As told by Kat

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!

Normal day

// September 1st, 2009 // 2 Comments » // Batten, Boston, John, Julie, Kat, London, Rachel // Batten, Boston, John, Julie, Kat, London, Rachel

Rachel was really normal today.  I know that was partially because I didn’t see her all day but before her first day of school she was pretty good getting ready and after school all the way until bedtime she was pretty manageable.

When your daughter with a rare and fatal disease has a normal day you wonder if the bloodwork somehow got screwed up at the lab.  And then you realize how crazy that sounds and question your sanity.  She has the symptoms.  She is going blind.  She has this disease.

In happier news we are going to Disneyland due to the absolute kindness of Make a Wish!

And… JULIEBEAN starts Kindergarten tomorrow!!

October 2001

// August 30th, 2009 // 3 Comments » // Rachel // Rachel

I wrote this to Rachel and the paper with board backing (twas the cover of a sracp book)  I wrote it on is falling apart.  I’m typing it out here to remember it.  In October 2001 I was a single Mom, divorcing from my first husband and had not yet met my John.

Dearest Rachel,

I completed this book for you on October 1, 2001.  I want you to k now that I love you more than anyone or anything in this world.  You are a brilliant little girl who will thrive and succeed at anything you choose to do.  Just don’t grow up too quickly, I’m enjoying every moment of your childhood.

Love, hugs & kisses,

Your Mum XOXO

This

// August 29th, 2009 // 8 Comments » // Life // Life

is a nightmare.

Thursday

// August 27th, 2009 // 1 Comment » // Batten, Boston, Julie, Kat, Life, London, Rachel, School // Batten, Boston, Julie, Kat, Life, London, Rachel, School

Best day of the week!

We’ve had a small virus passing through our kidlets starting with Boston and London and yesterday moved to Julie and Rachel.  Fever, lethargy and all gone after a good night of sleep.  Rachel had a headache yesterday and then got a fever and went to bed before 6pm and still (at 715am) has gotten out of bed yet.  Julie fell asleep at 7pm with a newly acquired fever and has been up for at least an hour.   Now I am sitting here wondering if Rachel has had this virus for longer because of her disease?  I made chocolate chip cookies for the kiddos and a pot of tea to try and make her headache go away (caffeine) but she wouldn’t touch any of it.  London was thrilled for the cookies.

Holly is doing well.  Its still a learning experience having a dog.  Knowing how much to feed her and at what times, knowing when to take her to the bathroom.  Seriously like bringing a new baby home from the hospital.

Julie is excited for Mrs Buttkus, who I assure her, DOES NOT kiss butts.  I am sure that her new teacher has heard all of the butt and butt kissing jokes before and has a way to get them to stop.  LOL  I am going to probably cry my eyes out putting her on the bus (which I am not sure I want to do but am going to do anyways because I have to let go a little bit in order to be the best possible Mom to all 4 children).

London is so excited to start preschool.  He is going half days and will have the same preschool teacher that Julie had for the last 2 years,  He is going to LOVE it!

Boston who flipped out for several days about a Buzz Lightyear toy which I finally bought him, has since played with Buzz in the sandbox and then “washed” him when I wasn’t looking.  Guess which newly acquired Buzz Lightyear toy doesn’t work anymore?  So annoyed.  Hopefully we’ll get some sound back because he is, as you guessed, is flipping out again.  I should laugh more about this or he’ll drive me nuts all on his 2-year-old-cuteness own.

Woke up to a fantastically chilly morning today.  Perfect way to start the best day of the week.  Now to get the trash out…

We’ll see

// August 26th, 2009 // 11 Comments » // Batten, Kat, Rachel // Batten, Kat, Rachel

It is apparent to me that people who need sleep the most get it the least.  Either my mind keeps me up or I am too stubborn and need to finish just one more thing.  Just.   One.   More.   Thing.

John and I fell asleep early tonight and at 3am I shot out of bed and ran downstairs to take Holly out to walk.  What a good girl! No accidents!

Yesterday I had an intake meeting with Pediatric Palliative Care and I requested a Personal Care Attendant (PCA) to help Rachel get ready in the morning because we have had such a difficult time getting her ready in the morning for YEARS.  They also have a volunteer program and we talked about having someone come into the house and help me with the children while John is working at the shop on Saturdays.  We’ll see…

Today (Wednesday) I have an intake appointment for Rachel with Psychiatry at Childrens Hospital to discuss Rachel’s medical situation and behaviors at home and see what type of medication we can give her that won’t lower her seizure threshold but will take the edge off of her extreme moods (without making her a zombie).  Let’s hope this is possible, I kind of feel like I am asking for something that can’t happen.  We’ll see…

Tomorrow Night (Thursday) we have two volunteers from Make-A-Wish coming to talk with the 6 of us about Rachel and her wish.  They know about her cognition and attention issues but I’m hoping she will have a good night and be clear.  She often has heightened anxiety when in new places or with new people and can become extremely silly.  We’ll see.

All of this is such a surreal experience, like a really bad dream.  The weather yesterday felt very cool and I briefly forgot about the diagnosis and felt so good and hopeful about all of the fun shit we’re going to do this fall and winter.  I quickly slapped myself into place and felt really down.  I think the funny part about this is I sound really unhappy and miserable, maybe like I am not getting out of bed or showering.  I WISH.  I don’t really wish but I’m certainly not letting this stop me from being alive and participating both as a person and mother.  Just about all of the horrible crap I am feeling is contained within, while I am hanging out with the children, making dinner or running errands.  Except for the occasional set of puffy eyes, you would never know if you saw me out with the kiddos.

Julie had a meltdown while Palliative Care was here.  It started off as she needed my help playing Webkinz every 60 seconds but then it progressed to where she was crying over tasks on that game I know she can complete.  I had to send her up to her room so should could have a good flipout and Annie (the social worker from Palliative Care) suggested that Julie was feeling upset and was simply seeking my attention.  It never dawned on me before but it makes complete sense.

Boston WONT STOP asking (well beyond the point of obsessing) about a Buzz Lightyear toy so I caved a few days ago and bought it on amazon.  It comes today.  Hopefully he will be satisfied for a few days and will stop following me around in misery while carrying his HTML printout of the toy and description.  Fingers crossed because that sucks the life out of me.  Maybe he is seeking my attention as well?  I kind of think that he is just being a regular 2 year old and DRIVING ME CRAZY!

The boys have their birthdays coming up and I am trying to decide whether to do a “movie theatre party” at the  new cinema that opened up at the Hingham Shipyard or to have a home-grown party and invite everyone.  If I can have my Mom on Rachel duty (which I am sure she is reading right now and saying “yes, of course”) I am leading toward the joint, home-party.  Any suggestions?

I have been really, unimaginably lucky to have found the BDSRA and all of the kind parents that belong.  The amount of information I have received from them has been tremendously helpful and the only saving grace of this shit diagnosis that I still have a hard time accepting for my Rachel.

If you’re reading my blog, leave a comment and say hello.  If you want to trade blog links, be sure to let me know.

I turn 31 today

// August 24th, 2009 // 8 Comments » // Batten, Kat, Life, Rachel // Batten, Kat, Life, Rachel

It doesn’t feel much like a happy birthday.  The crap I was pondering last night in regards to Rachel, the disease, school, etc is really, really depressing.  I guess moving past the constant crying stage moves you into the anger and depression stage.

This is a nightmare.