Face of Cannabis

Can you spot baby Winston?  :) (Photo credit The Salt Lake Tribune.)

Just a quick post to let you know we are alive but busy!  Yesterday we hosted an event for the "Face of Cannabis" Project on behalf of the Epilepsy Association of Utah and Hope for Children with Epilepsy at the wonderful Holodeck.  It was a joy and honor to work with the talented Nichole Montanez and the 22 Utah families who came out to share their stories and support.  Together, we will keep fighting to make treatments available to all kids who need them!

Utah Hemp Extract Registrant #1

This morning, Bertrand became the first person in the state of Utah to receive a Hemp Extract Registry Card.

While Bertrand may not receive CBD oil for quite some time, we wanted to show our support for the new law on the very first day.  

The only time I could fit a trip down to the Utah Department of Health was at 9:45am, after school bus pick-up, camp drop-off and some errands, but before school bus drop-off, camp pick-up, and a doctor's appointment.  (And, let's not forget, nursing baby.)

Since the Office of Vital Records opens at 8AM, we did not expect to be the first in line.

There was a bit of confusion from some of the staff when we first arrived, but things were quickly straightened out.  

Since all of our paperwork was in order ahead of time, getting the card only took 30 minutes (and should be faster for subsequent registrants).

The Hemp Registry Instructions on the Utah Department of Health website were very thorough and easy to follow.

The Department of Health is accepting applications via mail or in-person.  

For the first week only, walk-ins are welcome, but appointments will be required in the future.

Please see the Utah Department of Health website for more information.

I broke my no-selfie rule to show Bertrand one of the kind registrars at the Dept. of Health, Leisa Finch, who is helping kids like him get access to these cards.  

A big hip hip hooray for the state of Utah, our representatives and senators, and the fantastic folks at Hope 4 Children with Epilepsy who made this all possible!

Seizure Smart-er

Matthew and I attended the Epilepsy Association of Utah's Seizure Smart Conference.  Given Bertrand's impending travel, we could only attend a few sessions.  The topics were cannabidiol extract, new legislation, and the genetics of epilepsy.  It was an informative and motivating conference.  We were glad we went!  The folks at EAU did a fantastic job, as did all of the speakers.

Look Niki!  We missed you.  You should have been here!  :)

The amazing Heather Jackson with The Realm of Caring.

The fantastic Jennifer May with Hope 4 Children with Epilepsy.

TOMORROW: Get Seizure Smart!

2014 Epilepsy Education Conference

Get Seizure Smart! Educational ConferenceJoin us for the 7th Annual Get Seizure Smart! Educational Conference.

June 7th, 2014
8am to 4pm
Little America
500 S Main St
Salt Lake City, UT
84101 

To Register - please fill out this form and remit payment.

"Change is the end result of all true learning."
- Leo Buscaglia

This past year, the changes that have occurred in the epilepsy community include new diagnostic tools, new treatments and more research into what causes epilepsy and what can help stop seizures. The EAU is pleased to have some of the experts in this field of study and practice, speaking at our annual Get Seizure Smart! Education Conference.

Some of the respected speakers include:

Keynote Address:

---

Genetics of Epilepsy: The Role of Dravet Syndrome
Dr. Ian Miller - Miami Children's Hospital


Special Guest Speakers:

---

Cannabidiol for Seizure Management
Heather Jackson - Executive Director - Realm of Caring

Epilepsy 101 for Adults
Dr. Jeffrey Bigelow, MD, MPH - Neurologist at Intermountain Medical Center

Epilepsy 101 for Pediatrics
Dr. Matthew Sweney, Assistant Professor, Pediatrics and Neurology - Primary Children's Hospital

The State of Cannabis Oil
Jennifer May, Co-Founder: Hope 4 Children with Epilepsy,

The genetics of epilepsy and personalized medicine: What does the future look like?
Dr. Reid Robison, Chief Executive Officer, Tute Genomics Inc.


