A Scary Tuesday

We had a very scary moment with Mira on Tuesday afternoon. After a fantastic weekend (2 seizures in three days with none on Sunday) she had 5 seizures within a couple of hours on Tuesday afternoon. After her sixth seizure around 3:30pm, she started twitching and jerking repeatedly for a while afterwards (not uncommon), but then began having what seemed like a status seizure. We are not sure exactly what it was, but it could have been what is referred to as complex partial status epilepticus. She began squeezing and tightening her hands, arms and legs repetitively and consistently every 30-45 seconds.

We didn't want to be too concerned at first, as her breathing wasn't labored and she didn't seem to be in any pain. Although she had been quite distressed after each tonic-clonic ("big") seizure she was really zoned out from the start of this "episode." It became apparent to Sarah after a few minutes that she was having clusters of activity and was staring off repeatedly, tensing her arms and legs over and over.

We called neurology to get a hold of Mira's nurse to try and understand if this was a prolonged seizure or if she was going into status or what. I had left work early and was headed to CMH at that point to pick up Mira's Keotcal and was able to discuss her situation directly with our neurology nurse, Lynn. She told me that it sounded like a status seizure of some sort and recommended administering Diastat regardless, which we have as a rescue/emergency medication. Sarah gave it to her as I was leaving the hospital and her clusters and squeezing stopped almost instantly. This episode went on for about an hour and 15 minutes total.

Needless to say, we have never seen this sort of seizure activity with her before. If there is one thing that is consistent with Mira is that her seizures are inconsistent. Different types, different times, ever changing patterns. We were watching her like a hawk today and ironically, she didn't have a single seizure or episode all day.

This Week

Not much has really been happening over the past week. Mira is still averaging about 1-2 seizures a day, mainly when she is waking up or falling asleep, which is very common, according to her neurologist. We are still having difficulty getting her ketones up and within a 'therapeutic' range (so to speak) which could account for her seizures not being completely controlled. We are going to take her in to CMH next week to have her labs done again, just to see where she is with the diet and to see how ketotic she is. Chances are, we are going to have to supplement with Ketocal in order to boost her ketones. Overall, the diet is proving to be a success, we just need to get her into better ketosis. We are moving forward, slowly but surely.

We did get her Jenx Monkey Stander last week and she has been using it for PT at home. She broke it in the first day by throwing up on it! Probably not a good idea to get her in it right after breakfast........

Back in the Swing of Things

After some travelling last weekend and more this week for business, things are settling back down for this weekend. I was trying to come up with a witty title and all I could come up was this? Secretly I have the 1985 'hit' by A-ha stuck in my head. Yes, random, very random.

Mira has been doing well over the past week. She is still averaging about a seizure or two a day, but has really been in a good mood all week. She seems to be handling the tweaks with the diet, especially the MCT oil. We are gradually increasing her intake to get to 1 oz. daily. She has been off of the Lamictal completely for a week and we haven't noticed any negative changes yet. We will start weaning the Zonegran very soon.

We all had a great time in Saint Louis last weekend and especially at Siena's (Mira's cousin) birthday party. Thanks to Tom and Nikki for the animal farm fun!

(Pics from left to right: Eli and the tortise; Siena on a wagon ride; Sophia with the rabbits; Siena ready for some cake)

Roller Coaster Loop

Mira is down to her last day of Lamictal tomorrow. We always get anxious when she is coming off a medication, since you never know what to expect with starting one or ending one. She has consistently been having good days over the past few weeks, only having one or two seizures, then yesterday, she threw us for a loop. She had 7 seizures, 4 or 5 of them within about 20 minutes, right after dinner. She has never had so many so close together for a long, long time.

Today, though, she had a great day and was smiling and laughing some. Eli, Mira, and I went for a walk she was staring up at the trees and was giggling some. She even decided to belt out an unprovoked belly laugh during dinner time. Didn't think my cooking was that bad...........

We are heading to Saint Louis for the weekend for Siena's (Mira's cousin) 1st birthday party - can't wait to see everyone!

Slowly getting there.....

We are still tweaking Mira's MAD (Modified Atkins Diet) by adding, subtracting, and calculating ingredients to raise her ketones, with the help of her dietitian. Her labs came back from last week and we had mixed results.

The good news is that her cholesterol and her lipid levels came down, not to within a normal range, but they are certainly lower, which is great. Also, her alkaline phosphatase level is still normal, leading us to believe that her off-the-chart lab from early August was a fluke.

