Two oldest maine-iacs

Two oldest maine-iacs

Thursday, June 30, 2011

Vacation Days

Ahhh, Summer vacation. Daddy, Ava and Henners have been spending alot of time doing fun day trips. Michael is at day habilitation at Star Reachers. Before you feel sad for him for missing out on all the fun, let me assure you he is having the most fun out of us all. He has been rock climbing, to the University, swimming, to the park, playing sports, doing science experiments, having treat after treat, playing games you name it. It's definately not 3 more months of school.

Derek took Henry and Ava downtown to Cascade park. It is right next door to my new job. I'd be kidding myself if I thought I might have enough down time to wander down there and check it out. But I am so glad we have such beautiful parks all over town.

Derek said Henry got soooo excited when he saw the water. He LOVEs to kick and splash in teh sink or at the pool. He's another fish! 

Cascade Park.

Wednesday, June 29, 2011

EB- viewer discretion is advised

Every once in a while I get a spare 2 minutes and look around at blogs of different people. Mostly my mom, sister, best friend, cousin... But sometimes I even get a minute to look at friends of those friends and that is how I found Tripp and his mom, Courtney. This is how I first learned about EB. Viewer discretion is advised because the pictures are so graphic. My heart goes out to Courtney, who with the support of her family (parents) has been handling this by herself after her husband couldnt take it any more. She is one super tough cookie. She has earned a special place in heaven for her and her little angel, Tripp.

About Junctional Epidermolysis Bullosa

Junctional EB
What is the cause of Junctional EB?
Through research it is now known that mutations in the genes encoding alpha 6, beta 4 integrin, collagen XVII or one of the three chains of Laminin 5 contribute to defects in the formation of hemidesmosomes or anchoring filaments.
Defects within any of those components of the skin allows for the separation of tissue and blister formation whenever there is friction or trauma to an area. In many instances blistering can occur spontaneously.
There are three major sub-types of Junctional EB. Herlitz, non-Herlitz and Junctional EB with associated Pyloric Atresia. Though Junctional EB is considered a non-scarring form of EB, tightening and thinning of the skin does occur. In many instances residual atrophic scarring occurs.
How is Junctional EB Inherited?
JEB is an autosomal recessive condition. This means both parents are healthy carriers. Healthy carriers are non-symptomatic and will never develop the illness. When each parent has a copy of the altered gene, there is a 25% or 1 in 4 chance the child will be affected by Junctional EB. Unfortunately, there is no test to detect carriers for JEB. We are made aware that the parents are carriers after the child is born.
Junctional Herlitz EB:
Junctional Herlitz EB is a very severe form of EB. These infants often die during infancy due to overwhelming infection (sepsis), malnutrition, dehydration, electrolyte imbalance or complications resulting from blistering in the respiratory, gastrointestinal or genitourinary tract.
Some babies develop a hoarse cry and breathing difficulties which indicates internal involvement as well. These infants often fail to gain weight. These are usually symptoms of the severe form of Junctional EB.
Blistering is usually present at birth, however, there have been instances of infants being discharged to home, with a small blister on the finger or lip. After they are home, the blistering becomes more apparent warranting a visit to the physician. Skin blistering and ulcerations can occur spontaneously on the arms, hands, finger tips, back of the head, neck, shoulders, trunk, buttocks, legs and feet and toes (generalized distribution). Nails may be ulcerated or dystrophic. Warmer climates can exacerbate blistering. Blistering is noted on perioral (around the mouth) and mucosal surfaces as well. Oral lesions may affect eating causing weight loss.
Electron microscopic evaluation of the structure of the skin in a patient affected with JEB-H usually shows skin separation in the lamina lucida within the basement membrane zone. Absent or reduced amounts of hemidesmosomes may also be apparent.
Junctional Herlitz EB mutations are present on the genes encoding one of the three chains of Laminin 5.
Junctional non-Herlitz EB:
Generalized blistering and mucosal involvement may be evident at birth or soon after. Blistering may be mild to severe. Erosions on finger and toenails, nail dystrophy or absence of nails may be evident. Erosions and loss of hair (alopecia) upon the scalp may occur. Granulation tissue around mouth and nares may be seen. There may be some scarring and thinning of the skin on affected areas (atrophic scarring). Warmer climates can exacerbate blistering. Though laryngeal involvement (hoarse cry) may be experienced in early infancy, respiratory distress is a rare occurrence in this type of Junctional EB.
The infant may suffer complications such as infection, dehydration, electrolyte imbalances, respiratory, gastrointestinal, and/or genitourinary tract involvement. These complications may lead to death.
Electron microscopic evaluation of the structure of skin in a patient affected with JEB-nH shows skin separation at the level of the lamina lucida of the basement membrane zone. Variable appearance of hemidesmosomes may be visualized as well.

