Is wonderful and yet it sucks. I don’t think anyone has a truly blissful life like I thought possible when I was a child. I’m going to focus on the good things right now because I think they outweigh the bad at this moment. The Good We’re (I’m) selling lots of candles for the holidays to raise money for Batten Disease Research I’m taking the kids on another HUGE road trip in a few weeks and we’re going to see the Flying W The Good Geeks (our business) is about to open up another shop with another in the works Rachel’s medication seems to be helping without making her eat and eat and eat Julie can READ!!! Things are going well today and while they don’t go well a lot with a “sick child”, I wanted to write this one down to reflect on in a day or two when we’ve had a major catastrophe (relatively speaking, of course).
How did Thanksgiving creep up so silently? I can’t believe that we’ll be celebrating Thanksgiving in a couple of days and kicking off the Christmas season. I’m thankful that my Mother is having us over for Turkey dinner because I am not prepared. I’ve been overwhelmed with a tremendous amount of work, making candles for Batten Disease and juggling family life. I look forward to the day of rest this Thursday and will enjoy every single minute. I am also thankful to Annie, our Pediatric Palliative Care Caseworker who brought us our Thanksgiving Turkey with all of the “fixins” necessary.
Lots of good stuff going on with work, Rachel changed to a new medicine and London was covered in spots (the chicken pox). Rachel has lost about 6lbs (I’ve found at least that much) and we ended this week with a ham dinner with my parents and brother. Rachel watched “Ice Princess” tonight which is a movie that she used to watch A LOT but hasn’t seen it since going blind. She kept on saying things and asking questions that only a sighted person would be able to ask / say but it was obvious that she was recalling the visual memories of watching the movie years ago while listening as a newly minted blind person. I can’t describe the complexity of how that broke my heart in at least 3 different ways. You may not know that I often refer to myself as “The Iron Maiden” because I “never” cry (it is a rare event) but thinking about what it must be like for her to not be able to see, how confusing things are for her. It sends me over the EDGE and my iron clad tear ducts fly open. Tomorrow I am taking the girls for haircuts. Julie says she doesn’t want hers cut but will come along for the ride. Rachel needs her bangs trimmed. I believe Julie is declining the hair cut because she is obsessed with becoming a princess and I am guessing that she thinks all princesses have long hair. I should show her pictures of Lady Di and cross my fingers that she agrees to getting the wedge cut again (she looked so cute in that!).
I feel so cool blogging on a Friday night. 🙂 I took Rachel to her monthly girl scout meeting tonight and she had a good time. It was hard to watch her try and weave a bracelet (to raise $$ for Juvenile Diabetes) because she can’t see and has a hard time with mental processing activities that require sight. I promised her that I would finish the bracelet AND she could buy it off of me towards Juv. Diabetes. Such a good kid. She is on day 3 of the lamictal. She is definitely more alert now that the risperdal is totally out of her system and her appetite is MUCH lower. I wouldn’t say that she is totally under control (behaviorally) but I think even the baby dose of lamictal (25mg for the first two weeks) is helping somewhat. She hasn’t been in school since Monday but I think she will go back next week. There is a possibility that the Juvenile Batten Disease drug trial – the first one ever – will be starting soon. There has been talk of it as long as I’ve know about Batten Disease so I’m holding onto a shred hope. Hope, even if only a shred, is a good thing. The drug trial won’t be for a cure, it it to test out the safety of using Cellcept (Mycophenolate) on JNCL kids. Cellcept has shown that it can slow down the effects of the disease on mice that have been genetically altered to have JNCL (Juvenile Batten Disease). (jist of JNCL and cellcept usage in knockout mice) Featured Picture: That’s me (Kat), Rachel, Julie and Auntie Jacquie. A park ranger took our picture on Peddock’s Island… (wiki on Peddocks)
The doctor did their prior authorization leg work and insurance rejected it. Rachel’s rash is worse today from her old medication. I called the pharmacy and asked them to fill the prescription ($289) and I’ll just pay it out of pocket. With only prozac in her system today she did really well. I’m not looking forward to the rest of the week but I’ll just take each day as it comes (like we have any choice in the matter)… If she gets through the lamictal without a rash and we can get her on the regular pills the price might lower as the pharmacist explained, there are no guarantees. There are never any guarantees in life I am told. Why the hell not? Now if only I could get my hands on some Cellcept. Cellcept is an organ anti-rejection medication that U of Rochester has been organizing a Juvenile Batten Disease Drug Trial for…. for years but the trial price has gone up and postponed. If I remember correctly from our trip to the U of Rochester a year ago, the main requirements of that are 1. You have to have Juvenile Batten Disease 2. You have to be able to walk ten feet When I heard the second requirement I was all “we’ve got that in the BAG” when can we get started. Now I wonder if her central nervous system will be too deteriorated by the time this trial starts. She has years for walking still (knock on wood) but I’d like a crystal ball. Picture of today: I believe it was taken in 2003 and I called it “Sunshine” back then. Pictures like this literally kill me because I can’t stop wishing I had done more, spent more time, showed her more things back then. Literally kills me.
