I spent a lot of time in the car yesterday driving. Driving to see the kids new school (which is on the other side of Denver) driving Rachel to respite (and back. Twice.) and running a bunch of errands.
When I got home from running my errands I found that my friend’s husband, who will probably kick my ass for not referring to him as my friend (which he totally is but we don’t admit it, or hug lol), had built Rachel this:
My parents, with the help of my brother, built something similar for Rachel back in Massachusetts so Rachel could enter my parents home. I never expressed my thanks because it’s too painful to realize that that is her future. Yes it is inevitable that she is completely losing the ability to walk but sometimes I just can’t face it.
So for what its worth: Thank youRead More
So my name is Kat (Kate) and I have four children. My oldest is Rachel. She is 14, blind, developmentally disabled and can’t eat, toilet, bathe or do very much on her own. She is unable to maintain her temperature on her own and is having a harder time walking and will yell multiple times throughout the day until you figure out what she wants to get her needs met.
To say she is a handful, is the understatement of the fucking century.
She was healthy. Perfect. Sweet. Sighted, Never a brilliant child but certainly normal for her age (well, medically speaking, she was “within normal limits”).
She and I have been on this journey together for 14 years. It has been a rough road. When I was pregnant with her I lost my shit, mentally, and although I don’t often admit this, I was induced 6 weeks early because I didn’t care whether either or she or I lived…. or died.
Very dark days back in 2000.
When she started to show signs that there might be something wrong I had three mains sources of guilt that I hated myself for. Daily.
1. I lightly smoked when I was pregnant with her.
2. Her biological father made zero effort to be in her life either emotionally or financially (he has been consistent like that for nearly the entirety of her life. Apparently he thought they would have a relationship when she grew up. He is an idiot who went on to have two beautiful and perfectly healthy daughters).
3. I remarried and had 3 more children “back to back” and for a while chalked up her difficulties to some kind of rebellion to her younger siblings.
None of those three things actually matter. It took me a long time to get through those. July 2009 I found out she had Juvenile Batten Disease and was dying.
The whole process has sucked for her. She has lost so much, we have lost our normal lives. She is losing her ability to walk and talk and has a very hard time with bathing and toileting. Both activities she and I dread everyday. Because of accidents and period issues she has gone from underwear to wearing adult pull-ups.
I see other parents with children who have the same disease and I compare. I never compare with the parents who have infantile or late infantile batten disease because they truly are very different diseases that have their own set of problems.
I see other parents who pray for a miracle cure and I steer clear of them because I know that the miracle isn’t coming.
I see other parents who will keep their child alive for as long as they possibly can and while I support their level of intervention, I know that isn’t what I want for my daughter.
I see my face when I am helping her into the bathroom, I feel my arms ripping to safely lower her nearing 200 pound body into the bath tub. I feel my frustration level rise when I have to hear her yell yet again because she is upset.
She hates being bathed very much like a cat does. The getting wet, the getting in and out of the bath, the feeling cold while she is dressed, the hair brushing. I could write a whole paragraph on how much she hates having her teeth brushed. I don’t blame her (close your eyes and have someone else brush your teeth just once) but it has to be done even though she and I would rather skip it.
I see the other parents of Batten children who have the same form as Rachel and I feel like the other kids are happier. Rachel has been consistently unhappy for a long time. She loves my mother back in Boston when they spend time together and she has moments of happiness and giggling but for the most part she is either yelling or unhappy. I can’t imagine what it is like to rely on another person to do everything. To eat, to pee, to watch a tv show, to be washed. But I look back and think of her time with her teacher of the blind, Anneliese. She loved her time with Anneliese. I want the Rachel that appeared when she and Anneliese worked together to bake, to craft and to make music.
I want a happier daughter because I know that will lead to a happier Mom and a happier life. I have employed an external respite facility to have her for a few hours twice a week and she starts school next week.
Now I have to look inside myself, inside my tired body and figure out what I can change to make this happier Rachel happen. I’m open to suggestions because if I can figure this out it will change our lives in so many ways. I know most teenage daughters hate their Moms…. but Rachel, mentally, isn’t a teenager and she certainly didn’t go through puberty and exhibit the “NASTY” that I may have been seeing in my younger daughter entering into “the change.” (Not naming any names. Right, Julie?)
I also need to teach my younger children to have more patience for their sister…. and I must lead by positive example. This is the hardest thing I will ever, ever go through. This is the hardest thing they will go through. Oh hell. I hope this is the hardest thing my other, unaffected children will ever go through. I can’t even think about that.
