Shriner's Hospital Evaluation

The following is an update on Luke's appointment at the Houston, Texas Shriner's Hospital for Children:

Luke has pectus excavatum (sunken chest). Many people live with this, never needing surgery. In the past year, Luke’s chest has become increasingly worse and now affects his breathing and causes pain. Dr. Dietz, the doctor that monitors his heart at the Johns Hopkins genetic clinic, referred Luke to a thoracic doctor in order to correct the pectus excavatum.

After being examined at the thoracic doctor’s office we were informed that Luke’s pectus excavatum was severe and he needed corrective surgery. We were sent home to have Luke’s CT scan, pulmonary heart function test performed and to raise the needed funds for the surgery.

We immediately began brainstorming the most effective ways to raise the funds needed for this surgery. In the meantime, I also applied to the Shriners Hospital for Children. We were initially denied help at the Shriners because of Luke’s heart aneurysm. I then reapplied, along with a letter from Luke's doctor at Johns Hopkins stating that Luke’s aneurysm was “slow growing” and that Dr. Dietz was referring Luke for this pectus surgery.

Not every Shriners Hospital performs pectus excavatum surgery so we ended up being accepted at the Houston Shriners Hospital. We met with the surgeon on March 18th, and he informed us that Luke's pectus excavatum was "unique", meaning the Nuss procedure (the less evasive surgery/the one I was fighting for him to have) would not be enough for Luke. The Nuss procedure would place a bar through an incision in both sides of Luke's torso and push the sternum out. He would have had to keep the bar in place for 2 to 4 years. Due to the fact that Luke's sternum is twisted almost 45 degrees, the Nuss procedure would not correct all of the problems.

Instead, the procedure Luke will have performed will open him across the front, going under muscle to realign his ribs and straighten the sternum in the process. The "normal" patient with this type of surgery typically has 3 ribs realigned, but again Luke's case is "unique" and requires the realignment of approximately 5 ribs. Luke will also have approximately 2 plates to hold everything in place. His surgeon expects a successful surgery that will improve Luke's quality of life.

Luke will be scheduled for surgery in a few months and we will be sure to keep you updated on his progress.

Thank you!

Support Ehlers-Danlos Syndrome Research

Special thanks to Maya Brown-Zimmerman for allowing us to republish her blog post below. Maya has been volunteering with the National Marfan Foundation since she was 14 years old. Currently she serves as a teen program coordinator for the NMF and local group leader. She also works with the Coalition for Heritable Disorders of the Connective Tissue. Maya was first suspected of having Marfan when she was 8 years old, and that diagnosis was confirmed by genetic testing at age 21, after she was also tested for Loeys-Dietz and vascular Ehlers-Danlos. She loves playing with her toddler son and spending time with her husband, Mark.

Support Ehlers-Danlos Syndrome Research

Today I write on behalf of my friends with Ehlers-Danlos syndrome, a disorder related to Marfan syndrome. There are multiple types of EDS (affecting 1.3 million people worldwide) and NONE have real treatments available to them, let alone cures. One of the most serious of these, vascular-EDS, is particularly close to my heart.

At the end of my freshman year of college, during a routine exam, my geneticist said he was SURE I had vascular-EDS. He was reluctant to give me any details about the illness until I threatened to go home and Google it myself.

He was hesitant because vascular-EDS is a death sentence. With vascular-EDS, aneurysms develop anywhere in the body and organs can spontaneously rupture. There is no way to predict when this will happen, nothing to do to prevent it. The average life-expectancy is in the mid 40s. Children have died from just doing a cannonball into the pool.

However, hope is on the horizon, and that’s where YOU come in. The Ehlers-Danlos Syndrome Network has been named a finalist in Chase bank’s Community Giving Challenge. They are up for a $100,000 prize. What would this money do for the foundation? Well, Dr. Hal Dietz has designed a “mouse model” of Marfan syndrome (basically, mice with Marfan). Our mice have dramatically increased doctors’ knowledge of Marfan syndrome, such that we now have an average life expectancy. $100,000 would be enough money for Dr. Dietz to begin work on a vascular-EDS mouse model.

