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A little sunshine…

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PCC 2012 (225)I remember when I first saw that bubbly smile…the joy that emanated from this little lady…

Abby Jones, is a bright and exuberant six year old.  She loves to play and laugh.  Just like any six year old, she has dreams and aspirations.  However, Abby is not your ordinary little girl.  Abby has Recessive Dystrophic Epidermolysis Bullosa, one of the most severe forms of EB.  Each day Abby faces challenges, she must endure things that no child should ever have to endure.

According to DebRA of America:

Although in some cases this form of EB can be mild with generalized blistering, typically the recessive forms of EB tend to be more severe. Onset is usually at birth with areas of missing skin. Generalized blistering then scarring can occur on skin surfaces and mucous membranes. Scarring may limit range of motion of extremities. Fusion of fingers and toes and contractures cause deformity and loss of function.

In some cases there is relatively mild blistering on hands, feet, elbows, and knees; these cases are very similar to dominant dystrophic EB. However, recessive dystrophic epidermolysis Bullosa typically is characterized as follows:

Blistering onset is at birth or soon afterwards. In some cases, nearly all skin surfaces and mucous membranes (from mouth to anus) are covered by blisters. Large areas may be devoid of skin. There is widespread scarring and deformity. Fingers and toes may become immobile. With recurrent scarring, fingers and/or toes may fuse together. Hands and arms may become fixed in a flexed position with resulting contractures. There is usually loss of the nails of the fingers and toes. Teeth may be malformed and delayed in appearing through the gums. Because routine dental care can raise blisters, many persons with RDEB have a higher than normal incidence of cavities. Blistering on the mucosal surfaces often cause scarring within the mouth and gastrointestinal tract. The ingestion of food may be limited due to microstomia (inability to fully open mouth due to scarring and contractures of the perioral region), painful swallowing, difficulty chewing, (due to poor dentition) esophageal webbing. In many cases chronic malnutrition, growth retardation and anemia may ensue. Involvement of the eyes can include eyelid inflammation with adhesions to the eyeball, as well as inflammation of the cornea or the conjunctiva (the mucous membrane covering the eyeball and the underside of the lids).

Six years ago, Abby’s family (like most EB families) were thrust into this whole newPCC 2012 (238) world.  Doctors were unsure of her condition or how to care for her, as is the case in many hospitals.  EB is so rare that most doctors will never see it in there lifetime.  Her family would have to learn how to care for their daughter, how to protect her skin from blisters and infection that could potentially be fatal.  This was of course unexpected and undoubtedly caused much anxiety and fear.  Imagine how her mom Valerie felt when her daughter was whisked away after only holding her for an hour, the agony of not knowing what is wrong or what to do.  As a mother, I can tell you that I would be horrified.

PCC 2012 (232)Abby’s family struggled though those first days and finally were able to bring her home after nearly three weeks.  They knew that life would be never be the same, but naturally they did everything that they could for their little girl.  And it is evident when you see her that she is loved so very much, she has a very special family.

Upon meeting Abby, you would almost never know that she suffers every day because she has this incredible spirit that just shines; the joy just flows from her!  As I watched her playing, I just soaked up all of the sunshine that she brought into the room.  Her attitude is beyond contagious!

Abby inspires me because through her struggles and her pain she continues to 185821_10150983073307810_1038996416_nembrace the joy in her life…a lesson so many of us could stand to learn.  Abby may have EB, but it certainly does not have her.  Thank you Abby for making me smile and bringing me joy.  I hope you know how very special you are.  You are such a beautiful little lady and you are going to do great things when you get older, in fact, you already are!

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A gift to the EB Community…

I remember when I first starting advocating for those with EB.  I was a bit overwhelmed and to be honest, nervous.  I had no idea what to expect when I started to reach out to these families.  Despite my trepidation, I started to make some connections.  One of the first and one of the most profound was when I “met” Sara Denslaw.

