a different kind of perfect

i'm a mom...on a mission...to enjoy life everyday...appreciate the little moments...try not to get so easily annoyed...raise my kids with a faith legacy that leads them to continue to be amazing people...and to make my husband feel as special as he makes me feel.

What is Cystic Fibrosis?

CF is an inherited chronic disease that affects the lungs and digestive system of about 30k children and adults in the US (70k worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that both clogs the lungs and obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. [www.cff.org]

Saturday, June 30, 2012

Yes! Yes! Yes! Phase 3 of VX-809 & Kalydeco

Yes!  Such exciting and positive news announced last week about the clinical trials that are going on for Dean's mutation.

Here is the link to the article and I'll summarize the excitement:

A little science to explain what is going on...Cystic fibrosis is caused by the mutation to the CFTR gene which hinders the transfer of water and salt across cells affecting both the digestive and respiratory systems.  To manage the digestive system properly, Dean takes pancreatic enzymes before snacks, meals and milk since his pancreas doesn't produce the enzymens needed to digest fat.  On the respiratory side, oatmeal-like mucus is produced in the mucus producing organs and since last August, we've been giving Dean daily respiratory treatments to prevent a buildup of mucus in his lungs as well as thin any mucus that is making a home in his lungs.  We do this to help him breathe easier and because bacteria like to breed in warm, sticky environments and we're trying to prevent the bacteria from causing any damage to his lungs.  So, back in January, the FDA granted approval for Kalydeco, which is the first drug for CF that doesn't treat the symptoms but rather treats the disease by targeting the CFTR gene by helping to open the cell "gates" to allow better transport of ions across cell membranes.  Dean's issue though is two fold.  Not only do the cell gates not open but the protein can't even get close enough to the cell surface to get out!  So, the Cystic Fibrosis Foundation partnered with Vertex and developed a drug (now called VX-809) that they intend use to get the CFTR protein to the cell surface, where Kalydeco can then work it's magic, open the gate and allow for the correct flow of ions.  On Thursday, final results from the phase 2 clinical trial of the combination of these drugs were released which showed significant improved in lung function for patients that had the same mutation that Dean has.  Lung function is measured by how much air can be expelled from the lungs in 1 second.  Although Dean is too young to get measured for this now, these trials in CFers 18+ with the same set of mutations showed very promising results.  So, the next step is the pivotal phase 3 trial which will be the mose rigorous evaluation of the safety and efficacy of the combination of these 2 drugs in patients who have the double deltaF508 (in preparation for another FDA filing).

We are beyond hopeful and prayerful that God guides these scientists to continue making the future look bright for our son and so many others with this disease.  We are so thankful for all of your support and wanted to explain what $75million of research dollars can do for one little man and 30,000 other people in the United States alone.  It's the passion of the people that care so much to do everything to help us raise money that is allowing this to happen.  As a "orphan" disease, meaning one given very limited federal funding, the Cystic Fibrosis Foundation is at the mercy of private donations and the families and friends of those affected to raise money.  We truly won't stop until CF stands for Cure Found.  Thank you for helping us.  Our next fundraiser for Team Eat. Pray. Dean, Chillin' & Grillin' for a Cure is on July 14th from 3-10pm.  Please say you'll come and be a part of this amazingly huge part of our lives.  We thank God for you and for your kindness and your prayers.

With love and appreciation,
Jim & Erin

check out http://www.eatpraydean.com/ for the schedule of events on July 14th
email us at findacure@eatpraydean.com if you will join us and need a link to the evite
click here to get these posts by email
Dean 2 years old

the awesome kids that will do anything to help Dean-o

"God is not unjust; he will not forget your work and the love you have shown him as you have helped his people and continue to help them.” ~Hebrews 6:10 (NIV)

Monday, June 25, 2012

Check out the lineup - don't miss Chillin' & Grillin'

Hi Friends,

We wanted to send out a reminder about our “Chillin’ & Grillin’ for a Cure” block party fundraiser to raise money for the Cystic Fibrosis Foundation.  As you know, we are striving to raise as much money as we can in the hopes of advancing research to find a cure for our little man.  Over 90 cents of every dollar donated goes directly to fund vital research and development.  If you didn’t get a chance, log onto our website (www.eatpraydean.com) to find out more about Dean and to watch him in his very own video.

Put in on the calendar and invite your friends and family to the Block Party at our place on Saturday July 14th from 3-10 pm where we will have a rib cook-off, basket raffles, games and activities for adults and kids!  Below is a timeline of the events that we have secured so far.

