Catching up!!

Wow it's been a while! I know I say that each time but blogging these days is tough for me to accomplish! These boys keep me on my toes for sure! And when I have a free moment I tend to put my feet up and close my eyes! 

A LOT has happened since I last wrote. First and foremost, Evan is now a SIX YEAR OLD!!!! We celebrated his birthday on June 9 and then had his whole class over for a party that following weekend. He was a very excited and happy kid to have all of his buddies come play and celebrate. 

After our many birthday celebrations, Evan finished off his school year as a Kindergartener. So he is officially a first grader! Ahhhhh! I can't believe it! Evan's school is very special. He is surrounded by love and support and we couldn't ask for a better community to live in. This year, as the 6th graders moved up to the high school, they choose someone to dedicate their yearbook to. And can you take a guess at who they chose?? Check out this video. My little man walking up to accept the honor!!

(Sorry about the quality, the sound is all you really need!)

After the school year was over we kept busy doing summer activities. We took a family vacation to the Cape and then up to the Green Mountains in VT to camp. Evan just LOVES to camp. I was always a bit worried how we would work it out considering, camping isn't the cleanliest   experience. Fortunately our good friends live a mile from a beautiful camp area. We have the experience of true camping with a home and bath tub at our fingertips when need be. 

Between trips to the lake, trips to Action Wildlife, adventures walking through the grocery store to pick out snacks (which is his first choice) and the therapy/summer school he attended, we kept pretty busy. Now the summer is winding down and we have a few weeks left to enjoy. 

Another very exciting change for Evan is that he finally got his wheelchair! He's been very anxious to get it and couldn't be happier. I am so proud of him and how well he can maneuver this thing! He will be bringing it to school as his main source of independence. 

Ichthyosis Awareness Month 2016

It's Ichthyosis Awareness Month! I know I am about 30 days late, but better late than never! I have been without a desktop for months and writing on the blog using a small device is rather challenging to do! Nonetheless, here I am trying to do what I set out to do by starting this blog.

Learn more about ichthyosis and how you can help at the FIRST Skin Foundation!!!! 

Here is an update on the boys:

Evan is doing amazing! He is wrapping up his Kindergarten year with a bang! He started the year off with little mobile independence and now he is RUNNING around the halls in his walker. He is probably the only kid in school who is "allowed" to run! He has accomplished his Kindergarten goals academically and has pretty much accomplished his gross/fine motor goals as well. Evan continues to have a extreme love for school and I can only assume a lot of that love is thanks to the amazing care he receives from his aide, nurse and teachers. 

As for Evan's ichthyosis, it is the same as last year, the year before and the same as he was born. Ichthyosis does not change or get better over time. Luckily we have been able to avoid any serious skin infections. I like to attribute that to our obsession with keeping his skin in its best condition possible (bathing 2x/day, min of 5 head-toe aquaphor applications/day and intense exfoliation: keeping him as scale free as possible). We still battle with the dry conditions in the winter and the heat, now that summer is at our doorstep. I honestly wish the AC fairy would come and magically install central air in our home! Fighting with window units all summer is quite stressful and nerve wrecking. But as stressful as it can be, ichthyosis is our normal and something that just comes with motherhood, for me.

Evan is delighted to be a big brother and is such an amazing brother to his baby bro. Though the tables are turning a bit since Cenzo is able to walk and move about. Evan, still, has never seemed to be frustrated or discouraged that his baby brother is more mobile than he is. Evan only encourages Cenzo and cheers him on when he accomplishes the milestones that come so easy to most children. Evan truly is such an amazing child!

Side note on Cenzo: after his most recent cardiology visit, he "graduated" to annual check ups rather than quarterly! Everything seems to be the same (with the tumor) and not causing any issues for his heart! Good news on that end! Phhheeeewwwww!

Now for your viewing pleasure, my boys rocking out to Evan's favorite song! 

 

Our MicroSilk Tub: Part 2

The last time I wrote about our tub was in October 2013. We got our MicroSilk after we bought our first home in 2011. Evan was only months old when he tried the tester tub and a year old when we had our own. So MicroSilk has pretty much always been a part of his routine. We have had our tub for over 4 years and I now can say that Evan is finally using it "on his own". Since Evan has been behind on the development track, could not sit independently for over 3 years, it was challenging to give him a bath in the MicroSilk tub. Even with the proper adaptive equipment for him to sit in, it was still nerve wrecking having him in a huge tub full of water. Then when I was pregnant in 2014, it made it impossible for me to reach and lean over to bathe him in the MicroSilk tub.