Photo Courtesy of Sara Denslaw

Photo Courtesy of Sara Denslaw

In all of my searches to find a story with a happy ending, I stumbled across “In Garrett’s Memory“…not exactly the happy ending that I was searching for.  I remember the sadness I felt when I saw his picture.  I could not imagine

Despite this, the site provided me with a wealth of information.  Information I had not seen on the other sites, this was information written by a mom who knows EB, not only are her children affected, but so is she.  I immersed myself and literally got lost in her blog, gaining knowledge along the way.  In addition to all of the information,  Sara’s blog also led me to the stories of so many others, including her “Family Blog”  Four Busy Bees and Two Little Butterflies.  It was in this blog that I saw hope, I saw a family affected by EB and not being defined by it.  This was truly a unique situation considering that Sara must contend with her own pain while she also tries comfort and ease the pain of her children.  I was totally captivated by this story and this mom.  I really needed to connect with her, so I did and it changed the way that I thought about Epidermolysis Bullosa and she definitely clarified many misconceptions.

Photo Courtesy of Laurie Sterner

Photo Courtesy of Laurie Sterner

Sara, is a very busy mother of five.  She along with her daughter Sami and son, Joey all suffer from Epidermolysis Bullosa Simplex Dowling Meara.  It is EB that also took the life of Garrett when he was less than two weeks old.  Approximately 43 babies each year are born with EB Simplex in the US and of those about 10 of those babies are born with Dowling Meara, the most severe form of simplex.  Often times I think that Simplex is downplayed because it is a milder form.  Joey TrunkHowever, it still presents many challenges and those challenges are not without pain, not without anxiety.

Despite the challenges that Sara faces everyday dealing with EB and just being a mom, she is dedicated to helping families navigate through their situations.  When there is a new family, she is one of the very first to reach out and offer help and guidance   The knowledge and support that she provides is definitely invaluable.  I know that in my own experience she has be a guide and blessing.  I know that when I need a better understanding or if I am struggling, I can reach out to Sara…she is always there.  It is such an honor to know someone who is so giving of their time and energy in the midst of their own struggles.

We spend a lot of time posting about the new babies and I really wanted to focus on this woman who has risen above EB and is using her own experiences for the good of an entire community.  Her situation is so unique and I really wanted to get her perspective. I recently asked Sara if she would share some thoughts with me…

I think a lot of people assume that since I have EB myself, I would know how to care for a baby with EB.  That assumption couldn’t have bee further from the truth when my oldest was born over 10 yrs ago!  Sure I knew what EB was and a good deal about it; but I had no idea how to care for a healthy baby let alone one with EB!  I brought non-stick pads and rolled gauze to the hospital with me.  I had no idea EB as an newborn would be so severe that she would need to spend 13 days in the NICU.  By the time she was four months old, she was missing over 75% of her skin.  She required bandage changes that took two hours everyday.  This resulted in $5000 a month in special wound care supplies! 

Then when Garrett was born I had no idea simplex could have such severe complications that those complications combined with unknowing doctors would cost him his life.  I know many people didn’t believe he had simplex.  The phrase I heard the most after he died was ‘ those with simplex can’t die’.  Well they do and he wasn’t the first and certainly hasn’t been the last either. 

PCC 2012 (132)Then came Joey and I know I was judged a lot by that decision, but I don’t think my life is worse off because I was born with EB; nor my children’s lives.  If I hadn’t been born with EB I wouldn’t know such wonderful people and I would know as much as I do to help others.  But even with all I know, I wasn’t prepared for what Joey came out with; no skin on his knees, ankles, wrist and feet.  He was Sami and Garrett rolled in to one.  Since we knew more about EB than the doctors did, he was able to come home with us,only to end up back there because his airway closed off due to blisters/inflammation a week later.  I never expected to be watching doctors try to save another one of my children when they were 10 days old because something as simple as eating caused so much irritation their airway swelled shut.  Our knowledge allowed us to do dressing changes in less time, however they were still time consuming and tedious.  His skin seemed to slide of from getting wet.  His skin was certainly worse than Sami’s.  He had to be wrapped head to toe, his skin was that fragile, it would just slough off.  I am not sure we will ever know why he was so much more severe than Sami, although we have our suspicions.

