I am sooooooo excited to announce that Evan has started moving forward in his pony-walker. He is still working on sitting, rolling, propping and crawling but has finally reacher his "Ah Ha" moment with the 'walking' part. Last night I recorded this video which was the the first time he took more than one step forward (for the past two weeks he has only moved backwards). Any movement is a major achievement for this little guy. I can definitely say that I am "One Happy Mama". Yay!
Love my lil guy
Off to finish up the hurricane prepping. Hoping the power doesn't go out but at least this year I am more prepared than last. Stay safe East Coast!
"Epidermolysis Bullosa (EB) is a rare genetic skin disease that causes the skin to be so fragile that the slightest friction can cause severe blistering—inside and outside the body. Today there is no cure. Severe forms of EB cause patients to live with constant pain and scarring. The worst forms of EB lead to eventual disfigurement, disability and often early death.There are many patients who are diagnosed with milder forms, which, while they can be extremely difficult to live with, are non-disfiguring and non-lethal.
The only treatment for EB is daily wound care and bandaging. The daily routine is a grueling, multi-faceted daily regimen. Caregivers, often parents or family members of EB children, must work in tandem with medical professionals to determine and administer different treatment methods to care for EB wounds.
With skin as fragile as a butterfly wing, EB patients are dubbed “Butterfly Children”. On the outside physical wounds prevent them from normal daily activities enjoyed by other children. On the inside, their dreams are the same as any child who loves, plays, learns and grows despite the pain and impediment caused by their disease. With the programs and services of Debra of America, EB’s Butterfly Children and their families find the support they so desperately need. Debra of America works to ensure that a life of struggle is also a life of hope for the 1 out of every 50,000 live births in the United States affected by EB." Learn more at www.debra.org
EB is pretty much the exact opposite of what Evan has. Evan's skin grows at an excessive rate and requires consistent exfoliation and moisturizing to prevent a tight crippling armor. The slightest friction to someone affected with EB can cause that skin to blister, leaving open painful wounds. It is a vicious skin condition that needs more advocates to help promote awareness.
I was oblivious to the variety and the severity of skin disorders and diseases that existed before Evan was born. A week after his birth, the National Geographic Channel aired the show Extraordinary Humans: Skin. The episode focussed on two individuals who were living with a severe, life threatening skin condition. My husband and I were anxious to see this show since one of the individuals was affected with Harlequin Ichthyosis (I wrote about that in an earlier post here). What a coincidence it was to gain this information so quickly after his birth. The timing could not have been more perfect. But the reason why I bring this up is because the other child interviewed on the show was a young boy with Epidermolysis Bullosa.
I was a bit overwhelmed after seeing what Harlequin Ichthyosis entailed. But once the next story began, my heart began to ache even more. I do remembering saying to my husband, after seeing what a child with a severe form of EB goes through, "Evan has it bad but it could be worse." Learning about these two skin conditions has opened my eyes to not take anything for granted. As much as I did not want my child to suffer with the many challenges he faced, I felt so empathetic for that child with EB. Having a slight idea of what Evan's skin care regimen would entail, I could not imagine spending hours bandaging and cleaning wounds which would endure an enormous amount of pain. To think the slightest touch could be devastating to their skin is heartbreaking; even a kiss or hug.
Fast forwarding about a year and a half, Facebook led me to a mother's story about her son Tripp. From a share of a share this story came up in my feed. Courtney's blog "EB"ing" a Mommy has touched me deeply. I began to read her story about a month before this precious boy went to heaven. She is such a wonderful mother and cared so much for her baby. There has never been a time where I read her blog and did't cry. It has made me truly appreciate everything I have in my life and has made me realize that these severe skin conditions need more awareness so a cure can be found.
EB needs a cure just as much as Ichthyosis does. But in my personal opinion, I hope they find one for EB first. The suffering these children and adults have to go through is horrible. Even when taken care of so gently and carefully, their skin is just too fragile. Those who have severe forms of EB have inner tissues that are just as delicate and require feeding tubes to avoid friction from chewing and swallowing. Everything about it seems so painful. As much as I want a cure for my baby, I know that right now he is happy and 'comfortable'. A cure for those affected with EB is essential. Any donation is a step closer to a cure. DONATE TO HELP CURE EB
Help educate yourself and others by sharing this post or any of the EB related sites in honor of Epidermolysis Bullosa Awareness Week. Here are a few sites, blogs, facebook pages and videos focussing on living with, caring for or educating others about EB.
