Our Bizarre Stranger Encounter

Yesterday Evan had his monthly ENT appointment. These visits are crucial for us since I am obsessed with keeping his ears clean and free of skin debris. You can see in the video I posted, My World & Ichthyosis, that it is a never ending battle removing skin from his ears. Not only in those nooks and crannies but down his ear canal, up to his ear drum. There is an ENT office a few miles closer than Yale, cutting the round trip drive down to 100 miles. To top the morning off, it was snowing and raining. But the drive wasn't too bad, what happened in the waiting room was what made the trip weird.

Once we walked into the office, the waiting room was packed. It wasn't our typical appointment time so I wasn't surprised. Of course, as when we go to most places, everyone had to have a look at Evan. Most of the reactions or expressions on others faces were typical. A few of the 'eagerly trying to look again' folk too. I ignored it as I usually do with a smile. So I checked in and sat down rolling Evan's stroller up to me. He was directly facing another man, who ironically enough was one of the 'eager lookers'.

I grab a bottle and gave Evan a little drink. I talked to him and had him play with his teddy bear. I noticed the man next to us had his phone and was playing on it. Or I thought. All of a sudden I hear the snapshot of his phone camera. I jolted my head up and looked at him then instantly and obviously turned Evan's stroller in the opposite direction. It was instantly awkward but I didn't say anything. I looked around the room and with the way things were positioned and the way he was holding his phone, unless he took a picture of himself, it was of Evan. Really? People really do things like that? So weird. And if he did take a picture, what exactly would be he do with it? Show people he saw a red baby? I don't get it.

I am not the confrontational type of person but of course now I wish I said something. Luckily, I happened to be texting with Courtney (Brenna's Mom) and she was quick to defend us which made things less awkward for me in the waiting room. :-) Our doctor came right up to us to greet us and chatted with Evan before we went in. I was tempted to mention the situation in the waiting room to him but I didn't. And though it still bugs me, we move on because it unfortunately will probably not be the first or last bizarre encounter with a stranger. I want to teach Evan to stand up for himself but when necessary. I feel that killing others with kindness is a more appropriate way of dealing with awkward stranger encounters but obviously defending oneself when needed. I'm a 'glass is half full' type of gal and would rather keep things positive and try not to let insignificant things get to me. Apparently, I have a thick skin too.

And here is some Evan and Bruli cuteness!

Yes, I did it again!

 

Cute Stuff..

Here are a few super cute videos of Evan. In the first video he takes a few steps (with a little help from Mommy) and the other video is how Evan reacts to his crazy chocolate lab, Bruli. Both must sees! Also, I recently updated my page Cute Stuff. There you can find a bunch of photos of Evan, including progressional pictures of him from when he was first born. And 2 NEW pages!! POWERFUL POSTS and  VIDEOS. I made a page of links to all of my favorite posts and I organized a page of all of the videos I have posted on this blog. All the videos are linked to its original post. It's nice to look back on some of posts and videos and see how much Evan has developed in a short amount of time!

Showin off to our friends in Springfield!!!

Apparently, Bruli is hilarious!

 

Our Journey to Springfield

This past weekend my mother, Evan and I traveled to Springfield IL to visit a special family. I have been looking forward to this trip and could not wait to meet Courtney and the Westlake crew. Brenna, as I mentioned before, is almost 11 months old and also has Harlequin ichthyosis. So many things have seemed to connect us from the start. Obviously, both having a child with the same condition but also Courtney and I are the same age, our birthdays both fall on the 11th of the month AND her husband's name is EVAN!!! So cool!

We got off to an early start Friday morning, leaving my parents house at 3:45 am. Unfortunately we had a layover, but thankfully everything went smoothly. We arrived into St. Louis, MO at 9:30 am. (By the way, Evan was such a charmer on the plane. We had so many compliments on how well behaved he was and he was trying to give the man sitting next to us fist bumps!) We rented a car and headed to Springfield.

