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:

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


Special Guest Speakers:

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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:

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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 

Preschool Twilight Zone

Victoria loves Titi Karen and R!  R is one of her best friends.

Today we went to visit a preschool for Victoria.  Oddly enough, this is the preschool I'd looked at for Bertrand aaages ago when we first moved to Utah.

The school is still fantastic.  (Victoria is now on the waiting list.)  But, the experience was surreal.

Victoria didn't hesitate to start playing with the teacher and other kids.  Despite some uncharacteristic shyness, she loved it.

Bertrand could barely sit on his own the first time he was there.  Even now he can barely sit on his own.

It was weird to feel time fast forwarding for one child, but frozen for the other.

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Later at lunch with friends, Victoria had the time of her life playing with her best friend, R.

R is a little boy Bertrand's age, and I am constantly amazed by how sweet, kind and nurturing he is with Victoria.

It's a little bittersweet at times, since I wish Bertrand could play with her like that.  I can see how much his attention means to Victoria.

I am grateful that we have good friends like R to fill Victoria's desire for friendship AND to take the intense little sister heat off Bertrand.  :)

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We're recovering from our colds.

Next week once Bertrand is stabilized, we will adjust his medication as per discussion with his (new) neurologist.  We'll be tweaking his lamictal and depakote dosage and timing.  Our aim is to increase seizure control while minimizing cognitive impact.

Nothing ventured, nothing gained.

Keeping things interesting - Take 2

Remember that post yesterday where I said, "Matthew and I are increasingly optimistic that he could be discharged tomorrow"?

HA. HA. HA.

Bertrand's discomfort and heart rate skyrocketed this morning.  His heart rate broached the 180s and stayed in the 170s for a large portion of the day.  He was transferred to Peds and then the NICU team got involved.

After lots of oxycodone, epinephrine breathing treatment, intravenous methylprednisilone (a.k.a. SoluMedrol), intravenous morphine, intravenous antibiotic, 3 boluses of fluid on top of his regular maintenance fluids, (and more steroids on the way)... Bertrand's heart rate is down to the 140s and he is much more himself.

We still don't know what went/is wrong.  Many of his labs, including the blood culture looking for signs of infection, are still pending.  However, we do know that his white blood cell count is high but his cortisol is low, which isn't helping any if he is trying to fight an infection (hence the additional steroids on the way).

So, yes, Bertrand is still keeping things interesting.  ;)  Fortunately, he is in good hands and we expect he'll be feeling better soon eventually.

PS - Happy birthday, Titi Saby! We wish we could be celebrating with you with all our hearts.  We love you!

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.

Parent Education Night: Seizure Medications

Pediatric Neurology Parent Education Night: Seizure Medications

Friday, Februay 24th 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:

• Colin Van Orman, MD
• Jeffrey Ekstrand, MD, PhD
• Denise Nielsen, MD
• Shari Combe, PA-C
• 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.

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Bertrand has a 2-day video EEG scheduled that weekend.

Conveniently, he'll be inpatient at PCMC, which means we'll be in attendance!

The Happy Mystery

Bertrand is the medical mystery that keeps on giving. Here are two of his most recent puzzles.

The Case of the Disappeared Blink.

At a recent ophthalmology appointment, a doctor noted that Bertrand doesn't blink. His lack of blinking is new. This appears to be the new cause of his corneal erosion. While Bertrand produces tears and can cry now, his corneal erosion has persisted. I immediately flashed to this post by my friend, Carrie, about her daughter Hannah's loss of blink reflex. Lack of blinking is a symptom of Parkinson's disease, with which Bertrand's condition shares some similarities. Blink rate has a direct correlation with dopamine levels. In low dopamine conditions such as Parkinson's, individuals may barely blink. In high dopamine conditions such as schizophrenia, individuals may have a very high blink rate. Certain medications have as a side-effect dopamine reduction and resulting blink reflex loss. The ophthomologist, citing possible drug interaction, decided to write a note to inform Bertrand's neurology team of the finding.

With the neurologists both M.I.A. (yup, the new one is equally unresponsive and Bertrand's EEG has yet to be scheduled), I called my dad. He played the reasonable Watson to my flighty Sherlock. Bertrand's CSF dopamine has tested normal. His movement disorder doesn't present like a low dopamine one. However, corneal sensitivity is what triggers the blink. Both Bertrand's corneas have scarred. Scar tissue is not sensitive. Perhaps it is that simple? We like simple and non-invasive. Bertrand will undergo a non-invasive corneal sensitivity test at his next ophthalmology appointment.

