Great Local Businesses

We have some really fantastic neighbors.  As it turns out, we also have some fantastic neighborhood businesses.  We really lucked out!

Today, our local bike shop saved the day--and our upcoming vacation--by fixing Bertrand's sport wheelchair.  Shriners Hospital couldn't do it.  Our medical device company wouldn't do it (we didn't order the chair through them).

The guys at CycleSmith not only did the job well, they did it cheaply, same day, and with a smile.

(A man in a wheelchair came in for some tire stuff too while we were waiting.  Were we the only ones who didn't know about bike mechanic versatility?!  It's definitely worth keeping in mind if you have problems with your kid's wheelchair.)

Oh, and I can't talk about awesome local business without mentioning our pharmacy either.

University Pharmacy was our savior while Bertrand was on the ketogenic diet.

Since it is a compounding pharmacy, the pharmacists were able to source or create carbohydrate-free versions of all his medication.  They also have a great selection of high quality supplements.  And like Cheers, they always know your name.  ;)

But by far, the most memorable customer service experience at University Pharmacy was when we forgot to pick up Bertrand's seizure medication one weekend.  The pharmacy is closed on Sundays, but a pharmacist (the wonderful Trip) came in to open shop just for us and let us pick-up it up.  That day made us customers for life.

There are probably some fantastic, small businesses in your area too, so check them out.  :)  And if you're in the Salt Lake City area, check out University Pharmacy and CycleSmith.  By the way, I hear CycleSmith does bicycles too.  ;)

2010 Year End Review

Fern (my cousin), Bertrand, me, Matthew, Belinda and Aury (my sisters).

We are currently busy in Florida with several family events (such as my aunt's wedding, pictured above), but I just had to check-in one more time for 2010. :)

Closing out the year, I feel like our family is staggering across a metaphorical finish line. Any year that starts with your child having brand new seizure types on the very first day is bound to put you through the wringer.

Borrowing the idea from my friend Fawn, here is a representative post for each month of 2010:

January

Tonic seizures

February

How did Bertrand break his arm?

March

Developmental Evaluation: No Progress

April

ACTH, here we come!

May

State of the Pookie Report: May 2010

June

Hospitalization Update

July

The new Bertrand, and our biggest fear

August

Post-Infusion Report

September

Pneumonia Scare 2.0

October

Falling Down

November

A Big Change

December

Holiday Update

Re-reading all the 2010 posts this morning was quite the effort. And honestly, I was too disheartended, exhausted and sick for most of the year to write regularly. Much of the really hard stuff was left out. Hopefully, 2011 will bring positive changes to our family as well as many of our friends.

We're wishing you all much love, happiness and peace in the new year!

Bertrand watches "How To Train Your Dragon"

For a change from his typical Elmo fare, Bertrand sat down to watch "How To Train Your Dragon". It is a DreamWorks production that came highly recommended from other special needs parents for its portrayal of disabilities. Several characters in the film, including the title dragon, have need of prosthetic limbs, but this doesn't define their characters or limit the greatness they are capable of. It's a cute movie and, as you can see from his youtube review above, Bertrand enjoyed it!

Emily Dickinson: Poet, Recluse, Epileptic**?

Photograph by Jack Illingworth.

"Hope" is the thing with feathers—

That perches in the soul—

And sings the tune without the words—

And never stops—at all—

And sweetest—in the Gale—is heard—

And sore must be the storm—

That could abash the little Bird

That kept so many warm—

I've heard it in the chillest land—

And on the strangest Sea—

Yet, never, in Extremity,

It asked a crumb—of Me.

Emily Dickinson

Emily Dickinson's words have inspired generations. In particular, the poem above seems to have struck a chord within the special needs community--perhaps because Emily Dickinson is one of us?

Known for her reclusiveness as much as her creativity, it was long thought that Ms. Dickinson suffered from mental illness. New research revealed in her biography Lives Like Loaded Guns: Emily Dickinson and Her Family's Feuds indicates that her condition was more neurologic than psychiatric. According to author Lyndall Gordon, the drugs prescribed to Ms. Dickinson were ones used at the time for epilepsy.

With the diagnosis of epilepsy, many strange aspects of Emily Dickinson's life tumble into coherence. Her reclusiveness transforms into the common fear of having a seizure in public. Her unmarried state is due to the stigma associated with seizures--in many states "epileptics" were forbidden by law to marry. Her white wardrobe and adherence to routine? An attempt to reduce overstimulation and resulting seizures. Even her creativity? Temporal lobe epilepsy has long been associated with creative genius.

This book doesn't focus solely on the poet's epilepsy; it includes the many familial struggles she faced. But, given the hurdles which individuals with epilepsy still face today, the beauty that Emily Dickinson managed find and create is made even more astounding.

Thanks to Lyndall Gordon's investigation, Emily Dickinson, who has long inspired us with her words, now inspires us with her life.