Fortunately with a new baby came some milestones for Evan. One huge milestone was his new found love of "crawling". He started army crawling around the house just at the perfect time; just when he could use a little independence as the new baby arrived. Another huge milestone was THROWING AWAY (recycling) the baby tub. The poor kid still got one bath a day in the baby tub, mainly because he was small enough to fit in and it was easier for me to bathe him without bending over. He obviously was getting uncomfortable in the little tub so we asked him if it was ok to get rid of it. He had no objections.

Since this spring, Evan has only gotten a MicroSilk bath and many times two of them a day! Thanks to his progress in the motor department, he is stronger and sits much better and safer in the tub than in years past. He has also grown a little bit, so now the water level isn't an issue when sitting on the tub floor or in an adaptive seat. And boy does this kid love his tub!

The benefits:
As I have written before, it is undeniable that this tub has benefits for the maitnence of his skin. The small oxygenated bubbles help with exfoliation and easily remove small scales without pain. The tub looks like a shaken snow globe within minutes of starting! Scales seem to lift easier and it is a much gentler way of exfoliating rather than aggressive rubbing with wash clothes or mitts. The oxygenated water must be a benefit to his skin becuase it is very noticeable when he gets a bath in a regular tub while on vacation or visiting family for the night.

Evan is in the MicroSilk 2x a day with a minimum of 12 baths per week. Many weekend nights we tend to "skip" baths due to busy schedules and becuase Mom and Dad need a little break. On weekday mornings he only recieves one cycle in the tub (20-30 min) due to our tight schedule- getting ready for school. On school nights, Evan takes a very long bath with at least 2 MicroSilk cycles and rarely gets out of the bath before an hour of time. As wonderful as this tub is, Harlequin Ichthyosis is relentless and that skin grows almost too fast to keep up with!

Another benefit of this tub is how it keeps the water at a constant temperature. To have a kid who can't regulate his body temperature, in a bath tub for an hour, would be tricky without this feature (never mind draining our water tank!) Thanks to the tiny oxygen rich bubbles, it keeps heat energy in the water maintaining the temp as it was filled.

Tub Maitnence:
This tub is known as a "green" tub. No cleaners or additives are needed when using the tub. But since Harlequin Ichthyosis is so severe, I tend to continue with my typical maitnence routine before, after and during the bath. While using the tub I will occasionally use a splash of bleach as directed by our dermatologist. Since Evan does not have the necessary proteins on his skin to kill harmful bacteria, it is important to use this in any type of bath. We use to use baking soda in every bath but now only use it in non MicroSilk tubs.

Before and after every bath I wash the tub with dish soap. I want to be sure the bath is clean before using. Afterwards, dish soap helps to cut the greasy Aquaphor and skin residue caked on the walls and floor of the tub. I will occasionally use bleach as needed while cleaning out the bath.

To maitnence the jets, Jason sells a green cleaner that can be run through the MicroSilk. I usually do this every few months or when I notice the cycles will not run a full 20 minutes. As we have a first generation MicroSilk, we also unscrew and clean out the filter every bath or the jets will not turn on.

Read more about our experience with MicroSilk here!!!

Learn more about the science of MicroSilk Here!!!

 

The Kindergarten Life

I can't believe my boy is in Kindergarten. And it is no surprise that he LOVES it. I am so grateful for Evan's love for school; interacting with his peers, independence from home and his love & dedication to learning! He is so proud of his accomplishments whether a worksheet completed, drawing created or story to share. We are very fortunate to live in an amazing community and that Evan's peers are so kind, warm and accepting.

Dancing in music class!

The biggest change going into Kindergarten was getting use to a FULL DAY of school. Since Evan attended the same school for Pre-K 3&4 we had already gone through the process of transitioning into a school setting (were ichthyosis is concerned). But I was a bit worried how Evan would manage on an energy level for an entire day, as well as how his diet would be compromised from the "distractions" in the cafeteria. Luckily his energy has been great and to my surprise he comes home seemingly energized! Though all the fun of eating lunch in a cafeteria full of kids has been more enjoyable for Evan than Mommy. Eating well has never been his forte but we are still working on it!

