersman, both real and fictitious, have made him a legendary figure in Texas history and a folk hero of American culture.

Born in Kentucky, Bowie spent most of his life in Louisiana, where he was raised and later worked as a land speculator. His rise to fame began in 1827 on reports of the Sandbar Fight. What began as a duel between two other men deteriorated into a melee in which Bowie, having been shot and stabbed, killed the sheriff of Rapides Parish with a large knife. This, and other stories of Bowie's prowess with the knife, led to the widespread popularity of the Bowie knife.

Bowie's reputation was cemented by his role in the Texas Revolution. After moving to Texas in 1830, Bowie became a Mexican citizen and married the daughter of the vice governor of the province.  In January 1836, he arrived at the Alamo, where he commanded the volunteer forces until an illness left him bedridden. Bowie died with the other Alamo defenders on March 6.

We weren’t allowed to take photos inside unfortunately.  It's also a big no no to touch the walls!

This is a photo I found on the internet to show what the inside looks like.




I like this following website which shows how the Alamo has changed over the years.

A L A M O  I M A G E S
CHANGING VIEWS OF THE MISSION SAN ANTONIO DE VALERO

http://www.tamu.edu/faculty/ccbn/dewitt/adp/history/alamo_images/images.html

I have introduced Russ in a previous blog along with Betsy, one of my nurses.  Betsy bought him his Gators outfit. 

Here he is again at the Alamo;
Can you find him?






While we were walking around outside the Alamo looking at the sights, a lady commented on my “New Zealand” tee-shirt.  I said “I’d recognise that OZ accent anywhere”!  Turns out she was from Hamilton! *palm smacks forehead*!  She was with her family, and had driven from the West Coast and was heading to Florida, having a driving holiday.  So I had a yarn to her for a while.  I wonder how many other Kiwis I pass without knowing it. 

On the way back to the hotel after our Alamo visit, I fell off Road Runner!  What an idiot!  I was going up a gutter, it had rained a LOT the night before and there was a big puddle that I ended up in.  Fortunately I was with some people who picked me up off the ground; thanks Theresa, Tim and Brad!  There was no way I could have gotten out from underneath the scooter by myself.  I'll have to get a helmet if I keep that sort of thing up! 

And talking about rain in Texas, I have never in my life heard thunder like they have there.  When they say everything is bigger in Texas, it is certainly true of a thunder storm!  It rumbled and cracked like there was no tomorrow!

Thinking about tomorrow, I have my 6th infusion.  I have noticed that my pain is lessening a lot and I even managed to roll over in bed one morning without having to pull myself up with my blankets or sit up to turn over.  This may sound like nothing to a “normal” person, but to me it is a LOT!  It’s only happened once, and it’s early days, but it’s an improvement!

Until next time, hope you all have a great week :) 

A BIG Happy Birthday to my son Ben who was 10 this week :)  10 years has flown by. 

What a lucky Mum I am to have 3 lovely sons.  You make my world wonderful.  Love you love you love you xoxoxo

Hi y’all!  A little bit of Texan speak there…

I’m slowly coming back down to earth after the conference in Texas.  I’ve been so busy with a new exercise routine plus the ongoing campaign back in New Zealand to get treatment funded for Pompe patients.  The Prime Minister refuses to speak with anyone about it and the first day of this round of the campaign saw our supporters asked to leave Parliament by security guards.  I’m just at a total loss as to why rare disease sufferers are not considered worth saving.  Everywhere we turn we hit a brick wall.  Maybe it is because our numbers are so small that we cannot make a big enough noise to be heard?  Anyway, I’m not giving up the fight!  Ever!

So, blog time…I still have lots on Texas. 

The San Antonio Riverwalk; what a beautiful place to visit.  Our hotel was across the road and around the corner.  You can either take stairs down to the riverwalk or an elevator if you are wheeling your way around.  There are so many shops, cafes and restaurants.  Lots of tourists but not too crowded.  The people are all friendly and cheerful, enjoying their time out and about.  We were told it is a very popular city to have conferences, and hence there are a ton of hotels there!

The San Antonio River Walk (also known as Paseo del Río) (sounds like a great name for one of my miniature horses!  "Half Pints Paseo del Rio"  I like it, i'm using it!) is a network of walkways along the banks of the San Antonio River, one story beneath downtown San Antonio, Texas. Lined by bars, shops and restaurants, the River Walk is an important part of the city's urban fabric and a tourist attraction in its own right.

Today, the River Walk is an enormously successful special-case pedestrian street, one level down from the automobile street. The River Walk winds and loops under bridges as two parallel sidewalks lined with restaurants and shops, connecting the major tourist draws from the Alamo to Rivercenter mall, to the Arneson River Theatre, to Marriage Island, to La Villita, to HemisFair Park, to the Tower Life Building, to the San Antonio Museum of Art, and the Pearl Brewery.

The area was noted for being extremely dangerous (at one point, it was declared off-limits to military personnel). Support for commercial development of the river bend grew, and crucial funding came in 1939 which resulted in the initial construction of a network of some 17,000 feet (5,200 m) of walkways, about 20 bridges, and extensive plantings including some of the bald cypress (others are several hundred years old) whose branches stretch up to 10 stories and are visible from street level.

Thank you Wikipedia!

One afternoon we walked to the Tower of the Americas. This is a 750-foot observation tower/restaurant. The tower was designed by San Antonio architect O'Neil Ford and was built as the theme structure of the 1968 World's Fair, HemisFair '68.

The tower was the tallest observation tower in the United States from 1968 until 1996, when the Las Vegas Stratosphere Tower was completed.

It is located in the middle of HemisFair Park and has an observation deck that is accessible by elevator for a fee - about $10. In addition, there is also a lounge and revolving restaurant at the top of the tower that provides panoramic views of the city.  It's pretty cold when you go out onto the outer deck of the tower!  The wind is pretty strong up there.  But you get an awesome view of every angle of San Antonio. 

Construction History

Construction of the tower began on August 9, 1966 and was completed in approximately 18 months, just in time for the opening ceremonies for the fair held on April 6, 1968. The tophouse of the building was constructed at ground level and hoisted to the top of the poured concrete shaft. As the tophouse was being hoisted into place, on October 30, 1967 some of the cables used to hoist the tophouse snapped leaving the tophouse resting on and precariously tilted on the Tower’s shaft. Eventually, oil field pipes were used in lieu of cables to complete the job.

Thank you Mr Google for some of the above information!

A couple of interesting bits just for fun:

Interesting bit #1.

One for the blokes.  We saw a stretch Hummer on the street near our hotel.  And in the back, a SPA POOL!  You wouldn't want to go around corners fast while it's full I guess. 

I just did a Google search for Hummers with hot tubs, and came up with this website.  It appears the one we saw is the only one in Texas.  Texas is a huge state!  Check this site out if you want to have a drool!  The photo at the top has the limos parked in front of The Alamo.

http://aclassluxurylimos.com/Hummer_H2.html


Interesting bit #2.

On our walk back from the Tower to the Riverwalk, Brad and I took a wrong turn and ended up slightly out of our way.  We turned to go back to where we were supposed to be and some strange man started staring at Brad and kind of bowing his head to him.  I was aware that the man was holding something in his hand but I was more intrigued by the head bobbing thing so I took no notice of what he was holding.  Once we past the strange individual I asked Brad if people often bowed to him in the street.  He said he was trying to sell us cocaine, didn't you see what was in the bag he was holding?  Um NO!  No I didn’t!  Colour me naïve!  This was only a block away from our hotel.  You really don’t need to go far out of your way to encounter some really seedy areas!  Scary stuff.  I think it was more good luck than good management that we didn’t get mugged!

The best news this week is...Mike went HOME Tuesday!!  Yaaaay!  Love and best wishes to you Mike, you rock!!

Here's a pic of Mike with his family on the day of his arrival home.  Couldn't wipe the smile off his face with sandpaper!  Great stuff :)

Firstly, my dear friend Brad from Canada, arrived here last Monday night and stayed with me for a few nights before we headed off to San Antonio, Texas.  Brad has Pompe also.  I had never met him in person before, but when we met it felt like we had known each other for a long time.  He is very special to me because the very first day that I was diagnosed, I went looking on the internet for information and support, Brad was the first person who spoke to me, gave me hope and carried me along with his great sense of humour, and we have communicated often for more than a year, sharing jokes, information, and even poking fun at our situation at times.  It is almost…almost…worth having this disease to meet wonderful people like him.  Thank you Brad, we will be friends for a long time.  Love you heaps.

Monday, the day Brad arrived, I had my 4th infusion.  I had all of the testing that morning, pulmonary function, quantative muscle testing and 6 minute walk test.  After lunch was my infusion.  All went well again, no bad reactions.  I got home that evening at 7.30, Brad arrived at 10.45pm and we went out for dinner at the local sports bar. A long day but all good!

Tuesday we went to the Harn Museum of Modern Art, the Museum of Natural History, and the Butterfly Rainforest here in Gainesville.  We must have spent around an hour in the butterfly rainforest.  The butterflies were so beautiful and the surroundings so serene.  There was a lady there who must have had some nice sweet smelling cream or something on her legs, because the butterflies were landing all over her legs and she was walking around with them all stuck to her.  It was pretty funny!

Tuesday afternoon we went to Paynes Prairie.  There were 3 alligators in the lake this time.  Of course Brad and I think they are cool because Canada and New Zealand don’t have alligators in the wild at all.  Whew!  We also saw lots of deer, squirrels and pretty birds.  I particularly like the bright red cardinals.

Wednesday we headed on down to Orlando arriving at Disneyworld at 9.30am.  We rode rides, we scootered around, we hung out with friends, we ate food, and I tried SUSHI for the very first time ever, and it was YUM!  I didn’t see Mickey Mouse but I did see Donald Duck, I do prefer Donald to Mickey so I was pretty rapt.  He was wearing a sombrero though so I don’t know what was up with that!  Didn’t he used to have a sailor's hat?  We stayed at Epcot (New Zealand had a food stall there!) at Disneyworld till 6pm then caught the monorail and ferry over to the Magic Kingdom.  We arrived at 7 just in time to trip over all of the people who were sitting along the footpaths waiting for the parade which starts at 8pm, so that wasn’t very good timing on our part!  So we went down the middle of the roads where the parade was to follow, it felt like we were our own little parade lol. 

You can't go to a theme park without getting a goofy photo of yourself and your friends!

The castle was all lit up with wonderful light displays.  At 9pm the fireworks began, it was so beautiful.  It’s easy to get caught up in the “magic” of it all, no wonder they call it the “Magic Kingdom”. 

Following the fireworks it was going home time.  We got the ferry and monorail back to the car which took us till around 11pm because so many people were all leaving at the same time.  We didn’t get back to Gainesville until after 1 in the morning…zzzzzz

Thursday morning we organised ourselves to get to the airport here in Gainesville and winged (wung???) our way via Atlanta, Georgia to San Antonio, Texas.  It was a tight connection and we made our flight in Atlanta with only 6 minutes to spare!  We arrived in Texas at 5pm and got a taxi to our hotel on the Riverwalk.  We met a few other Pompe patients in the lobby and went to dinner at a Mexican restaurant.  It was wonderful to meet all of the people we have been on facebook and other parts of the internet with for a long time.  And they all walk funny like me!  Made me feel kind of normal! 

The reason for our visit to Texas was to attend the 2011 AMDA/IPA Patient and Scientific Conference.

I’m extremely fortunate to have been funded by the International Pompe Association and the Acid Maltase Deficiency Association to attend this conference.  The expertise there was phenomenal.  I wish our government and the people who run Pharmac, who deny us the right to live, could have been there to hear the evidence and wonderful stories which have allowed people the world over to live long and relatively healthy lives with the treatment available to us.  Well when I say “us”, I mean people in 52 OTHER countries, NOT New Zealand!  I hope that one day John Key and his people in Pharmac will get with the 21st century and embrace these new wonderful treatments that are making their way into our world.  You never know when you might be the one who needs them.  I never thought I would be one of those.  We never do, do we?  Who has heard of Pompe Disease and Enzyme Replacement Therapy?  Not me!  Well, not until last year.

I had the pleasure of meeting a young man named Trevor who has Pompe Disease.  Trevor is 21 and lives in Canada.  He is totally wheelchair dependant.  Fortunately he is not on a ventilator.   Trevor has a wonderful sense of humour.  We had great fun racing our wheelchairs and scooters against each other.  I was always the loser.  He has 2 motors on his so he had a distinct advantage in my humble opinion!  One word Trevor never uses is the word “can’t”.  He said that when he was younger he could play hockey and do kid stuff and over the years as Pompe took its toll on his body, he said he might not be able to play hockey any more, but he would be “the best darned hockey watcher in Canada”!  I think we can all learn a wonderful lesson from Trevor.  Glad to know you Trevor, you are an inspiration!

Another inspirational person to me is Mike.  I have mentioned Mike in my previous blogs, yesterday he spent 10 whole hours off his ventilator…and guess what!  He’s going HOME to his own house on the 18th of THIS MONTH!  Over 4 years and 6 months since he found himself in the emergency room with respiratory failure.  Wow, brings tears to my eyes that story.  The same thing happened to me last year, I’m just so lucky they didn’t stick a tube in my neck!  We’ll be going to New Jersey to see Mike in his HOME in November.  Diaphragmatic pacemakers, look it up, totally cool!

