As told by Kat

Monday Mawn-in

// February 8th, 2010 // 1 Comment » // Kat

We had a good weekend. Friday I took Julie to Childrens and we got the “all clear” for her heart murmur! I wasn’t worried about it because there are countless people out there walking around with benign murmurs but it was really nice to hear good news.

Friday night Rachel and Boston had a sleepover at Granny’s because Boston is POTTY TRAINED! We promised him a sleepover as a reward and he had THE BEST time. I took advantage of being “half off” from kid duty and took Julie and London to dinner at Barefoot Bobs Friday night and then to the dollar store on Saturday. We managed a playdate and met a HUGE dog called MayDay and ran some errands.

Sunday I took John and the kiddos to the MFA and everyone had a good time. Karen, our tour guide, gave us gloves so we could TOUCH some of the exhibits. AMAZING! Even Boston had a good time.

Friday

// February 5th, 2010 // 2 Comments » // Doctor, Julie, Kat, Life

Today I am taking Julie to Children’s Hospital to have a recently discovered heart murmur checked out by a cardiologist.

Julie has spent the last week telling everyone she knows and then immediately following it up with “don’t worry, I’m not worried.”

She is such a good girl. I’m looking forward to having a few hours alone with her. Will update later.

Nightmare

// February 2nd, 2010 // 1 Comment » // Kat, Rachel

I refuse to spend my time with her overcome with the overwhelming feelings of living a nightmare.

But we are.

Living a nightmare.

This is so unfair.

She barely got started living.

The nausea / nightmare feeling hasn’t gone away since July and it never, ever will.

EVER

The Bucket List

// January 24th, 2010 // 11 Comments » // Batten, Bucket List, Colorado, Julie, Kat, Life, London, Maine, Rachel

So if you follow Rachel’s facebook page or know me “in real life” you’ll probably know that the idea of a Bucket List has been the topic of several conversations between me and the kiddos. They don’t understand that this has ANYTHING to do with death so please don’t comment or email me worried that I have a doom and gloom attitude that is negatively affecting them. My positivity is borderline disgusting. LOL

I’ve put “The Bucket List” on the top right of the blog and plan to add many, many more things to the list as the weeks go by. The plan is to take a very long (nearly) cross country trip stretching from Boston up north to Maine and all the way West to Kansas City then Colorado and back. We have free access to several amusement parks through September and I’d like to take advantage of this gift from “Give Kids the World” (the place we stayed in Florida on our Make A Wish trip).

I’m also setting up a FLICKR account to link our pictures from all of these successful missions to show the world that life is too short to wait on the important things in life. Life is too short, people. It’s a fact. Batten Disease is such a shit disease, I refuse to let it steal my Rachel until she has had the chance to use up her body and slides into home base covered in fattening food and exhausted from life.

The more afternoons I can get my children sticky with ice cream this summer, the better! I’ll leave you with a picture from Summer 2009 (the extra 9 year old boy in the picture is my nephew Ryan).

You keep that thumb in the up position, baby. Hold on tight because I’m going to drag you all over this country!!!

For the unaware, “The Bucket List” was a movie that came out in 2007. < link to IMDB>

Trip to Maine

// January 18th, 2010 // 2 Comments » // Batten, Boston, Bucket List, John, Julie, Kat, Life, London, Maine, Pictures, Rachel, Vacation

I scooped John and the kids up and took them to Maine for the weekend. We had a great time although I had promised to tray and do some sledding or snow tubing. We met a family of chickens and a herd of alpacas, went swimming and hot tubbing and took the kiddos into a sauna for the first time. We even brought home 3 dozen farm fresh eggs which have already begun the transformation into baked goods.

Here are some of the pictures which I will directly share from facebook (you don’t need a facebook account to see ‘em).

http://www.facebook.com/album.php?aid=374918&id=266424125552

We had THE BEST time! Here is my favorite picture of Rachel from the weekend. Just goes to show that you don’t have to see well to have a fun and full life. Experiences like this are more valuable to Rachel than to a sighted child because of her tactile senses.

I think I think I did a sufficient job at flipping Batten Disease off this weekend. Tomorrow morning we’re going ice skating for an hour. Can’t wait! Gotta get them into a swimming pool more often. They love the water (just like their Momma) and love the hottub (just like their Daddy).

Depressing

// January 14th, 2010 // 2 Comments » // Batten, Kat, Life, Rachel

I’m an upbeat person.

But this week when I was setting up 529 Savings accounts (through Upromise) for 3 out of 4 of my children, I became really, really depressed.

Her new meds are working well so right now I am focusing on TODAY and enjoying TODAY. Tomorrow might suck but as long as today is good I can handle the distant future.

What are you doing this week (or weekend) to enjoy the TODAY? You might not have a tomorrow so make sure TODAY counts.

Another update

// January 8th, 2010 // 2 Comments » // Batten, Julie, Kat, Life, Rachel

Good week. Everyone was exhausted at bedtime tonight since it was the week after vacation. I took the kiddos ice skating on Tuesday and we’re going again this weekend. Next week I am taking Julie and London for their first day of skiing at a nearby mountain. I can’t say that we have the money to do all this fun stuff, but I can say that life is so stupidly short that I don’t see a choice.