Dress up is so much fun

Her dad may mock her for the mary poppins, pocahontas and 1980's gear. but she loves to dress up and play pretend. I think it's adorable. She also loves to model- strike a pose Ave!

Tuesday, June 28, 2011

Too many things, not enough time

I always have this list of stuff that I want to do, that I should be doing, that I could do if only I had the time. I am pretty darn good at time management. There is only one person I might say is better at multi tasking and that's my sister Meghan. Although I love a clean house and I function better with organization there are so many things I would rather be doing. I forgot how hard it is to do these things with a baby. I love to scrapbook (its been ages), garden (such a disaster after ignoring them last summer when I was too fat to bend over and weed), make cards with Ava (this we have been doing periodically), write (I have 4 books that have been in progress for twice as many years), read (Ken Follett can you write some shorter books 1200 pages takes me forever!). But mostly I want to snuggle my baby, I want to play cards with Ava and listen to Michael read, I want to go running and take them on bike rides. I want to have dinner on the patio with Derek, try a new restaurant, watch a movie. I want to not fall asleep at 8pm. My summer resolution admist all of the job changing and house renovating chaos is to enjoy my family every moment and do one of my favorite things a week. I hope I can stick to it!

Sunday, June 26, 2011

I wanna talk tonight...

When out of work for 1/2 a year (maternity and paternity leave) we decided to take turns with one phone and cancel the other to save money. One of us was always at work and the other always with the baby and other kids. It worked out perfectly. As a treat we both got new phones recently. We love them! Henry got a new phone too. He knows you do something with it up by your mouth.


 Close Henry

 But that's no cigar.

Saturday, June 25, 2011

Daisy Scouts

Ava and I have had the BEST time being involved with the girl scouts this year. We went roller skating (eek!) we went on a nature hike, we made brownies for the Ronald McDonald House and so many more fun things. We are getting ready to go camping over the summer, which brings back fond memories of my childhood.
Ava was being sucky.

Marissa and ava smelling the "peppery" lupine blossoms.

Best buddies. This is why we joined, to meet a group of nice, friendly little girls.

Waiting to tell our guide about the lichen she found.

  Fields Audobon is awesome. It reminds me of the nature center at home. Theresa and I attended a yoga retreat here years ago and the atmosphere hasnt changed. So peaceful and relaxing!

Friday, June 24, 2011

Best Friends Forever!

Who wins the BFF contest?
David and Michael were friends first.

Ava and Grace dress alike.

These two cause alot of ruckus!

So do the girls!

These two know how to chill out.

So do the girls.

It's hard to believe it has been SEVEN years since the boys met and almost SIX years since the girls met. Although our BFFs moved far away they are still in our daily thoughts and always in our hearts.

Wednesday, June 22, 2011

The school bus

Uh oh! Henry has a school bus of Michaels. Michael would say - mom he's slobbing on my stuff!
And we all know how crazy he is about his school buses. (Right grammie?)
When my mom came to visit she mistakenly mentioned seeing one and Michael perseverated on it the entire trip.

I mentioned this to Henry - he thought it was HYSTERICAL!

And slobbed on it anyway.

This post was supposed to be about Henry's Roots hat.
You know you are a Canadian when you love Roots!

Monday, June 20, 2011

Bar harbor

On any free weekends we like to take a little trip down to Bar Harbor. Usually before the tourists have arrived or after they have dwindled down to a few stragglers. The shops may be closed but that's not our favorite part anyway. We love to skip rocks, eat ice cream and walk the shore path. (June 10, 2008)

Some of us prefer to "ride" the shore path

Daddy is an awesome skipper and is trying to teach Michael - there is alot of room for improvement (so far)

Some of the islands in Frenchman Bay, worth the 45 min drive, just for the view


Walking downtown

Some of us didnt get enough of the water on the beach.
The kid watching is the funniest. What is he doing?

Yeah! got it!
We had to have a talk about tossing them in, not digging the money out.

Sunday, June 19, 2011

Happy Dad's day!

Our dad is working on Father's day this year. But we had a moment to squeeze in some shots before 6am!
Our favorite things about our dad:
Michael: "Wrestling and watching sports!"
Ava: "He lets me do the dishes! And read in bed at night."
Henry: "He lets me eat Klondike bars!"
Mom: "He is the best cook! He cleans, does laundry, pays bills, goes to the dump, does the grocery, takes care of Henry 3 days a week. And is still fun to be around! He is the best!"
Happy father's day to my dads. Wish we lived closer. I hope it is a relaxing and fun filled day. You earned it!

