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.

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.

Rachel and her Mom

Posted by Kat in Kat, Life, Rachel

Rachel and I had a great day at Endless Summer talking up the crowd for our 10/23 road race!

Family, Life, Race Update

Posted by Kat in Batten Disease, Blog, Boston, Bucket List, John, Julie, Kat, Life, Rachel, Research

We are all back to school doing full-day classes. Rachel has settled into 5th Grade, Julie is loving 2nd grade, London can’t wait to go to Kindergarten everyday and Boston is enjoying going to full-day Montessori. Boston has been acting up A LOT so I am trying to find ways to give him 1:1 time and so far I’ve been taking him on morning bike rides before school. This morning we went to the beach because we had a few minutes to spare and looked for shells.

On my walk home from Julie and London’s bus stop I was lamenting to myself that getting all 4 kids ready for full-day school in the morning (NOW) is easier than it was when it was only Rachel who was going to school.  I can only imagine what the neighbors thought while Rachel screamed and cried and yelled and hit us while we got her dressed in the morning and that is pale in comparison to the intense sensory agony she was in while we brushed her hair, her teeth or put socks on her.  I am so grateful for medication and how it has given us intermittent good times with her at home and substantial amounts of good time at school, and with Granny or one on one with someone.

Tomorrow we are running our annual jewelry table for Endless Summer (www.endlesssummerhull.com) with a twist because this year we are seeking runners, walkers, volunteers and business sponsors as we are raising money for Juvenile Batten Disease.  The race planning is going very well and we are getting a lot of buzz around our town through all 3 schools, loads of residents and our customers.  I am very excited about this race, c’mon October 23rd!

We have put Rachel’s name in the hat for the trial and are waiting to hear as to when she can start  I have filled out all of the paperwork, caught her up on vaccinations and have gotten permission from Dr Sims (Rachel’s Neuro at Mass General) to be her local doctor.

Things we have planned?  Outside of the upcoming road race I have two pretty giant things in store for my kids.  A day or so after Christmas we are driving down to florida and renting a house with a pool and will spend our Winter break in the warmth.  :)

Next summer?  A few days after school lets out I am taking a complete cross country road trip with my cherubs going from Boston to California and back.  We are also planning to drive north for a few days and spend time with James and his family (James is Rachel’s biological father).  I know people have asked me why I would bother doing that as James has been out of her life (by his choice) for many years but really this isn’t about me or about him.  This is about the kids.  I think it will be great for my 4 children to meet their twin sisters.  Technically they are only Rachel’s half sisters but my motto has always been “You can’t have enough people love your kid.”  (Or in my case, kids).  I have a lot of planning to do for that but the details are all on hold until after this road race.

London especially wants to dip his toes in the Pacific ocean, his life’s dream is to live in California and drive a minivan.  I figure a good test drive through Cali could help him to see if this is something he wants to aspire to do or not.  Yeah, he is barely 6 but he talks about it everyday.

Life is too short to waste it waiting for things to happen so you can move forward.  Move forward, move things into place.  Make things happen.  Your life could be over tomorrow.  Live without a lot of regrets.  Live enthusiastically.  IF we only know, truly, how many people in this world, in America, in your own town, maybe even on your street, who are fighting for their lives…  Just thinking about it is very humbling to me.

- With love, Kat.

And school begins…

Posted by Kat in Julie, Life, London, Rachel, School

Rachel is in 5th, Julie is in 2nd and London is in Kindergarten!!!!!!

Today is a great day!

Posted by Kat in Batten Disease, Birthday, Fundraiser, Halloween, Julie, Kat, Life, London, Rachel

Today is my 33rd birthday so I imagine my Mom is once again thrilled that she is no longer pregnant with me (I am mostly joking lol).  I was born over 3 weeks late and weighed over 10 pounds so I really can’t blame her.  Thank  you Mom, aka Eileen, for giving me life and putting up with my crap, foul language and obnoxiousness all of these years.  :)

I also want to thank my Mom for being the first to sign-up for Rachel’s road race which is in 2 months.  I can’t say I was outright nervous that no one had signed up because I know people are really busy with “back to school” whether it is their child or grandchild headed back in the next few weeks.  I guess my Mom had mental telepathy and for that I am so grateful.  We have one official walker!!!!

This week I am finalizing the Sponsor information for businesses who can give over and beyond the registration fee as well as walkers and runners who will ask their friends to donate on their behalf.  Plus the prizes and raffle items.  I have never done this before and got some really great ideas at the Batten Disease Golf Tournament I took John and the girls to yesterday.  They raised about $30,000!!!!  My goal is to raise $10,000 this year for our walk/run/raffle.

Have a great day today if you read this entry!!!

Photo: Rachel (6), Kat (?) and Julie (2.5).  Not really easily seen is London who was 8 days past his due date and born the next day.  We were at a pumpkin patch in Colorado.

Xanax?

