Rachel started on her first day of the last two weeks of school today. She was not feeling very well yesterday and ended up waking up about 8 minutes before the bus comes to pick her up. She was very excited about going with Mrs Cross and she got ready fairly easily. Still had trouble with hair, teeth and shoes but I never expect that to go very well. I’m concerned about next years placement but I am going to try it out and see how she does. I think she might have had a seizure a few days ago because I found vomit in the upstairs bathroom sink and none of the children will own up to it. If the three little ones had puked they would have come into bed in the middle of the night. If Rachel vomited and doesn’t remember it very well might be seizure related. Its hard to pick out what is Batten related and what is regular stuff.
Julie started Park and Rec today, it is the town run summer camp that goes for another three weeks. I was terrified to send her but she really wanted to try it out and it is directly across the street from our house on the L Street field. I’m sure she is going to be fine because she is really independent but I still worry. A lot. It will be good for her to break out from the house for a few weeks before starting Kindergarten and get a good tan.
London and Boston are with Linda today until 3. London was very upset that he isn’t old enough to go to camp with Julie but he is excited that he is old enough to start preschool in the fall. He wears his backpack around every chance he gets. Boston just wants to be fed and watch “Tokyo Mater” over and over again.
John and I are hanging in there. I walk around feeling really sick to my stomach and he walks around with a splitting headache. Either of us can cry at the drop of a hat. We took the kids to see Gforce on Saturday and there was a scene that set me off but no one saw. It is nice to get out and do “normal people” stuff while we can with her. Taking her out with the other 3 can be totally overwhelming for her and she can’t enjoy herself so I am trying to take her out more by herself or just with Julie. People ask me a lot how they can help and the only thing I can think of, especially those who are far away, would be to send the kids mail. If you only send mail to Rachel the other three will feel left out and I’d rather them not know for as long as possible. There will be plenty of time where the other three kids will know something is terribly wrong but I’d like to prolong that time as much as possible. Here is our shop address , I’d rather not post our home address publicly:
The Good Geeks
667 Nantasket Avenue
Hull, MA 02045
Attn: vT Kids
The kids love mail in general, stickers, postcards, etc. This isn’t a request for mail so don’t feel obligated, I just wanted to share a way that you could do something since several have asked.
I took some pictures of the three little ones yesterday playing in our teeny pool. Might try and post them soon. I’m not feeling very motivated today, it is taking a lot of effort to put on a good show for the children (although I am doing it). Takes a lot out of you to look normal but still plan for Rachel’s future.
I’m trying to decide whether or not I want to get involved with the fundraising that this disease needs to get more information and eventually a treatment/cure. I need to think about it some more. I’m most focused on keeping life normal, making money and deciding what to do for Rachel and her education as things progress. I feel strongly that we need to live close to a school for the blind and have been putting some time into researching those options.
I’ve also put a call into “Make a Wish” to see if they can do something for Rach before she loses more vision and cognition.Read More
Many thanks to my friend Mary who organized Rachel’s haircut today via her friend Jess. Rachel loves it and I think she looks awesome!
Less hair + less hair in Rachel’s face = good for me!Read More
In the weeks leading up to her diagnosis, I did a lot of research looking to see how other Batten Disease kids present. What their eye symptoms were, previous testing, bloodwork, etc. One site in particular I found myself going back to a lot even although this family is touched by a different form of Batten Disease. Looking at Nathan’s medical reports was so helpful to me, I have decided to put the same type of information online. In the coming weeks I am going to have a column on the right hand side (you’ll probably have to scroll down to the bottom to get there) but I plan to make the medical stuff available in the interest of helping other parents in my shoes (with a child who has language, memory, visual problems and probably a cognitive decline) try and pinpoint if they should talk to their Dr about being tested for NCL (aka Batten Disease).
I’ll start by sharing the fax report of her diagnosis. Click here for the diagnosis PDF
Rachel was a little irritable today and very excitable. I have finally settled them in for a little while with Ice Age. I am about to start dinner in a few and then head to a nighttime Dentist appointment. Tonight I am meeting Lulu and her friend for a drink after the kids go to bed.
I also wanted to announce that London (who is turning 4 in October) is FINALLY POTTY TRAINED! Now we just have to convince Boston to lose the diapers and I’ll be a happy Momma.Read More
I met with Dr Rappaport today and am very glad that life can relax a bit and get back to normal (which is always a relative term, right?). Until she starts having visible signs of seizures and unless anything else crops up I plan to keep her out of the hospital and Dr offices as much as possible. Tomorrow they have a little bit of summer school in the morning and I am going to do a quick round of miniature golf (she has her heart set on it so we’ll make it as fun as possible) and probably an hour or two at the beach courtesy of Mrs P.
Maybe this is kind of a gift. If I spend as much time with her as possible and fit several years of life into a small amount of time I won’t have many regrets. Maybe I am fooling myself but at this moment in time, I am doing better than most people would (in my unprofessional opinion). I’m sure that will change and change back but I don’t care.
Now I need to figure out the next step as far as getting her into a school for the visually impaired. This is definitely something that will be immensely beneficial to her and hopefully keep her mentally PRESENT for as long as possible. If we didn’t live so far away from Perkins it would be a “no brainer.” I’ve figured out harder problems. I’m sure I’ll sort this one out soon enough.Read More
Had a pretty good day with the on kids on saturday. I nearly cried once but held it together. Rachel got to spend 3 hours with Mary’s dog today and was in LOVE. I was so impressed how she handled him and loved him. I can see how she will benefit from her own canine companion down the road. I’m able to better understand her now that I understand the WHY now. She is definitely responding positively to my new lease on our relationship.
