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 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 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!!!!!!!!!!!!!!!!!!!!!!!!!
// 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!
// November 10th, 2009 // 2 Comments » // Boston, John, Julie, Kat, Life, London, Rachel, School, Thanksgiving
Can’t think of anything to write about tonight. I still have a horrible cold / upper respiratory infection that seems to be a “super cold.” I have been having lots of irrational thoughts about never leaving the house again and wearing a face mask to check the mail. Definitely the virus “talking.” Julie thinks I need to wash my hands more.
The kids had microwaved hot dogs with ketchup for dinner. Kind of proves that I feel terrible.
Both Julie and London brought home “manilla turkeys” to decorate and I doubt that our dining room carpet will ever recover from all of the glitter it soaked up tonight. Julie’s turkey is so thick with glitter that I think it needs a custom piece of plywood just to support the weight.
Rachel had a good entrance and exit to school today. We brought Holly (our dog) to school with us and that most certainly lifted her mood and made the transition easier. I think I better bake some cookies for them tomorrow. You can never have a mom who bakes too many cookies, right?
// November 4th, 2009 // 4 Comments » // Batten, Julie, Kat, Life, Rachel
Thank you to those that emailed and commented on my blog. It does make me feel better to be heard and not “suffer in silence.” Really I am not the one who is suffering but it is very painful to have a front row seat.
And thank you to Veronica at ATT Wireless who kindly cancelled Rachel’s cell phone and waived the early termination fee.
Better day today. “Better” is a relative term I should clarify. I still had to lay her on the floor with a rolled up towel under her head for a pillow and pin her body and arms down so I could brush her teeth and she tried to spit in my face in the process but the bottom line is that I got the job done. I hope she is having a good day with the Melissa & Doug music set I got for her this week. She was unhappy with music class this week because she couldn’t sing YMCA (she can’t mentally keep up with the song and can’t see the arm movements) and she actually was able to tell me “I can’t keep up.” I can’t imagine. Hopefully her person (Mrs P) isn’t suffering from a headache right now.
Julie is at home with me today with a hacking cough. She isn’t sick, just sick of coughing. (And she is eating oreos next to me right now).
If anyone has any grocery coupons they no longer want / need I would be very appreciative to have them. I am trying to cut down our food bill as much as possible.
// October 24th, 2009 // No Comments » // Boston, Julie, Kat, London, Rachel
It is dark and gloomy today but soccer is still on. Julie still won’t play on the team that we sponsored and are coaching. My girl is, at this age, not interested in playing team sports. I prefer solitary fitness activities as well so I can’t say that I blame her.
Boston won’t stop eating the organic banilla yogurt. Gross! At least no one is fighting him for it.
Rachel is, right now, watching Nickelodeon as best as she can. Her blindness is becoming more and more apparent.
London is my cookie monster. Can’t keep fresh baked goods in the house for very long with London Jack around.
I am clearing out my email, ordering pictures via shutterfly and tying up some loose ends. I cannot say enough about having a regularly updated “to do” list, it really makes a difference.
gmail + google calendar + google docs = increased productivity and happiness
I love Google!
Rachel goes back to school on Monday. Her new schedule is M, Tu, W, Th. 8:45 – 11:15. I am excited for the new set-up.
// October 22nd, 2009 // 2 Comments » // Batten, Boston, Colorado, Julie, Kat, Life, London, Pictures, Rachel
Another trash day has come and gone.
This was a pretty uneventful week, the kiddos are all healthy (knock on wood) and Rachel went in to school for some OT and Speech therapy which she enjoyed. Next week she is going back to school on a modified schedule that fits in with London and his preschool hours. She hasn’t been going to school since the week of Disney (Make A Wish) trip.
Julie is sitting next to me playing with her new wooden stamp set that I amazoned her as a special treat. The weather is perfect for October and Rachel, London and Boston are playing on our porch, actually on the other side of the window I am sitting next to. Its very nice to see them playing. Rachel is doing some pretend play in their play structure, pretending it is a ship that she is sailing to California with her GPS. Its nice to see her imagination at play. She started a stimulant medication this morning and I’m hoping that we will see some long-term benefit and behavioral improvement. Her vision continues to get worse, which we know to expect, but it still sucks to see. The blind have their own world that I have never been exposed to but I’ve been thrown into the deep end of the pool and am trying to learn quickly.