Posted by Kat in Batten Disease, Boston, Julie, London, Medical, Rachel

Taking Rachel into her Primary Care doctor this morning to talk about Xanax as a possible med to help Rachel with her anxiety and obsessive thoughts. Something to help her like Risperdal did but without the weight gain (WOW!).

This weekend I think we should take the kids to the beach. We live a block away from it but we never go because I hate the texture of the sand. Most people avoid swimming situations because they don’t like the way they look in a swim suit but frankly I don’t care about that. Its the sand that keeps me away.

12 more days unti school starts for Rachel and Julie!!!! 13 days for London and and 20 for Boston. London should get “THE” letter in the mail this week to let him know who he has for his Kindergarten teacher. So exciting!

Picture:  This was taken earlier in the summer when the pool looked like pea soup.  Nothing can keep Juliebean out of the water.

Childhood Dementia

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

I came across a guide I hadn’t seen before detailing what its like to be a child with dementia.  This one was written by a Niemann Pick Group (Niemann Pick is like Batten Disease except those affected kids can’t metabolize cholesterol) but is fully applicable to Batten Disease and the kids who are affected.

http://www.nnpdf.org/documents/ChildhoodDementias-US-FINALVERSION.pdf

Many “bad” behaviors from dementia have trickled down to my younger children and there is no guide to fixing those… Hopefully as the little ones get older and mature certain behaviors and attention seeking issues will sort themselves out. Their big sister, their role model is losing her mind and we can’t stop it.

Here is the dementia guide that the BDSRA put out.  <link to guide>

iCarly

Posted by Kat in Rachel

Rachel went to see iCarly (Miranda Cosgrove) in concert with her friend Anthony. Rachel had a great time and came home with a real concert t-shirt! Thank you to the Berlin family for being so generous and buying tickets so she could go! Pretty cool to cross off a bucket list item that you didn’t know you wanted to do until you were given the chance. Life is pretty amazing like that…

Rachel shooting more hoops!!!

Posted by Kat in Batten Disease, Rachel

Today

Posted by Kat in Batten Disease, Life, Rachel

Today we went blueberry picking with our wonderful cousin (my neice) Sarah. We all had a good time and then went to Mickey D’s and onto Bridgewater State so Sarah (going into her senior year) could look around.

Today was also another day that showed Rachel is becoming more aggressive. I need to do some “studies” and see if her aggression is because of being out of the house for too long or having her siblings around or…. I don’t know.

What I do know is that watching your child slowly lose her mind has to be just about the most painful experience in the world. People who think that everything happens for a reason are completely mistaken (I’m being “nice” in my terminology) or haven’t thoroughly thought that statement out and/or haven’t had to watch their child suffer extensively. There is no good reason to put a beautiful, intelligent and thoughtful little girl through this. If you take offense at this declaration then feel free to turn around and never visit this blog again. The next person who tells me that “everything happens for a reason” can expect an ass kicking.

Rachel shooting hoops!

Posted by Kat in Life, Rachel

This is Rachel with her summer school teacher shooting hoops!!! Turn your head sideways to watch.

September 2011

Posted by Kat in Batten Disease, Bucket List, geocaching, Kat, Life, Rachel

After the recent heat wave affecting New England we (me and the kids) have decided to postpone our summer road trip  until September.  Its a good excuse to blow off a little school but more importantly the weather will be more temperate and I won’t have to deal with 110 degrees while strolling through Hays, KS or Terre Haute, IN.  We stayed there last year in our travels and both were little slices of hell on earth.  Neither my kiddos nor their Mom do very well in such high heat.

We’ll be driving out to Colorado and hopefully staying at the Garden of the Gods Campground (they don’t close until September 30th) for about a week and hanging out with incredible friends (they can only be found in one place apparently lol).  We were considering staying a while but the Colorado Springs School for the Blind isn’t sure if they’ll be able to provide programming for Rachel.

We’ve generally traveled route 70 and took route 80 home once but we are open to suggestions.  Any good road stop ideas?  I really want to go to Niagra Falls this time around…. but I hate the drive through upstate New York.

Planning to go to the Flying W for dinner, Santas Workshop for some fun, Geocaching, then float down the Arkansas River and we (me) are looking to do some stock friendly trails with the Jeep while we are out there.  Every summer seems to be a little more difficult than the last so we have no choice but to take life by the horns……………..

Cinnamon Buns

Posted by Kat in Food, Kat, Life, Rachel

What started out as my plan to make a special cake for our 2-year diagnosis anniversary turned into a desperate need of Rachel’s to have cinnamon buns.  Since we are a family that likes to roll with whatever comes to us, I switched gears and made what the little angel wanted.    (Anyone get the Lilo and Stitch reference to “little angel?”).

Here is the recipe for Cinnabon Cinnamon Buns:

Cinnabon Cinnamon Buns

Yeah.  I know the picture is blurry but you have to imagine my life.  4 kids, 1 great niece  helping with my oldest and constant email/work requirements and everyone is grouchy because I hadn’t fed them dinner yet.