Honored Speakers:

---

The Practical Application of Chiropractics for Epilepsy
Dr. K.C. Oliver - Board Certified Chiropractic Neurologist, Founding Partner - The Neuro Clinic in Lehi UT

Trusts and Living Wills
Jared Allebest - Adjunct Professor, UVU - Owner, Allebest Law Group

Empowering Parents: Caregiver resources and how to use them.
Kim Orton - Pediatric Epilepsy Care Coordinator for the University of Utah, School of Medicine at Primary Children's Hospital.

Funding, Medicaid and SSI
Jodi Hansen - Utah Parent Center and Utah Family Voices.

Neuropsychological, Behavioral, and Social Challenges Associated with Epilepsy
Dr. Jenise Jensen - Pediatric Neuropsychologist

My Personal Experience and Accepting Epilepsy
Chris Connor - Youth Leader, Epilepsy Association Board of Directors

Essential Oils and Mental Health
Leslie Reader - Wellness Advocate 

Epilepsy Education Conference

Purple Day 2013

Another year, another Purple Day. Purple Day is an international grassroots effort dedicated to increasing awareness about epilepsy worldwide. On March 26th people in countries around the world are invited to wear purple and host events in support of epilepsy awareness.  The Bertranator wore his purple today.  How about you?  :)

"I'll be back... after school."

Get Seizure Smart! Educational Conference

This Saturday, join the Epilepsy Association of Utah for the FREE 10th Annual Get Seizure Smart! Educational Conference.

Saturday, November 3rd, 2012
9am to 1pm
University of Utah
Clinical Nuerosciences Center
Auditorium Level
175 North Medical Drive
Salt Lake City, UT 84132
Topics Covered:

• Epilepsy 101 
• Latest Research and Development 
• Treatment Options 
• Art and the Brain Connection

ARTILEPSY: Visualizing Hope

The Epilepsy Association of Utah and the University of Utah Clinical Neurosciences Center present ARTILEPSY, an exhibition of original artwork, photography and home crafts by people of all ages living with Epilepsy. This evening illustrates the profound effect that epilepsy can have on human life and the courage, humor and imagination many show in facing it.

Entries due FRIDAY October 26th!

Exhibit Open November 2 - 30th.

Dine at IHOP for the Epilepsy Association of Utah

You know, some days, eating is just eating. Then there are the meals spent at IHOP. On those special days devouring at IHOP, why not help The Epilepsy Association of Utah (EAU)?

From 5:00 pm to 9:00 pm on September 26th, particpating IHOPs along the Wasatch Front will donate 20% of their qualifiying sales to help the 100,000 people in Utah living with Epilepsy.

Just bring in a copy of the flyer (Download it here)

to one of the following IHOP Restaurants and the EAU will get 20% of the total cost of your meal! Now that's a meal with a purpose.

This September 26th from 5:00pm - 9:00pm IHOP will donate 20% of their sales to The Epilepsy Association of Utah. All you have to do is print out the flyer to the right, take it in to one of the Participating Restaurants, present it to your server when you order your meal with a beverage and IHOP will send us a check for 20% or your total bill. How cool is that? Help us, while enjoying a meal with family and friends!

When: 
September 26th, 2012
5:00pm to 9:00pm

Where: 

IHOP Ogden
189 12th Street
(801) 621-7000

IHOP Layton
920 North Main Street
(801) 593-0433

IHOP Riverdale
947 West Riverdale Road
(801) 392-4467

IHOP Centerville
388 North Market Place Drive
(801) 296-8000

IHOP Sandy
10815 South State Street
(801) 523-8613

Parent Education Night: Alternative Medical Treatments for Seizures

Pediatric Neurology Parent Education Night: 
Alternative Medical Treatments for Seizures

Thursday, October 11th at 6:00 pm

Primary Children's Medical Center Auditorium - 3rd Floor

Parents are invited to participate in a question-and-answer format presentation about seizures. Topics may include diagnosis, disorders, prevalence, medication and others. This is a chance to ask any questions you may have and to meet other parents who have children with seizures.