I think a lot of people think simplex is mild, easy, simple, but anyone who has dealt with the more severe forms of simplex know that simplex is far from simple.  There is nothing simple about a three month old missing 80% of the skin on the body who must endure a two hour dressing change daily and only get Motrin because his doctor thinks that since it’s just simplex he doesn’t need anything stronger.  Or lying in bed at night when he his sick, worrying he will stop breathing in the middle of the night because his airway sounds swollen or even knowing what a swollen airway sounds like for that matter!  But I wouldn’t trade this for anything.  My kids are happy and healthy.  They enjoy life and they do not let EB stop them for doing what they enjoy.  I enjoy getting up each morning to see how my ‘other’ family is doing as well; reading about all the other EB kids and adults I know.  Sure some things I read are sad, really sad but a lot of the news and updates I read are happy, exciting, and milestone reaching.  While I wish there were no more babies with EB; I am grateful for the families who have allowed me to help, listen and advise them in order to make their journey through their new life with EB a little easier to travel.  -Sara Denslaw

I finally got to meet Sara this summer when I attended the PCC in Florida, it was certainly about time!  I also got to meet her family including Sami and Joey.  What amazing kids…both very happy!

Courtesy of Sara Denslaw

Courtesy of Sara Denslaw

Sami is a very active 5th grader despite her EB and challenges with Scoliosis.  She loves to play with her friends, sing in the school choir and girl scouts (she even goes camping with her troop)!

Joey, is a happy, VERY active almost 2 year PCC 2012 (304)old.  He loves to run, jump, climb on EVERYTHING.  To look at his bandages and blisters you know he has EB.  But to look at this activity level, you’d never guess it!  While is EB has gotten better with age, he is still no where near Sami was by age of two.  His bath and dressing takes about 45 minutes daily and he is still wrapped from his chest down to his ankles.

Sara does so much for this community and I really wanted to take an opportunity to thank her.  Not only for the guidance she has provided me, but the way that she is continuously giving back to the EB community.   I wanted those who have not had the chance to get to know her to learn about her and her family.  I really wanted her to be recognized for all that she does for so many.  Sara is constantly supporting other families and providing people with knowledge that we otherwise would not have.  She is a gift and a blessing to so many.   So Sara, I thank you…this community thanks you.

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Where have you been my butterfly friend?

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08-9-11
Today a little butterfly flew by me.
I thought to myself where have you been little butterfly.
You come into this world as a cocoon all by yourself and blossom into
this beautiful butterfly and fly off to see the world.
What you don’t realize little butterfly as you flutter through your days
is how you touch those around you in your soft gentle way.
You don’t even realize the wonder and awe you create around you.
He fluttered his wings toward me as if he was waving good-by as he
headed towards the horizon.
He looked very happy and content as he went on her way, as if to say
to me “Don’t worry I’ll be okay”.
I was sad to see him go for he had touched my heart in such a way that
I knew my life would never be the same.
He had left an imprint of all the beauty life has to offer.
I knew each time I looked at another butterfly or horizon I would
remember our moment in time when it was only him and I.
I knew I would be a better person all because this little butterfly flew by
me one bright sunny day.

© Barbara Ann Rogers

Tripp 2Interesting how we get what we need just when we need it, little signs that come just at the right time.  I had been thinking long and hard about writing a post to honor Tripp Roth today.  The words were not coming to me as easily as I had hoped and I was totally unprepared how emotional I would be trying to write about him today.  I thought I would search for a quote to help me get started and so I did a quick Google search, hit enter and went to get my coffee.  This is what was waiting for me when I got back to my desk.  As I read, the tears came to my eyes, for I knew that he was speaking to me and guiding me as he did so many times during his life.  It was exactly what I needed and exactly the right time.

