April 29, 2012

Targeted Dosing

April 23, 2012  The Wall Street Journal
Treating Epileptic Seizures in Kids by the Clock

Neurologist Develops Individualized Plans for Patients Who Don't Respond to Conventional Drug Therapies

By Dawn Fallik
Tobias Loddenkemper, a pediatric neurologist, works with some of the hardest epilepsy cases—the children whose seizures have been little helped by medication or surgery.

Nearly a third of epilepsy patients don't get sufficient relief from conventional drug treatments. But where advanced techniques don't help, Dr. Loddenkemper hopes a simple solution might: timing patients' medication to better coincide with their seizures.

Epilepsy, which affects 1% of the U.S. population, is a seizure disorder involving a surge of electricity in the brain. Its cause often isn't known, but in children the condition may be congenital or the result of a head injury. When medications can't control seizures, alternative treatments may be attempted, including surgery, strict diets and brain-stimulation techniques. But these have had limited success, according to the Epilepsy Foundation, a patient-advocacy group.

Dr. Loddenkemper, who works at Children's Hospital in Boston, is trying ways to make medication more effective by adjusting dosages based on when a person's seizures typically occur. This month the 39-year-old won the American Academy of Neurology's Dreifuss-Penry Epilepsy Award for young researchers for his work on treatment approaches.

Dr. Loddenkemper's approach seems basic. He asked his patients, or their parents, to keep seizure diaries, a standard practice. From those diaries, he noticed many patients had seizures around the same time every day, but often took the same dose of medication throughout the day. He changed their medication schedule so they took a higher dose when they most frequently had seizures.

"Why give the medication in the morning if the patient isn't having seizures then?" he says. "If they are having more seizures at night, give it to them at night." Although targeted dosing isn't new, "it's slowly growing" as a common treatment, he says. There has also been very little research on its effectiveness.

In a small study published last year in the journal Epilepsy & Behavior, Dr. Loddenkemper and several colleagues at Children's Hospital looked at 17 children who had seizures at night or in the early morning. The researchers varied the patients' doses to administer more of their anti-seizure medication when episodes were more likely to occur, while leaving the total dosage unchanged. After five months, 15 of the patients saw their seizures reduced by more than half, and 11 had become seizure-free.

Elaine Wirrell, director of pediatric epilepsy at the Mayo Clinic in Rochester, Minn., who wasn't involved in the Children's Hospital research, said targeted dosing is sometimes tried if patients have a predilection for seizures at a specific time.

"It's not something that's usually in textbooks," she says. Having studies on the practice "legitimizes the practice and encourages it."



Bertrand's big night seizures occur like clockwork sometime between 11:30-12:30am and 2:30-3:30am, (and occasionally between 5:30-6:30am) every night.  

Since we've heard NOTHING back from the neurology department, today we began the technique discussed in the article above (and in more detail in "Higher evening antiepileptic drug dose for nocturnal and early-morning seizures." Epilepsy Behav. 2011 Feb;20(2):334-7. Epub 2010 Dec 30. [PMID: 21195032]). 

Bertrand is shifting from 250mg of depakote at 7am and 5:30pm to 125mg of depakote at 7am, 125mg at 5:30pm, and 250mg at 10:00pm. So far, he was very alert and happy with no increase in daytime seizure activity. Fingers crossed for tonight.

Victoria's 1st Birthday Party

On April 14th, we celebrated Victoria's 1st birthday with a small party among family and a handful of close friends. We were lucky that the amazing Staci, from Sweet Envy Photography, was there to capture the day!
























April 24, 2012

Syndromes of Status Epilepticus

It was recently confirmed by Bertrand's pediatrician that his most recent EEG was abnormal, and significantly so, during sleep.  His epileptologist wants him to be admitted for a high-dose valium protocol (also known as the Riviello Protocol).  However, the admission process has been tricky since many of the neurologists on-call do not feel comfortable administering and overseeing this rarely used protocol.

It consists, in lay-mommy terms, of cycling between a month of high-dose valium and a month off.  The idea behind it being that it gives the brain time to rest and reset normal pathways.  That said, here is a good presentation on the issues with Bertrand's electrical status epilepticus of sleep (ESES) and the various potential treatments of it, including this high-dose valium (benzodiazepine) protocol.  Enjoy!