Go here to vote for the EDSN!!! Then, tell all your friends!

Below, please read what Dr. Dietz has to say:

“Vascular Ehlers-Danlos syndrome (or vascular EDS) is a disorder of the body’s connective tissue – the material between the cells that give the tissues form and strength. In vascular EDS the body lacks sufficient type III collagen, a molecule that contributes to the strength of the skin, intestines, uterus, and most importantly, the blood vessels. People with vascular EDS live with the knowledge that they will die from this condition at an age ranging from childhood to young adulthood. They are also told that there are no effective treatments. There are no medications that are known to strengthen the tissues or delay blood vessel rupture. Attempts at surgical repair are often delayed there is confidence that the patient will die within hours if nothing is tried. This is because the tissues are so weak that they often simply fall apart during surgery – akin to trying to sew together wet tissue paper. Of all the conditions that I care for, I hate this one the most. It not only drastically shortens the length of life, but also robs people of any meaningful sense of hope and quality of life – always anticipating that the shoe will drop at any moment. Indeed, all too often children with vascular EDS lose any sense of ambition and purpose despite truly remarkable talents and potential. To their mind, “Why bother.”

Fortunately, there is now strong reason for hope…It is now our goal and intention to make mouse models of vascular EDS in order to learn more about the condition and to test this and other therapies. If someone had suggested 5 years ago that a pill might be able to treat a connective tissue disorder, I would have considered them crazy. Given recent breakthroughs and sufficient resources for further research, I will be shocked if a revolutionary new treatment for vascular EDS is not in general use within 5 years. I have already begun to share this sense of optimism with children with vascular EDS. Our job is to bring this goal to fruition. Their job is to begin dreaming big.”

Harry (Hal) Dietz, MD
Johns Hopkins University School of Medicine
Institute of Genetic Medicine

From The Beginning

Hello All!

My name is Vikki Pier and I am Luke’s mother. I have decided to try my hand at blogging in order to keep you updated with Luke’s progress, needs, and who knows - this may be therapeutic!

Luke is the 7th of my 10 children. He has always been tall, thin, energetic, tender hearted, mechanically minded and extremely flexible. An orthopedic doctor Luke was once seeing, because of pain in his joints and dislocations, called him “rubber band man”.

In February 2007 I took Luke to a cardiologist because he was complaining of heart pain. The pain was so great at times it would stop him in his tracks. Even with this pain I never dreamed Luke had any problem with his heart. Instead I believed the cardiologist would refer us to a doctor that would correct his sunken chest. During this visit we learned Luke had an enlarged aortic root and a possible connective tissue disorder.

After my initial shock and fear I began researching in order to make sure Luke received the best care possible. I discovered that one of the best alternatives for Luke to have, when surgery was needed, was the valve sparing procedure. This operation is not done at very many hospitals and the few I found would not accept us because of insurance reasons. Johns Hopkins Hospital in Baltimore, MD did welcome us into their genetic clinic and Luke has been seen there since 2007.

Luke’s aneurysm, at this time, is “slow growing”. As of his last visit in June 2009 he has been referred to an orthopedic doctor at Johns Hopkins in order to have surgery on his chest. The doctor said Luke needs this surgery because the progression of his chest deformity has been great and it is now interfering with his breathing. The doctor said it would be best to perform it now because of his age. He would perform the Nuss Procedure costing approximately $70,000. This figure includes the doctor and hospital fee.

Here I go again – I am not in shock and I am not fearful – overwhelmed and desperate is a better description. I am overwhelmed at the prospect of raising $70,000.00 and desperate to do just that. We (The Luke Pier Foundation) must raise this money! Luke does not have a choice; this procedure is not for cosmetic purposes. I contacted the Shriners Hospitals for Children and they have denied Luke because of his aneurysm and there is no insurance to pay for this.

