Category: (Team) Rachel & Batten Disease


To put it as directly as possible: my oldest daughter is dying, I’m a single Mom and life is a bit tough at present. I realized that I was due for a magnificent stress relief yesterday. I was at the bus stop putting my daughter on her bus with my Mother at my side. It was not a regular moment, my Mom was in from Boston visiting myself and my children. I had let my daughter stay home all week from school so she could spend every waking moment within fingers’ reach of my Mom. As the bus driver and bus monitor were raising my daughter in her wheelchair on the bus lift I looked over and saw my Mother’s eyes starting to well up with tears. My Mother has exceptionally bright blue eyes and I’ve noticed over the years that, for my mother, it makes it nearly impossible to hide tears. For me, I hate crying. Once in a while I will let it out. However, if at all possible I make sure this embarrassing event only happens when I am completely alone, or within the company of my 4 children. Intellectually, I know crying is healthy and good and all sorts of other bullshit, but I hate doing it for an audience. I think I would rather shit myself in public rather than cry in front of other people. So as my daughter was being raised up by the wheelchair lift into the bus I noticed my mother started to sprout tears. I had to take action. Seeing her cry will make me cry and I panicked. I so lovingly looked over at her and said “If you make me cry, I’ll punch you.” It sounds pretty harsh. It was pretty harsh. But I think she, too, would rather cry (if at all) in private. My Mom has seen a lot of people she loves pass away. Both of her parents, her brothers, my brother’s wife, friends, friends children and lots of family. I can’t imagine how many silent cries she has had of her own. So my threat was real but the punch would have been pretty weak. Seeing her cry makes me upset and once I let go, it might take a week to pick myself off the floor. When my daughter was secured on the bus it drove away and we went inside. This was my Mom’s last day visiting us in Denver and she was rushing me to get her to the airport. My mother (and my father for the sake of record) like to get to the aiport suuuuuuuper early. I, personally, like to arrive at the gateway to the gangway just as they are calling for final boarding and are about to close the door. After we had gathered my Mom’s things and she said goodbye to my 3 other children we headed to the airport. She isn’t a big fan of listening to music in the car (I like to blare it) so naturally our time together turned to conversation. I asked her why she was upset at seeing my Rachel going into the bus and the answer was pretty simple. She was upset seeing Rachel upset. Makes sense. We’ve been watching a formerly healthy Rachel decline for quite some time, affected by a really terrible, genetic, terminal neurological disease. Batten Disease. It doesn’t get worse than that. We were talking about my Mom’s house back in Boston and their downstairs master suite they created. I had always thought that the room, my father’s former office, was converted to a giant bedroom to keep things neat and tidy. My Dad likes to collect stuff. A lot of stuff. Yesterday I realized that my Mom created that suite, which was complete with hospital bed, to accommodate my daughter. Rachel had lost the ability to see and has been rapidly losing the ability to walk. My Mom felt it was unsafe to have Rachel climb a flight of stairs for their famous “Friday Sleepover” so she orchestrated a Rachel / Granny / Granddad retreat. This whole time I thought my Mom made my Dad’s office into a bedroom because she wanted to prevent him from messing the room up. The very moment I realized that the whole thing was for my daughter was the moment I switched back into a different stage within the 5 stages of grief. I usually reside at 4769 Denial Drive out here in Denver Colorado but I was quickly moved onto 35 Grief Street. And I started to cry. My eyes welled up, my voice cracked… And I left the room. Being the brave “Queen of Denial” I kept it all together for the rest of the day. I hatched a mental plan during my drive home from Denver International Airport to watch an older movie that I very much loved. A movie I knew was sure to completely clear out my tear ducts. The movie date went well, I put out (tears) and I glued myself back together before sunrise. If you ever need a good cry, consider watching 1998’s What Dreams May Come which stars the late Robin Williams. My eyes have been blissfully swollen all day. I feel a renewed energy today to forge ahead and keep my shit together for my kids and for myself. Here is a quote from the movie which I personally identify with (just read all of the “He” as “She”) “He was a coward. Being strong, not giving up. It was just his place to hide He pushed away the pain so hard he disconnected himself from the person he loved the most. Sometimes when you win you lose.” – Robin Williams (What Dreams May Come)


Julie pulled this video up over the weekend of Rachel and Julie doing the weather. I cannot believe how much she has declined. It was a huge kick in the gut watching this. Life is so fucking unfair, I just can’t stand it sometimes.

Rachel, in a pink onesie, my sunshine

The merry go round that is my life seems to be spinning faster these days. John moved to Denver on Friday and is staying with us for 10 days. It is pretty interesting to have him in my home after so many months apart and so many bad feelings between us. This is for the kids and hopefully we will be able to be somewhat nice to each other for them. The triplets are doing well in a relaxed homeschool environment. The boys are super close and Julie has been struggling with feeling left out. She has a sister but not the sister she needs and its hard for a child (10 years old) to deal with the emotions surrounding watching your sister die. Some kids are remarkable with special needs kids but I can only imagine how hard it is for Julie. She is such a huge help with getting Rachel in and out of the wheelchair van. Julie does this for me and it is her biggest contribution in helping with Rachel. I tell her everyday “I don’t know what I would do without you” and that statement is very true. Rachel is very much getting worse. She is in a good routine between her Teacher Jane at school, with 2 paraprofessional’s named Sam, her bus monitor (Casey) and respite at Megan’s Place with Bryan and Kristin. Tonight when we picked her up at respite she was noticeably neurologically affected by her disease, more than normal. Her drooling was excessive despite the transdermal scopalamine patches she has to reduce her saliva secretions, her walking was only somewhat weight bearing and she kept falling forward and back with no trunk control. I got her into bed and she nearly threw her pills up because she was unable to swallow. We pushed through and she is sleeping soundly. I have been talking to this guy for a year or so and over Thanksgiving he and his beautiful daughters came and stayed with me and the kids. We had an incredible time and commented to each other how normal and beautiful it was to have a “regular family” if only for a long weekend. Julie and I are going out to visit them soon as she really liked being around his girls and I really like being around their Dad. LOL I’m pretty sure he will read this (as will my Mom) so I will leave this at that. His children stole my heart… Life is moving along. It is a living nightmare but I feel that I am making the most of it. Some days I want to crawl in a hole and die but how can you not feel like that (sometimes) when helplessly watching your child succumb to the most brutal neurological disease. Just for a day I wish I could have my Rachel back. Just one day as a normal 14 year old. Just one fucking day. The work to take care of her is substantial but it is the front row seat of a slow death that is the absolute worst. I get jealous of the people who believe in God and have that comfort but it is not a possibility for me. Science is my “religion” and tonight I hate science.

