Back in the saddle

Posted by Kat in Batten Disease, Kat, Life, Rachel

Another preschool mom came up to me and said she had read my blog from when we were trying to figure out what was wrong with Rachel through getting her diagnosis and beyond.  And it dawned on me that I really haven’t blogged in a while and I really should.  I have a hard time with some of the comments I get from people when I post on Rachel’s Facebook page or even entries on here.  I’m 33 and I have 4 kids and I rock.  Sometimes people are going to say stuff that rubs me the wrong way and I need to learn to ignore them and remind myself that they’re only trying to be supportive.  Its hard to know what the “right thing” is to say.

So last week I took Rachel in for an EKG at Mass Gen. (an amazing hospital in Boston) and got the results this week that her heart is fine. Her Neuro (Dr Sims) requested that Rach get the EKG to make sure her meds aren’t affecting her heart.  Later on in the same day of her EKG I took her to Spaulding Rehab hospital and got Rachel fitted for her first wheelchair.

I remember a time about 3 years ago when I was hoping and hoping that my child was “just” going blind.  And now we have a wheelchair soon to be added to the family.  It’ll be pink with wheels that we won’t need to maintain and folds up.  Rachel is having a harder time with balance while standing and walking and her knees are turning a bit more inward while she does this shuffle walk.  When I walk with her we go arm in arm or I put my arm around her.  She is also having a harder time with the stairs and getting into the jeep and her school 13 passenger van.

Her appetite is very healthy.  She takes seizure meds 3 times a day, just started on Abilify (an anti-psychotic) in the AM to help with her agression and at night she takes Klonopin and Prozac.  Kids with her type of Batten Disease struggle with sleeping as a part of the disease and dementia so we kind of have to load her up at night to get her to fall asleep (and give her a book on tape to listen to).

This disease sucks and there isn’t a damn thing I can do about it other than just be as supportive of her as possible, make sure her school stuff is appropriate and ever changing and keep on top of her meds.  I also try to remind myself daily that when she tells me for the 5th time in a day that she hates me or that I am a bad mother its just the monster of a disease living inside her head.  I’d give up everything I own to spend a single day with a normal version of my child just to see how badass she would be.  She is amazing in her own way now but I’d love to see a non diseased version of my child.  She’d probably kick my ass and make me make her 10 batches of cookies.  Just a daydream….

I’m also in the process of trying to sort out our living arrangements.  We have a 4 story house (3 sets of stairs inside) and a flight of stairs to get to our front door.  She won’t be able to navigate it forever so I need to come up with a solution to this before it becomes too dangerous.  A ramp? Maybe… but we don’t have the room in our first floor to put a bedroom and handicap accessible toilet with a separate living area from the “normal” kids once her brain becomes more affected.

Between work and the kids my marriage is kind of in the toilet right now but I’m hoping we’ll just hold on until it gets better because we just don’t have the time for each other that we should.  Divorce rates amongst batten families are very high and its a fact I ignore and hope for the best.

Thanks for reading if you’re still reading.   My other  3 kids are healthy and happy, albeit always asking for stuff and eating me out of house and home.  I hear that is what normal is.  :)

Photo:  Last weekend we went to the Drowned Hogs swim (in the Atlantic Ocean) to raise money for our local community service organization that helps families in our town with computer training, legal advice, food, thrift shop and book store.  John, Julie and I swam while my parents and Sue kept the kids.  It was such a cool experience.  Julie went as a vampire, I was the devil and I dressed John up as Redfoo from LMFAO.

A long overdue thank you

Posted by Kat in Life

I just sent this little letter into the Hull Times and I can’t wait to see it published:

Dear Santa Clause (by Rachel)

Posted by Kat in Batten Disease, Life, Rachel

I would like an American Girl Doll and clothes.
Sewing needle plus wool to make things.
A new collar and leash for Boise.
For my sister, Julie Bean she would like a Mrs Clause outfit because she is a girl.
New clothes for my brothers London and Boston.
Make-up for Rachel and Mommy.
Aftershave for Daddy John
New shoes for Granddad
New bed for Great Granny which is comfortable and easy to get out of.
New pair of shoes for Auntie Dorothy and Great Granny.
New pair of eyes for Rachel Eileen von Tungeln.
Rachel wants a new guitar.

Love Rachel

PS  I have been a good girl all year.

