Emma's Grace

Gosh, will it ever end.

Emma spent a week teething.  And then she gets a cold during the last couple of days.  It still hasn’t gone away.

I’ve got a lingering infection from my cold a couple of weeks ago.  It’s killing my throat enough that I went to the doctor yesterday and got an antibiotic.

Jon had poison ivy for over a week now.  His poor arms are the worst of it.  He’s on a steroid to kick that from his system.  Today is his 3rd day on it and he’s hoping to see some signs of it going away soon.

Matthew came home yesterday and said his head hurt and ended up with a slight fever.  He’s already had a cold.  Let’s hope this doesn’t turn into anything more.

Please pray for good health.  We could all use it so we can enjoy the beautiful weather.

And Jon & I have a test coming up at the dojo in a couple of week.  It would be really nice if we could be healthy for it.

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Today, at 8pm, it will have been 2 years ago that our precious Emma Grace was born.

Happy Birthday my sweet angel!  You are a blessing in so many ways.  Each day we look forward to every little thing you do.  Your smile is beautiful and automatically spreads to everyone around.  You mean so much to all of us and to so many more.  We love you dearly!

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It’s been a crazy week.  Emma has been cutting a huge amount of teeth … 3 molars & 2 eye teeth on the bottom.  She has spent many days in pain.  We’ve been trying to make her as comfortable as we can.  It’s been difficult since the only things she likes to put into her mouth are food, her cup, and her hand.  We’re hoping that this will pass quickly since it would be horrible to feel so bad on such a special day.

Because of how Emma’s been feeling, we’re not sure how we will celebrate this weekend.  But Matthew is very excited to bake Emma a cake and to help decorate it.

Please feel free to leave comments.  I’m sure Emma would love to hear from everyone.

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We had an appointment with Dr. Chugani today.  Or as my friend, Danielle, calls him, Dr. Rockstar.  This was our follow-up appointment after Emma’s MRI and PET scan.

The MRI showed that Emma’s white matter in her brain is not as mature as a normal 2-year old child.  She doesn’t seem to have any obvious defects of her brain.

The EEG that was done with the PET scan showed the hypsarrhythmia pattern during the time that she was sleeping.  Other than that, there were no seizures.

The PET scan didn’t show a focal point.  Her entire brain didn’t process the radioactive glucose correctly.  Because of this, she isn’t a surgical candidate.

Dr. Chugani didn’t have a whole lot to say today.  Emma’s obviously not a surgical candidate because of the PET scan.  Because Emma’s not having any seizures, he wouldn’t mess around with her medication at the moment.  He didn’t think there was really any genetic testing that we’d be able to do.  So, right now, we’re just waiting and treating the seizures.  He has a sample of Emma’s DNA, that will eventually be sent off to do a bunch of genetic testing on it.  If they find anything, they will let us know.  But we don’t know when that will be done.  Maybe next week….maybe next year.

Here is what the actual reports say…

MRI of the brain without contrast
There is evidence of diffusely increased T2 signal throughout the deep white matter bilaterally, most evident within the right parietal and both temporal lobes.  The degree of increased T2 signal is out of proportion for the expected amount of myelination at this age.

There is no evidence of focal mass, mass effect, hydrocephalus, or extraaxial fluid collection.  There is no brain herniation seen.

No obvious cortical dysplasia or neuronal migration anomaly is identified.  The abnormal T2 signal is best seen on image 12 of series 2.

The corpus callosum is also of diminutive size.

Ventricular size and overall brain volume, however, is within normal limits.  The globes and both optic nerve sheath compleses appear symmetric.

Impression:
1.  Diffusely increased T2 signal within the deep white matter as described which is bilateral.  Possibilities could include various causes of delayed myelination or dysmyalination.  An underlying metabolic disorder is also not completely excluded.  Clinical correlation is recommended, a short-term followup in 6 months is also recommended to further evaluate.
2.  There is no evidence of acute ischemia on the diffusion-weighted images.