Once we got to the Westlake house we relaxed and talked and it felt so comfortable. Seeing Brenna was so exciting and Connor is absolutely adorable! Evan was due for a bath so I 'set up shop' in the kitchen as I chatted with Courtney about his bathing routine. Before we headed to Springfield, my husband and I worked really hard on Evan's skin so it would be easier for me to manage on my own while away from home. So I did not have to put in as much 'elbow grease' as I normally would. After his bath, I was able to see what their routine was with Brenna. I asked questions and was impressed with their set up as well. They got right down to business!

Before we went out to dinner for some Horseshoes we snapped a few pics of our freshly bathed cuties together. Evan was so interested in Brenna and kept trying to touch her face and hold her hand. I really feel like he knew that Brenna was just like him. It was sooo adorable. We were melting. :-)

So precious..

And if this one wasn't blurry... but absolutely adorable!

After dinner, my mom took Evan back to the hotel so I could spend some time with Courtney and Evan W. I couldn't tell if I was imposing and should have called it a night but I was so excited to be there and couldn't wait to talk some more. It was great to talk in person about our birth experiences and Courtney was nice enough to share lots of Brenna's birth pictures with me. Although, I knew Brenna's story and related to Courtney's birth experience, it was totally different hearing her story in person. 

Saturday we had an eventful day out on the town, or I should say city. Courtney was nice enough to do a little photo shoot of Evan at her studio. Then we stopped at a park to snap a few of me and Ev together. After our photo shoot we all headed out for lunch in downtown Springfield. It was a gorgeous day so after lunch we walked from the cafe to the museum. All of the buildings were so beautiful and historical. We then arrived at the Abraham Lincoln Presidential Museum which was incredible. It was great to relearn the history of Abe Lincoln and learn many new things as well. There was so much to see and many of the displays and exhibits made you feel like you were back in the 1800's. One very clever room was set up like a TV studio, recording the Presidential election campaign on a station similar to 'CNN'. Courtney and Evan stayed with my Evan so my mother and I could go into one of the exhibits that was not so baby friendly. It was a play/movie/presentation of Abe Lincoln which was very theatrical with gun shots, explosions, smoke, and vibrating seats. (So glad we didn't bring Evan in!)

Saturday night we snapped some more pics of the kids playing together. Evan was again very interested in Brenna, especially whichever toy she had in her hand. He was acting like that typical two year old who only wanted what he didn't have. And Brenna was doing some excellent reaching to try and get her toy back! Priceless!

Happy and content with a toy...

Wait a minute, what do you have Brenna?

Hey, Evan that's my toy!

And I'm gonna get it back!

Saturday night my mom took Evan back to the hotel again so us parents could go out for a drink. It was so great to have another evening where we could talk and relax without worrying about the kids. I was able to meet some friends of Courtney and Evan who were very nice. I felt as if I fit right in and knew all of these people my whole life. It was such an amazing weekend and I felt so comfortable with Courtney, her family and friends. Not only did we have such a connection prior to this visit but meeting them in person really made me feel like I had a new family. My husband was unable to come due to his work schedule so I cannot wait for him to be able to meet this family as well. We will definitely see them again at the FIRST Family Conference 2014 if not sooner. :-)

Thank you Courtney and Evan for a wonderful weekend. You both are amazing and are doing such a wonderful job raising your beautiful children. Connor is absolutely precious and Brenna is doing so well thanks to all of your great work. I would also like to thank Courtney for inspiring me to finally reach out to the Ichthyosis community. I have met life long friends who we will be forever connected to. She also inspired me to create this blog to help raise awareness for Evan and others. Courtney wrote a post about our visit as well and has some fantastic pictures that you should definitely check out! www.blessedbybrenna.com

 

One Happy Mama

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!

 

Raising Awareness for EB- Epidermolysis Bullosa

Epidermolysis Bullosa Awareness Week 

October 25-31

"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

EB Awareness- www.ebawareness.com or their Facebook page

I Refuse EB- http://irefuseeb.org or their Facebook page

Courtney and Tripp's story- "EB"ing a Mommy

Support Baby Easton- Support Baby Easton Friedel

The Butterfly Fund- Facebook Page

A video share I found on Support Baby Easton's Facebook page
Video Link



 

Evan's Skin

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.


 

Continuous Support for Evan

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.

Time to celebrate