(Can’t stop crying reading the part about new eyes)

Rachel Scarecrow Story

Posted by Kat in Life, Rachel

I had a scarecrow and his name was Joe.  One day Joe came to life!  He was a real scarecrow person.  Joe met another scarecrow person named Sophie.  Joe and Sophie both wanted to walk to the ocean.

They called up a Limosine Service to pick them up at the farm.  Farmer Dave gave them some money for their trip.  It took them 3 weeks to get to the beach.  They ran and ran and jumped into the water.  Then a whale ate them.

Rachel made this story up on 10/6/11.  Thanks Tami for writing it down.

Back on track

Posted by Kat in Batten Disease, Life

Rachel is back on prozac and I am seeing more and more of my “normal” Rachel.  She was very difficult at home during her break from that medication and I don’t intend to ever take her off of it again.  Rachel won the Fireman Poster Contest for the entire 5th grade because of the poster that she and Tami made… and won a trip for me and her to ride the fire truck to school.  Fireman Evans was pretty sympathetic to her diagnosis and picked us up first.

Featured Photo:  The picture is me with all of the other winners, Fireman Evans with his daughter Hannah and Rachel (far left, obviously lol).  :)

JJ and Mel

Posted by Kat in Batten Disease, Friends, Kat, Life

My dear BFFs from Colorado Springs sent me a beautiful margarita bouquet yesterday to cheer me up from the rough day I had last week.  I found out that Rachel is #9 of 30 in the study for Cellcept and that the earliest she would be able to start is late spring.  It was expected to everyone but me, I had unacceptable high hopes and felt really let down afterwards (my own fault).  This is what the card said from my dear friends (I’m writing it here to keep for forever and maybe to steal and reuse on someone else one day):

We may be separated by miles,

but your tears do not fall alone.

Your triumphs we celebrate,

Your grief we share,

We love you. xoxxoxoo

- JJ & Melissa

My race speech

Posted by Kat in Batten Disease, Life, road race

I am reposting the “jist” of my speech here for those that were too far to attend or those folks who came but were way in the back…. Here goes:

Hello everyone.

My name is Kat von Tungeln and I am the proud Mother of 4 really great kids:  Rachel is 11 and Julie London and Boston are 7 6 and 5.

About 3 years ago,  and truly looking further back it was  longer than that we noticed Rachel having difficulty with her short term memory and simple activities like planning out getting ready in the morning for kindergarten.  After all was said and done and her symptoms progressed to crazy vision loss (I mean try to imagine your child going blind – it was truly an out of body experience for me) I finally got confirmation of  her terminal diagnosis of Juvenile Batten Disease.  In the most basic terms one of her chromosomes is a teeny teeny tiny bit shorter than it should be and as a result she has the inability to dispose of excess waste material in her brain and is causing Central Nervous System and Brain cell death.  I won’t talk about down the road as my kids are only a few feet away from me but feel free to ask me any questions after you have done the race or email me at any time.

I am so grateful to the International Brotherhood of Electrical Workers Local #104 and to Clean Harbors for becoming Platinum sponsors.  Julie Miller and Liza Collier really came through and thank you to our silver sponsors,  your support has made all of the difference in our success.

I was expecting maybe 100, 125 to register and we have tremendously exceeded the 300 registrant mark.

Thank you to my Mom, Tami and Becca Petrocelli, Janet Donato,  Mary Johnson, Briana Johnson (with one N), Sam Walsh, Jen Duran and my niece Haley for being a tremendous Assistant Race Director.  Thank you to my brother Adrian and dad Andrew for helping get things set-up today, could not have done this without you!

I also want to thank my husband and kids for dealing with my insanity and absence these past couple of weeks. You guys mean the world to me.

This is a very nasty and unforgiving disease and I urge you to research it and share it with your friends because people like me and the families I am about to introduce to you are relying on private fundraisers to give the scientists the funds they need to help kids like Rachel.  At this time I would like to introduce my local Batten Family and then we’ll be ready to roll with this race.

And if any of you Hull parents want me to have an easy to understand, kid friendly talk with your kids…. You know how to reach me.

Thank you so much for joining us today on our journey.

I am forever grateful.  Now lets get ready to go.  On your mark, get set  <AIR HORN>

Here is a picture of Rachel and Granny after the race:

UNBELIEVABLE!!!!!!!