Taken August 2007. I miss this. This girl is still in my daughter but the disease is taking over. I took Rachel to see a primary care doctor here in Denver and she is willing to treat Rachel both as a person and a person with a neurological disorder. This is a big weight off of my shoulders. As she is still having breakthrough bleeding despite the pill and extra estrogen I decided to try her on the depo shot and so far so good. She weighed 186 at the doctor and I am still getting her in and out of the bath with minimal help from her. As long as I can physically care for her I am keeping her endlessly chatty, yelling, hungry, sometimes irritable self at home (which is still an airstream – so cool).
I hate what this disease is doing. I absolutely hate it. Taking her abilities and making it hard to do things as a single-parent family. I won’t give up but unfortunately, Batten Disease doesn’t give up, either. My arms hurt from my muscles ripping to grow larger as her muscles give out more and more each week. Being built like a brick shit house has its advantages that I never dreamed of.
The weeks after the road race have been super busy for Rachel, me and her siblings. Rachel finished up her home schooling with several weeks of seeing Anneliese the awesome TVI (Teacher of the Visually Impaired) at our home and baking. Once school was out I loaded up the kids, pets and the Airstream and we drove out to Colorado. Rachel had a hard time over the 4 day journey as it was very disorienting for her but once we arrived she has been doing much better. I sent her IEP (education plan) to the school district here and they started her in summer school the Monday after we got here. She likes going to school as much as she ever has (says she hates it but has a good time when she is there). A huge difference for me was putting her on a big yellow school bus when I am used to putting her on a 13 passenger van.
I have been making her walk everywhere but I let her use the wheelchair to go to school with for her safety. Yesterday she went 4 wheeling which tired her out from having to keep her core balanced. She also went pee in the woods! This morning she slept in until nearly 10am so I know she had a work out. She is definitely losing her ability to walk but I am trying to fight it as much as possible. Soon I will let her sit (she wants to just about always) but for now, while I think its in her best interest she is stuck with me and walking.
Here are a few recent pictures:
Thank you to Gary Wyrosdic of Wyrosdic Builder Design & Construction for sponsoring Team Rachel Race for A Cure!!! They are returning sponsors of our race and we are so grateful!
Wyrosdic is located in Lynfield, MA and graciously put in ramps to both our front and back door at Rachel’s home.Read More
Thank you to John and Good Geeks for sponsoring the T-Shirts for Team Rachel this year! People love the shirts and they last forever, we appreciate the donation and support of Rachel and Batten Disease Research!
Website: www.goodgeeks.coRead More
My last post was about following my gut and taking Rachel out of her residential school, which I did. Tonight I will briefly write about the last few weeks and the immediate future.
Rachel has been in her wheelchair twice since leaving school. Once to walk to have pizza around the corner and once to come to the park on our street. While at the park Rachel was able to swing in the “disabled people” swing for several minutes and could not have been happier.
Since she has been home I have put our house on the market! As a result of selling the house I moved her bedroom back to the second floor and she is sharing a room with her 10 year old sister, Julie. Rachel’s lower body strength and endurance for walking has dramatically increased. She is happier due to a lack of repeated transitions and having a verbal translator (me) near her at all times.
The school is trying to force the issue and send Rachel to a “Collaborative” school. I have requested, for the time being, pull out services. If Rachel’s Dad accepts her “Collaborative” placement then I will be forced to send her (unless she has diarrhea all of those days of the week), or I have to get a Dr note every 60 days requiring school to bring school to home. Since the house is on the market and the kids and I are moving, I think I am going to go for the homeschool option.
Rachel is happy at home. I am doing just fine with her. This is such a stupid “fight” and one which I am completely unwilling to lose because advocating for Rachel is at the top of my priority list. I know what she wants, I know what she lives and I know what she is trying to say when the words fail her. I guess I am up at bat. I haven’t swung a bat (literally) since high school. Luckily this is a metaphor and I will do my job as her Mom.
In 50 years this won’t matter to anyone. But the next few years mean everything. For Rachel, for me and for her siblings. Standing up for what you know is right for Rachel is the right thing to do.
I shall stand tall and proud and wearing a gas mask because Rachel sure does love to fart. <3Read More
I’m watching Rachel stuff herself a few feet from me with some pancake donut holes that you can buy at Trader Joes. It’s a Tuesday morning at 8:40 and my triplets (my other three children who are unaffected by this disease (I call them the triplets) are all at school. A little over a month ago I sent Rachel off to a new residential school and we lasted a month. They offer some amazing activities there for kids who have a wide range of disabilities and I highly recommend the place.