Discussion Lead by a Panel of Experts:

• Matthew Sweney, MD
• Francis Filloux, MD
• Paula Peterson, RN, MS, PNP
• Eliza Hamilton, RD, CD
• Kim Orton, RN
• Parent Advocate Marin Bywater, LCSW

Sponsered by the University of Utah Pediatric Neurology Division and Medical Home Demonstration Project as part of a quarterly education night series.

Purplelicious Pageant

Did I really just enter Bertrand and Victoria in a pageant?!  Yes, yes, I did.  (Eep!)  But this is not your typical pageant!  The "Purpleliciuos Pageant" is both an event for epilepsy awareness and a fundraiser for epilepsy research.  Tickets are $5 in advance and $7 at the door.  Come watch Victoria destroy the stage and Bertrand win the "Best Hair" category.  ;)

It's one thing to wear purple, it's quite another to be Purplelicious! Do you have what it takes to strut your stuff and show the world your fashionista? Maybe the Purplelicious Epilepsy Awareness Pageant is for you! Application deadline moved to August 3rd. 

What:   Purplelicious Epilepsy Awareness Pageant 

When:   September 15, 2012 (DATE CHANGED!) 

Where: Best Western Cotton Tree Inn in Sandy, UT 

Time:   9:00am - Registration, 10:00am - Pageant 

The Purplelicious Pageant is the brain child of Patty Gannon and her Granddaughter, Courtnie Bartholomeusz. Patty and Courtney are working to get the word out that Epilepsy is nothing to be feared, it is something you have, not something that has you. Be prepared, not only should you be lovely, you have to be lovely IN PURPLE. That's right, it's Purplelicious! 

Visit Purplelicious Pageant Application to download your application today!

Dr. Dravet to visit Salt Lake City

Salt Lake City, UT June 21, 2012 – At the invitation of the Epilepsy Association of Utah, world renowned French Epileptologist, Dr. Charlotte Dravet, will visit Salt Lake City on August 15-18, 2012. Dr. Dravet has agreed to meet patients affected by Dravet Syndrome, a rare form of Epilepsy named after her research efforts. Patients are invited to meet one on one with the doctor on Monday, August 13. Dr. Dravet will also be presenting an “Introduction to Dravet Syndrome” on August 14 from 9 – 11am for all medical professionals wanting to attend. Please RSVP to this event by visiting www.epilepsyut.org/dravet_registration.html.

“The opportunity presented to the people and medical community within Utah is unparalleled”, said Kris Hansen, President of The Epilepsy Association, “Never before has the Epilepsy Community in Utah had the occasion to meet someone of her caliber. We are honored that she agreed to spend so much time here. It’s a once in a lifetime chance”.

Schedule of Meetings/Events

Monday, August 13:
9:00am – 4:00pm     Patient Visits at University of Utah

Tuesday, August 14:
9:00am -11:00am     Introduction to Dravet Syndrome
                               Dr. Dravet presenting along with doctors from UofU and Primary Children’s
12:00pm                  Lunch – By Invitation Only
2:00pm                    University of Utah Research Lab Tour
7:00pm                    Dinner with Dr. Dravet - A minimum donation of $75.00 a plate is suggested

Wednesday, August 15:
10:00am                   Grand Rounds at University of Utah

If you would like to participate in any of these events or have any questions, please contact:

Jenn Whiting
jenn@epilepsyut.org
or
Kris Hansen
kris@epilepsyut.org
(801) 566-5949

ABOUT DR. DRAVET
Dr. Charlotte Dravet was mentored by Dr. Henri Gastaut (Lennox-Gestaut Syndrome) at the Centre St. Paul in Marseilles. Her interests were focused towards the explanationof syndromes in childhood Epilepsy, the psychological consequences of Epilepsy, the progressive Myoclonus Epilepsies and their genetics, the congenital malformations of the offspring of Epileptic women and related studies. Her main contribution was in Myoclonia Epilepsy; with emphasis on benign and severe Myoclonic Epilepsies in infancy. Dravet Syndrome, is now recognized as one of the most malignant syndromes at this age and is widely studied by other authors and clinicians as well as geneticists.