Posted by Kat in Batten Disease, Life

I am tallying the results this morning after passing out from exhaustion yesterday afternoon and we’ve raised over $20,000 with approximately 350 participants.

As a mother I want to sit and hug each and every one of you with tears streaming down my face. I know this won’t save my daughter but this is exactly what this rare genetic brain disease needs to bring some publicity to it and give the scientists the much needed funding they require to do experiments that will one day save these kids.

As a member of Hull’s community I want to say how proud I am for all of you coming out yesterday: either as a walker, runner, volunteer, sponsor or donor.

I am extremely grateful for everyone’s help, support and hugs. It only took 33 years to turn me into a hugger but I’m not going back

Thank you especially to my Mom and Dad, my brother Adrian for being my delivery man, to the Durans, the Johnsons, the Donatos the Petrocellis, Sam Walsh, Sue Bradley, Lulu, The St Gelais + Kelsey, the Rotary Club, Steve, Cath Morgan, Walter and The Broders. Extra special thanks to my neiece Haley. She is simply another version of myself and got all TYPE A on this race. Without our race committee this race would never have caught on fire like it did. And Liza Collier of Clean Harbors and Julie Miller of IBEW 104 for getting me the Platinum Sponsorships!!!!!!

THANK YOU SO MUCH!

And thank you to my Batten Family for coming out and showing the Town of Hull that Rachel is not the only one!

Getting ready for my speech

Posted by Kat in Batten Disease, Life, road race

I plan to thank Clean Harbors and the IBEW #104 as well as my totally badass Team and everyone for coming out and donating.  And briefly (since I have been told to be brief but precise) I will be mentioning and honoring all of the families who are joining us for race day and the boy in the next town over who died of Juvenile in 2002.  Here is the list:

Curran:  Fran & Glen of Duxbury with college age twin daughters who are unaffected and 19yo Nick (JNCL)

Daniak:  Heather and Chris of NH with son Nicholas (LINCL) and William (unaffected). 

Habershaw:  Preston Habershaw passed away from JNCL in 2002 in Hingham, MA (next town over)

Landers:  Nancy and Zia Landers of Cape Cod.  Nancy’s son (Zia’s brother) Barry passed away (LINCL) in 1998.

Montville:  Jessica of Worcester with daughter Nora (LINCL) and unaffected children Thomas & Alicia. 

Stancombe:  Maria & Mike of NH with affected (LINCL) daughter Gracie and son William died in 2010.

St Gelais:  Moe & Jean of Westport MA with daughters Kelsey & Kate, affected (JNCL) sons Jordan and Zachary.

von Tungeln:  Kat and John of Hull with children Julie, London and Boston and affected (JNCL) Rachel (11).

Alice’s Attic becomes a Silver Sponsor!

Posted by Kat in Life

Thank you so much to Laura and Jillian for sponsoring Team Rachel through Alice’s Attic.  We love you guys and appreciate your support of our family and of this race.  :)

Website: http://alicesatticonline.blogspot.com/

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!

Lynda’s Restaurant Sponsors Team Rachel

Posted by Kat in Life, Sponsors

Thank you to the folks (Jim, Denise and Joseph Jr) at Lynda’s for sponsoring Team Rachel and they are also donating a percentage of their week’s profits from the race week to our cause.  Thank you so much for supporting us!!

Website: www.lyndasrestaurant.com

IBEW Local 104 becomes a Platinum Sponsor!

Posted by Kat in Life, Sponsors

Thank you so much Julie Miller for getting the IBEW Local 104 to become a silver sponsor of Team Rachel!  You have helped us reach our $10,000 goal!  Thank you!!!

Website: http://www.ibew104.org/

Kaplan Painting Sponsors Team Rachel

Posted by Kat in Batten Disease, Life, Sponsors

Thank you to Kaplan Painting of Hull, MA for becoming a Silver Sponsor of Team Rachel.  We live on the same street, wave at least 10 times a day and are so grateful for your support.  See you on race day!!!

Website: www.kaplanpainting.com

Alma Nove – Our 11th Sponsor

Posted by Kat in Life

Thank you to Alma Nove, Chef Paul Wahlberg’s signature restaurant and bar inspired by the creative blend of Italian and Mediterranean cuisine for becoming our 11th sponsor.

Website: www.almanovehingham.com