Tag: batten disease

rachIts 10:51 on Monday night and I haven’t been able to shake how much I miss Rachel since I shipped her off on the van yesterday afternoon at 4:00pm.  I pushed for her to go to this school (she has never wanted to leave home) and now I can’t stop missing her.

I have some big decisions to make.  I have an appointment tomorrow with the Pediatrician on staff at her new school to go over the MOLST form.  Grab a box of tissues and imagine filling it out for your formerly healthy baby girl:

http://www.molst-ma.org/sites/molst-ma.org/files/MOLST%20Form%20and%20Instructions%208.10.13%20FINAL.pdf

I want to pick her up and bring her home.  The last day has been the hardest since she left.  I left a voicemail for school at 7:20 tonight for her to call me but no luck.  I will definitely be bumping into her tomorrow. She drives me crazy but I love the shit out of her.  I usually get upset and just want my healthy daughter back but the last 48 hours has kind of been me realizing that I will take any version of her because sooner rather than later there will be NO version of her to have back.

This disease is relentless.

.

[dropcap] THE ROAD RACE  IS BACK!!! [/dropcap]

2014-03-14 17.09.45Last week I decided (after some gentle nudging from a few people that I love as much as the sun loves the moon) to hold a second road race for Batten Disease Research. The third Team Rachel Race 4 A Cure is being held on May 18th (which happens to be my Rachel’s 14th birthday) in Hull, Mass. We are looking for everyone to chip in either as a walker, runner, volunteer, sponsor or raffle donor.

Please get in touch with me if you can help at team@teamrachel.com – click here for more information on the road race!

We’ve raised a lot of money and donated it all to research and would love to see several thousands more!  I know this is a rare disease and it seems hopeless but research into Batten Disease also helps other brain / neurological diseases like Alzheimers, Parkinsons, Multiple Sclerosis, Huntingtons and so many other rarer diseases which you hopefully never have to learn about.

So excited! Road Race meetings are being held every Wednesday night at my house 8pm starting Wed, March  26th!  <3

2014-03-19 17.29.45Rachel’s new school is full of people just like me and just like you with feelings, hopes, laughs and tears.  Young people who have been admitted to the school because they were born with (most) a disability or acquired one along the way like my Rachel.  I know that some of the kids there will probably attend the school until age 22 and will transition into the mainstream world.  I also know that due to privacy the staff is unable to divulge medical information to the other children about Rachel or her fate.  When I am visiting, different kids will strike up a conversation and I have started to tell them things about Rachel.  That she was totally normal and healthy, reminding them (especially the boys) that she is only 13, that she is blind.

Last night I told two of the other students that Rachel is terminal and the name of her disease (Batten Disease) so that they could read more about Rachel.

There is a boy at her school who is truly amazing.  He can maneuver his iPad with his feet faster than I can with my fingers.  He took a liking to Rachel over the summer when she was in summer camp at her new school and I have reminded him, specifically, that she is only 13 and a MINOR.  🙂 Last night at the theme dinner I told him about her disease and that she is terminal.  I also told him about her website that you are reading right now.

I think its only fair that the kids who care about her know that the Rachel you see today is not the Rachel that will be in a few years (and not the Rachel that I knew when a few  years ago).  It is heartbreaking, but only fair to let the other students who are cognitively capable of grasping her disease know what is to come.

Reality sucks but I’d rather be a realist.  Living an honest, pragmatic life has made living with this stupid disease much more bearable for me.  The picture is from dinner last night.  I had to threaten her to get a smile because she was so busy letting me stuff her face with mashed potatoes, gravy and chicken. <3

2014-03-08 09.44.19-1I feel like I have turned into a caterpillar these last few weeks.

I feel silly using the caterpillar to butterfly analogy. Like, please don’t bring it up to my face, but it’s such a valid feeling for me.

The last few years of my life have focused on taking care of my oldest daughter (first) and her younger siblings (second). I have tried to take care of my husband but mostly gave that job up except for a bout of vertigo this year.

I have sat at the very bottom of the list for a very long time. I think that this is a common practice, for women (especially mothers) take care of everyone else before themselves.

It’s time to re-evaluate things and put myself at the top of the list.

2014-03-04 16.46.40Last week London hit his head really hard and was unable to remember the last few weeks and every 15 seconds was asking me the same questions over and over again.  What is today?  Why am I not at school? Where is Dad? What happened? Why am I bleeding?

