Best Sunday Ever!

Posted by Kat in 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

Posted by Kat in 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

Posted by Kat in 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?

Santa Claus!

Posted by Kat in 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!

Rachel and John read the news

Posted by Kat in 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

Blank

Posted by Kat in 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?

The stages of grief

Posted by Kat in Birthday, Kat, Life, Rachel

I just found myself googling the 4 stages of grief only to find out that there are 5. Who knew?

I’m assuming that its normal to bounce around from one stage to the next and back again?

Anyways, here are the four FIVE stages of grief.

1. Denial (Check) and Isolation (No) (Doesn’t that make six stages?)
2. Anger. (Check)
3. Bargaining. (No)
4. Depression. (No. I’m so sad for her and what she will miss out on but I won’t give into depression and miss out on the good moments and the good days)
5. Acceptance. (No)

I can’t imagine the day that the warm sun rises and I actually feel acceptance.

I tried to spend a few moments today imagining what Rachel would do if she were in my shoes. What would be her plan of attack if it were her daughter with this disease. What would she do? Because whatever *that* is, is what I want to be doing. I need to get out of the overwhelming sadness I feel for her and the speechlessness of it. I need to start fundraising so there is never another child lose their eyes, lose their mobility, lose their mind. In Rachel’s honor I need to make a difference.

I changed the “About me” on the top right hand side of the blog to sound a bit more cheery. I don’t feel that cheery yet but I’m hoping to get there. I want to spend my “Kat time” (when I’m not working or with the children) on promoting awareness and fundraising for the www.BDSRA.org.

I start most of these paragraphs with I. I don’t know why.

I have talked about fundraising a few times since July but I don’t know where to get started. I haven’t gotten closer to figuring the “getting started” part in the months since July so I figure I better get started NOW or I might find that 2 years has passed and I’ve done nothing. And doing nothing is simply not an option.

Please email me at mrskatvon@gmail.com or comment if you have fundraising ideas that have worked for you (or someone you know) in the past. Also please contact me if you are willing and able to help. I definitely plan to have a raffle. I also plan to contact Rosie.

Thank you!!!

I hate being sick

Posted by Kat in Batten, Kat, Life, Rachel

Over the past two days I have developed some kind of lung infection with a sore throat. Julie has a cough that keeps her up coughing at night. I need to feel better tomorrow. NEED TO.

We had a good Sunday, our only family day together ever week. Each kiddo took a bath and while Rachel (who was the last to go) had a good soak we talked a bit about her eyes. You’ll have to remember that she has made very few comments about the loss of her eyesight in these last months which was another symptom of something “major”going on with her.

She was about 20/40 at the beginning of this year, tested at 20/125 and 20/320 in May 2009, then tested at 20/400 (with lighthouse cards) in early June 2009, then 20/250 in July (but was inconsistent with her answers, as she had been at the other two eye places). Her “certificate of Blindness” is signed 7/13/09.

Her eyes have progressed welllllllllllll passed 20/200 in these last couple of months with substantial loss of peripheral vision. You can literally tell by looking at her eyes that they are substantially more affected than they were in early October.

Still can’t get over it. She was born and was normal! (There aren’t enough exclamation points to emphasize that point, in my opinion).

I had to remind her that glasses can’t fix her eyes and that there is nothing that we can do to make it better. I’ve talked about this with her a couple of times before. This time her reply was “You mean I’m gonna be a blind kid?” I said that yes, she is (trying not to let her hear me cry). She then became concerned that she was going to be alone. I reassured her that she won’t be alone and she became concerned that I am going to die, that Daddy will die and that Granny will die.

Then she asks me: “What if you and Daddy and Granny and Julie, London and Boston all die?”

I then went through all of the people that will help take care of her and she kind of mentally walked away from the topic to play in the bath water.

Tomorrow is another school / work week. Hopefully it will be a bit brighter.

Thank you

Posted by Kat in 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.

Saturday

Posted by Kat in 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.

Thursday

Posted by Kat in 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:

Wednesday

Posted by Kat in Boston, Rachel

Went out last night for a couple of drinks with a few friends and had a very good time. I definitely need to do that every week as it is really hard to fall deep into the horrible feelings that come with having a terminally ill child.

I was trying to explain to someone yesterday or the day before that I don’t treat Rachel like she is dying. I still give her a hard time, still throw her on the couch and tickle her until she begs for mercy and I still like to pretend that I forgot her name is Rachel (while I call her Julie). She is a good kid and is upstairs at this very minute babying Holly and playing with Boston in her room.

That boy, Boston, is a heart attack. My heart nearly stopped when I found out I was pregnant with him, and every time he jumps off the couch and especially the time when he took a header into the coffee table. He is wonderful and obnoxious and sure can eat. And more than being best friends with London, he is a therapist to Rachel. He doesn’t run off to be mean because she can’t see, he doesn’t call her stupid or say things like “you don’t even know what a battery is?”. All he wants to do is play with her, be loved by her and team up with her (mainly against me).

Boston Fenway – I am so glad that you are here on this earth.

Village Idol

Posted by Kat in 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.”

Back from New York

Posted by Kat in Batten, Rachel

First of all, Happy Belated THIRD Birthday to Boston and Happy EARLY 4th Birthday to London. My boys are awesome and I feel so lucky to watch them grow into little men, drive me crazy and eat my cookies. Here is to another crazy year, guys!

I took Rachel to the University of Rochester yesterday and today to be seen (and to see) Dr Mink and the Batten Disease Clinical Research team. I was lucky enough to have this short trip planned during the time that Heather and Tyler had planned so not only did we see the Batten Disease Experts but Rachel and I had the HONOR of meeting up with another Batten Disease child (Tyler) and his Mom! Meeting Tyler and Heather was a great experience for me and I feel so lucky to have had that opportunity and tie it in with going to the University of Rochester.

The gist of the meeting with Dr Mink is basically that Rachel is showing mild Batten Disease symptoms for a 9yo (which was AWESOME to hear) and that he feels her inattention (ADHD symptoms) is quite severe which is unusual for Juvenile Batten Disease. It is possible that her ADHD symptoms are actually separate from Batten Disease but regardless he suggests that we try her on a stimulant medication to try and reduce those symptoms. Reducing the ADHD symptoms could drastically improve her quality of life.

I’m all about quality of life!

Here is a link to the Batten Disease Clinical Research Center: http://dbb.urmc.rochester.edu/labs/pearce/bddcrc/index.htm

Hopefully the information that they gained from meeting Rachel and me will help them in their research and help other families that are facing the same disease that we are. Ultimately I (as would all of the other Batten parents) would love a cure and hopefully adding Rachel to the stack of data will get us one step closer.

We’re home!

Posted by Kat in 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!

http://www.facebook.com/pages/Rachel-von-Tungeln-LIVING-with-Juvenile-Batten-Disease/266424125552?ref=ts

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.