Thank you,
Jim & Erin
21831 Yellow Finch Lane
July 14th Chillin' & Grillin' Schedule of Events

9:00 - Fire up the smoker and start cooking your ribs!
3:00 - Let the party begin!  Food and raffle ticket sales
3:30 - 4:00 - Music for the kids by
4:00 - 6:00 - Ribs and food sales
4:00 - 6:00 - Caricature, Face Painting and Balloon Artist for the kids by Art4Clowns
6:00 - 6:30 - Music for the kids by Poochamungas
6:30 - Dessert station
7:00 - Bean Bag Tourney
7:30 - Final call for raffles
8:00 - Basket Raffles, 50/50 (winner need not be present), Paddle pull raffle

Kids games and activities will take place throughout the day
Time TBD:  ATA Frankfort will host some mini Tae Kwon Do sessions for kids and adults

There is still time to show off your grillin’ skills – we have 6 competitors.  Let us know by June 30th if you’d like to join the Rib Cook-Off.  The rib cook-off portion of this event is sponsored by our friends from the Glen Ellyn Backyard BBQ Competition. Although a non-sanctioned event, this is for all teams, regardless of skill or competition experience, to compete, raise money for a great cause and either gain experience or try out new recipes and techniques.  Contestants must provide all their own equipment and bring 5 slabs of Pork Ribs (spares or baby backs) and ingredients. Charcoal and gas grills/smokers are welcome. A “boat” will be provided for each dish. Teams may arrive as early as 9:00 AM and begin cooking and setting up if you will cook your ribs here.  Rib Tasting will begin at 4 P.M. and will conclude at 6 P.M. or whenever you are out of ribs.  We will only sell 50 tickets to taste the ribs — with each tasting equal to 1 individually cut rib from each contestant. Each ticket sold will also include a vote for the best rib.




Monday, June 18, 2012

The winner of the Cubs/Sox raffle is...

We had an awesome weekend running the warrior dash, dancing away to a family wedding and celebrating the most important men in our lives on a special Father's Day.  We ended the weekend with Dean pulling the winner for the tickets to the Sox / Cubs game that we sold to raise money for the CFF.  We thank Karen for donating those tickets and for all of the people that supported us and purchased tickets!   Check out the video below to see who won...

Here are a few more pictures from the rest of our weekend...

Warrior Dash 2012

Congratulations on your wedding Tony & Shannon!
And a salute to the best Dad's in the world!

The world's largest Peanut in GA!

Pelican Beach in Florida 2012



Save the Date for the Chillin' & Grillin' for a cure Fundraiser Block Party on July 14, 2012 3-10pm.  Check out http://www.eatpraydean.com/ for details.

Thursday, June 14, 2012

Save the Date for Chillin' & Grillin' and Cubs/Sox Raffle

We are trying something new this year; something more relaxed and our style.  We’re going to host a Block Party on Saturday July 14th from 3-10 pm and we would love to have you join us as we are “Chillin’ & Grillin’ for a Cure.”  This isn’t just any block party, it is a fundraiser that we are hosting to raise money for the Cystic Fibrosis Foundation.  Along with help from many of you already, Team Eat. Pray. Dean has raised over $50,000 in the past two years.   We are striving to raise as much as we can in the hopes of advancing research to find a cure for our little man -- a cure that is truly within reach.  Log onto our website (www.eatpraydean.com) to find out more about Dean and to watch him in his very own video.

This awesome day will include a rib cook-off (entry deadline by June 30th if you're interested), basket raffles, games and activities for adults and kids.  Stop by for the day or for just a few hours – we’d love your support!  Help us plan for food by giving us your RSVP via this evite link:  http://new.evite.com/#view_invite:eid=026ANC2ZTL7RSYAS4EPBVLPPOUYR3Q

Next up...we have tickets to the June 19 7:10pm Sox/Cubs game at US Cellular that we are raffling off for $25 each.  You will win 6 seats (section 149) including parking pass!! Dean will pull the lucky winner this Sunday. Email/call us if you are interested in buying a raffle ticket.  Thank you to Karen and her hubby for graciously donating these to us!

Finally, if you missed our Great Strides thank you, you can read it here...we're still working on putting the pictures together but will have them all ready after the Block Party.  Team Eat. Pray. Dean in Maryland had their walk this past weekend and we're grateful for the crew that supported us and walked for Dean in Ellicott City, MD Great Strides.

Thanks for all of your unending support!