Many of us search our whole lives, seeking our purpose, and I was blessed to haveOct 21 2011_0262BW found it in the incredible life of a little boy.  It was again what I needed right at the right moment, a simple mouse click on a link and I was forever changed.  It was a gift that humbles me each and everyday.  I know that I am a better person today because of him and everything I do is because he came into my life and changed it.  He gave me the purpose I had so long been seeking.

I am only one of many who has been impacted in some way by Tripp’s life.  Through his suffering, he taught many about courage and faith.  Despite the challenges of life with Epidermolysis Bullosa, he taught all about love and gave people hope.  He has left a tremendous imprint on the hearts of many, and even in his absence, he is changing lives through his story.

Tripp Roth 12-2 thru 12-3-2011 (36)A year ago today, the news of Tripp’s passing shattered many of us.  The loss of this beautiful spirit rocked the EB community and the entire group of supporters who had been following his journey and praying for him.  I am one of many who never wanted him to leave and now he was gone…or is he?

I don’t think so, I think that he lives on in all of the work that we do each and every day fighting for those with EB.  I think that he continuously graces us with his spirit, inspiring us to continue to advocate and fight…to be a voice to all of those who suffer.

Today more people know about Epidermolysis Bullosa than ever before. There are advocates across the country who are raising awareness, people who are dedicated to finding a cure and end the needless suffering.  This is not a coincidence, it is the spirit of one very special butterfly guiding us on a path of compassion and love; helping us to be better people.

Today my heart is with Courtney.  So many of us have been inspired by her unconditional love, her unwavering strength and commitment to her son.  Courtney,  thank you for sharing Tripp with the world, for trusting us to love him too.  Thank you for facing criticism and negativity so that we could experience the many blessings and the joy he brought.  Opening yourself up to us all so that we could share this journey with you.  I love you today and always my sweet friend.  Please know that Tripp is our hearts always; his spirit still shines.

Tripp Roth 10-21-2011 (49)In parting, God said, “Do not forget little soul that I will be with you always. Although you have agreed to bear the pain, you will do so through my strength. And if the time should come when you feel that you have suffered enough, just say the word, think the thought, and you will be healed.” Thus at that moment the brave little soul was born into the world, and through his suffering and God’s strength, he unlocked the goodness and love in people’s hearts. For so many people dropped their differences and came together to show their love. Priorities became properly aligned. People gave from their hearts. Those that were always too busy found time. Many began new spiritual journeys, some regained lost faith – many came back to God. Parents hugged their children tighter. Friends and family grew closer. Old friends got together and new friendships were made. Distant family reunited, and every family spent more time together. Everyone prayed. Peace and love reigned. Lives changed forever. It was good. The world was a better place. The miracle had happened. God was pleased.  – an excerpt from The Brave Little Soul by John Alessi

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A lesson of love, learned…

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As we start the new year, I have so many beautiful stories to tell, each one so very special to me.  As I get to know so many new families, I am always so humbled by the lessons that I learn; lessons of love, gratitude, faith and hope.

PCC 2012 (94)This is Blake, a bright and cheery little guy who is full of personality!  Blake was born on March 7, 2011 and was diagnosed with EB Simplex Dowling Meara.  EBS-DM one of the most severe types of EB Simplex, and as I have learned each and every case can be and is usually different.  Often those with Simplex are “forgotten” because they do not appear as severe as some of the other types of EB, however despite the fact that Simplex is a milder form of EB, it is still very difficult to live with and presents numerous obstacles.

According to www.eb-nurse.org:

“Infants often are born with widespread blisters on the face, body and limbs.
Congenital localized absence of skin (also known as aplasia cutis congenita and, historically, as Bart’s syndrome) may be present at birth and usually affects the arms and/or legs. Often, with severely affected infants, parents report the occurrence of as many as 200 blisters per day. The widespread blistering of EBS-DM may lead to serious infection, along with feeding problems and the development of failure to thrive. Death rarely has been reported during infancy.