No school today and I’m going to have to keep Rachel out tomorrow. LOTS of work to do for work which is awesome and I look forward to the quiet late nights. Working on getting my “ole faithful” mini christmas tree working again (fuse?) because I find the lights so relaxing late at night. The girls and I carved our pumpkins tonight (yes, we know it was late but we ran out of time on Halloween). Better late than never is my motto on Halloween because we run out of time every single year. The girls were so thrilled to stab and gut the beautiful squash which are now glowing the night away on my doorstep.
The rash from the Risperdal is getting worse and the Lamictal “starter pack” was denied (I got the letter today). I can’t keep her on the Risperdal with the rash like that so I’ll be stepping her down starting tomorrow, as quickly as possible. I put a call into Rachel’s doctor within a minute of getting the denial letter so I am hoping she will call something else in (or a different version of Lamictal) tomorrow. The Lamictal starter pack costs about $250 out of pocket…. Rachel is going to be (and going to feel) HORRIBLE this week so will likely miss school. Send me a shout if you don’t hear from me for a while. The picture: This picture is of Rachel on her 5th Birthday with my Mom (Granny) and her baby sister Julie.
Rachel’s rash from (presumably) Risperdal has been developing over the last few months and covers a good portion of her body. Its not an angry rash, I would say it looks more like goosebumps that are red in a few places. This medication has been good to get the ADD symptoms of Batten Disease under control and help make for a happier day for her and everyone… BUT… she has put on nearly 50 pounds since starting it. At her age she was expected to gain some weight as puberty is around the corner but 50 pounds is crazy. I’ve been working on coming up with the next step for her medication (she, thankfully only takes Risperdal and Prozac) because this weight makes her uncomfortable. I brought a list of possibilities to the Doctor 10 days ago and we agreed to start her on Lamictal which is an anti-convulsant medication that is the only medication certified by the FDA to treat Bipolar disorder (other than Lithium). Apparently the “starter pack” of the medication is quite expensive and insurance is still taking its time… I find this endlessly frustrating and time consuming. Making multiple phone calls to get a medication that she NEEDS for an organic, degenerative brain disease seems terrible. I’m absolutely positive that I’m not the first person with a sick kid who had to climb through hurdles and I’m quite positive that I’ll look back at this entry in a few years and laugh with all that is to come.
I love the anticipation, the weather, the thrill my kids get when someone asks “So, what are you being for Halloween?” So much excitement and easy happiness that I simply cannot get enough of this time of year. John is making a cheesecake with Rachel right now (its 8am) and I have plans to make candy apples with them later on. My brother and nephews are coming up and we’re having a small, family Halloween thing on Sunday nigh and a friend is having a birthday party for their freshly minted 8yo daughter. 🙂 Life would simply be perfect if it weren’t for… Can’t let myself think like that today because there is nothing I can do. We, as a family, must force ourselves to ignore the disease and enjoy this weekend in spite of the challenges brought to Rachel through her diagnosis. Happy Halloween, everyone. Do me a favor and tell me what your kids are going to be for Halloween and/or what you’re going to do this weekend to make it amazing. (Note: If your “amazing” is going to involve baking a cake, I’ll be happy to hand over my address. LOL)
From a dear friend who, if possible, Rachel would steal this friend’s husband and marry him one day. 🙂 The complaint is that the website background is (well, was) too grown-up and “isn’t this a website to represent a child?”. This friend is right… so I’ve decided to keep the website theme in tune with upcoming Holidays and Seasons. So if you hate the new Halloween theme you’ll only have to stick with it for a week and then we’ll be onto Thanksgiving. The picture in this entry was taken of Rachel with her brothers about a year ago. To everyone offering to help with the 5k, thank you so much! I will definitely be calling on all of you to help and I really appreciate the offers!!!!!! Thinking of dropping the candle prices to $10 as a few people gasped at the price. I’d rather sell more and raise more research money than hold my head high and refuse to bend. What do you think? Is $10 a better price?