He cut his lip open and was bleeding and the questions didn’t stop coming and his short term memory didn’t return so I drove him to the hospital.

I will never understand how I have the ability to stay completely calm but I am very glad that I am not one to “freak out” in an emergency.  I drove him to the emergency room for an exam because I wanted to make sure his brain was okay.  After a cat scan and a popsicle he was ruled to be okay and sent home.  Gradually that afternoon and evening his short term memory started working normally again (the biggest feeling of relief in my life).  Having one sick child does not protect your healthy kids from harm, disease, accidents. That would be fair but life doesn’t work that way.

Life is so short and London’s head injury brought everything I am working towards to the surface.  In some regards I have been putting off my morphing to keep my marriage together, to keep my house together, to keep my sanity together. How can you really live if you put off your life for other people?  Its time I stuff myself with leaves, wrap myself up, hang from my feet and wrap my body in silk.

Keep taking baby steps and before I know it, I will be myself again.  Myself, only happier and more wise.  I am so glad London is okay and that I no longer suffer from depersonalization and derealization.  Feeling outside of my body was very, very scary but if I “get it” again, I will look back at that time and know I survived it once and can survive it again.

 

sleeping_rachelI gave up working on the steps I am taking to a somewhat normal life during the last 7 or 8 days. This past week has been a blur and has felt so strange, very not normal. The only two feelings that keep coming to mind are:

1. I feel like I have left my baby in the maternity ward and I am home. I should be with her. It is painfully quiet at night without her yelling for me.

The first night I had the wind taken from my lungs when I snuck past her bed (in the dining room) to grab a glass of water before I went to sleep. I looked over toward her bed on my way into the kitchen and saw she wasn’t there. The resulting feeling was terrible. That feeling was actually worse than how I felt when I had unpacked her stuff and settled into her new room and walked away from her hospital cottage with an empty suitcase. AN EMPTY SUITCASE.

2. This is similar to how I will feel when she is gone forever. I’m sure friends who have lost children will tell me I have no idea what I am talking about and I respect that but this is a real feeling and it is mine. At some point, long before should ever be possible in a parent’s worst nightmare I will lose my Rachel and my house, her room, her voice will be quiet forever.

I’m holding up incredibly well. She had a rough couple of days and I am sure the nursing staff is sick of seeing my (303) area code number show up but until I feel fully confident I am going to call as often as I think I need to. I did this job for 5 years with her disease and I know every facet of it. They forgot to send home her weekend medications on Friday but I was able to purchase what she needed for the weekend from our locally owned pharmacy. There were a few other issues which they are hopefully working out. A transition this giant can’t be perfect.

I want to lay in the street in front of my house and scream “I just want my baby back” but she is not coming home. This disease has robbed her of so much and none of us will ever be the same.

Letting her go was the hardest thing I have ever done. Admitting that someone else could take care of her better than I could was the strongest thing I have ever done.

I’m exhausted but so glad to be alive and healthy.

Friends from all over the country wore their jeans to support Rachel for National Rare Disease Day. The idea is that you wear jeans to show support of rare genetic diseases because the disease is in our genes. I asked all of my friends and family (well, any of them on facebook) to tell at least 5 people about Batten Disease and my Rachel. Here are the ladies and gents who wore jeans for genes!

BECCA

becca

NICOLE

nicole

MEREDITH

meredith

MELISSA

melissa

KERRIE

kerrie

KATIE

katie

Me (Rachel’s Mom)

kat

Heather

heather

Cyrille

cyrille

Chris

chris

Tami

tami

The Brightson Family

brightson

Susie

susie

Apryl

Apryl

Kelly

kelly

Rachel and her StuffieIt’s a different kind of Christmas this year.  There are 5 stockings hung, the house is absent one husband and has gained one puppy.  Rachel’s health is continuing to decline on a continual basis and my other three kids are aware of some of the harsh realities of our life, more than last year.  Rachel’s speech is becoming harder to understand and she is mostly in a wheelchair or on the floor crawling but I have her walk to the bathroom and to her wheelchair on the front porch.  Often when she is in an angry mood she gets upset easily and screams like a toddler when her needs aren’t immediately met, other times she will sit content for hours listening to her favorite shows and movies.