The bad news is that her beta-hydroxybutyrate level (measurement of ketosis in a sense) is still low, meaning she isn't burning enough ketones to put her into adequate ketosis. Therefore, our dietitian recommended adding a certain percentage of MCT oil to her diet. MCT can be hard on the stomach, so we will go slowly with it.

In the meantime, we started weaning Lamictal, and she should be off of it completely in about two weeks. We haven't noticed any difference yet, but it is early in the game. Five out of the last six days, she has only had one seizure per day. This is promising news so far with the diet, considering she was having 5 or 6 a day a month ago.

A Great Labor Day Weekend

The weather is giving one last Kansas sweat-fest kick before it starts nestling into fall. After a wonderful week of mild weather, we are back into the 90's with lots of humidity.

We started doing some tweaks to Mira's diet over the weekend and we think it is starting to show. First, we eliminated all of the flavored waters and Atkins shakes she has been having. We found that most of the prepackaged and store bought drinks have lots of additives in them, which can mess with her state of ketosis and make the diet less effective. Instead of the shakes, we now make our own, with Stevia (all natural no-carb sweetener) heavy cream, and all-natural flavoring through Bickford Flavorings. Sounds disgusting, but it is actually good. Sarah disagrees, calling the shakes 'bizarro-juice'. It's about as natural, carb-free, sugar-free, and simple as you can get and it will have a minimal effect on Mira's ketones.

It is extremely difficult to find flavors for drinks that don't either have alcohol, glycol, corn syrup, or some other bizarre additives I cannot pronounce. Most parents who try their kids on the Ketogenic Diet or the Modified Atkins Diet swear by the Stevia and/or Bickford add-ins for flavors and sweetening.

We also created a very strict schedule of ingredients and measurements for all of her meals and have really been calculating her carb intake. Since these tweaks over the weekend, she has been doing pretty well, only having one seizure Sunday and one today. Last night, she slept for 12 hours straight too.

Neurology Update + Questcor Soapbox

We had our neurology appointment this afternoon and it turned into our usual 2-hour marathon of what has been happening with Mira and trying to determine where to go next. Neurology at Children's Mercy is going through some staff transition over the past few months but fortunately, our neuro is still there.

First, Mira's Biotinidase Deficiency labs, drawn from July 2006 (I can't seem to get my dates right - thinking it was May 2006) were normal. I really just wanted to make absolutely positive that we had not overlooked something so easily treatable.

Second, our neuro gave me a briefing on MTHFR and basically said that based on her labs, the likelihood of her having some sort of connection to such a genetic issue would be slim and none. I ask dumb questions because I am not a neurologist; nonetheless, it doesn't hurt to ask.

Basically, we are going to do some minor tweaking with the diet and continue with it. We are also going to start weaning her off of Lamictal. Our neuro said there is no reason to continue ineffective medications, so the less the better. We will wean her off of that first, then look at weaning Zonegran. Perhaps once she is off those medications, we will see how effective the diet is and just take it day by day. We area also considering Vigabatrin again, but we will cross that bridge when we get to it.

After an exhausting day, we met up with friends at Avelluto's for a carb-fest-romper-room dinner. Seven kids, seven adults and none of the mess to clean up. Thanks Avelluto's!

On a side note, Questcor Pharmaceuticals, the manufacturer of ACTH (which is the first line of defense against IS and used for MS as well) announced this week that they have decided to raise the price of a vial of ACTH from $1,000 to over $23,000 in an effort to stay 'profitable'. This absolutely makes my stomach turn. For so many children and families, ACTH is the first line of defense and often the only medication that helps combat Infantile Spasms. It now has been put completely out of reach for most families without insurance. Mira did ACTH and had we not had insurance, her 4 week trial would have cost us around $5,000 for the medication alone. It will now cost over $100,000 for a child to go through a typical round of ACTH.

I will end my soapbox on this: something has run completely out of control in our health care system so much so that it is just surreal to me. The cost of medications, therapies, testing, and hospital visits for Mira would have bankrupted us in about 15 minutes. Praise to God we have good insurance. We now worry only about lifetime caps. It saddens me to hear so many families on the I.S. message boards wondering if they can afford to treat their child or what alternative(s) they might have to pursue to get help, both medical and financial. It seems the new corporate pharmaceutical motto is: 'how much are you willing to pay for something that might save your life - in other words, how much is your life worth to us?' It truly sickens me how bad it has become.

Neurology Appointment Tomorrow

We are gearing up for another neurology appointment tomorrow around noon at CMH. I usually email our neurologist prior to our appointment, just to get him thinking about what we want to discuss.

