As told by Kat

I forgot to update

// March 5th, 2010 // 1 Comment » // Batten, Rachel

Rachel did not have the surgery yesterday, I cancelled it at the last minute. I couldn’t get over how terrifying the post-operative clean out appointments would be for her. I tried very hard to get her a Valium type medication from both the ENT and our Primary Care but no one felt comfortable prescribing that for her because they are respiratory suppressants.

I am going to push harder for her to use saline and maybe try to pin her down and use a neti pot on her. Increase the humidity in her room and possible get her a hospital type bed so she can sleep reclined. I’m also going to see if I can get our primary care Dr. to prescribe a steroid nasal spray to try and decrease her inflammation that way.

I have no doubt that the end result of the surgery would have improved her breathing but I couldn’t get past how terrified she would be every time I took her back for another “clean-out.”

I feel good about my decision.

My brother comes out to visit next week…. better get the lobster order ready.

I promise a “non Rachel & Batten disease” entry later today.

Depressed

// March 4th, 2010 // 2 Comments » // Batten, Rachel

Rachel seems to be going into a depression. Who wouldn’t be depressed, losing your vision, losing your mind.

I’m looking at old pictures of her, seeing that smile, trying to remember what the BEFORE was like.

This just can’t be real.

Well

// March 2nd, 2010 // 6 Comments » // Batten, Kat

I pretty much feel dead inside tonight and I thought I’d be nice and share it all with everyone who reads my blog. John went last week and got tested to make sure he isn’t a carrier of Batten Disease. Those results should be back in another week or 2.

I’m terrified, literally terrified that he will come back as a carrier for JNCL. Its really unlikely, but I have heard of this happening before in a conversation with another Batten Parent who got their diagnosis some years before we did.

My healthy other 3 children can sometimes be what get me out of bed in the morning, knowing that they have this future I must look forward to. I can’t imagine what it is like for the other parents that have multiple children affected. Please don’t ever let me find out what that is like.

I know my mind is getting the best of me tonight. I’m going to bed.

Oh and Rachel is having sinus surgery on Thursday. I haven’t told her. It is going to be hell. A more acutely experienced hell than normal. I’d like to add some curse words at this time but I don’t want to lose my most regular commenter (my Mom). So assume I said them so I can temporarily feel better.

‘Nite

I

// February 11th, 2010 // 1 Comment » // Batten, Kat, Life, Valentines

I am working on accepting that some children’t won’t grow up and live happily ever after. It is a concept that I live with and I struggle with every single day.

My children don’t see the struggle. This evening for dinner they had their choice of Mommy’s homemade chili or an unlimited supply of cupcakes. 3 / 4 chose cupcakes and my Julie chose both.

Who doesn’t love cupcakes? (Answer: No one I care to know).

Some kids don’t have a “happily ever after.” They just don’t.

The Bucket List

// 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>

Trip to Maine

// 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).

Depressing

// 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.

Another update

// 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.

Exhale

// 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!!!!!!!!!!!!!!!!!!!!!!!!!

Christmas week

// 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?

The week in review

// 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!

Word of the day

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

I hate being sick

// November 8th, 2009 // 4 Comments » // 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.

Can’t sleep

// November 7th, 2009 // 7 Comments » // Batten

Can’t get over this and what is in Rachel’s future.

Can’t get over how unfair this is for her.

Can’t get over how hard it must be for her.

Can barely breathe.

I just can’t believe this is happening.

Thank you

// 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.