(Regardless, the course of action is lubrication, lubrication, lubrication. Drops don't cut it. Big B is a gel and ointment man. Good times.)

The Happy Mystery

Since Monday, Bertrand has been happy. REALLY happy. Like, perhaps the-happiest-he-has-ever-been-in-his-entire-life happy. Obviously, this is a good thing. And, alert--he stopped taking naps during the day. That is HUGE. However, we don't know what triggered it. And, it bothers me like you wouldn't believe! I lay awake at night trying to figure it out. With Bertrand, we take nothing for granted. If I don't find the root, then I'll keep waiting for the other shoe to drop. We may accidentally mess it up. Or, we could miss the opportunity to make him even happier by doing more of whatever we did right. Neither is acceptable to me. There are a lot of variables, but here are some candidates:

• Stopped administering ursodiol (Friday afternoon)
• Switched to a more complete multivitamin
• Resumed application of the B12 patch
• Changed brand of l-carnitine supplement
• Restarted dairy after 8 weeks since he's not allergic (Sunday night)
• Added whey protein smoothies for lunch
• Started riding school bus (Just kidding! That was Tuesday)

Despite our beloved gastroenterologist's claim that ursodiol is a fairly benign bile salt, it is my primary suspect. This case remains open.

Inflammatory Bowel?

Bertrand looking enthusiastic.

"You poke my belly and now you want to do WHAT to my butt?!"

At Bertrand's GI appointment today, the doctor told us to stop his medication ursodiol/actigall because his liver enzymes have normalized. (Bertrand was on 180mg 3 times a day for over a year and a half.) At the follow-up appointment in 6 months, if Bertrand's liver enzymes have risen again, he will reinstate the ursodiol. But for now, that's one less medication! Yippee!

Also, Bertrand's faecal calprotectin test came back elevated. This usually calls for a colonoscopy. But, because this test has some false positives and the colonoscopy is a sedated procedure, the doctor ordered a repeat of the test, which is just a fecal sample. The reason this test was ordered in the first place was to check for inflammatory bowel (not to be confused with irritable bowel). The bowel is where many vitamins are absorbed, and chronic inflammation interferes with vitamin absorption. Causes could be anything from Crohn's disease (not likely) to metabolic disorder (duh), so now that's on the table. Fingers crossed for a normal result!

P.S. - Bertrand's weight is much better but the doctor still wants him to lose more. :(

Timeline

At Bertrand’s neurology appointment last week, the doctor laid out the following timeline:

• Follow-up appointment in 4-6 months
• Revisit medication in 4-6 months
• Repeat EEG in 6 months
• Repeat MRI in 2-3 years

I’ll admit to feeling some frustration upon hearing this but, as I noted in an earlier post, some times it takes doctors a bit longer to get on the right page. ;)

Fortunately, the neurologist is no longer key to understanding Bertrand’s case management. She never was. Bertrand was the key.

While some extra non-invasive tests, like an EEG & MRI, would give me peace of mind and validate the vitamin deficiency theory, these test are not necessary. I can see, with my own eyes, Bertrand’s seizures disappear, and I can see him progressing developmentally.

This is just a crazy theory, but I think that if we didn’t completely miss Bertrand’s growth spurt, his head measurement may increase since being on the vitamins B12 and A. He has microcephaly (his head measures 48 cm), but that can be caused by B12 deficiency. In young B12-deficient children, the microcephaly can correct itself in a few months with supplementation. I hope this is the case with Bertrand.

Last but not least, Bertrand’s seizure medications have already been decreased (without MD buy-in) with no additional seizures. And, in another few weeks, given continued seizure freedom, we will cautiously and slowly reduce the medication further. I would love to have a doctor’s help with this, but she wants us to wait months just to discuss the drugs!?

Despite the fact that Bertrand has been on Lamictal since November (9 months) and Depakote since February (6 months), she implied that the sudden disappearance of Bertrand’s seizures, within one week of starting the vitamin-B12 in late August, was most likely due to the lamictal and depakote! If we don’t lower the drugs ourselves, Bertrand’s neurologist will claim that the drugs are to thank for the seizure control. And, she is wrong.

She is not listening to me. If she doesn’t listen to me, then how can I listen to her?