(Debra) Dystrophic Epidermolysis Bullosa Reasearch Association of America- www.debra.org
When people see Evan, many say that his skin looks great, including his Dermatologist. About 95% of the time they do not notice that he is scaly. There are many reasons for this and the number one reason for it, is his skin care. A few other reasons are that his scales are white and he does not have the most severe form of Harlequin Ichthyosis. I was told by one of his doctors that since they found his mutation at the end of the protein, it was likely, that it was a milder form of HI. And if the mutation was found in the beginning of the protein it would be a more severe case. (Don't quote me on that.. still have A LOT to learn in the genetics department.) Also, Evan was born at 30 weeks 5 days. I can't imagine how much MORE skin would grow if he was born 9 weeks later. He started receiving skin care when he was 31 weeks old. I see how much skin he grows in one day so I can't imagine another 9 weeks of untreated skin manipulating his features, digits or the tightness of his chest.
Things obviously happen for a reason. Evan fought and thrived through his NICU days and continues to thrive in his own way. Though, the help he has been given from his Mommy and Daddy are helping to contribute to his success (not to talk myself up or anything ;-p). Joe and I work very hard on skin care. We have been consistent with our routine and Evan has been cooperating, for the most part. We have been very, very, very lucky that he has not gotten a skin infection (knock on wood) and has only had two mild eye infections. Evan still tolerates the 40-60 minute baths with exception to exfoliation of his hands or feet. He will relax for the most part, play with a toy, splash his feet and hands, giggle, babble or sign along to songs. Once the hands are scrubbed he will fuss, whine or even scream. And once you let his hand go, he smiles and goes back to what he was doing. He'll react the same way when we scrub his feet. His hands and feet are very scaly (very scaly to me, is when I struggle to get it off since it is thicker than card stock). Not being able to properly exfoliate his hands or feet due to his intolerance allows for quick build up. We have been using Tazorac (topical skin retinoid which helps breakdown scale) on his palms and feet a few times a week when necessary.
Before a bath & not even that scaly
If I didn't have Joe, Evan's feet and hands would be even worse. Joe ignores the fussing and I help distract Evan so he can exfoliate these areas. Usually, Joe will not stop until his feet and hands are 'smooth'. We obviously will not let exfoliation really upset Evan. If he is actually crying we will stop to avoid making it a completely negative experience. Not only is Joe great during bath time he is a constant picker. He seems to have an addiction with peeling scale. There are obvious pieces that will come off easy with no harm. But there are pieces that I would not dare attempt and Joe does. Evan doesn't fuss because it probably feels good to get the tight & itchy 'flakes' off. This picking contributes enormously to the quality of his skin but does have its risks. Besides moisturizing, descaling is the next most important thing.
During a bath splashing away
We use A LOT of Aquaphor (healing ointment). We are fortunate that the company supplies us with 50% of what we use. It is amazing that this company helps those with Ichthyosis by providing a case every three months (read more about that here). When Evan was little, it was enough but now we will go through at least two cases every three months, which averages out to at least 2 jars a week. I can't imagine having to pay for all of that ourselves. Having that reliance from this company is a major relief. I use a lot of Aquaphor because it is the only thing that keeps his skin moist. If Evan's skin is left dry it could easily break, rip or tear leaving him susceptible to infection. Lotions are not effective for Evan. Seconds after putting it on was as if you never put anything on his skin. He sucks it up like a sponge and then its gone. So things are pretty sticky around here! Evan obviously likes the Aquaphor since he acts like goof ball during applications and diaper changes. It makes a mess and is hard to clean up but it is helping to keep my son alive so I can deal with the greasy clothes, toys, blankets and smudges around the house. But it will be interesting once he is motor!
After a bath chillin' out
The only time I let Evan get really dry, is before a bath. Trying to bathe him with a fresh coating of Aqauphor on is near impossible and a waste of time. Once he is dry and very flakey we are much more successful with exfoliation and he is able to easily soak up moisture. We are lucky that Evan has been so well behaved with baths and skin care and I hope he continues to be tolerant. I have been holding off on compound creams or other types of retinoids (which help with thick scaling) unless is it absolutely necessary. I cannot imagine Evan putting up with all of the scrubbing when he is at an age where he'd rather play. So when exfoliating or descaling is unsuccessful we do have options to help with it.