The doctor and hospital at Johns Hopkins will work with us but we must raise the money for Luke’s surgery. Please pray for The Luke Pier Foundation as we brain storm our next fundraiser. Yes, I am overwhelmed, but I will not continue in that state because Luke needs my help. I have chosen to believe that the funds will be raised and Luke will receive this needed surgery.

• Proverbs 3:5 & 6 Trust in the Lord with all your heart; and lean not unto thine own understanding. In all thy ways acknowledge Him, and He shall direct thy paths.
• Jeremiah 32:27 Behold, I am the Lord, the God of all flesh: is there any thing too hard for me?

That should get you caught up on where we are today. I will continue blogging as we navigate through this journey. Feel free to post your questions and I will try to answer them in future posts.

Vikki

People Are Talking!

We are excited about our 3rd Annual Softball tournament tomorrow, & we're even more excited to read that people are talking about us!

SJB Weddings & Events has been a sponsor of The Luke Pier Foundation since our inception, & they're still talking about us after all this time! Click here to read their great post about us!

We're also being talked about on our new Twitter account. If you are on Twitter, feel free to follow us! Our ID is @TLPF, & we post updates about our fundraisers as well as important info on the latest news & research of connective tissue disorders.

Thanks again, SJB Weddings & Events, for your continued support!

Your Chance to Make a Difference

We now offer four options for our softball tournament sponsors! If you are interested in sponsoring our 3rd Annual Softball Tournament, please contact us at info@thelukepierfoundation.org. Thank you!

$1,000 Level - Grand Slam Sponsors

• Logo on www.thelukepierfoundation.org


• Link to your website


• Brief paragraph about your business on www.thelukepierfoundation.org


• Top level logo on volunteer t-shirts


• Top level logo on sponsors banner displayed at tournament

$500 Level - Home Run Sponsors

• Logo on www.thelukepierfoundation.org


• Link to your website


• Mid-level logo on volunteer t-shirts


• Mid-level logo on sponsors banner displayed at tournament

$300 Level - Triple Sponsors

• Link to your website on www.thelukepierfoundation.org


• Lower-level logo on volunteer t-shirts

$100 Level - Base Hit Sponsors

• Your company name on www.thelukepierfoundation.org


• Your company name on volunteer t-shirts

Payment Methods

• PayPal *preferred method (Select the 'Donate Now' button below, or on our website, to sponsor the tournament)


• Check


• Money order


• Payment must be received by April 25, 2009

The Softball Tournament is the Foundation’s largest fundraiser each year and is crucial to our operation. We have set an aggressive goal of raising $35,000.00 in 2009. We need your support to reach this goal. Funds raised this year through the tournament will be used for the following:

• Funding for life-saving scientific research focusing on, but not limited to, connective tissue disorders
• Funding to individuals for medical treatment they may not otherwise receive due to limited resources or a lack of health insurance
• Encouragement to individuals with medical needs

Thank you for your support!

Volunteers Needed

We are looking for volunteers for our 3rd Annual Softball Tournament on May 2, 2009. Volunteers are needed to help us:

• Pass out flyers at local ball fields to advertise tournament
• Make phone calls to potential tournament sponsors
• Tournament set-up & tear-down
• Entrance gate (collect entrance fees & direct teams to the check-in tables)
• Food service during tournament
• Track games & report final scores back to main table
• Other tasks as needed

We will work with your availability! Please contact Katti (katti@thelukepierfoundation.org) to sign up!

It's That Time of Year!

Contact us to sign up your team or sponsor this event!

Luke's Next Appointment

Many of you readers like to follow Luke's progress & hear the latest news on his condition, so we wanted to let you know when his next appointment at Johns Hopkins is scheduled for.

Luke will have another echocardiogram on June 23, 2009, to monitor the aneurysm. Immediately following that appointment, he will have a routine exam at the genetics clinic as well.