Clarifying the Syndromes of Status Epilepticus 

I'll be talking about the off-label use of valproate, clonazepam, benzodiazepines, ethosuximide, prednisone, adrenocorticotropic hormone (ACTH), and intravenous gamma globulin.

We've heard some talk tonight about status epilepticus and convulsive and nonconvulsive status epilepticus. I refer to what's been discussed as overt status epilepticus. This would be the status epilepticus syndromes that are associated with acute seizures or an acute encephalopathy.

I call some of these epileptiform encephalopathies. These would be specific epileptic syndromes that are associated with frequent epileptiform activity on the electroencephalogram (EEG). Frequently, we may see these during sleep, so we may have an EEG that becomes very sleep-activated. This would be called electrical status epilepticus of sleep (ESES). There's also an epileptic syndrome that's called ESES, which is important to differentiate from an EEG, which may be a very sleep-activated EEG. These can be referred to as epileptiform encephalopathies.

I use epileptiform encephalopathy to define something in which the primary clinical manifestations result from the epileptic activity, the epileptiform features on the EEG, and its resultant dysfunction, rather than an actual clinical seizure. Sometimes it can be difficult in differentiating these.

What we're focusing on are patients who may clinically not have many seizures, but have EEGs that are very epileptiform EEGs. Not all of these patients may have overt clinical seizures.

One thing that can be very important in these disorders is regression in either intellectual or cognitive ability. If you see regression in either intellectual or cognitive abilities, that has to raise the suspicion that we may be dealing with some of these very sleep-activated epileptiform encephalopathies.


April 16, 2012

The State of Bertrand's Care

Today, Matthew and I met with Bertrand's wonderful pediatrician to discuss the state of his care. She was 100% on the same page as us, and is now organizing a meeting with all of Bertrand's providers through the palliative care team.

While the neurology department issues were discussed at length (and are to be continued...) we discussed all aspects of Bertrand's care including: an impending trip to Duke University for study results, functional electrical stimulation (FES), and potty training.

Upon examination, his pediatrician remarked on Bertrand's improved attention span and muscle tone. She also ordered labs to be drawn when Bertrand goes in for his dental and eye exams this month July.

We were all very pleased with the outcome of this visit, with the exception of Victoria* who received her 12 month vaccinations. :)


*As usual, Victoria stole the show by wearing fairy wings, running through the hospital, smiling and waving at everyone. She remains 50th percentile for head circumference, only 10th percentile for height, 15th percentile for weight, and well ahead of her peers developmentally in every aspect. Whew! I doubt we'll ever stop worrying, but at least she tries to make it on us. :)

April 14, 2012

Happy 1st Birthday, Victoria!

Our darling daughter turned one today.

Yet, over the past year, she has become more than just a daughter.

She is also a symbol of healing--and of hope fulfilled.

For Bertrand, she has become his constant companion and his very best friend.

She encourages him to push his limits, and he returns the favor.

We are so happy to have Victoria in our lives.

We look forward to many birthdays to come.

April 11, 2012

Thin Mints

It's pretty much a universal fact that Girl Scout cookies are addictive. I don't even like them that much, but an entire box sleeve will disappear. I think that's why Matt found this display of altruism and care-taking on Victoria's part so impressive. It's one thing to share a cookie. But, to share a Girl Scout cookie? Now, that's LOVE! :)

April 10, 2012

Sweet Success!

This evening, representatives from the MOMS Club of Salt Lake City participated in the first ever Little Miss Hannah Foundation Lollipop Workshop! The lovely participants learned how to make, decorate, and package lollipops at home. They proceeded to make 55 delicious treats to serve as a fundraiser for the Foundation, in the following flavors: cinnamon red hot, pink lemonade, sour apple, sour grape, and guava. I am so proud of these ladies! :)

I took this picture after half the group had left! Oops!

April 8, 2012

Natural History Museum

Today, we went to the new Natural History Museum of Utah with titi Aury, titi Sabrina, and uncle Steve. This was our first time going with the kids. While we knew that the adults would love the museum, we didn't anticipate the huge hit it would be with the kids!

Bertrand insisted on getting out of his wheelchair to participate on many of the hands on activities! He *stood* and played at the water table, played with puzzles, and spun many of the interactive signs. Of course, Victoria was running and playing with him, as well as pointing out all the animals (you can see her trying to feed the wolf below).

