As told by Kat

My friend for life

// October 14th, 2009 // 1 Comment » // Batten // Batten

She better start reading my blog, damn it! This is Heather (Mom of Tyler who is 8 with JNCL) that I met during our trip to the University of Rochester Batten Disease Research Center. She is awesome, I love her and I will be a pimple on her nose for the rest of our lives!!!

Me and Heather in NY.

Wednesday

// October 14th, 2009 // 5 Comments » // Boston, Rachel // 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

// October 12th, 2009 // 1 Comment » // Batten, Business, Good Stuff, Humor, John, Julie, Kat, Life, London, Rachel, Vacation // 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

// October 9th, 2009 // 2 Comments » // Batten, Rachel // 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.

I remember some years ago

// October 1st, 2009 // 8 Comments » // Life // Life

I was in New Hampshire with Rachel spending the day with my dear friend and her family. One conversation that we had some years ago has been replaying in my mind over and over again but its funny because that conversation wasn’t anything “special” to me or her. As the winds of change seem to be blowing in my life I hear the conversation, I see it in my mind. My friend was talking about her children and the friends she has made with the other kid’s parents. The summary of the conversation was that you seem to make good, long-term friends with the parents of your oldest child’s friends and the subsequent children’s friends parents don’t usually become a big part of your life.

The parents that I became close with through Rachel have all moved on, keeping busy with their 4th graders and their activities. I have this beautiful 5 year old daughter and all of the parents of her friends seem to be taking the place of the friendships I should have through Rachel. I have to make the most of these new friendships and really make an effort because long after Rachel is gone I will have needed to invest in friends and life for Julie and the boys.

Its so sad. So unfair. So ridiculously depressing. I knew something wasn’t “right” with Rachel a long time ago. I just can’t believe it is this. Give me autism, give me an attachment disorder, give me bipolar disorder.

Just not this.

Lisa Gilbert

// September 28th, 2009 // 6 Comments » // Life // Life

Is a very talented photographer who has shared some of the pictures from our photo shoot with her a few weeks ago.

I LOVE them!

http://www.lisagilbertphotography.com/blog/?p=1003

We’re home!

// September 27th, 2009 // 1 Comment » // Batten, Birthday, Boston, Halloween, John, Julie, Kat, Life, London, Rachel // 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.

Cannot

// September 15th, 2009 // 7 Comments » // Batten, Boston, John, Julie, Kat, Life, London, Rachel // 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?

Venting

// September 13th, 2009 // 9 Comments » // Batten // Batten

I’m angry that despite my best efforts with my children, that there is a black cloud following us. We had a good weekend that was wrapped up with Rachel not being able to go to sleep for one reason or another (cause: dementia).

I cannot imagine believing in an omnipotent being. I cannot imagine believing in miracles. I cannot imagine believing in prayer. I cannot conceive that prayer actually does anything other than provide comfort to those that believe.

I don’t believe in prayer. I don’t believe in miracles.

I’m tired of having to recently deal with the vanishing act. People that don’t make any effort for several years to be in our lives… but the moment that something “bad” happens they come around and try to be a part of it (and possibly even shoving God down our throats). I won’t name names but the people who have invested in me, Rach, John and the other kiddos for a long time can easily guess that I am talking about the opposing coast.

I want to reaffirm my respect for people’s right to believe in God. I 110% support and respect your religious beliefs as I always have. This entry was to vent at all of the religion and “fast fixes” that people have been shoving down my throat recently. A multi level marketing scheme by the name of Glycol won’t fix her, neither will acupuncture, hypnosis or other crap.

And as much as I don’t subscribe to Christianity or another other mainstream faith I cry myself to sleep every night hoping that I will be reunited with Rachel after we both die. This can’t be my only chance to get to know her. The pragmatic side of me knows better than to believe in heaven but the emotional/mommy side really needs to know that I get another life to love my daughter who is clearly transforming from Rachel into a very angry, confused, blind and depressed child. Rachel doesn’t deserve this. Julie, London and Boston don’t deserve this.

I can deal with *ANYTHING* that happens but my children shouldn’t have to.

I might have to actually FLIP OUT on the next person who tells me they are praying for a miracle. This disease is ALWAYS, 100% fatal. Rather than pray for a miracle, donate to the BDSRA so they can help fund research. Science is Rachel’s only chance.

September 11, 2009

// September 11th, 2009 // 3 Comments » // Batten, Boston, John, Julie, Kat, Life, London // Batten, Boston, John, Julie, Kat, Life, London

Trash day was friday this week because of the holiday. Forever and always the best day of the week.

Rachel has been battling an uncomfortable itchiness and was sent home from school twice this week and was out on Thursday. Our primary care doc called in a prescription for “difulcan” and I’m hoping we’ve seen the last of this. Not fun for her and it has been interrupting her sleep. We NEED her to sleep. She needs her sleep.

We leave for Disney in a week and the kids are starting to get excited, Julie more than anyone! I am trying to gear them up for the limo, airplane and trip without causing Rachel any unnecessary anxiety. Our new “Neuro-Pharm” Dr aka Psychiatrist has given me some attavan to try in the event that we need it. The first two test runs produced no results but I will try a third time prior to the airplane so I have a trick up my sleeve if she “flips out.”

Julie started Kindergarten and her meltdowns have become frequent again but I am sure things will slow down once she gets back into the swing of things. The bus is so noisy in the morning that she now absolutely refuses to ride it so we have been driving her. She does take the bus home (more seats because the half-day Kindergarten students have already gone home). I think she likes her new teachers and all of her friends.

London started preschool and is enjoying it! That was totally expected because he has the same teacher that Julie had for the last 2 years! Mrs Menice made me smile when she told me that she sees a lot of Julie in London… I guess I never realized that they are so similar. Totally wonderful kid, I am happy that he has the opportunity to go to school!