Julie, Christmas at Fletcher Miller Breakfast with Santa 2014

This past weekend I brought the 4 kids plus their father who just drove out here from Boston to the Breakfast with Santa put on by Rachel’s Special needs school (Fletcher Miller in Lakewood, Colorado). It was a great time, huge value for the money and a nice, inclusive environment for kids with special needs and their families. Here are some pictures I took of my kiddos and a few of people that love Rachel (Hi Kristin and Becca).  


For Thanksgiving weekend my dear friend from Kansas (who I met through minecraft) came to visit us with his daughters after we’ve been talking on the phone for about 15 months. We had a great time at the Schnelker household who graciously offered party of “Cortus / Wasabi” room for 8! Here are some pictures taken over Thanksgiving:

Rachel with a magician on the stage at give kids the world in florida

I’ve asked my kiddos to write today about what they are thankful for.  This is a common theme in America in November that I think is total crap.  Many of us are selfish and take life and health and love for granted throughout the year. Boston and London realized last night that there are website statistics which compare their posts in views and comments. They asked me to assign them a topic which will hopefully get them the most post views so I had them jump on the “Thankful for” posts. I was thankful earlier today for having the common sense to record this video on our make a wish trip back in 2009.  Watching it feels like I am being split open and all of the happy is being shopped vac’d out of me but I’m glad to be able to see what my girl was like before this fucking disease activated inside of her.

rachel and kat von tungeln

She really enjoyed the long weekend visit with my Mom. I was sure that she was going to be nasty to me this week after my Mom left but I was pleasantly surprised (and wrong). Truthfully I think that is another sign of her cognitive loss. I knew the decline was coming, it is always coming and coming and coming. Her ability to walk is nearly non-existent but I was born to be somewhat of a “brick shithouse” so I have no problem maneuvering her. The order for a hoyer lift is in as well as a new air mattress and an inflatable bed bath for her hair. I’ve always planned ahead for the decline, having most things in place before she needs them. I hate that she needs them. This morning when I was getting her on the potty and cleaned up to go to school she said this (it was a bit broken but I am paraphrasing): Rachel: “Mom, I’m sad.” Me: “Because you miss granny?” Rachel: “No.” Me: “Nathan?” Rachel: “Yes. Remember when I went to the hospital and sat in his bed.” Me: “Yes, I do. It meant a lot to his Mommy.” Rachel: “Me, too.” She mentions Nathan everyday. Rachel only met him a handful of times and she knows he passed away. She has had this deep connection to him in her mind.


This year Rachel dressed up as a pirate, Julie was a midwife, London was Captain America and Boston Iron Man. We had a great day and was able to attend a few high school sponsored trick-or-treating events and also went to Rachel’s school for a halloween parade. Breaking my ankle a week earlier sucked but I pushed through and obviously the kids had a great time:


Rachel has been very difficult the past couple of days thanks to Batten Disease.  Constantly yelling, needing, demanding you come to her *NOW* and yesterday and Friday evening she was requesting to go to the bathroom several times.  Usually she only goes to the bathroom about 4 times a day (sometimes less).  Her walking is terrible and I am literally holding her up to make it the ten steps to the toilet. I’m at the point where I’m ready for her to start using her absorbent briefs.  I think that would be safer for her… and apparently my back.  There was no big event yesterday where I “pulled something” – but I woke up in excruciating pain that is not going away. Rachel was so upset last night.  She is still asleep now.  Friday and Saturday night she didn’t go to bed until midnight.  Saturday night she had her meds a 6:15.  I need to get some rescue medication for nights when she is extremely emotional, sad, angry, ADD, and all over the place.  :/


Today is like any other day.  Well, today is London’s 9th birthday and I let Rachel stay home today to hang out with all of us and simply relax.  I can’t believe my children are 14, 10, 9 and 8.  Seems like a happy day, which it is, but it is also a very sad day for me. This sadness I keep inside because my children don’t need to have a sad mother.    I have been avoiding this for a long time but today… Today I took delivery on Rachel’s hospital bed. She’s had a hospital bed at my Mom’s house and really quite enjoyed it.  Using the controllers to move her head and legs up and down. I don’t see this bed as a fun, good time.  I see this  bed as death coming closer to my door. I hate this disease. I hate this bed. Surreal to have a child’s stuffed animals on a hospital bed in OUR home.  I don’t like it. She is all happy because I made her ramen for lunch.  Ramen slides down her throat which has an epiglottis that likes to forget to close from time to time. Rachel listening to Disney Junior. Please someone wake us up from this nightmare.  All of the parents of dying kids are living in a nightmare.  Nightmares are for night and you’re supposed to wake up from them in the morning being grateful that it wasn’t real. Except this is real.