The museum is 5 minutes from our house, so I hope to get more photos in the visits to come. As we left, we purchased a family membership because anything that will motivate Bertrand to get out of his wheelchair like that is WORTH IT.

April 5, 2012

Working Out

Bertrand working hard with Miss A!

This afternoon on our porch, Bertrand walked 6 laps at the blistering speed of 0.075 mph!
I'm calling him "Lightning McQueen" because he is so fast. He is AMAZING!
I'm very proud of both my boys today. :)

April 4, 2012

Lollipop Workshop!

You too can make exquisite lollipops like these! For a great cause!

On April 10th, I'll be holding a Lollipop Workshop* with the MOMS Club of Salt Lake City to benefit the Little Miss Hannah Foundation!

Making homemade lollipops is fun, quick, easy, and delicious! (My husband says they taste more like a giant jolly rancher on a stick. Yum!)

I'll be teaching the basics of hard candy making. We'll be using basic quick release molds and cake decorating supplies, that you can find at party supply stores.

We will also discuss and practice with different forms of lollipop presentation, such as making lollipop pencil toppers and lollipop "fairy wands".

(I will furnish candy making supplies, but if a few participants could bring extra sauce pans, candy thermometers, and/or clean cookie sheets, we'll be able to make more batches.)

Everyone who attends will come away knowing how to make their own lollipops quickly and easily at home. You'll take home a handout with instructions, recipes, and of course a few, delicious samples for the kids.

I promise that even the "big" kids will be impressed! ;)

To top it all off, this workshop will benefit a fantastic cause! The Little Miss Hannah Foundation was recently started in honor of Bertrand's little girlfriend, Hannah. She was only 3 years-old when she passed away from a rare disease last December.

The goal of the foundation is to work with families who have young children with life-limiting rare diseases, undiagnosed complex medical needs, and children who have been placed in hospice or palliative care. The aim is to give parents the necessary tools to empower them to meet their child’s unique medical and lifestyle needs, as well as provide support resources and special attention for their other children.

To learn more, please visit littlemisshannah.org.

*Appetizers and drinks included.

April 3, 2012

Power Wheelchair Reconsidered

Yesterday afternoon, Bertrand went to Shriners Hospital to see the physical therapist in regard to the power wheelchair.

We have been practicing hard and at a minimum of three times a week, as per the therapist's direction. The therapist was deeply impressed by our dedication. Bertrand has made a great deal of progress. We couldn't be more proud of him.

However, the therapist at Shriners didn't see Bertrand's hard work and efforts in the same light.

First he said that insurance wouldn't cover the chair for Bertrand. So we found ways to get one donated and pay for one.

Then he said that Bertrand would need to practice 3 times a week. So we found ways to get Bertrand to practice at least 3 times per week.

Now he said that Bertrand's cortical visual impairment would likely prevent him from passing the safety requirement...

The pieces of the puzzle finally fell into place. He won't recommend a power wheelchair for Bertrand no matter what we do.

He had already made up his mind about Bertrand. He was just being obtuse about letting us know it.


Bertrand's cheerleading squad is vast. (And, I love you all for it!) So, whenever someone doesn't pick-up the pompoms, I am confused and, honestly, sad.

But, I think this is actually an opportunity in disguise. We were back to spreading ourselves a bit thin. (I know. I know. It's chronic. Apparently, I can't help it!) This situation has me re-evaluating the merits of a power wheelchair.

While Bertrand does enjoy the power wheelchair, he also increasingly enjoys standing up and taking steps--activities which are conveniently assisted by the TAOS. Such weight-bearing activity is great for his bones, his muscles, and his bowels. Overall, much better for him than using a power wheelchair.

Maybe the power wheelchair was a distraction? Maybe the therapist at Shriners actually did Bertrand a favor?

On our drive home from the appointment, I told Bertrand, "Forget that guy. He doesn't know you. You'll WALK. That'll show him!"

And when he does, you better believe he'll be surrounded and cheered by the people who believe in him.

April 2, 2012

TAOS Fitting

Bertrand was fitted for and received his TAOS today. He is the 6th child in the state of Utah to receive one. He was a great sport throughout the 2 hour fitting process, and was gracious enough to take some steps for the camera. We look forward to capturing more precious steps of his in the future. :)

Wow! This month has already been one of many firsts and it is only April 2nd!