Rachel is off the stimulant medication because I wasn’t seeing any difference. We’ve been waiting to see a NCL specialist neurologist in boston for quite a while but have just been informed that this neuro has gone on an immediate medical leave and Rachel’s appointment has been rescheduled for the end of april. I was telling our primary care all of this on Wednesday at Rachel’s follow up (which was also the same night that I had apparently scheduled Julie’s physical) and the primary care doctor agreed to let me give risperdal a shot. Quite a different medication than adderal but I am really hopeful that this medication will increase her quality of life and place Batten Disease on the back burner where it belongs!

Julie’s physical went well, she is absolutely perfect. She got her first shot (since she was about 4 months old) – the MMR, and had a lead test. The doctor heard a heart murmur that we had never heard before and has referred us out to a pediatric cardiologist so we can have it checked out. Its statistically going to be nothing but the doc and myself both agree that it needs to be checked out.

Last but not least, Rachel and Julie are selling girl scout cookies. If anyone wants to buy some, let me know!!!!!! Feel free to email me @ mrskatvon@gmail.com and you can either buy some boxes or sponsor some boxes and I’ll donate them to the next school function for the kiddos / teachers. Its up to you. The girls are excited to sell a couple of boxes, I am NOT excited because I cannot control myself near the caramel delights.

Exhale

// December 30th, 2009 // 4 Comments » // Batten, Boston, Julie, Kat, London, Rachel

Having to explain to my 9 year old daughter who can’t see very well that she can’t learn to ice skate really SUCKED! If only for one winter, I wish that Rachel can ice skate. Please let it be possible.

Earlier today Rachel told me the money she is saving is for a car. I gently reminded her that she can’t drive a car and she said her “person helper” would drive her in it. I told her she should have Julie drive her.

This sucks so hard. A nightmare. I have noticed she is having a harder time identifying her siblings. As of late she would confuse her brothers but now she can’t differentiate between her sister and brothers. She’ll walk into a room looking for me and if I don’t immediately answer her calls of “Mom, Mommy?” she immediately walks out because she can’t see me. Such a good kid that deserves a full life. I guess I better get moving because I don’t have as long as I expected. Its a very painful lesson. I’m currently looking into planning some mini trips for us when the weather warms up, doing things that can be DONE rather than just seen.

Lots of rage today from her. Her emotional filter is apparently on vacation (hopefully not a permanent one). I’m grateful, in some ways, that Julie, London and Boston are so young because they are doing great in the face of our daily life. But its hard because they cannot grasp the concept of her recent blindness and can’t remember to “keep the floor clear” (or she’ll trip and fall or break their stuff out of sheer frustration). Most adults I know can’t grasp the concept of her recent blindness, either. What does that tell you? My little 3 are awesome with her. I’m starting work with having them identify themselves to her but that will take time.

DO NOT TAKE YOUR LIFE FOR GRANTED!!!!!!!!!!!!!!!!!!!!!!!!!

Christmas week

// December 27th, 2009 // 4 Comments » // Batten, Birthday, Boston, Christmas, Food, Julie, Kat, Life, London, Rachel, Valentines

is drawing to a close and I’m up by myself tonight (a special shout out to our cat, Krabs, who is sitting next to me while I type). Christmas was good, we went to my Mom’s for Christmas eve and raced home to put out the reindeer dust, milk, cookies and carrots. Santa brought Rachel the “babydoll” stuff she asked for, PIXOS for Julie, an army tank for London and the Buzz Lightyear “yard” that Boston has been talking about non stop. It was a really nice day and the kids have finally calmed down from all of the excitement.

Today I brought up our Geotrax stuff that has been sitting in the basemenrt for at least a year and the kids played with it for HOURS. For whatever reason we don’t have a complete remote control train for the set (either the RC or the train but not a matched pair) and I looked everywhere. The kids manually pushed the trains for hours and had such a good time. I was going to play with the wii fit tonight but I didn’t have the heart to put away their train stuff. They are such good kids

I am absolutely broken over Rachel and what her disease will mean for her future. It is so unfair and I try not to think about it because I just cry hysterically and it doesn’t do me any good. I’m very good about keeping my emotion hidden, especially when I have to. She made me a box for Christmas at school with her paraprofessional with a little note that she had me read out loud. And here I am sobbing but doing everything I can to not let her hear my voice crack. Her hearing has become quite acute as her vision has been failing and she picks up on things she didn’t used to.

I hate this disease and how powerless she is. There is no fighting what is to come. No treatment. No hope. My child is slowly fading away and is generally unhappy, anxious, unsure and not living a life filled with quality and happiness.

And all I want to do is crawl under a rock and die for every moment that I fuss at her for things out of her control. The natural guilt of motherhood has always been difficult for me but it is borderline suffocating now. Balancing 3 presumably normal children who make a lot of noise and hopefully have very long lives ahead of them while taking good care of Rachel and making sure she has good moments and opportunities in her short life. That balance is absolutely impossible to strike and fills me with incredible guilt.