A professional photographer, aka Lisa Gilbert, had the daunting task of taking our family pictures this past Wednesday. Professional family photographs was a first for our family of 6. Lisa was very nice and energetic and has a great personality, I was really impressed with how well she meshed with our chaos. I would imagine that she can mesh well with every family and that is part of what makes her good at what she does. Once we have some proofs I will be sure to share them here. This is her website: www.lisagilbertphotography.com

1 week until Disney. I hope Mickey is prepared for us. Maybe I should send them our mug shot before we head down there? Ha Ha. Maybe I should just sent a picture of Boston. He wore the “devil ears” during our photo shoot with Lisa, definitely a fitting head piece for him (he rocks!).

This is where we are staying: Give Kids the World. It is this amazing place for children with life threatening illness and is a treasure as much as Disney World is: http://www.gktw.org/

I HATE that we, as a family, are eligible for something like Make A Wish but SO GRATEFUL that these organizations (both M.A.W and G.K.T.W) exist. So, so grateful.

There are many people that I am grateful for. Mrs Petrocelli and Mrs Donato, Mary, my Mom, Ceil at Sunshine Pet for Holly, Amy at BDSRA for the information and Batten Disease DVDs, Lauren at Make A Wish for expediting Rachel’s wish to “beat” the blindness, Pamela in MN for the postcards, Auntie Janelle for the stickers, Ed for the VEGGIES, Xina for organizing the dRaffle, for Lauren and Mommaville for hiring Lisa to photograph my family, Uncle Adrian for coming to stay and being great to our kiddos.

The University of Rochester is having Rachel (and me) come for a clinical rating in October. I had originally planned to drive until I realized that the trip takes about 7 hours so we’ll fly out there instead. U of Rochester pays for the travel and one night in a hotel. I have managed to get Rachel’s appointment on the same day as my new Batten Mom friend (Heather) is bringing in her (also newly diagnosed) son. It will be nice to meet her and observe another JNCL child who is about the same age as Rachel. It is such a rarity to meet another JNCL child and I am grateful for the opportunity to meet him and his Mom.

The week after Disney and the week before University of Rochester we are having a birthday party for the boys turning 3 and 4. I can’t believe the boys are turning 3 and 4! Every occasion is being treated like this is the last one that Rachel will see. Long-term memories will be important down the road so I want to make them as best as they can be.

One day the memories will be all I have left of her. Such an extreme and almost intangible concept to process.

Canobie Lake Park

// September 6th, 2009 // 4 Comments » // Life // Life

My Mom wanted to do something nice for us and “sponsored” a trip to Canobie Lake Park.  My Mom said we could go anywhere but I figured doing a “test run” at a smaller amusement park would be good practice before our big trip to Disney in (LESS THAN!) 2 weeks.  It went really well, better than I expected and Rachel kept it together emotionally and didn’t complain of headaches on the way home.  She did mention her head hurting a few times while we were at the park but quickly went on to other things.  We did get her checked in to Guest Services at the Park and got a wrist band that allowed her to jump ANY line.  It worked pretty well, John went with her a few times on a few big kid rides and the little ones alternated with her and some rides they all went on together without waiting.

Here are the pictures, I put them in my new Picasa Gallery. Click on my little London Jack to see the pictures. I would have taken more but I left my camera behind, these are phone pics…

Canobie Lake Park 9/2009

dRaffle to Benefit Rachel

// September 6th, 2009 // 2 Comments » // Batten, Kat, Life, Rachel // Batten, Kat, Life, Rachel

Some of my Mom friends online have very kindly put together a dRaffle (think Drawing / Raffle) to benefit Rachel (my daughter who was recently diagnosed with Juvenile Batten Disease).  The tickets are $1.00 each and  are on sale until September 18th.  Here is the link if you wanted more information;

http://www.rachelsfriends.com/

The weekend

// September 5th, 2009 // 1 Comment » // Life // Life

I took a few minutes out of the morning and brought Rachel and Holly to Sunshine Pet and bought Holly some food and a new harness.  Holly was so excited to see Ceil and was trying so hard to get into the doggie area.  Such a sweet dog.

Ran a few errands and took the kids to the park and hit up the grocery store on the way home.  I made grilled cheese sandwiches for lunch and bought John the stuff to make salsa this afternoon and I’m making a new pasta with broccoli and chicken dish.  Hope its good.  Looking to take it easy today.

Enjoy the weekend!

Friday

// September 4th, 2009 // 4 Comments » // Batten, Boston, John, Julie, Kat, Life, London, Rachel // Batten, Boston, John, Julie, Kat, Life, London, Rachel

Just a little shout-out to yesterday, Thursday, which is the best day of the week.  Yay for Trash day!

Julie and Rachel are on their buses and off to school.  John sent me a picture this morning to let me know that the convenience store across the street now has a BLOCKBUSTER VIDEO MACHINE inside.  I need to get a bit of work done today so the boys are getting ready to go and rent a movie.  So exciting!  I love when things get easier.

John, Rachel, Julie, London and Boston don’t know this yet but I am taking them to Canobe Lake Park on Sunday.  My Mom is sponsoring the trip and I am looking forward to a good day with them.  :)

Please don’t forget to spread the word about Rachel’s Facebook Page: <click here for Rachel’s Facebook Page>

Have a great long weekend, everyone.  We are going to be closed on Monday and are going to have 2 straight days together!!!  Just about 2 weeks until we go to Disney World and Give Kids the World because of the kindness of Make a Wish and all of those people who fundraise, volunteer and donate money to them!  :)

Kindergarten!!

// September 3rd, 2009 // 1 Comment » // Life // Life

Julie has started!