Blisters occur more frequently the weather is hot and humid. Increased physical and emotional stress also have been reported to cause an increase in blistering. Interestingly, some individuals with EBS-DM have reported an improvement in blistering when they have a fever. Blisters tend to decrease in number and severity for most patients as they grow older..”

Unfortunately, Blake’s blistering is wide spread and requires hours of daily wound 1355629657023care; an incredibly painful process for him.  His blistering has not only affected the outside, but it has cause numerous feeding issues.  He has been in feeding therapy, but continues to have issues.

In addition to feeding issues, 1355629605903.  his feet are severely blistered and yet he walks enduring pain that most of us cannot even imagine without complaining.  Blake faces multiple challenges daily as a result of his EB, and despite this, he still enjoys being a little boy and doing the things that a little boy should.  He loves to play and laugh…not to mention bugging his little sister and doing everything that she does. It was such an honor to meet 557986_10150979297147810_1984649274_nhim and his family this summer after “meeting” online months earlier.  I remember the first time that I saw his big eyes this little pout.  There were people everywhere and he was no doubt overwhelmed.  PCC 2012 (36)He clung to his daddy where he felt safe and loved.  However, it did not take him long to find a new little friend and let that little personality shine. PCC 2012 (99) I could have watched him and his new little friend Eleanor play for hours, both of them so excited and full of this exuberance!  You could tell by watching Blake and how he interacted that he had a very loving little heart and that despite the fact that he suffered the pain of EB he was loved deeply.   Tammy, like so many EB moms that I have gotten to know, is truly amazing.  You can see the love that she has for Blake in everything that she does and just the way she glows when she holds him. PCC 2012 (354) Her connection with him is so apparent and it is truly something that inspires me and the way I parent my own children.  When I see the abundant joy of this family I am just in awe.  I often find myself taking for granted how easy I really have it.  This past holiday season everyone was sick and I remember feeling so defeated and just exhausted.  I was frustrated and overwhelmed and relieved that my kids were in bed.  Then I sat to write this post and I broke down in tears as I looked at the smiles on Tammy and Josh’s faces. PCC 2012 (353) For in their smiles I see the gratitude that they have and the unconditional love that flows freely from them despite the many struggles that they must endure each day.  Through this family I am reminded to appreciate the many blessings that I have been given and to love every moment regardless of the situation.  Tammy and Josh…it is truly an honor to know you…thank you for allowing me to share Blake with others and for teaching me tremendous lessons in love!

As we enter into this new year, I hope that we can all resolve to love a little more an worry a lot less.  That we can be compassionate and caring not only towards those we love, but those we may struggle to love.  May we be reminded of the countless blessings that have been bestowed upon us and embrace them, looking beyond our struggles to see the joy.

Namaste

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2012 in review

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Thank you so much for all of your support in 2012.  I have so many more stories to share and I hope that 2013 will be better than ever.  I hope that my posts will inspire you and touch your heart.  I hope that you will be called to action to help make a difference and follow your passion!

Namaste

Christie

Here’s an excerpt:

4,329 films were submitted to the 2012 Cannes Film Festival. This blog had 16,000 views in 2012. If each view were a film, this blog would power 4 Film Festivals

Click here to see the complete report.

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With deep gratitude…

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Along this journey, I have had the opportunity to meet some pretty amazing families. Families that inspire me and drive my passion. Each and everyone touching me in different ways, helping me to be a better person.

This latest family was no exception.  To be quite honest, I did not expect to be affected in such an emotional way, but this young boy has captured my heart and I have just fallen in love with him and his incredible spirit.

I would like to introduce you to Eduardo.

Eduardo has been braving the challenges of EB for eleven years. That is eleven years of pain and suffering…eleven years of bandages and blisters. He has seen more pain in his short life than any of us will ever know and yet he just keeps smiling this beautiful smile. Eduardo’s skin certainly shows the evidence of his pain and the effect that EB has had, his eyes however are full of wonder and HOPE. Those beautiful eyes captivated me and stole my heart.  As did his tremendous gratitude.  Despite everything he has endured, he continues to be thankful…really an amazing and beautiful thing.