EEG (done with the PET)
The patient was awake and asleep during the recordings.  The background activity during awake was disorganized and consisted of a mixture of delta and and theta activity ranging from 2 to 4 Hz.  During sleep, hypsarrhythmia pattern was noted and no well formed sleep architecture was noted on either side.  Very frequent multi-focal interictal epileptiform discharge was seen in the left and right anterior as well as posterior quadrant which was slightly dominant in the left hemisphere.  No ongoing seizures were captured.

Impression:
This was an abnormal EEG recording.  Disorganized background activity with hypsarrhythmia pattern during sleep and absence of well formed sleep architecture suggested diffuse neuronal dysfunction.  The presence of very frequent interictal epileptiform discharge in the left and right anterior as well as posterior quadrant suggested increased risk of epileptic seizure with multi-focal onset.

PET scan
The EEG performed during the PET scan showed background slowing with hypsarrhythmia in some of the sleep EEG segents.  There were also very frequent multifocal spike and wave activity from the left and right hemisphere, perhaps more dominant on the left side.

The PET scan showed severe hypometabolism of the parietal, temporal cortex bilaterally, more pronounced onthe right side.  There is a milder hypometabolism of the frntal cortex bilaterally.  The occipital cortex appears to be also less affected but is nevertheless hypometabolic.  The basal ganglia appear very prominent because of the surounding hypometabolism.  The thalamus, brainstem, and cerebellum appear normal.  The medial temporal lobe structures appear symmetric.

Impression:
This is an abnormal cerebral glucose metabolism study showing bilateral hypometabolism affecting virtually the entire cerebral cortex but most pronounced in the parietal and temporal cortex on the right side.  This finding is most consistent with an underlying genetic or metabolic condition.  We suggest further testing in this regard.  Based on this scan, it is unlikely that this child would be a surgical candidate.

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Well, since I’m sure most of you have noticed, posts have been a little lacking lately. Ok, a lot lacking.

Since I have such a problem with 2 blogs, I’m going to stop posting at A Magic Mommy and just post here, on Emma’s Grace. So, if I ever get around to it, you’ll not only get posts on Emma and how she’s doing. You’ll be getting posts on Matthew, our family outings, recipes, and other various things I may post on.

I hope you all don’t mind the intermingling, but I feel bad each time I post something on Magic Mommy because it should go there and then Emma’s blog doesn’t get seen, which is what I really want. And maybe I’ll actually manage to post more often.

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Let’s break the silence. Wear purple to support your loved ones with Epilepsy. It’s National Epilepsy Awareness Day. It’s time to support and educate. Show the world how many people are affected by Epilepsy.

Wear your purple. Let people know that you love Emma.

I’ll be wearing purple. Will you?

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The past months have been busy, to say the least.  Emma has had her meds adjusted several times, with the end result being a return to Topamax and Vigabatrin.  Her seizures are mostly under control again, but not completely.  She has also developed a new type of seizure, a head drop.  If you don’t know what that is, she basically goes totally limp (thus causing her head to drop) for about a half-second.  She also has some cute new glasses, which have helped her vision tremendously.

Along with all of that, which would be plenty all on its own, we have also started some more forward-looking consultations with the neurologists.  Last week Emma saw Dr. Chugani, one of the top (if not the top) neurologists for IS.  He is the Chief of Pediatric Neurology at Children’s Hospital of Michigan, where Emma ends up when she has to go to the hospital.  Following up on that visit, Emma will be going in for a PET scan and an MRI, after which we can discuss what options are available.

Emma continues to have a very busy schedule, with school two days a week, and private therapy another two days a week.  She’s been doing well, despite a regression during the period of uncontrolled seizures we had in December and January.  Along with plenty of help and persistence from Mommy, she is even mastering drinking from a sippy cup; this is a major step for Emma, as the only drinking she was doing before was nursing.  She even seems to be communicating when she wants a drink, with a sort of licking motion.  Learning these types of communication behaviors is an important step for all of us, as it is a tremendous help for quality of life and skill advancements.