Being home with Evan has been a huge benefit for both of us. I am fortunate to be able to take care of Evan, participate in all of his therapies and be in control of his skin care and feedings. I feel most comfortable being responsible for his complete care. In less than a year he will be headed to preschool where he will receive his therapies. This stresses me out beyond belief. I know I will eventually have to let go and let someone else care for him. I just hope that when we make that step towards preschool he will be communicating better and will be moving around more on his own. We have a lot of goals to reach and achieve in the next 10 months and I am optimistic that we will succeed.
This past weekend a good friend of ours ran the Hartford Half-Marathon in honor of Evan and to support the FIRST Foundation. We were able to go and support Leif as he accomplished this goal. Not only was it a very cold morning but it was an early morning for Evan since Leif had planned on making excellent time. Leif finished his 13 miler in 1:29:44. Wowzers! That is less than a 7 minute mile. Impressive.
Of course once Leif finished, we all headed straight for the beer garden. Granted it was only 10am, we had to celebrate Leif's amazing accomplishment. It was great to hang out for a bit and snap some pics while drinking our morning beverage. Good thing I ate breakfast! Leif even made his own shirt with Evan's picture on it which read "I ran in support of Evan and the FIRST Foundation". We are very proud of Leif and are very grateful that he has helped support FIRST. Thank you to everyone who supported Leif by giving donations for the FIRST Foundation. Every penny is a step closer to a cure.
First big news: Evan got a pony walker. Yay!!! I am so excited that he has the opportunity to be mobile on top of getting a bit of independence for the first time in 2+ years. He still has a long way to go but getting his feet onto the floor is nothing but good for him. And to see his face light up as I help him step and walk is sooooo adorable. It is obvious that he enjoys it and he is starting to 'get it' already. It has only been one week since we got it and he is pushing backwards to move. I'll take anything at this point. He will learn to move forward with more practice.
I think he likes it
This past weekend was our bi-annual family wine crush. I married into a pretty cool family I must say! The wine crush is always a nice celebration where we get to eats lots of yummy food, listen to traditional Italian music and drink some home-made wine while makin' some. Evan's first wine crush was just about a month after he came home from the NICU. So it was one of the first real outings he went to. It was nice to see the how far he had come since his wee days. This year he had his little cousin Alison to entertain. It was great to see them interact. And of course we took plenty of pictures.
Wine Crush 2010
Wine Crush 2012
(Evan is actually sitting in Joe's arms. His legs are twice as long.
And good thing Joe's mustache didn't last!)
Fall is my favorite season. I consider myself to be a bit of a 'leafer' or what some call 'leaf peeper'. Most people refer to 'leaf peepers' as the tourists and out-of-towners that come around this time of year to check out the foliage. I, on the other hand, consider myself to be a 'leafer' because I am obsessed with watching the foliage and taking pictures of it. I get so excited when I see a nice vibrant red or orange tree. I honestly need to pay more attention when driving instead of looking at all the nice color.
The start to some color
Last year we got gipped on the foliage and Halloween. Thanks to Storm Alfred, we had almost 2ft of snow on the ground along with huge branches, trees and power lines. The storm pretty much knocked out the whole state for close to a week, more in some parts. The foliage, still on many of the trees, was the culprit; the trees couldn't handle the weight of the snow and ice. Not only did we have no power, water or heat but Halloween was approaching and it was taking a long time to restore power and clean up the damage. Most towns had to cancel Halloween and others rescheduled trick-or-treating to another night. (I was super bummed and I can't even go trick-or-treating anymore!)
Our backyard- Storm Alfred 2011
Our backyard- Storm Alfred 2011
I am very excited for Halloween this year. Finally, Evan is at a developmental stage where he can enjoy a hayride, pumpkin picking and maybe even a little trick-or-treating? (Ok, ok that would really be for me) When I dress Evan for Halloween, I haven't been crazy about putting him in an elaborate costume. I usually just stick to something comfy and easy on his skin. Once he is older the choice will obviously be left up to him. Evan's first Halloween he was dressed as Superman and during the blackout last year he wore his cowboy sleeper to bed.
Evan's 1st Halloween 2010
(gotta give props to Cassie for an excellent costume choice!)
I have many things to look forward to, the weeks following Halloween. For one, my husband won a trip to Puerto Rico!!!! It was pretty unexpected but no complaints here. Though I was extremely excited when I heard the news, it happened to be on the same weekend I already had a trip planned. And there was no way I would completely cancel this trip either. Luckily, I was able to reschedule it to the following weekend.