Eduardo is currently at recovering from his bone marrow transplant at the University of Minnesota Amplatz Children’s Hospital, where I picked him up last Friday.  Normally when I am meeting families, they are families with a baby who has EB. I rarely have the chance to spend time with someone older, someone who can articulate their pain in such a profound way.  Never once did he complain, he just expressed himself in such a way, his attitude was so powerful.

When I left Ronald McDonald House, I  had some much needed perspective.  Meeting with Eduardo was indeed a reminder to appreciate every single moment and embrace it with gratitude.  We take so many things for granted and yet here is this boy who suffers day in and day out and he is nothing but thankful for his day.

I am not sure if Eduardo will ever know the depths of his impact on me, but I am so grateful. I felt so much joy being with him and he gave me some pretty beautiful gifts that day. I would love nothing more than to be able to give him an incredible gift this Christmas.

Eduardo will be spending Christmas in Minnesota, away from his family and I would love to send some joy his way.  Would you join me in making his Christmas bright by filling his Christmas tree with ornaments from all over?  You can send any ornament you wish.  Or you can also place an order at www.personalizedfree.com and use the code PUCK to have a portion of the proceeds donated to the research that is helping to give Eduardo a better life.  You can ship ornaments or any gift directly to Eduardo Romero. 621 Oak Street SE, Minneapolis, MN 55414

If you are in Minnesota, you can meet Eduardo at this years Wings of Hope benefit on December 1, 2012.  You will also hear Quinn’s story from her dad Marc Seymour, Trisha Knuth will be sharing Charlie’s amazing journey, and Dr. Tolar will also be presenting.  It is sure to be nothing short of inspirational.  Please feel free to send me an email if you have any questions christie@puckfund.org

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Honoring Aubrey…

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I have spent the last day reflecting on my time in Michigan, trying to collect my thoughts and trying to come up with the words that could describe the last several days.  I thought that it would come easily, but I am finding that I am more overwhelmed than I had anticipated.

Aubrey Joy Courtesy of Dave and Angela Oberlin

Aubrey and Angela, Courtesy of Dave and Angela Oberlin

If you do not know her story, Aubrey Joy Oberlin was born on October 6, 2010. They noticed right away that Aubrey was missing skin on her left foot and that she had small blisters on various parts of her body.  She was airlifted to University of Michigan Mott’s Children’s Hospital and on November 1st was given the diagnosis of Junctional Epidermolysis Bullosa -Herlitz.  It is one of the most severe and often fatal forms of Epidermolysis Bullosa. This meant that EB was not only causing blistering on the outside, it was slowly affecting her body on the inside.  After six weeks, on November 17, 2010, sweet little Aubrey lost her battle with EB.

Although she was only here for a short time, she impacted the lives of so many and created purpose in the lives of some some very special people.  People like my beautiful friend Andrea Ness, who is so dedicated to the EB community and does many things in Aubrey’s memory.  Andrea was instrumental in the coordinating of Aubrey’s 5K, and it was such a privilege to be able to be there to support her, Aubrey’s parents and the rest of the planning committee.

It was such a beautiful morning…the sun was shining so brightly warming us from the crisp air.  It was amazing to watch as everything came together…this small community coming together for this special little girl, it was such a beautiful thing!

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I am not a seasoned runner by any means, but I have a few under my belt now and yet I was so unprepared for how I would be affected by this race.  It will be one of those that is added to the list of moments that have had a profound impact…very profound.