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**Disclaimer: I am using the word "epileptic" here for historical accuracy and grammatical consistency. The term "epileptic" is no longer used, as individuals suffering from epilepsy are not defined by their condition. After all, no one refers to someone suffering from cancer as a cancerian, right? That said, as I've witnessed some spectacular showdowns over the use of the E-word, please don't crucify me!

Guest Review: "Seized"

Bertrand's grandma Diane reviewed the book Seized by Eve La Plante. Please enjoy!

What might Van Gough, Dostoevsky, Flaubert, Lewis Carroll, Poe, St. Paul, Muhammad, Moses and many more have in common? Analysis of historical data by author Eve La Plante in “Seized” reveals all potentially suffered from a type of epilepsy called Temporal Lobe Epilepsy (TLE). La Plante takes the reader into the world of TLE partial seizures and their effect on the lives of both the extraordinary and the ordinary. This book is recommended as a good first read before further research into epilepsy.

TLE affects over 1 million Americans. The reader becomes sensitized to those with this disorder and how lack of knowledge creates inappropriate prejudice and discrimination against epileptics. Tragically the word “epileptic” often carries such stigma that many doctors initially refer to their patient’s condition as “seizures” only.

In between seizures key TLE symptoms may include hypergraphia (arts/writing), hyperreligiosity, stickiness (dependency on certain people), aggression, and/or altered sexuality. It is believed that TLE causes ordinary traits to be intensively muted or enhanced due to an “underlying brain scar which causes hyperconnectivity”. An over abundance of rapid connections in the emotional part of the brain cause hypersensitivity to environment much like a spigot that can’t be turned off.

The medical history of epilepsy is summarized with focus on important contributors to the field. Past barbaric treatments as well as current and future treatments are discussed. Although brain anatomy and function are reviewed, the reader is left with a desire to learn more as well as research the reasons for abnormal neuronal firing and the various types of epilepsy.

Since there is an organic basis for many mental conditions, the inevitable merging of psychiatry and neurology is addressed. Intriguing age old questions are posed such as “What is a thought?” and “Are mind and body dual in nature?” In addition, the concept in our society that everyone has the free will to control his/her actions is thrown into question. In closing, as a fun exercise before reading, draw the face of a clock at 10 after 11 and see how your clock compares to that of someone with TLE.

Review: "The Ketogenic Diet"

A few days ago I read The Ketogenic Diet: A Treatment for Children and Others with Epilepsy by John M. Freeman et al. The authors are members of the Johns Hopkins team which has developed, preserved and championed the ketogenic diet. The book is in its fourth edition and, after reading it, I can see why it is required reading for almost everyone commencing the ketogenic diet. I highly recommend it to anyone interested in learning more about this alternative, non-alternative treatment for epilepsy!

Due to decades of scientific research done on the effectiveness of the ketogenic diet, the authors stress that it is not to be viewed as “alternative” medicine. The diet IS traditional western scientific medicine. The mechanisms that grant the diet’s effectiveness are as well understood as (and even more well studied than) the mechanisms which govern modern seizure medication’s effectiveness. Sadly, by this I mean, that all epilepsy treatments remain a bit of a mystery to the scientific community.

The book is every book you’d want or need to start the ketogenic diet rolled into one. It covers the history of epilepsy and the ketogenic diet. It attempts to explain the possible biological and chemical mechanisms at work in the ketogenic diet and even the very promising, modified Atkins diet. The Calculations section is basically a workbook, which leaves one more confident about being the sanity check for your dietician. The recipes (both Ketogenic and Atkins) are excellent, and in this section and through out the book, the diet’s use in infants is heavily covered! Lastly, the book is peppered with anecdotes and examples drawn from a vast pool of humanity, which bring the content to life.

While the book is forthright on the limitations and expectations one should have regarding the ketogenic diet (I am now back to being pessimistic about Bertrand’s odds of being helped by the diet), I was heartened by a section entitled “Speculation: The Future Role of the Ketogenic Diet.” The authors hypothesize several additional uses for the ketogenic diet outside of epilepsy--the one that caught my eye was as a treatment for cancer. If I ever get cancer (brain cancer in particular) I have nothing to lose by trying this experiment!

“Brain tumors appear to feed on glucose. Indeed they “steal” glucose from the surrounding brain tissue, causing the surrounding tissue to die. Whereas normal brain tissue can use ketones such as beta-hydroxy butyric acid (BOH) as an energy source, tumor tissue does not appear to have the ability to metabolize ketones... Would it be possible to “starve” a tumor by reducing its glucose supply, while feeding the brain with ketones?”

Fascinating, huh? :)

Tracking Seizures

In order to objectively judge the efficacy of the ketogenic diet, we need to find a way to count Bertrand's seizures. This poses a BIG problem because he has several seizures (2 to 6) every minute, even in his sleep. That is close to 6,000 seizures (absence, complex partial with automatisms, drops, and startles) a DAY! (The number of XL spikes and XL events on his EEG are double to triple that, so I am glad I don't have to try and count those!)