Vitamin A Deficiency

Everyday since beginning supplementation, it has become crystal clear to us that Bertrand was extremely deficient in vitamin A.

We have seen a lot of changes in Bertrand and, almost every time we google, it turns out that vitamin A is involved.

Vitamin A is important for more than just tears. Pretty much every moisture producing gland in your body (and there are LOTS) uses vitamin A.

So what are some of the changes we see in Bertrand? (Beware, there may be TMI for some.)

• Tears - Self explanatory.
• Styes - These are blocked oil glands in the eye lid. Now that the oil glands in his eye are coming back on-line, he has had at least 10 of these bad boys. I have to scrub his eyelids twice daily so they pop on their own. Lovely, right?
• Cradle cap - Also known as seborrhoeic dermatitis, this is caused by overactive oil glands in the scalp. It has gotten better after using Head & Shoulders shampoo, but there are still plenty patches left to pick off. Double lovely.
• Cough - Turns out that the lung has moisture glands that help with respiration. If they are going into overdrive like all his other glands, I can imagine it's uncomfortable. He is coughing a LOT. Maybe all the productive hacking will FINALLY get rid of that patch in his lung from pneumonia over a year ago. I can hope, right? (Coincidentally, his oxygenation high value rose from 95% to 100% for the first time. This may also be B12.)
• Snot - Oh. My. Goodness. There are rivers flowing from this child's nose for the first time EVER. I wipe his nose, turn my back for 2 seconds, & turn back to see his face covered again. Every tissue box in the house is depleted. Moving on...
• Bowel movements - He is having at least one every day--no miralax needed. They seem to have a coating?! Google... yup, your intestines all have mucous glands. I'll stop here.

I didn't even realize how weird (and time consuming) it was to slather my child in Cetaphil and Aquaphor multiple times a day, shove Ayr up his nose nightly, add Miralax to his drinks, set-up the humidifier in his room, put drops or ointment in his eyes every 2 hours... until now.

And, to be honest, I am angry. Furious, even.

It's more than the scar on the cornea of his right eye. It is every strained bowel movement he suffered. Every time we had to pin him down, screaming, to put drops in his eye, or dig un-yielding snot rocks from his nose. Every cold he couldn't shake. Every hospitalization. Every tear he couldn't shed.

All the pieces of the puzzle were there. So why did I have to be the one to put it together?! We've paid "experts" hundreds of thousands of dollars to poke & prod my poor child. And, at least for these symptoms, a simple vitamin supplement was what he needed all along?!

I am horrified. And, not just for Bertrand, but for all the other children and their parents, who are also overwhelmed, but lack the energy, stubbornness or chutzpah necessary to second guess the experts.

Corneal Erosion: Genetic Condition or Side-Effect?

Victoria "styling" Bertrand's hair this morning.

This is just a quick update (read: vent) on what I've been up to regarding Bertrand. Since his keppra wean, he has been having the least number of seizures since ACTH! As a result, he is also more vocal and interactive. Unfortunately, the seizures that he IS having are sleep-related and disturbing to watch. More on that later.

All summer Bertrand's corneal erosion has not improved despite eye drops or ointment every 2 hours. In case you are wondering, that is $50 worth of eye goop every week. It is also a very upsetting process for Bertrand and time-consuming for us. And yet, I am plagued by the feeling that I am losing the battle to save his eyes. If he is this bad now, in the summer, with a humidifier in his room, how bad will it get in the dry Utah winters?!

I've been feeling defeated. It's not a feeling I am comfortable with, so I got to thinking...

It was not always this way. While Bertrand didn't cry tears (alacrima), he wasn't battling constant eye infections and corneal erosion. This all started back in January, before depakote, but a month AFTER ADDING LAMICTAL. A quick google search confirmed that dry eyes are a common enough side-effect of lamictal.

After leaving 2 messages with neurology and 2 emails to her, I finally heard back from Bertrand's neurologist last night. I feel like she blew me off. Bertrand's eyes and seizures could wait until she could see him at his September 20th appointment.

NO. WAY.

My baby boy has suffered long enough. Corneal erosion is extremely painful. As per my last discussion with his ophthalmologist, Bertrand is facing surgery to sew his eyes partially shut to reduce the amount of surface area and thereby the moisture he loses by them. As it is, his corneal scar is occluding his pupil and damaging his vision.

This is unacceptable.

Yes, seizures suck, but Bertrand needs to be seen as a whole. It is possible that depakote may be enough to control his seizures. