The Not-So-Camping "Camping Party"

Sweet Colin turned 5 on January 20th. As is his tradition since his real "birth" day snow came our way and turned one celebration into two to accommodate all his out of town family. Of course my January baby boy would want to go camping of all things for his birthday. So we compromised on a camping theme. I totally turned into a pinterest junkie. All the clever camp themed birthday party ideas were there!


Decor: Nature and outdoors




Food:
Menu Kids in a Blanket, Campfire Flames, Bug Juice, and Sweet Treats for all the Happy Campers :)















Best Birthday Cupcake Cake I have ever seen! River's Edge took my theme of camping and ran with it quite literally. I was super impressed :)


The rest is just pure fun!








Dear Colin,
We may not get to spend as much time with you as we wish but you will always hold a special place in our hearts. You were the only little baby we got to have without worrying you might have CF. Even before we knew for certain, our hearts told us. You are a miracle. A blessing. You are funny, outgoing but sometimes still shy. You pose silly in pictures and you spell things goofy. You call trail mix, mail trix and only suck your thumb when you have your green 'b'.

All joking aside, I thought I was going to pass out when the nurse announced you weighed ten pounds. You were a big boy with a huge heart! God knew we needed someone just like you to fit the pieces together. You keep us "normal" when life is too tough.




Your blue eyes are the windows to your soul. I could look in them then just like I can now and know exactly how you are feeling.


We love you. We can't imagine life without you.
Love,
Mommy

Sigh.

The dates are marked on our calendar. In big blue letters.

February 3rd-Cayden done with TOBI
February 14th-Trinity done with TOBI

I love seeing the clear vials disappear from the fridge. It's always a big deal when a 6 year old exclaims. "No more TOBI, question mark. YES!"

In addition to inhaled TOBI, Dr. Green also treats pseudo with the oral antibiotic ciprofloxacin (cipro). The combination of antibiotics has shown in clinic studies to reduce the prevalence of PA in future cultures.

Here is a graph of an eradication procedure for early or recurring PA done in 2008. Colisin is another common nebulized drug used for the purpose of killing PA, but it is more common to start with TOBI.



I am such a CF geek. In fact, I know I am when I enjoy looking at charts like this.

Cayden was in a study as an infant using TOBI and a combination of Cipro (or placebo) in patients who grew PA before their first birthday. However we couldn't complete the study because Cayden's liver enzymes were too elevated to begin.

We're really hoping for good results on the 13th because, yes, we could get more use to an extra 2 hours in our day.

Next post will be most definitely an update, but Colin's out-of-this-world 5th birthday party will also be spotlighted. After all, he deserves his own post every now and then.

Exciting Day for the CF World!

This is such huge news. Today marks a momentous change in the way we view treatment for CF. The first drug ever to target the underlying problem in CF has been approved by the FDA. And while it doesn't address our specific type of mutation, it gives enormous hope as a similar drug is in the works.


FDA Approves Kalydeco (VX-770) — First Drug That Targets the Underlying Cause of CF - CFF

One box down, One to go

A week into Cayden's pseudo treatment we got word from the clinic that Trinity had also cultured for pseudo.

TOBI® (Tobramycin Inhalation Solution, USP) is a prescription inhaled medication for cystic fibrosis patients whose lungs contain bacteria called Pseudomonas aeruginosa. For the purpose of this blog post we will refer to it as PA.TOBI is given in 28 day cycles especially for people on it chronically. 28 days off and 28 days on. At the end of 28 days they will be re cultured. If they do not grow PA we will stop TOBI. If they continue to grow we will most likely repeat and possibly change to Colistin at some point. Many will never get rid of PA, but the important thing is to minimize damage to the lung tissue.

Between the ages of 6 and 10, about 40% of patients with cystic fibrosis have Pseudomonas aeruginosa infections

By the age of 17, nearly 60% of people with cystic fibrosis have Pseudomonas aeruginosa infections in their lungs

Up to 80% of adults with cystic fibrosis between the ages of 25 and 34 have Pseudomonas aeruginosa infections

To learn more about taking TOBI you can visit TOBI's OFFICIAL WEBSITE

Each of the kids is on a 28 day cycle and each treatment takes about 20 minutes. We start each day as close to 6 am as possible. With antibiotic nebs we have to start with chest therapy (30 minutes) followed by TOBI (20 minutes). We try to accomplish all this before 7 am so we have enough time for breakfast and getting dressed for school.

I have to admit as little of fun it is for our kids, I hate it even more. We have lost 2 hours of our day and I for one am not a morning person. TOBI is super sticky which clogs our nebs so by the time we get everything working properly we're looking at another 15-30 minutes a day. In the words of our favorite bedtime story "Where the Wild Things Are" PA "roars its terrible roar and nashes its terrible teeth!"

As much as we hate TOBI we have to be thankful for it, because not too long again CF patients who cultured PA were not given much longer to live. The fact of the matter is that when CF patients do not treat Pseudomonas aeruginosa, lung function declines about 2% a year. That could be about a 10% loss every 5 years.