But the place just wasn’t for us.
She’s home for now while we figure out the next step. If the decision were hers she would return to the Middle School she was attending but we have to consider another option as well before she (hopefully) gets to return to her old school. She is sitting in her recliner under an electric blanket and listening to Sesame Street on Sprouts. Happy as a clam. I followed my gut and I’m happy about my decision to bring her home.
My favorite part about her being home? She is walking up and down the stairs and sleeping in her old bunk bed in her old room with her little sister, Julie.Read More
For the third year in a row, BJ’s Auto Repair in Norwell Massachusetts generously sponsors Team Rachel’s Race for a Cure! BJ’s Auto is our first sponsor of the year!
Website: http://www.bjsautonorwell.com/Read More
Petrocelli Public Insurance Adjusters are faithful sponsors of Team Rachel Race 4 A Cure and has sponsored all 3 races! This year Tami made sure that Petrocelli was our first road race sponsor. Thank you!!!
Website: http://www.petrocelliadjusters.com/Read More
Its 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:
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.
Rachel’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. <3Read More
My apologies for not updating this website in months. I’ve either posted bits and pieces about how things are going on facebook or I have completely fallen off the face of the earth. Some days I would really like to just disappear and not have to watch as this disease slowly kills my daughter. So much of who Rachel is gone but I saw a glimpse of my “old” daughter today.
Tami (the lady who worked with Rachel on a 1 to 1 basis in elementary school) stopped by with her beautiful daughter Becca today and Rachel was bringing up things that we had forgotten. Rachel speech is terrible, her extremities are always freezing yet she lacks the sensation of them being cold and she can barely walk (and for those who are late to this blog she is also completely blind yet born totally healthy). She has become a danger to herself and those who work with her because she is so unsteady on her feet. If 190 pounds comes crashing down due to a motor planning or balance faltering moment, she can easily break a leg (and bleed out internally (hypovolemia)).
This is not a pretty picture and I will be completely honest (something I am known for, they tell me):
I have tapped out.
Rachel, my beautiful Rachel, will be starting at a hospital school about 30 minutes from our house in 8 days. She will live there during the week and come home on the weekends. I know it won’t be long before the weekend trips home will become too difficult.
Batten disease is so hard on myself and especially my other 3 children. As much as Rachel didn’t ask to be born with a rare, terminal brain disease, my other 3 children didn’t ask for Batten Disease to be front and center in their childhoods.
To listen to their sister scream, yell, demand and generally wreak emotional havoc on their Mom is really hard on them. A lot of her behaviors that I deal with are well worn grooves that have become deep over time. Things that Rachel does with me, at home, are behaviors that are much, much less with others (especially new people). The yelling, the death threats, the hitting, the everything. Its all a product of a little girl who’s brain is dying. Things got much worse when she started her period.
I love Rachel so much and wish there was something more that I could do but if you have to pick between your sanity and the emotional health of your children who will hopefully live long happy, healthy lives or a very ill and terminal daughter…
I had to choose. I HAD TO.
And I have chosen myself and healthy children.
And it kills me.
The school that she is going to be living at is awesome. She spent last summer there as a day camper and had THE BEST time.
Like legit. The best time (a few iPhone pics are below).
They have every piece of equipment you can possibly imagine to safely take care of Rachel. They have activities adapted to kids (under the age of 22) that you have to see to believe.. They swim, they ride horses, they do awesome crafts, they cook, they are a community. She has fought me on wanting to go, not wanting to leave home but I’ve worked the idea into her head with the help of my Mom. I know the transition will be hard on her I believe that this is the absolute best place for her to go.
As a Mom you want to protect your kids, see them grow and develop and harvest a passion for life. I’ve done so much with her yet I wish I could do so much more. I’ve been told that this transition will be harder on me than on her. And I believe it.
My other 3 kids need me and Rachel needs to be in a place that can physically manage her and have enough people in rotation to give her the best years of the rest of her life. This is the hardest thing I will ever do.
I wish it didn’t have to be this way.
I love her so much.
Here are some pictures of Rachel taken by her awesome person that was with her during middle school (Gina). Gina has taken a job at the same school Rachel is moving into and is helping Rachel (and me) bridge over into this new way of life.
It’s 4am. I need sleep. Vacation week is upon me and this is my last week with her.
For the 4th day of spirit week at school (Olympic Day), I sent her in as a Gold Medalist Snowboard (complete with board and all). She won a prize and $5 to the book fair. The smile on her face was priceless!
An 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!Read More