Today we have a birthday party to go to for one of the little boys in daycare with London and Boston. I am going to walmart to stock up on the things we eat a lot (and buy a present for the birthday party). I’ve been a little behind keeping things stocked but, again, I am thinking/hoping things will slow down.
Tomorrow I am meeting with Developmental Medicine to talk medication.
Lots of pictures tonight. Lots. I also wanted to share that:
Rachel’s favorite songs are “Sun Goes Down” by Kenny Chesney and “Honkytonk Badonkadonk” by Trace Adkins.
Julie’s favorite song is “Fidelity” by Regina Spektor
London and Boston’s favorite song is “Old Mcdaonald had a Farm” LOL
Couple of things.
1. If I could stop having the chorus from “Live like you were dying” by Tim Mcgraw playing over and over in my head I would really appreciate it.
2. Praying for a miracle won’t do shit. There is a 0% chance of survival once the disease has run its course. Rather than prayers send my family your thoughts. Knowing that you are thinking about Rachel makes me feel better. I guess its my anger that is making me sound so nasty in #2 and I apologize for that. Genuinely.
3. Everything doesn’t happen for a fucking reason.
4A. I’m tired. I can cry at the drop of a hat.
4B. The caseworker from the Mass Commission of the Blind was awesome today. Very friendly, intelligent and genuine. She saw me cry today. A lot.
5. Knowing that there is a cause for Rachel’s changes and behaviors has really helped give me patience to deal with her and to be close with her even when I have usually had a hard time feeling close. I have had a hard time with Rachel of 2009 and getting the diagnosis has made her easier to parent. I doubt that makes sense to anyone reading but it does to me.
6. Her MRI came back as normal. I’m very happy about this but I can’t help but wonder if the radiologists at childrens would be able to notice a different between the new MRI and the one from May 2008. I am giving the disk of the old results to Childrens on Monday when I meet with Dr Rappaport so hopefully we can have that question answered.Read More
MRI was a long day. She was, shall we say, very unhappy with me afterwards. I did run into someone I know on the radiology floor and it was unbelievably nice to see a familiar face in that building who doesn’t have MD after their name. Twas nice to see you, Mrs McG.
Julie is such a good sister and doesn’t deserve the abuse that seems to get a lot of recently. I don’t think there is a better sister out there for Rachel, Julie is so caring and compassionate. I’m not sure how she doesn’t internalize Rachel’s words and visuals but she keeps on going as if she has some deeper understanding. I probably don’t give her enough credit. I do and have for some time, whispered to Julie several times a day how great/kind/sweet/wonderful/helpful that she is.
Hopefully I will have the MRI results tomorrow. Our kitten, Texas, is being neutered in the morning and after I drop him off I go and meet with Rachel’s caseworker with the Commission of the Blind. I hope that meeting goes well and that she is friendly. I imagine that she’ll start reading this blog so I need to be careful in case she isn’t.
Walking around knowing that your child has a disease with a 0% survival rate feels very much like a living nightmare. I don’t know how other parents move on and live with it but I will try and find out. Like I said, thinking that your child has a neuro-degenerative disease and getting confirmation are two totally different things. Sorry if this entry doesn’t flow and my words are screwy, I can barely stay awake and my mind isn’t working normally.Read More
The girls are in school for a couple of hours this morning, London is playing Star Wars Legos at my feet and Boston is relaxing in front of Dora (apparently he had a long night and is a tired boy). I am leaving at 12 noon to take Rachel to Children’s for her MRI under sedation, the last of a several tests that I put her through for a long time. Whats the point of getting other tests right now, like the ERG? We already know what she has, we can pretty much guess what the ERG will show. She needs to be a kid as much as she can be right now. Once we get home (no matter how late that is) we (the 6 of us) are going to have lobster with my parents, my oldest brother and his wife/3kids and my middle brother.
Susan, my friend in Colorado who has three children (the oldest is the boy who died of cancer 2 years ago) posted an entry on her blog about Rachel and included a picture. I can’t look at it or even recall it in my mind without the tears welling up in my eyes. Rachel looks so “there” and so “present” in that photograph. The personality changes that have taken over her since she was 4/5 are overwhelming to me when I consider this all “in retrospect” and recall just how sweet and kind and gentle she was. Here is Susan’s entry about Rach: http://suzeo99.blogspot.com/2009/07/rachel.html
This kind of brings me to something else that I thought of this morning. If anyone who has met Rachel could forward me any full resolution pictures they have of her because I want to make some photobooks. I lost a lot of my older pictures a while back and would appreciate your help in getting me some new images so I can have some prints made. Its a long shot but I know there has to be some out there.
I am sleeping okay but you go to bed with it and you wake up with it. John is doing better than I am right now… But I really don’t think this has set in for him as I am the one who has done countless hours researching. Thanks for reading.Read More
You can find a whole host of links and websites if you’re interested in looking but I will copy and paste a link that I found. I’m currently taking a breather after settling Rachel in to her room with a snack and a drink after having a total meltdown. I actually googled “violent outburst batten disease” and came up with the link below. If you want to learn about Juvenile Batten disease it gives a lot of good information. Please keep in mind that there are 4 kinds of Batten Diseases and that Rachel has Juvenile. The clinical trials for the other types do not apply to her nor do the progressions or information. Here is the link:
This all makes so much sense now (well, the symptoms). I guess “sense” is a relative term because I would really like to know how this is even possible. I think this has to be just about the worst way to die. I just can’t get over it. I hope that this stage of my grief (I guess its called that) passes quickly. While I want to crawl into bed and never wake up I have lots of people who love and need me to continue, with Rach at the top of the list. I did put her in the boy’s radio flyer and take her out for about an hour after she calmed down after the above meltdown (this post was left open for a couple of hours, hence the gap of time).
I’m sure I will write again soon about this, at least in the beginning… I will get some recent pictures up soon.Read More