Fortunately neither Cayden or Trinity are symptomatic at this point, but it is still creeping its way into their lungs. As adults they will most likely always culture PA, but while we can do something before the damage is done we will.

It seems like everyone we know lately with CF has cultured PA. It's interesting to see the spurt of something like this so widespread in just our circle of the CF community. One of which is little Maddie a friend we have met via social network sites like Facebook and Cystic Life. Maddie is 3 and has had pseudo for an entire year. She is now hospitalized for 10 days and luckily her bronch came out with great results---PA is in her throat but not in her lungs. You can follow Sandy, Maddie's mom's blog at Mommy for Madeline.

Fingers crossed this will kick it...we have 2 weeks left to see the verdict!

The Trip of a Lifetime

Alright, you're going to need a big cup of coffee for this post. I have so much to share with you from our trip to Disney. Where to start...Wow! That's really all I can say about Cayden's Make-a-Wish trip. It was an absolutely magical week for our entire family and it was so hard to leave Give Kids the World (the village we stayed at). Chris and I both cried as we left for the last time. But we will always have a piece of our hearts there!

I'll try my very best to capture all this trip meant to our family and the highlights of each day. We are so grateful to our wish granters for the trip of a lifetime. Each day Cayden and I sat down to journal about our day's activities so we wouldn't miss an ounce of the memories we had just created.

For starters, let me share a little bit about Give Kids the World (GKTW). To be quite honest I would like to wish for a week at this place alone. Forget Disney, Hollywood Studios, Seaworld, Epcot and the like. This place is magic in and of itself. We could have stayed at the village all week long and never been disappointed.


Give Kids The World is a non-profit organization that exists only to fulfill the wishes of all children with life-threatening illnesses and their families from around the world to experience a memorable, joyful, cost-free visit to the Central Florida attractions, and to enjoy the magic of Give Kids The World Village for as long as there is a need. Since 1986, Give Kids The World has hosted more than 107,000 families from all 50 states and more than 70 countries. Give Kids the World was founded by Henri Landwirth, a Belgian Holocaust survivor who spent 5 years in Auschwitz and Matthausen concentration camps. Landwirth immigrated to the United States, where he studied hotel management. While operating a hotel in the Orlando area, he became passionate about helping sick children fulfill their dream of a family vacation.

This place was unreal! Ice cream was available all day with no limits. Their slogan at GKTW is "Ice Cream for Breakfast." No joke, the Ice Cream Palace opens before the Gingerbread House where you are served breakfast.

The Village operates with over 1500 volunteers each week (yes, you read the 2 zeros correct) and only 120 year round staff members. The "angels" as they call them are all there for one reason: to serve your family. The most amazing thing is to know that the other 200 families there are also there for the same reason, because they too care for a child with life-limiting illness. Each time you come back back the attendant at the gate says "Welcome Home." It is truly our home away from home now and will always have a special place in our hearts.

Ok, back to the trip.

Monday morning, the day after Christmas our limo arrived @ 4:30 am. The kids were estatic! Colin said "Hey mom, look at the fancy car! The president is walking up to our door!"


Mommy was so caught up in the magic that she forgot everyone's meds in the fridge so we had to turn around 10 minutes on the way. That could have really been a damper in our trip.

At the airport Cayden was greeted by Jay from GKTW! When do you ever got a personal escort?!?


Every Monday GKTW celebrates Halloween and the main street was decorated in pumpkins, spiders, and costume-making stations.

Tuesday was our most and least favorite day all rolled into one!
The morning began with the football teams from FSU and Notre Dame stopping by to visit GKTW and sign autographs, play and hand out ice cream.



Cayden loved hula-hooping with all the players.


Both teams warmed our hearts. Thank you for making the stop to make our day brighter :)


The kids were really excited for Magic Kingdom so we headed there next. For those of you who have been do Magic Kingdom before, you know the time it takes to a) park, b) get on the tram, and c) cross over with either the ferry or monorail. For those of you who don't, I think you get the picture. It takes a while. Just after we had gotten to Magic Kingdom and watched the Castle show it poured! We got so wet that we decided to head back and take the 45 minute trip back to where we had just come from. Here's our picture to remember fondly our most soggy moment:



Colin even took a picture with his 1st charcter! We were so worried about how he would do with his fear but he was so brave and really overcame it this week!


Wednesday the REAL Santa came to visit the Village. He was vacationing for the month in FL. I say real because the boys asked Santa if he was real and he said "Sure I am." But they investigated even more about his beard and if he was the REAL Santa his beard wouldn't move if they pulled it. Santa gave them the nod, they pulled his beard and well, it was REAL! They were in heaven.

Wednesday was full of horseback riding, SeaWorld, and a Pirate Dinner Adventure.
Feeding the dolphins was such a cool experience. Definitely my favorite part of the trip!



Thursday we met Minne and Mickey Mouse. It was so great that each day our village had different characters visit from the theme parks.