Let me start by saying that I have never run without my iPod…I depend upon it to help me pace myself and to assist me in NOT focusing on the time.  Simply put, it makes the time go by faster and I am far more motivated when I have it.  Shortly before the race, I discovered that despite being plugged in all night, my trusty friend was out of power.  How that could possibly be was beyond me.  What was I going to do, the race was just about to start…

Since I did not have any music, what could I do?  Well, I decided it was a good time to pray.  I thought about Aubrey and all of the other angels like Lucas, Quinn, Bella and Tripp.  I thought about their lives and what they meant, how their footprints have left such a lasting mark on me and the rest of the world.  As hard as I tired, I could not keep the emotions from creeping up on me.  I kept replaying Aubrey’s video in my head…

As I reached the end of the race I discovered that there was a reason my iPod did not work.  You see if I had been listening to my iPod, I would have never heard Aubrey’s song as I turned the corner to go pass the cemetery.  I would have never experienced that profound feeling that came over me as I listened to the words and felt her presence there.  Fighting back the tears was completely useless…I totally let the emotion take over as I thought about that fragile little life and all of the other precious angels.  I thought about their pain and about all that they endured during their lives. Then I ran hard…my determination took over. My motivation was strengthened and my passion deepened. The journey has only just begun, we have so much work that needs to be done and it is up to us to fight this fight and be a voice.

I am grateful to Angie and Dave for sharing Aubrey’s story and allowing us to fall in love with her and honor her in all that we do in the fight against EB. Her suffering will not be in vain, for she will continue to touch hearts and spark passion in many. Her story will bring awareness and help lead us to a cure.

This weekend was such a blessing and I am so grateful to have been a part of it…once again I have been touched by an angel.

This week marks the beginning of EB awareness week and I encourage everyone to try to tell at least one person what EB is. Do what you can to bring awareness to your community…you could be the one…

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A special EB princess…

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It was about a year ago when I came across a picture that completely drew me in.  It was an image of this beautiful little girl with the most AMAZING eyes, big gorgeous brown eyes…this is Savvy…

Savannah was born on November 8, 2010 and despite having no complications and a very quick delivery, something was wrong.  Within seconds, the room door slammed open and three NICU staff came in and swept Savvy up.  It was happening fast, and her parents knew something that things were not right. The nurses asked if they wanted to hold her, but insisted it could just be for a minute because they needed to take her away for some tests.  They handed Savvy to her mother Angel, and in that moment she knew that there was something desperately wrong.

Savannah had no skin from ankles down to toes or on her side.  It looked almost as if she had been shredded.   Before Angel could even find the words, they whisked her away and left her mommy and daddy without their daughter and no answers.  Confused and scared.

The doctors had not seen this and had no answers. Savvy would need to be transported to another hospital, it was the only way to determine what was wrong.  Angel was given Savvy to hold and through her tears, she took pictures and fed  Savvy not knowing what the future would hold.  What was going on, will she be okay?  Countless doctors and nurses came in to express their sorrow to Angel and her husband, but that certainly did not ease the pain or anxiety they felt as they watched Savvy being taken away in an isolette, hooked up to what seemed like a million of machines.  They signed some papers and blew a tear infused kiss their, their beautiful baby, and then she was gone.

The next week was spent with a sea of doctors and nurses and medically licensed people scratching their heads until finally they received the news.  Savvy had Epidermolysis Bullosa, a devastating blow to any parent.  In that moment, their lives were changed.  They now had to learn about the vital wound care that their daughter would need and all of the special care that would be required.  Finally though, Savvy got to go home to the family who was waiting to love her.

Almost two years later, Savvy is as cute and as sassy as ever! When I saw Savvy in person for the first time I was again captivated by her amazing eyes.  Then she held me with her personality and her sass!  She is such an incredible child who is undoubtedly loved and cherished.  I watched as Angel and Alberto cared for their little princess and it was truly so touching to watch this mommy and daddy work in unison.

Savvy is incredibly determined and she will do great things in her life…she will not allow EB to define her and it will not slow her down.

I think that her Mommy says it best: “Our daughter grows, and smiles and throws things, but she also bleeds and suffers and longs for walking abilities. And we love her. And she truly changed our lives for the better.”

Through the smiles, there is pain and it is a pain that no one should ever have to endure.  Savvy is one of many who suffer, one of many who need your help.  You can find out more about EB by visiting DebRA’s website www.debra.org You cna help to make a differnece in the lives of kids (and adults) like savvy by educating yourself, supporting those affected and raising awareness.  With your help, EB will no longer be “The worst disease you NEVER heard of”.

