Oops!

As I sit here in a hospital bed at Duke with Maddie, I realize that I never updated the blog after the last treatment. There was a lot going on and honestly, I kept thinking I’d wait and update when I was sure I knew what was going on. Needless to say, I never got there. But here we athe gin the second day of her second round of high dose steroids. So I guess that means that we felt it was enough of a success to give it another shot.

Looking back, it’s good I didn’t update then. We had a pretty bad hospital experience and I would have had nothing nice to say about it. As for the steroids and their effectiveness controlling the seizures, it’s been interesting……we have had good days and bad. But on average, we’ve seen significant reduction in the number of drop clusters she has daily. She still has them like clockwork upon waking. Lots of them. And they happen anytime she wakes, first thing in her morning or after a nap. But there are less drop clusters happening throughout the day when she is awake. And at this point, that’s enough improvement to give the steroids another shot.

Her biggest seizures, her strong tonics, are reduced too on average. Now she averages 1-2 per day, before it was 3-4 on average. I’d love for that number to get lower as these seizures now last a long time (sometimes greater than a minute) and they wipe her out. She sometimes bites her tongue during these and I’m terribly afraid she’s going to bite the tip off. I’ve tried to get my finger in there a few times to try to spare her tongue only to find out what an awful idea that is since it feels like she’s going to bite off my fingertip. We now carry Diastat with us (a seizure rescue drug) in the case that one of these goes longer than 5 minutes. There are some breathing abnormalities that go along with this type so I’d really really like to see these go away altogether.

Developmentally, we haven’t seen any major improvements yet, but she just seems clearer for more of the day. That may be due to reduction in drops or it could be that the steroids are helping to clear up her EEG (making her brain waves less abnormal). I’d like to think it is that, but we won’t know until we have an EEG in 4 weeks.

So we are trying this again and the plan is that we’ll do three days of high dose steroids every four weeks to see if we can get some serious improvement. The side effects that we have seen have been body rash, change in body temp (weird), some behavior changes, damage to her fingernails and some hair falling out. While those don’t sound great, in the scheme of things, they are manageable. Certainly if they all increase after this round, we might change our minds. I think we just feel that if we didn’t do the second pulse at least, we wouldn’t really have given this a fair shot. But we also expect to see more improvement. If we don’t, we’ll have a tough call to make. At this point any improvement is good, but there are some more intangible side effects of this that make it not our favorite treatment.

The intangibles……well, let’s see, for the first week after we got out of the hospital, I was a major stress case. I took her temp 30 times a day and had numerous visits to the Pediatrician. I tested her urine daily and felt at any moment we might have to rush back inpatient if she showed signs of a UTI. The fact that she had been septic after steroids in the past made me nervous…..or more aptly neurotic. I’m always vigilant in watching for signs of infection but this just put me on much higher alert than normal. I think that the studies are correct that parents of children who’ve had cancer have some degree of PTSD after the experience. I think I’m normally pretty controlled (some might disagree, but whatever…..) but this treatment makes me remember really scary times and it’s hard not to be a bit neurotic when you’ve been through all we have with miss Maddie. So it’s a bit of deja vu coupled with a rough hospital experience and talk of new diagnoses; and it took me back to a time when I felt less comfortable with our life. And I don’t want to live that way again….always packed to rush off to the hospital. But I think a lot of it was purely emotional on my part, and so this go around, I’m going to try to be more calm and collected about the whole thing (while still being vigilant).

So I mentioned new diagnoses: we have one definitive one and one we are testing for. After looking at her EEG, it is clear she now definitely has Lennox Gastaut Syndrome. We had suspected this but it is now confirmed. It doesn’t really change much; treatments are basically the same as for Infantile Spasms. But it means we can no longer really hope that she’ll simply grow out of her epilepsy. It was also the first time we had confirmed absence seizures, so she now really has a multitude of types of seizures. Uncool.