Our next adventure took us flying high. We got an air tour thanks to Mauiva Air tours and the boys both got to fly the plane. Colin and I got special permission to fly over Magic Kingdom since it is a no-fly zone!

The rest of our day we spent at GKTW and getting some very important things done!
The Castle of Miracles is home to over 86,000 stars of hope representing all the children and their families who have visited the village. Cayden got an exact replica to keep at our house to remind him of the magic and love he experienced on his wish trip.


We can always go back and visit to see his star using a special passport.

Friday we headed back to the [dreaded] MAGIC Kingdom. We say dreaded because lines had grown to over 2 hour waits for rides and the crowds were unbearable. Cayden's wish pin was amazing getting us to the front of lines as fast as possible. Cayden's favorite ride was Space Mountain. We met a ton of characters, but Trinity's favorite was Rapunzel!



After Disney we headed to Medieval Times. The kids loved eating with their hands (except for Colin)!

Saturday was our last full day in FL so we took in Hollywood Studios and met our beloved Toy Story, Phineas and Ferb, and Cars Characters! After lunch we met up with the Loof family from Jefferson to spend time swimming at their condo. The excitement got a little bit crazier with Cooper hitting his head, a trip to the ER and 6 stitches! We got home just in time to ring in the eastern New Year's!

As we packed up to leave we were in tears. Cayden was especially heartbroken to leave his balloon behind, but decided to leave his balloon he had bought in the lobby at GKTW for other kiddos to enjoy. It was so sad for him to say goodbye but it would have been too crazy to fly on a plane with his balloon!



Each day when we returned the wish fairy had left presents for each of the kids on our kitchen table. They go above and beyond with all the gifts. It was most special because for the first time Colin wasn't left out and not including because he isn't like his other siblings. For the entire week he was given the most special love and wasn't told no. That was what made our stay at GKTW so unique.

Thank you for letting us share our trip with you. It will be forever in our hearts and we will never forget the most magical week of our life. Thank you GKTW and Make a Wish! We will always have a special place in our hearts for you. Until the next time we get to hear "Welcome Home..." thank you for making it such a memorable trip!

Hustle Bustle

We are so busy right now. To say we can barely slow down to breathe is an understatement.

Tomorrow night is our Make a Wish party to get all the paperwork finished for our trip to Disney. It's easy to forgot we have Christmas to celebrate before this big trip. Here are some pictures from our this past month as we decorated for Christmas and did some tree shopping at the Kysely's. Always our favorite time of the year!

Our Nativity Stable is filling up with stickers each night as we pray our way through Advent.


Our Christmas tree hunt was an easy one this year. We found ourselves a winner!


Clayton and Cayden


Time for cutting...


Trinity's turn


Timber! This year we competed with soggy shoes instead of snow as it as too warm in WI still.


Time for the star. Our trees always look taller inside!


Colin proud of last year's ornament he made in PreK 3


Trinity decorating


Cayden


Cooper...well he slept the entire time tree hunting and was just not into the whole "decorating the tree" thing.

Here's a pic of all 4. So much fun in such a small wagon...

Reaching the Finish Line

Here's a long lost picture from Colin's first day of school.


It seemed as if the anticipation couldn't be any thicker. On Friday we headed off to Madison for the "weigh-in" that we had been working ourselves up to. And so, we did what all good CF mamas do. We went to McDonald's.

No. Not after. BEFORE!

Trinity ate half of a mighty kids meal with 2 slices of bacon, a chocolate milkshake and fries. We did the usual mask ups in the elevators and headed up to the second floor. They called Trin's name and without looking back we headed for the check-in room.

As I waited for the scale digits to finish calculating it was nearly killing me on the inside. Did we do enough? Could we have possibly gotten her to gain something? Anything?

The numbers stopped. Kilos. The nurse presses the conversion button.

My heart nearly jumped out of my chest with sheer joy. Deep breath. She did it. We did. Trinity gained almost 2 lbs in 6 weeks. In my heart of hearts though, I know she only did that in the last 2 weeks on her appetite stimulant. We weren't out of the woods yet, but I felt like I had finally reached a place of comfort.

Our plan is to continue with the appetite stimulant, however people's bodies can become immune to them. Our dietitian suggested cycling the drug if that happens. In the meantime we will also change her Zantac to a stronger proton inhibitor which for CF patients maximizes their enzymes effectiveness.

For all my fellow CF mamas, here is a really great new site clinic shared with us on Friday for CF recipes called CFChef
One serving of the Banoffee Stuffed French Toast is 1100 calories!

We won't be back to clinic until after our trip to Disney which seems too close to be real!

Monday our doctor from clinic called to tell us our three checks for MRSA all came back clear! After 2 long years we can finally say we are MRSA free again. The infection control head told our doctor to personally thank us for adhering so well to the eradication protocol. Remember those chlorohexidine baths?!?

It's great to have such wonderful things to take us through the cold season :).


This picture of Cooper says it all!