Wednesday, February 1, 2012

Kaya K.

As much as I wish this post was going to be about Kaya the Grand Canyon, it is not (that post will follow soon, I promise) This post is about my favorite little girl in the whole damn world. My little Kaya K.

As most of you know Kaya had an exceptionally rough start on her entrance to this earth.If that's news to you just read back a few pages on this blog to get the lowdown.
As fate would have it, her progression through this life is not about to get any easier.
Immediately following her birth this little peanut of a girl had so many problems that the NICU Doctors suspected a Genetic Syndrome. Testing was done looking at a specific Syndrome called Cornelia DeLange Syndrome. Those test came back Negative. The plan then became to watch her and see how well she developed. Obviously Tiff and I had great hopes that everything was ok with our little girl but we both knew it wasn't. Kaya failed to gain weight and is behind in some childhood milestones. These things gave our pediatrician cause for concern and in the first week of December Tiff and I accompanied Kaya on a visit to Dr. Viskochil, in the Genetics department at Primary Children's Hospital. Within and hour of his observing Kaya we had a preliminary diagnosis of Williams Syndrome. Williams is caused by a chromosomal deletion. In other words, when sperm met egg and first divided to start forming all the cells of Kaya's body, the first cell had a part on one of the 7th Chromosomes that was deleted. As each cell replicated and divided the deletion continues until every cell in the body is missing a piece of genetic code on one of the 7th chromosomes. A test has been developed to confirm the deletion on the 7th chromosome so mid December Tiff and I took Kaya to have blood drawn for the FiSH Test.
We received a phone call from Dr. Viskochil on the day after Christmas that the FiSH test had found a deletion on Kaya's 7th Chromosome.

Kaya Has Williams.
Those words hung in my ear. I continued listening but all I heard was "Kaya has Williams" The phone call and the following counseling session did not proceed like they would if it had been a diagnosis of some kind of disease that you can fight. With a genetic syndrome there is no plan of action, no surgical remedy, no pharmaceutical concoction that can fix, lessen, or heal the ailment. No. What you get with a Genetic Syndrome is a discussion of what WILL BE. A road map of what to expect, a discussion of Kaya's future that is already limited to a degree , by a little teeny submicroscopic missing piece of a gene.

Williams Syndrome occurs in approx. 1 in 10,000 births. There is no known cause. It happens at random. Our boys have no greater chance of having children with Williams than anybody else. Were Kaya to ever have children they would be at a 50-50 risk because every cell in her body is missing genes on one of the two 7th Chromosomes. Obviously, genetic syndromes have no cure.

If you want to look into Williams for yourself, and if you are a member of our family and friends I recommend you do. The Williams Syndrome Association has a website that is full of great and useful information. You can find it here

As far as Genetic Syndromes go, Tiff and I both feel like we hit the jackpot. Most of the personality traits associated with Williams point to extremely happy people, very empathetic and overly social. They are the people that everyone in the community knows and loves. If you live in Mapleton you already know someone with Williams although you may not know it. Kaya is going to be a happy, loving and lovable person. She will be a blessing in the lives of all those that know her. Her life will be full.

So, what does all of this mean? It means Kaya is my little girl. It means I love her as much if not more than before. It means her life is not going to progress in the "Normal Fashion" that most lives proceed. It means she may have serious medical problems that continue throughout her life. It means that after Tiff and I die, her brothers will have a huge responsibility to their sister. It means that the Mapleton Schools better get prepared for a Williams child. It means she won't likely be a brain surgeon or rocket scientist, she may never understand 4th grade math(but that is due to the fact that neither of her parent can do 4th grade math either) It also means that my daughter is going to go on river trips to the Middle fork of the Salmon and the Grand Canyon. She's going to learn to ride a bike and rally up and down Maple Street terrorizing the neighbors. It means that those same neighbors and family will have to keep an eye on her. It means we don't have to worry about her lack of following the weight gain growth charts or her trailing behind on "normal" milestones, and timelines.
Kaya is on her own chart now.
I love you little girl. Thank you for choosing me.

Monday, July 18, 2011

Kaya, year 1!

Kaya will be one on the 26th.

I love you little girl.

Tuesday, March 15, 2011

Is it Summer Yet?

What? You looking at me? What?

Take me to your leader!

I've had some phenomenal Powder days this year, and I hope to get maybe another this week BUT, these warm days are killing me. I paddled Westwater last week with Johnnie A. Shizznittle, and Wiley and the sun was out, 61 degrees, I got a sunburn. The weather has been perfect for running. Tiff's been digging in the dirt, we have the garden started in planter boxes in Kaya's room. I'm ready for
"The Warm".