According to Davet.org, “Dr. Charlotte Dravet first described Dravet syndrome in 1978 as Severe Myoclonic Epilepsy of Infancy (SMEI). The first seizures usually happen before one year of age, with no known cause other than fever or illness. Seizures progress to be frequent and do not respond well to treatment. They also tend to be prolonged, lasting more than 5 minutes. Other types of seizures, which are common in Dravet Syndrome, appear in early childhood, including myoclonic seizures, atypical absence, complex partial seizures and nocturnal seizures. Estimates of the prevalence of this rare disorder range from 1:20,000 to 1:40,000 births.”

Dr. Charlotte Dravet has been President of the French League Against Epilepsy and a member of the Commission on Classification of Epilepsies of the ILAE. She has participated in numerous congresses, lectures and is the author of a number of published works. Now retired, she continues to work in France and Italy to improve the care of people with Epilepsy.

ABOUT THE EPILEPSY ASSOCIATION OF UTAH
Founded in 1973, The Epilepsy Association of Utah is a 501(c)(3) charity dedicated to enhancing the quality of life for all individuals living with Epilepsy and seizure disorders. 1 in 26 people will develop Epilepsy at some time in their lives leading to over 150,000 people in Utah alone. Epilepsy is the 4th most common neurological disorder in the US after migraine, stroke and Alzheimer’s. The Epilepsy Association of Utah offers a public education program, statewide support groups, personal and professional advocacy, college scholarships, art exhibits, educational conferences, summer camp, and more. Visit http://www.epilepsyut.org for additional information.

Press Contact:     Lindsey Palmer
Email:                lindsey@epilepsyut.org
Website:             www.epilepsyut.org
                          www.dravet.org

Tonsillectomy?

Bertrand as he was discharged last week.

Bertrand wakes up a lot at night.  His prior sleep study was hard to interpret due to his epilepsy.

However, it's believed that he may have some sleep apnea.  He will be evaluated by an ENT since his tonsils are a bit large.

If the doctor believes there is merit, Bertrand may get his tonsils removed.  This may actually be easier and less costly than another sleep study.

Despite having a referral, getting in to see the ENT has been a nightmare.  I'll be going in person tomorrow.

Complications from Depakote

The likely culprit for Bertrand's recent hospitalization was depakote.  This has been the most effective seizure medication for him to date, but it has many nasty side-effects.  We were aware of many side-effects, including liver damage, and were monitoring him for symptoms.  Now we have to watch for a few more: hyponatremia (low sodium) and bone marrow suppression.

Bertrand caught a typical virus.  (He goes to preschool, therapy, dance, playgroup... he could've caught a bug anywhere.)  And then entered a vicious cycle, due to undiagnosed hyponatremia and bone marrow suppression.  The hyponatremia alone could've killed him.  Normal values are 135 mEq/L.  He was at 4 mEq/L.  The bone marrow suppression resulted in low counts for all blood cell types--white, red, and platelet.  To fight an infection, you need plenty of all of the above.

We are incredibly lucky that Bertrand got sick exactly when, where, and how he did.  He was already in the hospital (for a different procedure), with the A-Team for doctors.  They acted fast, and he is now on the mend.  Bertrand sure knows how to keep things interesting!



A few more items learned from this week:

The targeted dosing greatly changed Bertrand's nighttime EEG!  He is no longer in status epilepticus at night, which means the valium protocol is no longer right for him.

The looping episodes, of which multiple were caught on EEG, are NOT seizure activity.  In fact, Bertrand's EEG is almost normal at those times.  These are periods of alertness and a manifestation of his movement disorder.

Once Bertrand is well, we will conduct another sleep study to see if apnea is waking him at night, thereby triggering those looping episodes.

We will also see about lowering/eliminating Depakote, and/or we may continue to tweak his medication dosing to target the night seizures better.  As a substitute for Depakote, Bertrand may try Clobazam.



Next Monday, Bertrand has appointments with his pediatrician and his metabolic doctor to follow-up.  He'll be getting labs drawn then too.

