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>

Nearly 33…

Posted by Kat in Batten Disease, Life

I turn 33 next week which means the summer is almost over (woohoo).  Next summer I plan to drive the kids from the east coast to the west coast so we can see the pacific ocean!  My birthday wish is to raise a little  bit of money for the Batten Disease Support & Research Association so if you have an extra $10 to spare please consider giving to them.  Here is the link (and thank you!):  http://wishes.causes.com/wishes/352172

Meet John

Posted by Kat in John, Life

As punishment for being my husband and barely reading my blog, may I present to you this unflattering picture of him.

Meet Juliebean

Posted by Kat in Julie, Life

Meet Julie. She is a 7 year old 30 year old who is tremendously helpful and caring!

Meet London Jack

Posted by Kat in Life, London

He is nearly 6 and about to start Kindergarten. He is really excited!

Meet Boston Fenway

Posted by Kat in Boston, Life

He is my baby who is about to go to preschool full-time.

The Longest Summer

Posted by Kat in Life, School, Summer

I’ve been a Mom for 11 years now. 2 summers ago I spent a lot of it with Rachel at Children’s Hospital Boston and / or at a variety of doctors. Last summer me and the kids took a month long road trip visiting a pile of amazing places and doing other road trips. This summer we have spent a lot of it at home having days at the pool but not really doing a lot but having simply fun.

Simple fun apparently sucks! This has been the most difficult summer of my mothering career and it is a summer I plan to avenge. I know some of the difficulty surrounds Batten Disease and behavioral issues that trickle down to my other 3 children but that can only take so much blame.

School goes back in 3 weeks. All 4 of my children will be in school full-time! Rachel is going into 5th grade, Julie is going into 2nd (with the best teacher ever), London will be starting Kindergarten and Boston will be at Montessori Preschool. I have promised them their own individual “back to school” shopping trips for some new clothes / shoes and supplies. They are so excited for their turn to go shopping with “just Mommy” as opposed to dragging everyone along.

So the Back-To-School Countdown begins!

The trial begins!

Posted by Kat in Batten Disease, Life

The Juvenile Batten Disease’s one and only trial that they have been talking about for years has finally been opened! I found it yesterday at clinicaltrials.gov and have emailed the folks running the trial to ask them to consider Rachel. It will require that she takes a liquid medicine everyday as well as ranitidine (antacid). The trial is double blind which means she will definitely get the medicine but she will also get the placebo. We’ll have to visit New York 4 times and work with Dr Sims (Rachel’s Neurologist at Mass General) for clinical evaluations. This won’t be a cure but if this medicine does anything, and is safe then maybe we’ll be able to get her this medicine after the trial. Here’s hoping!

Trial Medication: CellCept (Mycophenolate Mofetil)

What does this medication do? It’s an immunosuppressant

Side Effects: See also Warnings section.Constipation, nausea, headache, diarrhea, vomiting, stomach upset, loss of appetite, gas, tremor, or trouble sleeping may occur. If any of these effects persist or worsen, notify your doctor or pharmacist promptly.Remember that your doctor has prescribed this medication because he or she has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.Because this medication weakens your immune system, you are more likely to develop infections which may be serious. Tell your doctor immediately if you develop any of the following signs of infection: persistent sore throat/fever, night sweats, flu-like symptoms, painful urination, vision changes, a sore or wound on the skin that feels warm/tender/painful and appears reddened.This drug increases the risk of a rare and possibly fatal brain infection (PML – progressive multifocal leukoencephalopathy). Tell your doctor right away if you have any of these symptoms: clumsiness, sudden change in your thinking (such as confusion, difficulty concentrating), difficulty moving muscles, seizure, difficulty speaking.Tell your doctor immediately if any of these unlikely but serious side effects occur: unusual tiredness, fast/irregular heartbeat, muscle weakness, easy bleeding/bruising, swelling of the feet or ankles, mental/mood changes, weakness on one side of the body, unusual change in the amount of urine.Seek immediate medical attention if any of these rare but very serious side effects occur: chest pain, stomach/abdominal pain, black/tarry stools, vomit that looks like coffee grounds, shortness of breath/rapid breathing.A very serious allergic reaction to this drug is unlikely, but seek immediate medical attention if it occurs. Symptoms of a serious allergic reaction may include: rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, trouble breathing.This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.In the US -Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.In Canada – Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.