Kaya has a new habit. It includes digging her fingernails deep into any fleshy surface that is mistakenly placed within her short reach. This usually includes my neck, cheeks, eyeballs, eyelids, lips and nose(it really hurts when she gets a finger in your nose and then pinches)

So the other day we were out in the yard, Tiffy was cleaning up the garden, I was packing for Weswater, getting stuff out of my shed which I Love. Kaya is sitting in her Bumbo chair watching Tiffy and in general just looking like a garden variety Garden Gnome in her Pata-guccie hoodie(thx Murdocks). Ani the big black lab is roaming the yard, chewing anything that should cross her path and knocking kids down with her waggin tail. She approaches Kaya and the battle for the number 2 female in the family was about to be decided. Ani got right down in Kaya's face, Kaya latched on to Ani's lips with her piercing fingernails and dug in. That big black dog just sat and waited patiently for the claws of death to release and the pain to subside. So I guess Kaya established her dominance early on, and Ani secured her place in my heart.

Chillaxin on the swing with little Miss Alien Eyes

Wednesday, February 2, 2011

New Year, New Post

I should hopefully be able to fulfill my New Years Commitment to update this blog at least once a year to keep my one reader (Tiff) appeased.

Been a day or 5 since my last update. Not much has happened. Max had Birthday #5 Noah had birthday #9 Tiff had Birthday #34 and I also had a birthday. Christmas came and went. The kids got a ton of "stuff". We got further in debt. Noah lost 2 more teeth, Max lost his first, Kaya is trying to grow some. Kaya got longer but not much heavier, she's 6 months now and just hit 12 lbs. most of which she carries in her cheeks. I on the other hand am trying to shed the lbs I got for Christmas. Got a wicked case of Achilles Tendinitis the last week of the year from running in my new Five Finger shoes, (the ones that look like stupid feetgloves) Today was my first day back running, felt great.
Back in November Tiff and I both got thrown off the horse, that was fun. Tiff's back is just now feeling better. I spent the last 3 weeks in frantic study mode for my annual "if you fail you lose not just your job but your career" test. I passed, whew.

Took some pictures of my favorite little peanut

In the immortal words of Tom Petty

"She had eyes so blue, they looked like weather"

That's my girl.

Thursday, October 14, 2010

MiddleFork of the Salmon, and random flotsam

Ok, granted it's been a while since I posted but it's because we have been busy, and I'm lazy, and no one really cares anyway. I'm sure there are a few of you out there that are eager for a Kaya update so I'll give you that first and then if you want to stop reading you can.
Kaya is doing very well. She has doubled her weight to 10 lbs. The Dr's looked at her lungs and said don't worry, they looked at her heart and said don't worry. She passed her hearing test, don't worry. She still has the facial palsy, we have yet to hear back on the Genetic testing. I emailed the Geneticist today looking for answers. We worry. I blessed her this past Sunday. For you non mormon readers I gave her a blessing but no "Christening" as Mormons don't buy into the "Babies go to hell if not baptized" theory. It was a beautiful day. Kaya wore a dress made specifically for her for this day made by Grandma Karen. I'm not much for fancy clothes and I don't get too emotional about dresses but this dress was phenomenal.
Yeah, we love her

Me and my girl
Many thanks to everyone who made this a special day for us.

Looking back on the last few months we really have had a great end of summer.
Took the boys and My dad and Greg and Tapeaty backpacking in the Uinta's

Learning the art of Flyfishing

Noah practicing his 4 count rhythm between 10 and 2

This was Max's first year playing Soccer. It took him a game or two to figure out the point of the game but then his competitive fire came out and he kicked ass.
Don't be fooled by the smile, he is a fierce competitor.

Took some family photos for the Christmas Card

Rode the ponies at least once a week.

Took Kaya on her first run in the jogger

We took Grandpa Brian's boat to Strawberry for the day but we forgot to take the camera with us. It was such a beautiful day, the Aspens were Golden, the sun was warm and the cutthroat were biting. We caught this guy on a HTF (that's Murdock speak for High Tech Fly). Max was determined that we bring it home and eat it, so we did. Noah was a little hesitant to watch as I thumped it on the head but Max was laughing a hysterical, maniacal, frightening laugh as he yelled "Die Fishy Die" It did make for a tasty dinner and an opportunity to talk to the boys about where our food comes from. For the record, you can keep one Cutthroat over 24 inches, so that's what this one was 24 1/2

As anyone who knows me is aware, every January I apply for river permits on the Middle Fork of the Salmon in Idaho, and every February I get back my Permit Denied letter. So this past February I picked up a post permit season MFS permit for Sept 20th.I sent out invitations for the trip early and often. Many people committed, many bailed out. In the end a hearty crew of 8 set sail on what is absolutely an annual trip now. All original participants are grandfathered in for every year to come, the rest of you will have to beg and plead for a spot on next years trip. Obviously the water in September is extremely low so this was a Kayak adventure only but Napes was able to get his small cataraft down. The weather was amazing, the fishing was fantastic, my camera spent most of it's time in a drybag in my boat so pics are limited. The rule about only one night camping a t a hotspring is not in effect after the season and so we camped at the sheepeater hot springs, Sunflower, and then Loon Creek. We boiled ourselves silly for 3 nights in a row. We threw a big Elk roast in Napes' cooler and cooked it on sticks over the fire. We ate like heathens for the first 2 nights. We saw no bears, no mountain lions, we did get checked out by the local river otter crew as they eddied out right next to us to watch us float by. It was one of the greatest river trips of my life. Thanks to those who came. If you weren't there it was truly your loss.