London and Boston are back to daycare 2 days a week and are staying until 5 so I can get some work done (which is quite busy as of late). Both boys are in preschool (London is where Julie went and Boston is in the little green school across the street from our house) and they are happy. London is going to be Woody and Boston is going to be Buzz Light year. Julie wants to be a “Girl Frankenstein” and Rachel wants to be a “Mummy.” WTH am I going to find a Girl Frankenstein??? The kids are convinced that I *NEED* to be “Jessie” from Toy Story. Looking forward to Halloween (which is also my last day as soccer coach for the season).
A very nice person is taking my girls out tonight for a couple of hours and I am sitting next to three hampers full of clean laundry with no chance I can get it folded before she comes. I guess, on the bright side, is that the laundry is clean.
Most people say “Calgon take me away” but I’d rather think about this:
// October 12th, 2009 // 1 Comment » // Batten, Business, Good Stuff, Humor, John, Julie, Kat, Life, London, Rachel, Vacation
I signed John up to do Village Idol and took the video (so I could hide behind the camera, I won’t lie). Rachel is hiding in the shadows, John is multitasking with Julie and Boston while London is sitting in the audience with another child’s father. This was during our Make a Wish trip at “Give Kids the World.”
// September 27th, 2009 // 1 Comment » // Batten, Birthday, Boston, Halloween, John, Julie, Kat, Life, London, Rachel
We went on our Make A Wish trip to Give Kids the World, Disney, Universal and Seaworld (all down in Florida). I still can’t get over how hot it is down there in the fall! It was stressful because the kids are young and Rachel is clearly not on the correct medication but we definitely had a lot of good moments and took some great pictures! I’m glad that Rachel was able to experience the things that she did while we have usable vision. Who knows how long she will have that for. We have seen another decline in her vision in the past couple of weeks and she is starting to feel things much more rather than look for / at them.
I posted some WONDERFUL pictures of our awesome Make A Wish trip on Rachel’s facebook page, feel free to look! We met Mickey, Minnie, held an alligator, took Rachel on lots of rollercoasters and met some wonderful volunteers and people at Give Kids the World!
Julie has been having lots of meltdowns that I would expect to see a few years ago. I think part of that is she is tired but certainly another part of it has to do with Rachel and how she flips out and is so demanding to manage and refocus. Julie is definitely attention seeking and it truthfully is not surprising.
Boys are pretty much the same wonderful / obnoxious little guys that they are. London is back to preschool tomorrow and Boston starts back at his 2 mornings a week preschool that he is so excited for! I’m happy for them. Boston was so excited to go to preschool the Thursday before we left for Florida so hopefully he will transition well on Tuesday.
This coming sunday is our double birthday party for the boys turning 3 and 4. Halloween theme! I want to make it really special for all 4 of the kiddos but not make it too huge. I think either way Rachel will be overwhelmed and I might have to have my Mom take her away early depending on how she does. Who knows, she could surprise us all, right?
I was really happy to see that the University of Iowa just published a report on 9/21/09 that they have found a way to get through the blood brain barrier and treat 2 kinds of lysosomal storage diseases (Batten Disease falls under this group). Here is a link to the article on the BDSRA website:
http://www.bdsra.org/docs/research/nm.2025.pdf
I am starting to plan a handful of fundraisers, some of which will take place this year and a few in the spring. I would so love to see some kind of treatment before it is too late for Rachel. We go to the University of Rochester next week.
http://dbb.urmc.rochester.edu/labs/pearce/bddcrc/clinical_eval.htm
This just doesn’t feel like it can possibly be happening.
// September 15th, 2009 // 7 Comments » // Batten, Boston, John, Julie, Kat, Life, London, Rachel
I cannot believe that I didn’t go to Julie’s “Book fair” slot today. The poor kid was there and didn’t have her Mommy show up and didn’t have any money with her. I’m sure I got the notice (twice, once for each girl) but I didn’t flip over the letter to get the book fair class times. I feel like the worst mother ever imaging my little girl alone and penniless. *cry*
I cannot believe that Rachel is dying, slowly losing her vision, her mind and her abilities.
I cannot believe that my Boston is going to be going to his very own preschool program. Nearly 3 years old!
I cannot believe that my London is about to turn 4, is totally potty trained and goes to preschool. Time flies!
I cannot believe that we are going to Disney World in a matter of days.
I cannot believe that I actually drive a minivan and have done so for 5 years.
Sometimes I cannot believe that this is my life, the good and the bad. Yesterday I was a teenager driving an ‘86 For Mustang and today I am a grown-up with some pretty giant responsibilities on my shoulders. How the hell did that happen?
Copyright © 2010 As told by Kat. All rights reserved.