And while I have this inner symphony of juggling and guilt and batten disease and giving them good childhoods, I put a smile on my face and take them places and cook them dinner and show them how to “whack and unwrap” a chocolate orange and making sure they use the correct “Terry’s” terminology. How do you have a normal life in the face of the disease? I’m not sure of the answer but I do know that I am working my ass off to try and make things normal and wonderful and happy.

The kiddos and I already planning to make edible valentines for all of their classmates instead of the lame box o’ cards.

We’re also planning to learn how to make the PERFECT homemade Carrot cake to celebrate the birthdays of my mom, brother and his girlfriend (in March). Anyone have a good recipe?

Christmas Week

// December 21st, 2009 // 5 Comments » // Christmas, Kat

Gosh this has been a busy couple of UNEVENTFUL weeks! Just the way I want it! We had THE REAL Santa and his Elves pay a visit to our house on Friday night, complete with presents for the kids, the cats and Holly (our dog). The kids were SO excited!

This week is a short week at school and on Wednesday they are having “Polar Express” day and I’ll be going in to help out with that. Today at 12:30 a blind man is coming into Rachel’s special ed class to talk about being blind but Rachel gets out of school everyday at 11:15 so I’ll be bringing her home for lunch then taking her back in. I hope I get to stay and listen to him speak.

I hope everyone is enjoying this Holiday Season. I have plenty of pictures to post and will get around to doing that this week before Christmas.

Now tell me, what are your family plans this week? My children and I LOVE this time of year! If you quickly type out your traditions I’ll read them to my kiddos and maybe we’ll adopt some of your traditions. Thank you!!

Santa Claus!

// December 13th, 2009 // 5 Comments » // Christmas, Good Stuff, John, Julie, Kat, Life, London, Rachel, Winter

Our kiddos had their picture taken with Santa near the Paragon Park Carousel.

Rachel, Julie, London and Boston with Santa

Rachel on the Christmas Carousel Horse

No Batten Disease in these pictures!

The week in review

// December 11th, 2009 // 3 Comments » // Batten, Kat, Life

Apparently I’ve been a little bit busy and I didn’t update for an entire week. First of all we must pay respect where it is due and proudly salute yesterday, Trash Day, as it is the best (BEST) day of the week. Yay for trash day!

We went to the Polar Express on Sunday night courtesy of the very, very wonderful Mother of one of Rachel’s favorite teachers. Thank you so much, we had a wonderful time. I took a ton of new pictures but haven’t been able to get any up for editing because my laptop was having issues. Sunday night I went to hop on the computer to take a look at the pictures from the train ride but ended up finding out that the hard drive COMPLETELY died. Not one shred of data can be recovered. Proof that it does happen to “computer people” as well as everyone else. I’ll pull the pictures up on the temporary laptop that John just set-up for me and put them on here.

On Wednesday the girls went out with the very, very wonderful daughter of the Polar Express gift giver and had an AMAZING time! I still don’t know what they did before they had dinner but I don’t think I’m supposed to know.

This week has been busy with work and getting ready for Christmas. John has been rocking bedtime and bathtime this week and it has really helped keep me sane. My Mom has Rach, London and Boston right now, yesterday she had Julie for a few hours and they got to do some shopping.

Tonight Rachel, Julie and I are going to see the Hull High School put on “Willy Wonka” and I’m so excited! Rachel and I were supposed to go up to Maine this weekend but Lulu’s Mommy is sick so we are going up another weekend. Instead of Maine I have already replanned the weekend and are taking the kids all to see Santa on Saturday and we’ll be having Lobsters for dinner on Saturday night. Sunday I need to get the kids out individually to buy a gift for their secret santa person and hopefully tie up some projects around the house.

As far as a Rachel update: She had a really good week at school and at home, much better than last week. Her vision is still actively going away but I would say at a slower rate this past week or two than the previous two weeks. Put it this way, she was standing facing the black couch where Holly was sitting last night and she was looking for Holly but couldn’t see Holly in her peripheral vision. The contrast between the couch and Holly isn’t great but imagine looking at your dog and not being able to see her? The fact that Rachel was able to hide her difficulties for so long as best as she did is so amazing to me. She deserves a medal. I guess thats why I’m springing for Lobster on Friday night. Batten Disease took a backseat this past week and I couldn’t be happier!

Rachel and John read the news

// November 30th, 2009 // 4 Comments » // John, Kat, Life, Rachel, Youtube

I need to read the manual on my camera, my goodness! Sorry for the crappy quality.

Feel free to subscribe to my youtube, I have more videos to add.

http://www.youtube.com/mrskatvon

Happy 6th Anniversary

// November 15th, 2009 // 9 Comments » // John, Kat

John and I were married 6 years ago today. I can’t imagine stuffing any more stuff into the time we have been together (8 years). If you had told me that this guy (John) on yahoo personals was the one I was going to marry, have 3 more kids with and everything else that has happened, I would have died laughing (and crying). Life is full of surprises.

I have

// November 11th, 2009 // 3 Comments » // Kat, Life

really great kids