Welcome! My name is Kat and I'm the mother of 4. Rachel is living with a universally terminal illness called Juvenile Batten Disease. Rachel, Julie, London, Boston and I are doing everything we can to suck the marrow out of life and looking to raise money for Batten Disease Research. Watch us make the most of the time we have.
// 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.
// 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>
// 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).
// 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.
// January 12th, 2010 // 3 Comments » // Life
Julie had a nasty migraine last night, her first one ever. I was looking up online and found that people who “get migraines” generally start getting them as early as age 5. I hope it was a one time thing because she was in a lot of pain through the night.
London has the sniffles and Boston is grouchy so I’ve kept all 3 of them home today. It was a half day for all of them today anyways (Rachel is the only one @ school today… weird change).
I went to look at a house for rent nearby, one that is all one level with no steps to get into it. I am going back tomorrow with some measurements to see if our stuff will fit (mainly our couches and king size bed). Its a small house but would be great for us as Rachel slows down and starts having more mobility issues. Not knowing when mobility issues are going to become a real problem is really hard because I’m a planner. She is already needing help to get up and down the stairs to get into our house (but she goes up to the second floor by herself).
Updated Entry at 5pm
Julie and I just made some cupcakes, London is playing Webkinz and Boston has been begging to look on amazon for toys all day. I made several donation runs of clothing (etc) today and did a good house clean out. I’ve started recycling but looking for free ways to recycle. So far I’ve found where to recycle clothes, paper, soda cans and plastic bags for free. And I’m trying to compost but haven’t bought an indoor composter (New England isn’t the best place to compost in the winter, I guess).
Do you compost? Share some info with me because I’d like to be better at it.
// 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.
// January 3rd, 2010 // 3 Comments » // Boston, John, Julie, Life, London, Rachel
Took the kids sledding in the morning, stood next to the shore in Hull watching the waves crash against the wall. Spent the afternoon taking all 4 children ice skating (RACHEL, TOO!!!). First time on the ice for Julie, London and Boston and they did great! I plan to go back next week with Rachel (and then some) during their free skate because it is supposedly very quiet during the week.
I asked the kids which activity they preferred and London was the only one who liked ice skating the best, the other 3 felt sledding was more fun. Rachel was a little hesitant to go sledding (can’t say that I blame her) but I went down with her the first time and shoved her ass down the hill the second time. After that she couldn’t get enough!
John went sledding for the first time in his life today and is quoted to say “that was fun.” I know he liked it more than that but we’ll take it.
Today rocked! I have a few iphone pics to share tomorrow.
// 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?
// 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!
// 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!
// December 4th, 2009 // 4 Comments » // Life
Trash day has been once again celebrated, and… Rachel is in great spirits. Life is good!
This weekend we have two girl scouts things (one for each girl), we’re taking Holly to the mall for Doggy pictures tonight and baking cookies for the drama kids. Sunday we are going POLAR EXPRESS!!!!!!!!!!!!!!!!!
This weekend, my friends, is going to kick ass!
// 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.
// November 25th, 2009 // 5 Comments » // Life
We have a customer that knows another family that has a son (within 30 minutes of us) that has Juvenile Batten Disease. The customer put us in touch some weeks ago and it was kind of nice to be able to talk to another person who understands (etc etc etc). Anyways, the customer put a note on our door last night and put a jar of her homemade tangerine / cranberry sauce underneath our tree on the porch. Very, very thoughtful.
Today John handed me a card from the photographer that took our wedding pictures and inside the card was a Gift Certificate for a free family portrait! So amazing! John and I think we’d like to do another family picture when there is snow on the ground. Totally amazing!
On Saturday the Mayor of Hull (Geri) is holding the annual Hull Holiday Showcase and, with the help of her kiddos, is putting together a “Batten Disease Ornament table” that will have Batten Disease info and offer kiddos the chance to make ornaments (including one for Rachel) and contribute to a paper chain for Rachel. The proceeds are all going directly to the BDSRA.
People are truly awesome. Seriously. I just sent our “Good Geek” Christmas cards to the printer. If you want one, please email me your address. The picture on the card is from our Disney trip and might look nice on your fridge. My email is mrskatvon@gmail.com
xoxoxoxoxoxo
// November 24th, 2009 // 4 Comments » // Life
I realized today that trash day isn’t until Friday this week. Its kind of like a personal challenge to me. Waiting out an extra day, making sure I spent my extra day wisely. I’m sure its some kind of weird sickness to look forward to trash day as much as I do but I figure I’d rather have this sickness than something really bad. LOL
We’re having Thanksgiving with my Mom, Dad, Brother and 2 (favorite) Euro-relatives that are here for the week.
Thanksgiving is my least favorite holiday. I am looking forward to a couple of days break this year but Thanksgiving still lands on the bottom of the holiday totem pole for me. I feel thankful every single day of the year. I know lots of people say that but I truly do.
Thankful that I am sane, thankful that I am healthy with lungs that work and a heart that beats. Thankful that I have 4 (relatively) healthy children that adore me when I make them cookies and have 4 little faces that light up (mostly) when they see me. I make good decisions (finally), I am a good advocate for my children and I am extremely enthusiastic about the little things.
What are you thankful for, every day of the year?
// November 22nd, 2009 // 5 Comments » // Life
Need one more weekend to finish upbut this is how it looks. The kids are so excited!
Copyright © 2010 As told by Kat. All rights reserved.