And where might I be going, you may ask? Well, Evan is finally going to meet a special friend, Brenna. We are heading out to Illinois to meet the Westlake family. I found Courtney's blog only a few weeks after Brenna was born. They were the first family that I reached out to in the Ichthyosis community. I felt willingly obligated to share my experiences, caring for a child with HI, to help their journey with Brenna. I am very much looking forward to meeting the whole Westlake clan. (one month count down today!)
The next few months are going to fly by. Before you know it, it will be Christmas and hopefully then, we will be celebrating Evan's accomplishment of sitting on his own. Lots of things to look forward to.
Our good friend Jenny ran the Chicago Marathon this past weekend, in honor of Evan. This is the second marathon that she has completed all because she was inspired by our boy. In a recent post, I wrote about Jenny and her dedication to our family and to Evan. From day one she has been very supportive and has done so many things to help our family and to raise awareness for Ichthyosis. We are very proud of her and wish we could have been there to cheer her on.
Jenny's FB photo captioned "Evan's got my back"
Prior to the marathon, Jenny wrote about her journey and inspiration to run on a private facebook page dedicated to Evan. Her complete story can be read on that facebook page. Below are portions of what she wrote. Her story in her own words...
"....During training for the Chicago marathon I got injured. The injuries were serious enough to actually make me stop running and schlep off to see a doctor (trust me, it takes a lot for me to ask for help). I sat there as the orthopedist did he tests and I tuned him out as he lectured me on the dangers of marathon running and strongly encouraged me to give up on training for Chicago and marathons all together, blah, blah, blabbity, blah, blah and focused on the one phrase he said at the very end, “but if you rest and go to physical therapy, you’ll be back running your stupid marathons.” He was right, the rest was what my body needed, but the timing couldn’t have been worse. I basically had to start training from scratch, in the middle of the summer with only 10 weeks to get my body into marathon shape. ...I’m not going to lie, there were many times during my training runs that I walked and even where I just sat down and questioned why I was trying to accomplish such a lofty goal, but then I would think about Evan and his parents and a promise that I made to raise awareness and run in his honor. Evan’s condition is every single day...a training program that is so meticulous and requires the most patient, precise, loving care.... two years later; this boy has defied all odds, surpassed all predictions and motivated so many people along the way. Evan and I share a similar quality that has connected us in a special way; we don’t know how to quit. ...The cards Evan and his family have been dealt are quite the hand, although being around them, you wouldn’t feel that way for a second. Evan’s infectious smile, heartwarming giggles, strength to live and eagerness to learn are so inspiring it kept me going strong all the way to the finish line. Joe and Diane have given up so much in their young lives to keep their son alive and more importantly, happy. This two year old boy has brought so much perspective and clarity to my life that I will forever be grateful. Because of him, I am a marathoner. ...Knowing Evan has opened doors for me to meet other kids with skin disorders like his and taught me how much appearances can be deceiving. Now, instead of questioning or judging others, I tend to wonder what their story is, what their day-to-day is like. I try to judge people less and simply smile more. I’m learning through these kids’ stories to focus less on appearances and more on being a good person, the kind of person that people want to be around, the kind of person that adds value to others' lives and hope that attitude becomes contagious among my peers. I find that I’m not afraid of correcting ignorant people and educating those that do not know any better. If more of us adopt this attitude, the world wouldn’t be such a scary place for kids like Evan to grow up in. ...It is with great pride and vindication that I run (and COMPLETE) my second marathon in honor of such an amazing boy and family. Thank you again for providing me the platform to brag about a boy that has changed my outlook on life and made me a better person. The next time you see someone who might have a story like Evan’s, smile and thank the parents for such an amazing miracle, you will make their day and hopefully others will follow suit... -Jenny
Her words have touched me and make me proud to call her my friend. She is such an amazing woman. I feel incredibly honored to know that she has completed such an enormous accomplishment (for the second time) because she was inspired by my little miracle. Jenny has always had a special connection to Evan and I am very grateful for all of the support she has given to us. Thank you Jenny. To think of how my child inspires others is an indescribable feeling. If I had to describe it, saying it's a feeling of joy and heartwarming gratefulness is an understatement. I am grateful that I have been given a child who essentially lights up others spirits. Who so far in his two years of life has been such a well behaved baby, tolerant to skin care & doctor appointments, is always in a pleasant mood and goes with the flow after all the challenges he is forced to live with. He is an amazing warrior and is not only an inspiration to others but is such an inspiration to me as well.