As told by Kat

At home day

// January 12th, 2010 // 3 Comments » // Life // 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.

Another update

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

Best Sunday Ever!

// January 3rd, 2010 // 3 Comments » // Boston, John, Julie, Life, London, Rachel // 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.

Exhale

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

The sun is shining over our house

// December 4th, 2009 // 4 Comments » // Life // 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!

Youtube

// December 2nd, 2009 // 1 Comment » // Youtube // Youtube

I’ve started to add a few videos into my youtube account (mrskatvon) and take them out of my old, workish one.

www.youtube.com/mrskatvon

Word of the day

// December 1st, 2009 // 4 Comments » // Batten // Batten

Couple of days, actually. We’ve been having a rough go of it with Rachel. I’m crossing my fingers for a good couple of days starting tomorrow. We took Holly to be groomed today at Sunshine Pet (www.sunshinepet.com) and are about to go and pick her up. Hopefully that will lift the word of the day from her a little bit, or at least raise her mood.

Word of the day: dementia

Wiki: http://en.wikipedia.org/wiki/Dementia
Google Health: https://health.google.com/health/ref/Dementia
WebMD: http://www.webmd.com/alzheimers/tc/dementia-symptoms

Lots of anger, irritability, lack of concern for others, lack of reality, immediate (IMMEDIATE) screamed demands. Frustration, crying, throwing, hitting, flipping out, inability to prevent herself from acting out on intense feelings. And on and on and on.

Today this disease might have won but I swear that I am going to kick its ass this weekend and have a great, GREAT time with my kiddos. Stupid batten disease. Take a couple of days off because it’s my turn to have her!

Rachel and John read the news

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

Golden Tickets

// November 29th, 2009 // 4 Comments » // Birthday, Christmas // Birthday, Christmas

Yesterday was the Hull Holiday Showcase and there was a BATTEN DISEASE TABLE thanks to Geri Calos, her kiddos and the extremely mature “Hull Theatre Arts” (aka Drama) kids. Many, many thanks to the kids that sat at the table all day and helped little kids glue, stick and tape ornaments together to raise money for the Batten Disease Support and Research Association!

The Hull High School Theatre Arts is putting on a play in 2 weeks and we can’t wait to go! Willy Wonka and the Chocolate Factory is currently being advertised on Rachel’s new shirt that she bought at the holiday showcase yesterday. She is very proud to be advertising with the new shirt. I tried to take a picture but Rachel couldn’t stand still long enough for me to snap a picture of it.

I’ve been spending some time trying to learn ways to naturally clear out Rachel’s cells via diet. Last night she had a ridiculously huge portion of haddock. Julie and London wouldn’t try it and Boston gave it a taste but thought it was “yucky.” I’m not a huge fan of fish but I’ll learn to like it if that means we extend Rachel’s “good life” longer. I’m not going to try and extend her life just to keep her alive. Quality of life is my NUMBER ONE priority for Rachel.

Today is Sunday and we are having our usual lazy day on Sunday. Laundry, dishes, play at the park and maybe build another shelf for toys. I’m also planning to take Holly on a couple of walks and bringing a different kiddo each time.

Thinking about christmas and trying to think about what to get the kiddos. Does anyone know if they make a half a bike that I can attach to my bike for Rachel. I’ve found lots of tandem bikes but her legs aren’t long enough for a tandem adult bike. I’d like a second seat, wheel and pedals that can attach to my bike so we can ride together. She needs more exercise than she is getting and I know she loved riding her bike that summer.

Life rules. Enjoy your Sunday!

Thanksgiving 2009

// November 26th, 2009 // 3 Comments » // Thanksgiving // Thanksgiving

Thanksgiving should be renamed: “Key Lime Pie Day.”

Today was a good day. Good food made by my Mom and awesome desserts made by my other half. Rachel had a good day today, she has been “entertaining” my cousin and his wife who are here from England. I spent 10 minutes watching her watch TV before bedtime tonight and was humbled by how much she overcomes on a daily basis and how truly little (very little) Rachel complains.

Life is so humbling if you stop and look around for a minute.

Love my kids. Love Key Lime pie. Anyone else out there love a good key lime pie?

People are wonderful

// November 25th, 2009 // 5 Comments » // Life // 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