Math center on the carpet!

As far as his skin care goes while in school, I have to admit that we have the best crew there is! Being away for 7+ hours compared to 3 is a big difference when taking care of his skin. And yet again we are extremely lucky to have a nurse, aides, teachers & staff that are all working together to make sure he stays comfortable and safe. He now gets to enjoy lunch, recess and specials at school so planning for his skin in different environments, like the temperature of the cafeteria or gymnasium to what kinds of paints he can have on his skin in art class, have been considered.

When it's too hot, Evan and a few buddies eat in the classroom!

All in all I have nothing to complain about. Evan has gotten into a good routine, now, being in school for a full day. I know he is in good hands and his skin is the "least" of my worries while he is at school. He comes home happy, excited and has lots to share about his day. I can only hope his enthusiasm for school will continue throughout the years and he will continue to "jump for joy" when the bus pulls up!

Evan's favorite thing to play, GROCERY STORE!

**Side note** I usually offer to go into the classroom in the beginning of the year to talk about Evan's skin with his classmates. Since most of the students know Evan already and they have accepted him for him, I decided to talk about ichthyosis from more of a biological standpoint than talk about differences (which I did speak about on the preschool level). I did this mainly because Evan is older and more aware now and I didn't feel like it was necessary to have a discussion about how "different" Evan was when he really isn't. He's a kid just like all of his classmates.

Video: Explanation of Ichthyosis to Elementary Students

 

Goodbye Summer! Until Next Time!

Wow summer flew by! And we had quite a full one at that! So many fun memories, vacations and time spent at home with the family. Evan just headed out the door for his first day of Kindergarten. My emotions are going haywire but I am so excited for him and can't wait to hear about his day.

First on the list of summer fun was a trip down south to the Carolina beaches. Our entire family and extended family gather there each year. This year Joe was unable to join us again, due to work obligations, so I went down with the two boys. Thanks to the many helping hands, it was a fun vacation for all!

Listening to seashells!!

This one decided to wake up at 5am each morning. So we usually headed outside.

Evan played mini-golf for the first time!! And at an indoor course! 

AND we were with another child we met who also has ichthyosis!!

One morning it was cloudy and cool so as soon as Evan opened his eyes we went to the beach. 

Still in his PJs too! So convenient staying at a house on the ocean!

The next big event this summer was a vacation to Cape Cod! The weather was much more forgiving than the south and a cold front had come through creating ideal outdoor conditions for Evan. Unfortunately, he was a little under the weather himself so Joe and I were a bit worried to travel. Of course Evan said he "felt fine" and "didn't feel sick" to make sure we would still go on vacation. Luckily it all worked out and it was a great visit with more family.

The second time this guy got to play mini-golf!

AND GOT A HOLE-IN-ONE!!!

Don't mind my sqeaky excitement!

It was a happy day!

Beach time was perfect and cool! To the point Evan asked for a blanket!

What's a Cape Cod vacation without stopping for some School House ice cream! 

Another big event this summer was our family camp out. We started the summer off with some backyard camping but Joe and I braved the wilderness to do some real camping with the boys. We headed up to the Green Mountains in VT and camped at a beautiful park with some good friends. Luckily they lived close by so we knew we had a house (aka bath tub) to go to when we needed it! Evan just loved camping! His favorite part was helping his daddy make the fire! And again we got beautiful, cool, comfortable weather which included some chilly nights! It was a great family outing!

We had a gorgeous spot on top of a grassy hill with a little nook of trees for shade!

Evan picked flowers for us!

Vincenzo enjoyed the camping life!

Evan LOVED the campfire!

The benefit to an early rising baby!

Brothers!!!

Family!!!

I probably could go on and on and on about how eventful our summer was. It really was the busiest summer we ever had. Which included lots of picnics, pool parties, birthday parties, playground adventures, Action Wildlife visits, LOTS of blue berry picking, fishing at the lake and the usual home fun. We were always doing something or going somewhere but also had time to wind down and rest at home after busy trips and adventures. AND did I forget to mention Evan was in the summer school session and had physical therapy at the house? Yes we fit that in too! Next post will be all about Evan's Kindergarten experience!!!

And I couldn't not post an Action Wildlife pic! We spent half of our summer there!!!