Her decline has been hard for me because I have always liked to get out of the house and DO stuff.  We’ve spent more time at home this past year than we have ever done before and I find it depressing.  I’ve avoided the thought of obtaining a wheelchair van because I’m able to lift her as she still has some lower body strength (although it is considerably weaker since this time last year), although she weighs 185lbs.  I started looking at them this past week on ebay and there are actually a lot of them in the Northeast for well under $10,000.  I like Wasabi the jeep but it doesn’t fit Rachel nor her wheelchair and I am starting to face the reality that I need to find a way to purchase a used wheelchair van or get sucked into a depression from staying home.  Its good to get out of the house, its good to do things (even with a 13 year old that has a terminal brain disease).


I want to go camping with the 4 kids in the spring and have been looking at 1970’s airstreams and realized that between a wheelchair van and a camper, we can stay together as a family of 5 and go on trips together.


This life is certainly far from perfect, Juvenile Batten Disease likes to try and ruin lots of everyday things in life.  Some days the disease wins and some days we win the fight.  Rachel is definitely the one suffering from the disease but I’d be lying if I said it didn’t directly affect the rest of us.  Some days I feel crazy from the constant needing, some days I feel sad for the loss of her health and the loss of my marriage, some days I spend absolutely grateful for my good health.  Most days is a mixture of the three.


We love her.  The yelling, the seizure watch, her seemingly constant needs that have to be met, the depression of knowing that its going to get much worse, her frustration at not being able to be understood when she speaks makes it hard for me to remember who she was before this disease and see the person underneath that I love. The guilt of motherhood is the worst part of being a Mom.


Life goes on and we will forge ahead on this path. The tree is up, the stockings are hung, Santa has been written to. We all look forward to Christmas this year and enjoying a small, quiet celebration, grateful for what we do have and not sad for the things we lack. All we need is a cure for Batten Disease.  The cure is a long time away and out of our hands but the van is something I can tackle after the new year.

paintAn oldie but a goodie, dear friend Geri Calos has organized a night of painting and is donating $5 from each registration to Batten Disease Research!  Please consider signing up if you can, this is located at Sea Dog Brew Pub in Hull, Massachusetts! Click to register!

We recently, quickly updated the New England Chapter of the Batten Disease Support & Research Association from a Drupal based website to WordPress. Continue reading “N.E. Batten Disease Chapter” »

And a conversation about the road race and her Halloween costume!


Notice her speech and cognitive decline…. Just showing what Batten Disease is currently doing to Rachel.

We are currently in Colorado Springs, staying just one more night before we head west. Colorado has been riddled with multiple forest fires because of the extreme heat and lack of rain, dry lightning and a suspected arsonist. We were staying at the beautiful Garden of the Gods Campground but because of the high heat and the eventual smoke and nearby evacuations I moved the kids and I to a hotel in the eastern part of Colorado Springs (near Peterson Air Force Base).

Today we are having lunch with some of my favorite people then we’ll come back here and do some swimming and tonight we are going to a Heather’s house of pets (haha Heather, I love you) and a simple, PASTA dinner. Pasta is one thing my kids really miss on the road.

Tomorrow morning, before the roosters come out to crow, we are driving up through Denver, through Wyoming and staying the night in Utah. After one night in Utah we are driving to Eureka California and spending two nights there. We can’t wait to get to California, visit the pacific ocean and see / feel the Redwood Forest. That will effectively cross 3 more items off of our bucket list…

Another Batten mom named Cheri arranged for Rachel and the kids to go horseback riding at the Colorado Springs Therapeutic Riding Center at the Mark Reyner Stables. When we arrived they were waiting and I was a little hesitant at first because most people don’t get Rachel, especially people who don’t know her. About 5 minutes into our session with the ladies and Bubba the horse I realized I could take a deep breath because they are very experienced with helping special needs kids with riding. One lady (Corey) led Rachel and Bubba and Nancy and her daughter walked on either side of Rachel and helped her with balance. I was most impressed with their handicap accessibility and the ramp they had to help kids mount the horses.

It was a wonderful time for all of the kids and a memory I will treasure forever.

Here is a link to their website: http://markreynerstables.com/

Today we met up with Heather, Nick and Sky (her boys) at the Omlette Parlour. Great food, even better company!

Gotta love a place that serves up good, vegetarian omlettes! Check it out: http://www.co-spgs-omeletteparlor.com/

Picture: Heather and her son Nick

Due to the excessive heat in Colorado we have checked into a hotel for the remainder of our stay. Rachel had the pleasure of sitting in a hottub for a little while, something she really enjoys but doesn’t get to do too often.

Life is good!

1 2 3 4 5