I have been trying to get Mira's results from a Biotinidase Deficiency (BD) screen she had in May of 2006 and to get our neurologist's thoughts on Methylenetetrahydrofolate Reductase (MTHFR) which is a genetic defect that causes the body to not properly process folic acid. I am bringing up the results from her past BD screen to make sure that the proper test was performed and to discuss the results with a metabolic geneticist I have been corresponding with in Detroit. At the time of the test, Mira was on multiple medications and we were in the process of switching neurologists. Both deficiencies are sort of long-shots in terms of Mira's condition, but fairly easy to detect and diagnose through screening. Missouri happens to be one of the few states that doesn't perform newborn screening for BD. MTHFR is detected through genetic testing. Some of her past labs might some insight into potential folic acid issues.

We are planning on also discussing medications and give neurology an update on how the diet is going. Mira did have some decent days (her 1 seizure-free day) and a couple days where we have only seen one seizure per day. Better than a few weeks ago!

A Seizure Free 12 hours

Mira has not had seizure free day in a long, long time. Today was her first. She was in a really good mood all day too. I cannot tell you how refreshing it was to see her have such a great day.

On another positive note, the seating company dropped off her Manatee bath seat today and it was like a dream. The chair was donated to the company and is brand new. It is adjustable, flexible, lightweight, and can grow with her. I was so happy to throw away her broken down baby 'sling' tub, that was five sizes to small for her. She was so much more relaxed in the Manatee and bath time was a breeze tonight. No more back pain and hunching over for Daddy!!!

Ahhhhh.....................Barf!

Nothing spells fun like spit-up and vomit. For some reason, over the past few days, Mira has been spitting up a lot. We feel like we have finally started getting into a groove with the diet over the past week, but perhaps the full-swing diet is making her nauseous and creating stomach issues for her. We were initially having trouble finding snacks for her, but stumbled on the Atkins Shakes, which have only 1 gram of carb. She plowed through an entire 8 ounces in one sitting the first time we gave it to her. I suppose she is tired of all of the flavored waters.

Her days over the past week are hit or miss. Some days she does pretty well and even laughs on occasion if Sarah tickles her in the right spot. Other days, she is irritable all day, perfecting her LGF (Low Grade Fuss) and nothing seems to help her. Good days 2 seizures; bad days 7 or 8 seizures.

We added 300mcg of B12 and 400mcg of folic acid to her diet, since many AEDs (anti-epileptic medications) tend to deplete these vitamins in the system.

On a side note, Sarah and I escaped to a concert on Monday night, seeing Crowded House at the Uptown Theatre here in KC. We haven't been to a concert since Richard Thompson, which was 5 years ago. It was surprising to hear Neil Finn and company really 'rock out' - some of their newer material drifted off into some harder edge stuff that I wasn't expecting. Liam Finn (Neil's son) was one of the opening acts. His son?! Yes, they are getting old.

On another side note, what makes my day is visiting Sarah's new favorite site here. Cats are funny.

Ketocal Woes

A year ago, when we tried to start Mira on the Ketogenic Diet, we were given a sample of Ketocal for her to try before they were supposed to admit her to Children's Mercy. She tried it and liked it just fine. Little did we know, Ketocal is actually made out of GOLD. Ketocal is very expensive and the irony is, insurance typically does not cover it (including ours).

Now that we have her on the Modified Atkins and need to use it potentially as a supplement to her diet, we are trying to get Mira's neurologist to write a letter of medical necessity to our insurance company. Since she won't be on it exclusively, we shouldn't be going through it like we do with say, sour cream (lately), but it still doesn't really make sense to me that insurance companies view Ketocal as the equivalent of SlimFast.

Anyway, Mira had a really rough day today, have a half-dozen seizures or so. She was pretty irritable all day and wouldn't eat much. We think her stomach has been bothering her. Tomorrow is another day.......

Dietician Visit

We had a visit with Mira's dietitian for some guidance this afternoon. We were able to get some recipes and other information to help us successfully move forward with the diet. We haven't been able to hit the 10 gram limit lately, since our meal planning with Mira has been difficult.

One of our trouble spots with the diet thus far also has been getting Mira to drink enough fluids, so we will be supplementing her fluid intake with Ketocal. We are going to start preparing meals in advance, more within the 4:1 ratio of classic Ketogenic Diet, the difference being in that she can eat as much as she wants, so long as it is within the ratio and/or the 10 gram limit.

There will be a considerable amount of tweaking with the diet over the coming weeks.