Please pray for the doctors' wisdom as well as for Luke's condition.

We also wanted to mention that we have our 3rd annual softball tournament coming up on May 2, 2009! We are really excited about this event and will post more information very soon!

A Big Thank You!

We had a great time at Holiday From the Heart at Mercedes-Benz of South Charlotte! Approximately 50 people attended, & we were able to get our name out to that many more people in the Charlotte area!

The silent auction table was amazing - so many great businesses & individuals donated to the auction this year, & we are so thankful to them for their participation! Check out our website for more information about the sponsors of this year's event!

We thought you would enjoy seeing a few pictures from the event. If you were not able to attend, you really missed out on a wonderful night of food, fun & spreading the word about The Luke Pier Foundation, aortic aneurysms & connective tissue disorders. Click here to view the full album.

Live cooking demonstration by Blue Restaurant.
The Kimberly Carper Band donated their time to the event & did a wonderful job singing some amazing blues & jazz!
A large view of the dealership, including the great silent auction table right in the center.

Thank you again to all who came & donated their time & money to our great cause. In all we raised approximately $1900 from the silent auction, & Mercedes-Benz of South Charlotte gave us a generous (& unexpected!) donation of $2500. THANK YOU TO EVERYONE!

Holiday From the Heart

*Click on the image to view the high-resolution invitation.

We are still in need of silent auction items for this fundraiser!

Please consider donating* a gift basket from your favorite store or gift card from your favorite restaurant to The Luke Pier Foundation to help make our silent auction a success. We are also accepting cash donations that we will use to purchase items for the silent auction! Please contact us today if you are able to donate* something to this exciting event! *Donations are tax deductible as permitted by law.

Save the Date

To contact us about this event, please send us an email!
*Click on the image to view it in a larger format.

Our Next Event!

Well, fall is officially here, & we here at The Luke Pier Foundation are busy planning our next event. It's hard to believe that this will be our 4th event already!

We have a meeting with the event hosts on Friday, & we hope to be able to give you more details after that planning session. In the meantime, please pray that the event is a success & that we can all pull together to finalize the details!

We have a lot of work ahead of us, & if you are able to volunteer your time & energy to helping us make this event successful, please let us know!

Stay tuned for more details about this exciting event!!!

What a day we had!

We had a successful tournament on Saturday, May 3, 2008. It's hard to believe it's over, after so many weeks & months of planning. We estimate that 275 people came out to watch & play in our 1-day tournament, which is definitely more than last year!

We had 14 teams play this year (10 men's & 4 co-ed's), & it was a day filled with some really great softball. There were some glitches because two of the teams didn't show up, but we learned a lot about what to do next time & will be ready for it if it happens again. We want to say a special thank-you to all of the men's teams that were so patient & flexible with us as we configured the new brackets!

In all, we raised approximately $6,300.00 - not bad for our 2nd tournament! We feel like each one will be better, & we can't wait for the next one. We do welcome your suggestions, so please send any suggestions to info@thelukepierfoundation.org!

Below are some great pictures from the day. We hope you enjoy them, & we look forward to seeing you at the next Luke Pier Foundation event! (If you are reading this via an email subscription or blog reader, you may need to visit the blog home page to view the slideshow.)

444Don't forget...you can subscribe to our blog so you don't miss out on any of the exciting news about The Luke Pier Foundation. Simply enter your email address in the box at the right of this post. Once you confirm your email subscription, you will receive notices whenever the blog is updated. It's a great way to stay informed!

Thank you, Volunteers!

We want to send out a special THANK-YOU to all of the volunteers who came to help us at the softball tournament this weekend! We could not have done it without you!

And the Winners Are...

Players started filing in around 8am on Saturday morning. The first games started at 9am, & the last game ended at 8pm. It was definitely a day full of some really great softball for the 2nd Annual Luke Pier Foundation tournament. We will post a slideshow of pictures soon, but we wanted to acknowledge the winning teams first!