The other potential diagnosis is for a type of mitochondrial disease called Kearns Sayre Syndrome. This one I really don’t want. The blood test was mailed off to Baylor and we won’t hear results for another month or so. I’m not good at waiting by the way. And this truly is one we don’t want her to have. Lots of bad things come along with it. You can google it if you want, but I am not spending much time and energy researching it until I hear that it’s a fact. And for now, I’m telling myself that they’re wrong to suspect it. So I’m not freaking out about it as much as I would if I thought it was a perfect fit for her symptoms. At some point, I just have to say, enough is enough…..nothing else new to treat, please. But, it is what it is. If she has it, then frankly, she has it and she’s had it and it hasn’t resulted in all the possible symptoms yet. And maybe it wouldn’t ever, because our kiddo definitely knows how to be unique.

That’s why it has taken me a bit to digest that hospital stay. I hoped to update once we had results of her second piece of EEG done while we were at Duke last time, which could have already shown improvement. But our new Neurologist has not returned a single phone call since we were there. Not one. So I still don’t have that result. So I’m not highly impressed with his communication skills at all. If that isn’t remedied soon, we will not be Duke patients much longer. We could certainly continue this treatment with our old Neuro at UNC. I hope it doesn’t come to that, since our drive to Duke is easier…..but I can’t handle doctors who think they are above having to communicate with their patients.

So right now we are flushing dose three of six infusions of steroids. We’ll drive back to our house soon, then return again this afternoon for her second dose. Then repeat two more doses six hours apart tomorrow, except in between she has an appointment with an opthamalogist here at Duke. That should be fun.

There’s lots more going on to talk about…..marathon training, ongoing, fundraising, and a fun trip we recently took to DC, but that will have to wait for later.

Love to everyone,
Liz, Brandon, and Maddie

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6 thoughts on “Oops!

  1. Oh Liz, missed ALL of this. My mom passed away on July 7th and well, life has been fast and furious ever since.

    All I can say is is that you all will remain in my thoughts and prayers as this latest journey begins. Hoping with all my heart that this latest treatment yields all that sweet Maddie deserves. And, that absolutely nothing new comes to fruition, as you all do not need one more new thing to treat. nothing.

    I certainly cry ‘uncle’ on your behalf. Enough already.

    Keep running my friend. As I know for you, as with me as well, running has been my salvation during some of our most difficult days.Will be watching for an update and until then, thinking of you.

  2. Liz: Think of you often and sad to hear of the recent poor experience with Duke but prayerfully the end result will be worth the frustrations and stress.
    Take care of yourself too!

    C Stephenson-Gibbs

  3. Thanks for the update. This was certainly one with lots and lots of happenings and possibilities. Hope that you can have some positive news soon. You’re so positive yourself – you deserve some good stuff!
    Brucie Harry

  4. As always, so sorry for everything you and your family have to endure. Thinking and praying for you and that the steroids will help Maddie with minimal side effects.

    Thoughtfully,
    Cindy Janay

  5. Hi Heather! Big hugs to you! I saw that your mom passed and I should have written sooner. I have been pretty absent from blogs too and just trying to keep up with our life here. After moving to Raleigh in May, I’m having to start all over with doctors, school, therapies, etc. Luckily marathon training is keeping me focused in one area of my life at least.

    Read your blog today and it sounds like you are trying really hard to be in a good place emotionally. I hope that starts to get easier soon. Glad you’re running again, maybe that’s the missing piece.

    Thanks for crying uncle for me. I know you, greater than most, know the feeling when it seems the hits just keep on coming. I just want to be able to protect her from something. Anything. I guess I just have to do everything I can. But it still makes me angry. Ugh.

    Hugs to you and your beautiful family. Glad you are soon to be a Mimi again. That’s got to be amazing.

    Take care! Liz

    Liz Gorman

  6. Liz — It is exhausting just to read what all of you have been through. I am so sorry for your bad experience at Duke — I hope you got some feedback to the right people there. I am glad you have Brandon with you now as you start all these new treatments, doctors, etc. I will always hold on to hope that these latest treatments will work for your precious, beautiful Maddie. All of you are in my prayers. Love, Loretta

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