The world seems to be spinning faster and faster

Posted by Kat in Life

Rachel had another grand Mal on friday night, this one was while we were at a carnival sitting in the very top of the zipper. We were tipping back and forth as they were loading people into each car waiting for the ride to start and she starts convulsing. John was on another ride with Julie, London and Boston on the other side of carnival. I got Rachel away from the cage so she wouldn’t bang her head and started yelling down to the short line “Stop the ride, stop the ride, my daughter is having a seizure.”. Not sure if the ride attendants understood me but someone got them to stop and bring our cage back to the ground so I could get out with her as she was still seizing. When we got down I looked at the most capable teenagers to go to John (good thing he was wearing a lot of lime green and is really tall so is easy to recognize) and he came running.

She went home with John and I stayed with Julie and the boys and after my adrenaline stopped pumping we finished out the night on a good note. It is very important to me that Julie, London and Boston keep as normal as possible and not let Rachel’s disease overshadow every childhood memory.

The best part is that we all went back on Saturday night and Rachel and Julie both rode the zipper… TWICE!

What do people live for?

Posted by Kat in Boston, Bucket List, John, Julie, Kat, Life, London, Rachel

I took Rachel to her second appointment with her Pediatric Rheumatologist and he confirmed that she does have Lupus, also known as Systemic Lupus Erthymisomethingerother. How does a beautiful and innocent kid end up getting an early death sentence and Lupus? It’s total bullshit and rather than be angry this fine evening I would like to pose a question to those folks that still read my infrequently updated blog.

What do you live for?

Last year I spent many weeks living to cross off items on the bucket list, taking my kids to multiple states and experiencing all sorts of awesome things. Already this year I have driven my kids down to Florida and back and watched the miracle of Rachel and London learn how to swim. I have personally been living to make sure Rachel, Julie, London and Boston get to experience countless amazing days while Rachel can.

This year I want to continue this mission but maybe travel a little less (my new Jeep is not so good with gas mileage). Planning on sharpening our camping skills, maybe get a little sailboat. I’m looking for more adventuring ideas, things to enjoy, experience, to LIVE for.

So… What do you live for?

Took this picture of Rachel today. We clocked 100 miles with the Jeep’s top down today. It was 65 degrees in Massachusetts today!

January

Posted by Kat in Batten Disease, Blog, Bucket List, Julie, Kat, Life, London, Medical, Memories, Rachel

Can’t believe it is January already. Julie started back to school a few weeks ago and is enjoying her social time, she is pretty overwhelmed with all of the stimulation but loves it overall. London is starting preschool next week in the afternoons and they are all going to Art Class again starting later this week. Rachel was kind of upset about not going but I said she could bring some art supplies to Granny’s house as she’ll be at my Mom’s for her regular Friday night “date.”

Rachel is about 95% blind. The other five includes a spec of vision (literally a spec) out of the corner of at least one eye and she still has “light / dark perception.” She is handling it as well as can be expected. I try to get her to talk about it and how she feels and if she has any questions about her blindness and for the most part she is as happy as can be expected (and then some). She and I are going to the University of Rochester next week for a “check in” to see how she is progressing as they are studying Juvenile Batten Disease. My fingers are crossed that her disease progression is considered slow for her age.

The day after we get back from NY I am driving down the kids to Florida to stay the week in a house I booked months ago with a heated pool in the back porch. One day at Disney (can’t believe how expensive it is) and another day at a petting zoo. Our Make a Wish trip was amazing yet difficult for Rachel (and us) because she wasn’t on the right medication and it was so hot. My brother, nephews and my other brother and his girlfriend (Andrea) are all planning on coming down during the week.

Get out your fine china (I am so not fancy enough to ever own any). Wear your best jewelry (I made Julie wear her new princess earrings today despite her fear she might lose them) and treat this day like its your last on earth. Some days are harder than others but you should try every single day.

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?