Evan Tea, happy to be.

Dagger Falls at low water

Unbelievable fall colors at the ramp. For the first couple days we all just floated with our mouths open in astonishment at the beauty we were surrounded by.

The crew gets ready to launch
L-R Steven LeGrande Wiley, Colby Frazier, Alexander John Napier III, Nash Way, Johnnie Allsen(in boat), Fat Newty, Evan Tea, Jason Tea

The Good life at Sunflower Hot spring.
You'll notice my Tillamook Cheese and Dry Salami waiting for me.
I honestly cannot think of anything better than this.

Colby in Mid Jump off of the White Creek Bridge

Napes in Mid Contemplation at the confluence of the MFS and Loon Creek
That's my boat and gear in the foreground. There is something very fulfilling and elemental about carrying all of you gear inside your kayak.

"Newty, Newty, I caught my first Trout!!
Napes was so excited he dragged this poor fish over rocks and trees on a punishing trip to the camera. The little guy did survive the ordeal, the fish also survived.

End of the water, start of the "It's about 2- 2.5 hours from here to Stanley." Road trip that lasted closer to 5 hours to Stanley, about 13 to home. Every one of us would do it again without hesitation. Thanks again guys for a wonderful trip. For those that missed it, better get your resume and letters of recommendation in to us soon.

Wednesday, August 18, 2010

Kaya has been doing very well here at home. I attribute her success to two things. She has an amazing will to be well. She has a Mother who's eye is single to the life and love of her child. I love them both.
Two big brothers who love their sister.

As the bretheren from Coldplay say,
"We live in a Beautiful world"

We tried to keep things as normal as possible for the boys while we were at the hospital and we're making a serious effort to let them know they are not forgotten. Last week before I had to go to work I took Noah on a horse ride up Day's Canyon in the right hand of Hobble Creek. It was his first ride outside of the pasture. I wanted it to be a good experience for him and he did fantastic. We rode for a little over an hour and we both had a great time. Hopefully it will be something we can continue. This week we're going backpacking in the Uinta's.

Boy's+ dog+ Horses= Nuttin but good times!
(yes those are wranglers)
(yes I'm still a Kayaker)

Thursday, August 5, 2010

So Far...

We've had Kaya home now for four days and she has managed to survive a constant barrage of well wishers, Grandparents, Great Grandparents, Aunt's, Uncles and Cousins, bringers of food stuffs and so many damn pink things I think I might just vomit. Honestly though, we appreciate all of the support we have received. Her first days at home have been remarkably unremarkable. She has settled nicely into the steady baby rhythm of Eat, Sleep, Poop, rinse, repeat. She's a pro at the eat and poop parts, we're working on the sleep portion. For my part, the absence of lactating breasts has been a huge boon to my sleeping vs. getting up every hour ratio. Tiff on the other hand is getting her sleep where she can, mostly during daytime naps. Kaya went to the pediatrician today. He had a good look at her and thinks the hearing problem is due to the crap(yes literally) in her ear. Poor kid looks to be getting an ear infection already in the good ear. The Pediadoc also clipped her tounge. It was held down too short so he clipped away. These are the reasons we don't remember being this young, it's to traumatic. If we could remember the first days of life every one of us would have post traumatic stress syndrome. So as far as the Pediatrician goes he said she looked fine, needs to gain some weight. Come back in a week and look at the ear infection again and weigh her again. So things here are going quite swimmingly so far. We love having her home with the rest of us. We do not miss traveling to the NICU. I'm headed to work for a few days so the pictures will be few and far between but Tiff has loads of helpers here to make sure things go well. She'll probably have more help than when I'm home. I'm sure you're all sick to death of reading my drivel so here come the pics.

Sleepin. Max and I both sleep like that, with our hands under our cheeks.

Just my luck, she's a natural born cheerleader.

Early onset of runner's knee

This one is part Heisman Trophy pose,
the other half reminds me of the words of George Castanza
She looks "Like an angry old man, trying to send back soup in a deli"

Thanks again for the prayers and concern. I'll try to keep you all updated regularly.
Goodnight, and good luck.