There are many more levels of details to all of the above, of course.  But this at least gives an overview of how things have been going for our little angel so far in 2009.

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As you all can see, I am horrible about doing these updates lately.  Mostly, I think I’ve been avoiding it because I get tired of thinking about it.  But I really need to keep up with this.

Anyway…

Emma is still having seizures.  We’ve increased her Topamax, which didn’t help.  She was up to 75mg in the morning and 90mg in the evening.  That didn’t seem to be working though.

Then we had our little trip to the hospital.

After the holiday week was over, and getting in touch with her neuro, we decided to decrease her Topamax back down to 75mg twice a day.  We also introduced Emma to Trileptal.

An intro dose of 0.5ml was give twice a day for 4 days.  After that we moved her up to 1ml twice a day.  That didn’t seem to be doing the trick either.  We increased it again to 1.5ml twice a day.  Again, no real change.  We have increased her dosage to 2ml and then up to 2.5ml twice a day.  Still no decrease in seizures.

Tomorrow, the 23rd, we will go in for an EEG.  We’ll see what that tells us.

Emma’s seizures have been changing since before Christmas.

Before our trip to the hospital, she would make chewing motions with her jaw, scrunch up her face, and drool a lot.

And then there was the seizure that caused me to take her to the hospital.  I found her in bed, with her eyes fixed up and to the left.  Her forehead was twitching.

After the hospital trip, she was relatively seizure free for a couple of days.  Sadly the seizures came back, but in a different way.  These seizures were similar to her original seizures from over a year ago.

She has been doing a different things with each seizure.

At first, her hands would shake.  Her arms would bend and go up, but she would tremble.  It was almost indiscernible.

Now her arms bend and her hands go up.  Sometimes her arms fully extend, sometimes they don’t.  She trembles.  Her face scrunches up.  Her left leg bends, sometimes it doesn’t.

She seems to be cognitive during some of the seizures.  And she isn’t really sleepy after each one.  She’s quite happy and active some of the time.

But worst of all….she has started to jackknife.  A classic IS seizure.

There were even a couple of times where she would cry out after a seizure.  Also typically IS.

I don’t know what the EEG is going to tell us tomorrow.  But I have a feeling the IS is back.

And so will the Vigabatrin.  Hopefully it won’t damage her already diminished vision.

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Just a quick note.

I was able to bring Emma home tonite. She is sleeping soundly in her own bed.

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…another visit to the hospital.

Our Christmas day was going well, but Emma seemed pretty tired.  Everyone else in the house has been sick lately, so we thought she was coming down with the family cold.  She did have a small seizure during the day, but it didn’t last long, and wasn’t much more than her drooling.  She was also running a slight fever, so got some ibuprofen.

She was taking a nap in the evening.  At dinner time, we went to check on her.  She was sound asleep.  Andi and I discussed that if she was fighting off a cold it was probably best to let her keep sleeping.  After the rest of us finished dinner, Andi went to wake Emma up so that she could have dinner and get her meds.  Andi found Emma in the middle of a seizure, her eyes and forehead twitching.  The Diastat seemed to bring her out of the seizure, but she still seemed off.  Either she was having another seizure, or she was just so wasted from the aftereffects of the seizures and the meds that she was zoned out.  Not wanting to take any chances, Andi took her off to the ER.

Emma has been dehydrated or nearly so the last few days, but it has been difficult to get her to drink anything, except when nursing.  Even then, she hasn’t been drinking a lot (mostly playing, as Andi says).  So at the hospital they put her on an IV to give her some fluids.

As of lunchtime, Emma hadn’t been having anymore seizures, but Andi was still waiting to see what the doctors wanted to do.  Several options were presented (try a new med, Video EEG, etc), but we really don’t want to start anything new without discussing with Emma’s neuro.  So now Matthew and I are home waiting to hear from Andi, and see how long little Emma will be in the hospital this time.

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… I took Emma to the hospital.

It was the beginning of a horrible holiday season.

We are very thankful today that we are not in the same place.  And our holidays are looking to be quiet and snow-filled.

*knock on wood*

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