Warnings: Mycophenolate decreases the body’s ability to protect against illness and infection, and may also increase your risk of developing lymph node tumors (lymphoma) and other types of tumors. Therefore, this medication must only be given under close medical supervision. Discuss the risks of taking mycophenolate with your doctor. Notify your doctor immediately if you develop signs of infection (such as persistent sore throat or fever) or other symptoms such as weight loss, night sweats, enlarged lymph nodes, or skin growths.

Seizure #5

Posted by Kat in Life, Seizure

Rachel has a Grand Mal today on Nantasket Beach under the care of my 16 and 15 year old neices who also had Julie, London, Boston and their little sister. The girls handled Rachel very well, Sarah took care of Rachel while Haley ran to the beach bag and grabbed the Diastat and “did what had to be done” with it. So, so impressed with them.

The seizure lasted about 2 minutes. (Just writing this down for documentation’s sake).

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. 

Nearly 2 years

Posted by Kat in Batten Disease, Boston, John, Julie, Kat, Life, London, Rachel

Tomorrow marks the 2 anniversary of Rachel being officially diagnosed.  Its been a hard 2 years but I’ve worked my ass off to make it as fun and as successful as this time could possibly be.  There are huge marks of decline in her but at the same time we can’t ignore her infectious laugh, her record setting appetite, her love for her Granny and dozens of other things that prove she has a good quality to her life.  That is all that this is about now: Quality of Life.

I have this ridiculously high in calorie cake that I plan to make tomorrow to mark the occasion.  (Chocolate Snowball Cake).  If I die from clogging my arteries with this delicious cake please note that I died happy. 

Here is a picture I took of  my A-Team last week which visiting our new shop in Weymouth (they never look at the camera at the same time, lol):

Rachel, Julie, London and Boston (July 2011)

This is a picture of the wonderful person (Sue) who took my husband and kids out on her boat last week.  We went to fascination and she won a WHEEL SPIN!!!  She might kill me for putting this picture on the blog but I wanted to give her an honorable mention because there are very few people in the world who are so giving and I really appreciate her in our lives.

Sue at Fascination

I took Rachel and the kids to the Hull Fire Station for their safety day.   Her siblings climbed up onto the Jetski (on trailer) and where they go, Rachel has to go  (my rule lol) so I heaved her up:

Rachel on the Hull Fire Jet Ski

Have a good day if you are reading this.  I’ll post another entry with more pictures tomorrow.

Geocaching

Posted by Kat in geocaching, Life

Oh my goodness, I am hooked! If you don’t know what geocaching is, there is this great website www.geocaching.com

What is geocaching: Geocaching is a real-world, outdoor treasure hunting game using GPS-enabled devices. Participants navigate to a specific set of GPS coordinates and then attempt to find the geocache (container) hidden at that location.

Yesterday I took John and the kids on an adventure. We finally have the third row seat in my jeep so we were able to go – all 6 of us together. First time since January. We went to 5 sites and found 4 of the caches. We traveled to beaches and woods and parks that we have never been to before. It was beautiful, the warm sun felt so good on our skin and it was great to get everyone some exercise.

Of the 4 caches we found, John found 3 of them. I was mostly on Rachel duty and ended up about 50 yards behind the rest of them but we didn’t give up. Cache #4 was found by Rachel at a very old cemetery in Cohasset, Massachusetts. We were on the site for about 15 minutes and just couldn’t find it (about to leave) and Rachel was hanging on to this old iron gate and feeling all around it and what did she find???! Yes!!! It was this micro cache (about the size of a fat watch battery) that unscrewed with a very teeny tiny paper scroll inside. It was magnetized and was “stuck” to this gate. Couldn’t have been a more perfect find. Leave it up to my beautiful blind daughter to find what all of us sighted folk couldn’t see. I am so proud of her, was a great boost to her self esteem.

I think I might steal the kids away from their summertime boredom to do more treasure hunting today because I am hooked. HOOKED! We are also going to make a few of our own caches and hide them nearby for others to find.