Last day

Posted by in Kat, Life, Rachel

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.

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Lots of pictures

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

Julie (5) in the swimming pool at a birthday party

Julie (5) in the swimming pool at a birthday party

Rachel enjoying the pool at Tony's party

Rachel (9) enjoying the pool at Tony's party

John at Tony's 4th birthday party

John (too old to admit) at Tony's 4th birthday party

London with a sandy face

London (nearly 4) with a sandy face

Boston (nearly 3) pointing at an airplane

Boston (nearly 3) pointing

Rachel listening to Mary

Rachel listening to Mary

Boston (nearly 3) pointing at an airplane

Boston (nearly 3) pointing at an airplane

Rachel (9) with Trevor

Rachel (9) with Trevor

More walking Trevor

More walking Trevor

Rach is thirsty after walking so much

Rach is thirsty after walking so much

Julie (5) the Duck Whisperer

Julie (5) the Duck Whisperer

Julie (5) with a face you can't say no to

Julie (5) with a face you can't say no to

Julie, Rachel, Boston and London

Julie, Rachel, Boston and London

Juliebean, Rachey, Boss man and Jack

Juliebean, Rachey, Boss man and Jack

This is Rachel, 3 weeks ago at her Granny's house

This is Rachel, 3 weeks ago at her Granny's house

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Sunday morning

Posted by in Boston, Julie, Kat, Life, London, Rachel

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

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Friday Night

Posted by in Kat, Life, Rachel

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.

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Thursday night

Posted by in Julie, Kat, Life, Rachel

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.

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Thursday

Posted by in John, Kat, Life, Rachel

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.

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What is Juvenile Batten Disease

Posted by in Kat, Life, Rachel

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:

http://books.google.com/books?id=VRIMSb8u5-YC&pg=PA55&lpg=PA55&dq=violent+outburst+batten+disease&source=bl&ots=uS3M8VzQwa&sig=5uvEeZ_6zHEgT7eAJZLUuIlayBE&hl=en&ei=2IFnSueGKM3DlAealJXdDA&sa=X&oi=book_result&ct=result&resnum=1

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.

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Tuesday

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

Its been the best part of 24 hours since I got the news that I had been half expecting.  I told John last night and it went as well as could be expected.  I feel really sick to my stomach today, I’m not sure about him.  My oldest brother, who is out here from KC, MO visiting for a week spent the day working on our large living room (the front room for the people that have been inside this house).  It looks really good and is nice to get that big-ass project nearly completed because I am sure having less on my plate will help me deal.

Still haven’t cried.  I’ve spent the day working at home, keeping the kids from doing anything dangerous but not really doing much with them and I have also called and made lots of “next steps” and informed the people that needed to know.  I’m pretty much on “Worst Case Autopilot” because I need to get the right things in place and can’t lose focus because she needs my focus right now.  I will do everything in my power so that she never finds out that she has Batten Disease.

Thursday: MRI under sedation (which might or might not show brain atrophy)
Friday:
I meet with Rachel’s “Commission of the Blind” case to get started with a ton of paperwork. (KID FREE)
Monday:
I meet with the Chief of Developmental Medicine to talk about what medication (if any) would benefit her right now.  I have, for quite some time, wanted an SSRI for her.  I still want to go ahead with that and hopefully we can get that started.  (KID FREE)

London still isn’t walking on his leg but has learned to do this “inch worm” thing around the house and is somewhat mobile.  His knee is definitely still swollen.

Julie and Boston are doing well.  Boston is consistently obnoxious and totally untrustworthy but I have finally learned that he was my first “typical 2yo.”

Life is so fucking unfair.  She did nothing to deserve this.

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847pm

Posted by in Rachel

When you get a call at nearly 9pm from the head of Neurology you have to kind of figure that it is going to be bad news.  Dr Darras called this evening to let me know that Rachel’s Juvenile Batten Disease test came back positive with the standard DNA presentation.

For those who are new to the road I have been traveling with Rachel, Batten Disease is 100% fatal.  There is no cure, no real treatment… from what I have read up on it within the last few weeks all they can do is treat the symptoms.  I will leave you to google “Juvenile Batten Disease” because while I am ok right now  (I was pretty sure that she had something neuro-degenerative) I will not let myself think of the future right now.

And to anyone who reads this please take heart in the fact that you can’t say the wrong thing to me.  I’d much rather you stick your foot into your mouth than ignore me because you couldn’t come up with the right words.

The next step:  MRI on Thursday which will give us a good baseline and comparison from her other MRI 16 months ago.

John will be home in a few minutes so I have to prepare myself to tell him…

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Diagnosis

Posted by in Batten Disease, Blog, Medical, Rachel

This is a repost from Rachel’s Mom’s blog @ www.mrskatvon.com

Title: 847pm

When you get a call at nearly 9pm from the head of Neurology you have to kind of figure that it is going to be bad news.  Dr Darras called this evening to let me know that Rachel’s Juvenile Batten Disease test came back positive with the standard DNA presentation.

For those who are new to the road I have been traveling with Rachel, Batten Disease is 100% fatal.  There is no cure, no real treatment… from what I have read up on it within the last few weeks all they can do is treat the symptoms.  I will leave you to google “Juvenile Batten Disease” because while I am ok right now  (I was pretty sure that she had something neuro-degenerative) I will not let myself think of the future right now.

And to anyone who reads this please take heart in the fact that you can’t say the wrong thing to me.  I’d much rather you stick your foot into your mouth than ignore me because you couldn’t come up with the right words.

The next step:  MRI on Thursday which will give us a good baseline and comparison from her other MRI 16 months ago.

John will be home in a few minutes so I have to prepare myself to tell him…

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