The 2nd place winners are:

Total Combustion from Piedmont Natural Gas in Charlotte, NC won 2nd place in the Co-Ed's tournament. It was their first time playing together but you wouldn't have known it!JDC Builders came in 2nd Place in the Men's tournament. They played some really great games & were able to beat out 8 other teams for the 2nd place spot!

The 1st place winners are:

Double Play came in 1st in the Co-Ed's tournament. Here they are with Luke after he presented them with their trophy. They said they're ready to play again in the next tournament, so all you Co-Ed teams out there...watch out!

Broome Sign Company won 1st place in the men's tournament. Some of the players on this team were also on the winning team at our October tournament. They are proving themselves to be the team to beat! Their attitudes were apparent when they prayed for Luke, & then turned around & gave Luke the 1st place trophy because of the hard work he is doing to make The Luke Pier Foundation a success.

We want to say a special thank-you to all the teams who came to play on Saturday! Stay tuned for the slideshow...

Brackets are Here!

We have the brackets for the Men's & Co-ed's game available now! We are so excited because we officially have 12 men's teams & 4 co-ed's teams. To put this into perspective, we had 11 teams total last year!

• Click here for the Men's Bracket
• Click here for the Co-Ed's Bracket

Here is some basic info about the tournament:

• First Men's game starts at 9am
• First Co-Ed's game starts at 10:30am
• $5 minimum donation at the gate (Players, Under 18 & Volunteers free)
• Bojangles & Owen's Bagel & Deli are catering (all proceeds will benefit TLPF!), so please bring cash to purchase any food & drinks throughout the day
• Tournament is at Renaissance Park Athletic Complex at 1200 W Tyvola Road, Charlotte NC

Thanks again to all of you for helping us get closer to our fundraising goal of $50,000 in 2008! We'll see you Saturday!

Coming Soon!

We are only days away from our softball tournament, & we wanted to let all of you softball players know that the brackets will be announced SOON! (as early as today)

Please check back here or on our website often to see if the brackets have been posted. We will also contact all of our teams via email.

We are so excited about the tournament this year - it's already bigger & better than our first one - & that is thanks to all of our supporters' hard work!

THANK YOU!

It's Not Too Late!

We are still accepting teams for the May 3, 2008 softball tournament!

Please send us an email to sign up today! Registrations will be accepted thru April 29, 2008.

Don't miss out on this great event! We are still in need of both men's & co-ed's teams. You can check out our website for more details.

We hope to see you there!

Luke, The Radio Star!

The interview with Allen went really well yesterday! Luke did a great job talking to Allen about what life has been like for him since his aneurysm was first diagnosed. Luke also talked about the lifestyle changes he has had to make and what he misses most. Allen also focused on all the May 3rd tournament details to help us reach more people!

Tune in to 95.1 or 104.7 on April 20th & 27th from 6am-7am to hear the interview! You don't want to miss it!

Luke, we're proud of you! Allen, THANK YOU!

Hitting the airwaves!

We are so excited to announce that WBT, our Official Media Sponsor of the May 3rd softball tournament fundraiser, is now airing 30 second promos about the tournament on their radio station! They are helping us reach so many people with the information about our fundraiser!

We also have some other exciting radio news! A local Charlotte radio DJ, Allen, from Kiss 95.1 is conducting an interview with Luke & the Foundation today! The interview will air on April 20th & 27th from 6am-7am. Kiss 95.1 will air it on one of those dates, & 104.7 will air it on the other Sunday.

It's early for many of you in the Charlotte area, but maybe while you're sipping your coffee you'll remember to tune your radio to 95.1 or 104.7 & listen in!

The interview itself will be approximately 30 minutes long & will give us a chance to discuss the tournament, Luke & the Foundation in length. You don't want to miss it! Allen interviewed us last year & really helped us educate people on why we do what we do! Thanks again, Allen!