To be on the safe side, I've ordered some salt tablets for him.  Each pill contains: 215 mg sodium, 63 mg potassium, 11 mg magnesium, 22 mg calcium, 100IU Vitamin D.  Bertrand can take 1,200mg of sodium per day, so depending on what he has to eat on any given day, he may get 1 or several.

Paranoid much? me?!  Nah.

Valium Protocol - Failure to Launch?

Bertrand battles a possible UTI as well as seizures.

As mentioned way back when, Bertrand was to undergo the Valium (aka Riviello) Protocol.

Maddeningly, the neurology team at our hospital fumbled his admission for months.

So, the head of pediatric neurology decided to oversee Bertrand's initiation himself.

This afternoon, Bertrand was finally admitted for the procedure.

And promptly developed a fever and possible infection.

He is currently being evaluated for strep and UTI.

Depending on Bertrand's condition tomorrow, the protocol could be halted before it even began.

Seeing Bertrand sick is sad, but the thought of months more seizures is heartbreaking.  :'(

Here's hoping our little buddy gets well soon.

The Return of Atonic Seizures

The last 5 seconds of the video capture the drop attack.

For the past couple of weeks I've been trying to fool myself into thinking that Bertrand was just having some really big myoclonus. Nope. It's clear the drop attacks (atonic seizures) are back.

The last time we saw Bertrand have a drop seizure was in May 2010.  ACTH treatment, while it almost killed him, bought us 2 years of drop freedom.  Now, we're going back in the ring to fight them.

Doose Syndrome Research Study

The Doose Syndrome Epilepsy Alliance in coordination with the Gene Partnership at Children’s Hospital Boston and the Manton Center for Orphan Disease Research is proud to announce a new research project.

This is an exciting step for us. If your child has a Doose syndrome diagnosis (myoclonic astatic epilepsy aka epilepsy with myoclonic atonic seizures) then we want you! This is a new sequencing pilot project for clinical and research use. This initiative will allow for investigators at Children's Hospital, with approved IRB protocols, to submit samples for whole genome sequencing or exome sequencing. An inadequate understanding of Doose syndrome’s underlying causes is the greatest obstacle standing in the way of new treatments and cures for orphan diseases, like Doose syndrome. Many disorders are lumped together under a single diagnostic umbrella, making it difficult for physicians to identify appropriate and safe treatments. Further studies could uncover an underlying genetic flaw and open avenues of research to treat or correct the error.

Instead of individual scientists working in "silos" to understand specific orphan diseases, the Manton Center fosters collaborations between investigators to share ideas, resolve common challenges and attack orphan diseases from multiple perspectives. The Manton Center's environment also attracts new experts and resources to address challenging issues, accelerating the discovery process.

Armed with the necessary research tools, financial support and collective determination, investigators at Children's Hospital have created an interdisciplinary program to narrow the gap that separates the study of orphan diseases from mainstream medical research.

The first step to involve yourself in this critical research is to register your interest with Heather Jackson at info@doosesyndrome.org. Please call directly at 719-491-7340 if you have further questions. From there I will connect you with our contact to ensure that all of the data on our Doose syndrome children is submitted and ran together, which will strongly increase the probability of making links.  This research will be conducted at no cost to you, however the investigators will have to coordinate orders for blood work with your physician.

Best Regards,  

Heather Jackson

Doose Syndrome Epilepsy Alliance

www.doosesyndrome.org 

FAQ's

What if my child has "probable Doose syndrome" or their diagnosis has changed? Please by all means please register.

What if I am not in the US? No problem we will work with your physician to ensure participation.

What if I am not a patient at Children's Hospital Boston? Not a problem, you will consent.

Will I have to travel? No, you will do the blood work at your local lab with orders from the Manton Center.

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."

READ MORE.

*************************************

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.

"Surgery for Epilepsy Gains Urgency in Trial"

By NICHOLAS BAKALAR
The New York Times
March 12, 2012

Surgery for epilepsy is usually seen as a last resort for patients when medications do not work, and it is often delayed for many years after the failure of drug treatment. Now a randomized, controlled trial suggests that surgery as soon as possible after the failure of two antiepileptic drugs is a significantly better approach than continued medical care...

Read more HERE.