That's enough blogging for now.  Still got more on Texas and an introduction to "Russ" in the next blog...

A message from Sabrina.  Please read it and please do what you can to help us.

I don't think it's just patients and parents of patients who embrace the effects & benefits of Myozyme but likely it's those we surround ourselves with as well.  It takes just a few minutes for someone to draft a letter and I think the response could have a profound effect on the powers that be who don't see the need for this life saving drug.  I would greatly appreciate your consideration.  Please let me know of anyone else you guys know who blogs.  I also have requests in for AMDA & UPF.   Sabrina  Friends in the pompe community,  As most of you know, New Zealand is currently not on board with funding Myozyme even though more than 45 other countries see the benefit of doing so.  There are at least 5 patients in New Zealand currently suffering from this progressive muscle wasting disease. Monique Griffin, a fellow pompe patient, has been asked by Lysosomal Diseases New Zealand to join them in their fighton Tuesday with Pharmac (the Pharmaceutical Management Agency of New Zealand).  Whether she is talking directly to Pharmac or being interviewed by media, she is front & center in a major struggle that stands to benefit so many people.  Monique will be flying out on Saturday with this huge undertaking and we, as a community, can help.   For those of you who know Monique, she is a walking testimonial and a compelling public speaker but lets give her even more ammunition for this very important meeting.  

Please take a moment and write a brief story of what Myozyme has meant to you or what is has done for a beloved member of your family.   It's best if your story is kept short so that it'll be read.  Imagine if Monique waves in her hands hundreds of letters, photos, results and testimonials of patients who suffer from pompe disease who have benefited in some way from receiving Myozyme infusions.   There is great power in numbers, please take a moment to give voice to your story for our fellow pompe sufferers in New Zealand.  

Please send your story THIS WEEKEND to Monique Griffin at [email protected].

Thank you in advance for your support! Sabrina Low-DuMond Mom to Zack (16) pompe, Madison (14) unaffected, Kyle (28) unaffected So Cal. 

I had the pleasure of meeting John Crowley at a tailgate party at Dr Byrne’s house this weekend.  As you will see below, John Crowley is a hugely successful man, but when you talk to him, he is first and foremost a Dad with a huge love for his family.  As I said, it was truly a pleasure to meet such an inspirational person.

John has 2 children with infantile pompe and an older son, John Jr, who does not have pompe. 

In 1998, two of the Crowley's children, Megan and Patrick, were diagnosed with a severe neuromuscular disorder, Pompe disease. In the face of the children's deteriorating health, the family moved to New Jersey to be close to doctors specializing in the disease.  Frustrated with the slow pace of research on Pompe disease, Crowley took a position as CEO of Novazyme Pharmaceuticals, a biotechnology research company located in Oklahoma City  that was conducting research on a new experimental treatment for the disease.

In 2001, Novazyme was acquired by Genzyme Corporation, then the world's third largest biotechnology company, under Crowley's initiative. Crowley was in charge of Genzyme’s global Pompe program from September 2001 until December 2002. Genzyme’s work eventually bore fruit and in January 2003, Megan and Patrick Crowley received the enzyme replacement therapy for Pompe disease developed by Genzyme. John Crowley credits the experimental trial with saving his children's lives.   The acquisition of Novazyme by Genzyme, and Crowley's fight to cure Pompe's Disease, was documented in the Harvard Business School Case Study, Novazyme: A Father's Love.

Crowley went on to become President and CEO of Orexigen Therapeutics in 2003. In January 2005, he was named the President and CEO of Amicus Therapeutics, based in Cranbury, New Jersey.  He also serves in the United States Navy Reserve as an intelligence officer. He completed a six-month tour of active duty at the Center for Naval Intelligence in Virginia in 2007.  He is currently assigned to a Navy Reserve unit at the United States Special Operations Command.

Crowley was profiled in The Wall Street Journal by Pulitzer Prize-winning journalist Geeta Anand.  Anand expanded the profile of Crowley into a book published in 2006, The Cure: How a Father Raised $100 Million – And Bucked the Medical Establishment – In a Quest to Save His Children.

Harrison Ford and Double Feature films optioned the rights to produce a film inspired by Anand’s book and the Crowley family.  In April 2009, CBS Films began filming this major motion picture about the Crowley family's quest to save their children's lives. The film, titled Extraordinary Measures was released nationwide on January 22, 2010.  Extraordinary Measures stars Brendan Fraser as John Crowley and Keri Russell as Aileen Crowley, and also executive producer Harrison Ford as "Dr. Robert Stonehill" who is a composite character based primarily on Dr. William Canfield and inspired as well by other doctors Crowley worked with.  John Crowley has also written a personal memoir entitled Chasing Miracles: The Crowley Family Journey of Strength, Hope and Joy.