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Honoring an EB Soldier…meet Ben…

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This summer I had the opportunity to meet so many of the families and children whom I have come to know and love. One of these, is a very special little guy.

Meet Ben…he is an AMAZING little boy with the personality to match!  I just look at his huge, beautiful brown eyes.  They are gorgeous and they have a story to tell.

Photo Courtesy of Sirena Wiley

Photo courtesy of Sirena Wiley

Last October, Benjamin Joseph Wiley, Jr came into this world missing most of the skin on his hands and on his left foot. His mouth and throat were full of blisters. Ben was born with Epidermolysis Bullosa. The doctors had never seen anything like it. What was causing this trauma and how is it treated? They just did not have the answers and so baby Ben was transferred to Children’s Hospital in St. Petersburg, Florida where he would spend four weeks in the NICU.

Photo courtesy of Sirena Wiley

Because EB is so rare, it was difficult to find anyone who’s could care for his fragile little body. He was constantly covered in Aquaphor in order to prevent any friction that would cause the skin to shear off and blister. He was unable eat by mouth until they were able to control the excruciating pain he was in. As a mother, I cannot even begin to imagine the stress that this caused his parents Sierna and Ben Wiley.

By the time he left the hospital, he had been in the care of over nineteen doctors which included plastic surgeons, a GI specialist, dermatologist, skin teams, radiologist, respiratory team and nenonatologists. He was officially diagnosed with EB on November 8, 2011, his results were however, were inconclusive as to which type he is affected with.  So when he did finally go home, his parents were still left with many unanswered questions. A bittersweet moment being able to bring your baby home and yet being scared to death of all of the unknowns.

Ben Jr. Has been such a tremendous blessing to his parents, making them a stronger as a couple and stronger in their faith. They do not know what the future holds for their son, but they are determined to fight for him…doing whatever it takes.

Ben will turn one this October, a pretty special milestone to those who are born with EB. His parents still,struggle on a daily basis to cover expenses for his ongoing care. Please take a moment to help support this family and honor Ben on his first birthday. An online fundraiser has been set up HERE

If you are in the area, you can join in the celebration.  The details are are all listed in the Facebook event Honoring an EB Soldier. They will be hosting a wonderful party that will include an auction to raise funds for DebRA

You can also follow Ben’s story and receive updates on Facebook.

I want to thank Ben Jr.’s parents for allowing me to share his story.  It is my hope that his first birthday will be a smashing success and that he receives the support that is so needed and deserved.

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Seeing beyond EB…

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Not many of us have the privilege to be touched in a way that forever changes you.  I consider myself one of the very fortunate ones.  I have been given a very rare opportunity to capture some really amazing families, and I am forever grateful, for each one brings something beautiful into my life.  I have had the honor of capturing joyful moments of one of life’s most challenging and heartbreaking journeys and I can tell you it has been so very humbling.

When I was asked to photograph this year’s DebRA Patient Care Conference, I knew that this would be another life changing experience and it went far beyond that.

Most people would assume that I would go into something like this feeling sadness, but that is not the case.  Aside from great anticipation, I felt a sense of peace, almost a type of joy.  Joy you may ask?  How could one possibly feel joy a midst such suffering.  I see joy because I look beyond the EB and see the truly amazing spirit of these kids and adults.   So often we focus on the pain and the suffering (which is certainly part of the reality) and we allow EB to define the person when they are so much more than that.  Just look into their eyes and you will see that they too have dreams and aspirations.  Look at their beautiful smiles and radiant light beaming, to be in their presence is an amazing gift.  Just knowing and loving these families means that life can never be the same.

Just take a moment and see for yourself….

Again, I just want to thank DebRA for giving me this incredible opportunity…it is truly such an honor and blessing.  To the EB community, my love goes out to you each and every day.  I am dedicated to be sure that your suffering, your child’s suffering will never be in vain.  I will continue to be a voice until a cure is found.