Knowing that, is there any wonder why he is so severely developmentally delayed? He can barely follow through on his impulses or commit anything to memory! When I try to count and log all of his seizures, I literally can't do anything else. By the time I'm done checking off the kind of seizure and writing down the duration, he's already had another one and I've missed it. It is very frustrating--but imagine how much worse it must be for Bertrand. :(

Furthermore, due to the nature of his seizures you have to be staring Bertrand in the face--you can't be across the room or sitting on a chair--you have to be on his level at all times. Every eye roll, head jerk, mouthing motion, and meaningless hand movement indicates that a seizure is occurring. I'd gladly stand a 24-hour vigil for B, except that someone has to play with him, feed him, change his diaper, get his books/toys, stroll him, (not to mention, use the restroom, cook, clean, etc.). Besides, it's not fair to seemingly ignore a baby even if it is for his own good.

So, I've been searching for seizure tracking resources. There are all kinds of spreadsheets and programs. One that I thought would be particularly useful is called SeizureTracker.com. Seizure Tracker is a free online program. You can print reports out from it and there is even a mobile version that works well on my iPhone (if AT&T's EDGE network isn't acting up). Furthermore, multiple parties (such as Mom and Dad) can be logged on to the same account thereby automatically syncing the seizure log! Seizure Tracker is fantastic if you've got at least a minute or two between seizures.

Unfortunately for Bertrand, Seizure Tracker proved too cumbersome in the handling of rapid succession seizures. The online nature of the program resulted in seconds wasted waiting for the page to refresh and the inability to log seizures when there was no internet connection (damn you, EDGE!). Also, for our purposes, it was too much of a good thing. There was no option to hide unnecessary values such as "Mood" or "Possible Triggers", which while I'll agree are important, can't be filled out while the next seizure is already occurring.

Simply having a downloadable, offline app of the program which syncs with the online version (for when the network is down) would be a huge improvement. Integrating the app with the iPhone's timer or the computer's built-in clock would also aide in working with rapid fire seizures. And, while I know it asking much of a free program, the ability to hide fields such as "Mood" and "Possible Triggers" would be helpful. However, if you have a child with a more traditional seizure disorder, these aspects may not be as vital, so Seizure Tracker will work great.

So, how am I going to solve Bertrand's seizure tracking issue? I've decided to track his seizures for one hour every day and extrapolate from it. That hour will be from 2PM - 3PM. This time is exactly in the middle of both Keppra doses, so the medication level in his blood should be fairly representative. This is also after his lunch and before his snack, so it should be a good time to observe Bertrand au natural. Using shorthand I'll log his seizures in a notebook, and adjust my logging system as needed. By tracking just this one hour a day we hope to see the effects of the ketogenic diet, and if we're successful with the diet, we can graduate to using Seizure Tracker!

Review: "Keto Kid"

Keto Kid: Helping Your Child Succeed on the Ketogenic Diet is by Deborah Snyder. It was one of the few ketogenic diet books still in print on Amazon.com. At only 160 pages, I accidentally finished it in about two hours last night. You'll find what I liked and didn't like about the book below. If I could do it over, I'd try finding it at the library or buy a used copy online instead. The parent of an older child with a more conventional seizure disorder may find this book helpful, but, as the author makes many assumptions which do not apply in Bertrand's situation, I did not.

What I did like:

• The appendix contained useful templates of notes for a variety of situations to involve and inform stakeholders in your child's situation (friends, neighbors, teachers, grandparents, etc.). These ranged from dealing with classroom parties to dealing with trick-or-treating.
• There is a section on ketogenic recipes which would really help with an older child, including a one that Matthew is looking forward to: cooking with bacon. :)
• The book brought up that you can't use processed foods. I hadn't heard of this before, but at least initially with the diet, everything must be made from scratch. Even Matthew's beloved "bacon" is actually sliced side pork from a butcher because it cannot be salted or smoked like the store bought variety.

What I did NOT like:

• Even though it was published fairly recently in 2007, a lot of the information in it, product information in particular, was a bit outdated. (At least it points you in the right direction and google can do the rest.)
• The book does not cover the ketogenic diet in babies or very young children (under age 5 years).
  
• There is no mention at all of KetoCal, which is a standard formula used on the ketogenic diet.
• There are no ketogenic baby food recipes in the book, but there are some that could be modified for baby.
• The book does not cover the ketogenic diet with substances other than food--toothpaste was the only exception. In very young children on the ketogenic diet, there are concerns with the carbs in soaps, wipes, lotions, etc., especially given the amount of carb those items contain relative to a young child's size and weight.
• Before reading the book, Matthew and I had discussed going on the Atkins diet in solidarity, even if Bertrand is completely unaware of our sacrifice. However, it is not be safe for pregnant women or people with other health issues to limit their carbs! So the author's repeated insistence and deriding of parents who do not assume at least part of the diet with their child was hurtful and potentially dangerous.