For more about this inspirational family see link below:

http://www.crowleyfamily5.com/index.htm

Before we went to Dr Byrne's place I also went to another tailgate party at Katie and Dean’s sectioned off bit of roadside.  Great food, lovely people, some I have met before and some new ones.  It’s always interesting to meet new people and  find out about where they come from and what they do.  Most of them who have heard of New Zealand have heard about how many different terrains there are all in one small country, and the wonderful outdoor lifestyle we have there.  I’ve invited them all over.  If they all come at the same time our house is going to be PACKED! 

I had to include this photo of me with this man, because he is 6’8”!  That’s his wee daughter there pulling my hair lol.

I guess since I’m talking tailgate parties, I should mention the score in the big game following.  Gators vs Alabama…The Gators got chomped, #3-ranked Alabama beat #12-ranked Florida 38-10 in The Swamp.  At least the tailgate was great!

I took the OZs car for a drive this week.  It’s funny how you feel like you have this huge flashing neon sign on your forehead saying “look at me, i'm an idiot, I have no idea what I’m doing”! 

But, I took deep breaths, stayed on the correct (right…wait… yep the right hand side) side of the road and didn’t crash.  I didn’t go out on to the bigger main roads as they are a little scary.  But I will work my way up to that.

This week I went to the pet shop and they had an adoption day.  Lots of dogs, mainly large ones, plenty of cats and kittens.  I just wanted to bring all of the cats and kittens home…

Note to my husband: NO I didn’t! 

I saw a great sign on the cages of the black cats which read:

 
10 Reasons to adopt a black cat

10    You’ll save $$$ on their Halloween costumes

9      You can always find them in the snow

8      Holding a black cat is very slimming

7      Black cats will match any décor

6      A lint brush isn’t required for a black tie affair

5      When you love a black cat, luck is on your side

4      Black cats are like Onyx, a beautiful gem

3      Hey they don’t care what colour you are!

2      Love knows no colour

And the number 1 reason for adopting a black cat is…

...they are the least likely to be adopted.

I couldn’t resist putting this in because I think black cats are awesome.  Two of our cats are black, one which we adopted from the local SPCA.  Frances is one of the best cats you could ever hope to have.  So if you’re thinking of adopting a cat, try a black one!

Critter of the week

The Great Egret

I saw this bird down the road as I was scootering past.  At first I just noticed its head as he was in behind the low hedge I always go past.  He was sauntering along picking lizards out of the hedge and wasn’t worried one bit when I stopped to take photos.  When I went around to the other side of the hedge to photograph him he just carried on with his lizard hunting.  Felt kinda sorry for those cute little lizards though…

I had to do a little homework on this one.  When I saw this bird I thought it was a White Heron.  But when I googled that it wasn’t quite right.   It is similar to a white heron, but the egret has black legs, a thinner, bright orange-yellow bill and no head plumes.

The Great Egret is found across much of the world, from southern Canada southward to Argentina, and in Europe, Africa, Asia, and Australia. It's the largest egret in the Old World, and thus has garnered the name Great White Egret. In the New World, however, the white form of the Great Blue Heron is larger. In the United States, the Great Egret used to be called the American Egret but that was hardly appropriate, since its range extends beyond the Americas and indeed farther than other herons.

This Monday I have my 4th infusion, preceeded by a morning of tests for lung function, muscle strength, 6 minute walk test and belly dancing whilst standing on a beam like the gymnasts use…I’m kidding about that last bit!

Monday night my friend Brad from Canada, who also has Pompe, will be arriving.  Fortunately the All Blacks caned Canada in the game yesterday so I have a bit of ammo up my sleeve if need be!  Sorry about the Warriors, maybe next year!  Awesome that they got there though!

Anyway as I said, Brad from Canada (I have to differentiate because the OZ is named Brad as well) is arriving Monday to hang out here for a few days until we go to Texas on Thursday.  I've been very fortunate to be funded by the International Pompe Association (IPA) and the Acid Maltase Deficiency Assocation (AMDA) to attend a Pompe conference representing New Zealand next weekend.  Our New Zealand Pompe Network is now a fully paid up affilliate member of the IPA.  So I will have lots of info in next week's blog about our Texas adventures.  Would it be too "cliche" to buy a 10 gallon hat in Texas??

Until next week from Texas...Keep well, keep safe, treat each other with love and kindness...and get yourself a black cat :o)