Saturday, June 18, 2011

My Garden My Love

Anyone who really knows me knows how much I love to garden. We are getting ready to add onto our house and this means moving large sections of my gardens and even getting rid of two whole gardens! Just the thought gives me heart palpatations. (Seen below: Viburnum from my friend Gloria's husband's farm, and Clemantine white columbine.

Lupine is seen all over Maine in June. The roadsides and meadows are filled with it. The kids learn about it in school. The girlscouts learned that it has a peppery scent when they were on their recent nature walk.  I call it "slug salad," beacuse they LOVE it. Every year I threaten to rip it out for something less appealing, but they are just so beautiful with their umbrella leaves and long lasting flower spikes. They look fantastic next to my red papery poppies.

One of my all time favorites is my clematis. This one has been growing for four years and is finally blooming! It is called Henryi. I bought it because it reminded me of Derek's dad. Its so nice to finally see some of the pure white blooms. Gardening has taught me patience. What has it taught you?

Thursday, June 16, 2011

Great Grandma Hose

Everytime I sleep like this my mom always says you look like your Grandma. Ava sleeps like this, and looks like Henry does too!

Sometimes being a baby takes alot out of you. How cute is that bib from Auntie T?

Wednesday, June 15, 2011

The Best Book Club Ever

I love my book club. We have been together on and off for 8-9 years? Definitely before kids, although we took a little hiatus when the first set arrived. We have read some of my all time favorite books and books that I would have never read if it wasnt for our group.

Right now we are reading A World Without End by Ken Follett.  We read Pillars of the Earth last year and we all LOVED it, rather unusual since we usually have mixed reviews. No one has been scared off by the 1400 pages because Mr. Follett weaves such an beautiful and engaging story. Lucky for me my hubby and kids bought me a Kindle for mothers day, so instead of lugging around a giant book I am blessed to have a tiny little slip of an electronic. That combined with my fantastic friend Tammi, who helped me get Pillars and WWEnd for it. (I think she took pity on me after watching me carrying my work bag, breast pump, purse, lunch, and a giant hardcover. When I am packing the kids in the car in the morning it is even worse with two additional back packs, Henry's bag, his bag with clean diapers and sac for dirty ones, and his food and milk. It can take up to three trips, not counting the trip with him in his car seat.)

Some of my favorite books that our club has read are Time Travelor's Wife, Thirteenth Tale, Water for Elephants, 1,000 White Women, Ken Follett's books. Some of the books I wouldnt have ever read without my club: Loving Frank, Eating Kalamazoo, Poisonwood Bible, Olive Kitridge (which I loved!)... The best part about my book club? The women. Such a wide variety of backgrounds, careers, personalities. They bring so much fun and joy to the group. I always look forward to our next meeting, even when I think I know what people are going to report I am always surprised. Do you have a favorite book?

p.s. I see my sister and I are on the same wavelength today. I am going to look up her recommendation this weeknd.

Monday, June 13, 2011

Looking eyes

We are constantly working on Michael's "looking eyes." He cannot seem to find anything he is looking for, especially his clothes, his toys, his glasses... you name it, he will wander in endless circles looking for his stuff. We know that this is partly due to his diagnosis and so we are constantly reminding him to use his looking eyes. We do iSpy, we read Where is Waldo? books, trying to build some skills.  Recently he found TWO things in one day and he said "Mom I think I am starting to get good looking eyes!" Well, he forgot his good looking eyes when he went to visit Mama Sue this weekend. Riding on his fourwheeler, having a grand old time he missed their new puppy Lucky's rope leash and got it - right in the neck.  Poor guy. Lucky for him and Lucky it looks worse than it feels. Guess we still need to practice those looking eyes!

Sunday, June 12, 2011

First night away from home

Kids were off to visit Mama Sue, Patsy and Grandpa this weekend. Henry went too! First night away.  We packed the medicine, the breather (neb), clothes, diapers, kids... We thought of every last thing. Even the nasal aspirator just in case. An hour drive down and what did we discover? We forgot the baby's milk. All of it. Needless to say he had to try some formula. He did very well, he loved hanging out with the kids, flying with Grandpa, snuggling (sort of - he's so wiggle-y) with Mama Sue and Patsy. He ate them out of house and home (where does it all go?)and was a restless sleeper but overall he did great! We missed all three but enjoyed the break!