 

Happy Birthday Evan

We celebrated Evan's 5th birthday yesterday. It's hard to believe how fast time is flying on by. It feels like just yesterday he entered the world. And on that day we were filled with so much worry and fear; for his life and future on this Earth. Those feelings were uncontrollable and unfortunately not the typical feelings most parents experience with the birth of a child, their first child. Luckily we stayed strong and positive for him. Now, Evan is living his life happy and loved.

It has been quite an eventful few days of celebrating! Over the weekend we had Evan's birthday party, Monday we attended his preschool celebration/graduation and yesterday was his birthday! Not to mention he only has one day of school left before summer vacation!

Evan was just thrilled with all of the birthday attention this past week. He was very excited about his birthday party and just loved blowing out the candles on his cake. And as most children are, he was ecstatic about opening presents. He is such a sweet, loving, happy child. I am so proud of how far he has come!

 

Ichthyosis Awareness Month 2015: Evan's Skin

Sometimes it is hard to wrap my head around the fact that Evan cannot live without Aquaphor. Seems hard to believe that something as "simple" as putting on lotions/emollients is keeping him alive. Or that bathing multiple times a day not only hydrates him and keeps him infection free but makes it easier to move around, without pain. Having healthy skin was something I definitely took for granted before Evan came along. I never in a million years would think that not having healthy skin (excluding cancer) was something that could be life threatening.

Evan's skin cells are not functioning properly. The mutation in his ABCA12 gene disrupts the transportation of lipids to the epidermis which is necessary to keep the skin pliable and hold in the body's moisture. His body's automatic defense is to make new skin, for protection, which is growing much too quickly to shed on it's own. Untreated skin will encase the body restricting movement & breathing, cause fissures and open wounds susceptible to infection, cause dehydration at an accelerated rate and manipulate facial features & digits, just to name a few.

Over the years, medical treatments have vastly improved for those with ichthyosis, especially Harlequin. Prior to 1984 no one, affected with HI, survived birth. It is just mind boggling that, in my lifetime, the evolution of medical care for HI has advanced so rapidly. I can only imagine the kinds of treatments (or cure :)) that will be available when Evan is 30!! I am very optimistic about the future medical advances that will be made for this disorder. And thanks to foundations like FIRST, stepping closer to a cure is possible!

Evan's skin care these days is the same ol', same ol' regimen. I think of caring for ichthyosis not as "lather, rinse, repeat" but "bathe, exfoliate, moisturize, moisturize, moisturize, repeat". Evan gets two 40-60 minute baths a day to exfoliate and hydrate, he's lubricated with Aqauphor (head to toe) 5-6 times a day, receives eye drops while awake and eye ointment while sleeping and gets frequent ear care and nail care when he's tolerant. And due to his hydration and caloric needs, he drinks a high calorie prescripted formula for nutrition,  a minimun of 36oz of water each day, and is offered unlimited snacks and "meals" (this kid is so picky he will only eat 3 specific meals/foods).

I think Bruli would totally jump in if I let him!

The biggest change in Evan's skin care, these days, is his MicroSilk tub. We have had it for almost 4 years and Evan has used it many many times. But now that he is finally reaching some gross motor milestones (sitting), it is easier to give him a bath in his big tub. One flaw of having a (almost) 5 year old that is the size of a skinny 18 month old, is that I have been getting away with using the infant tub one time too many. Since Evan is finally able to verbally communicate, he makes it very clear that he doesn't want to take a "blue bath" and I don't blame him! Evan LOVES his MicroSilk tub. This tub emits millions of oxygen rich bubbles, so small they are intended to deliver oxygen to your pores. Since Evan essentially does not have pores because his skin is so tight, I hope and wonder that this type of bath technology is making it possible to treat his skin in a new way. Not only does this tub significantly help with skin management but Evan is happy, relaxed and comfortable when using this tub.

I am very thankful that Evan was our first child. Ichthyosis and motherhood coexisted. It was all I knew what being a mother was like. There was never a time when Aqauphoring, after a bath or diaper change, seemed anything but normal. Now as a mother of two, Evan's baby brother being unaffected, makes me appreciate the little things. I probably would never think of how easy and quick a diaper change was if Evan was the younger brother. I am glad that I am able to appreciate these types of changes between caring for both boys instead of being discouraged about how easy it "use to be" if Vincenzo was born first. I like how ichthyosis has made me appreciate the finer things in life because with all considering we really do have a wonderful one.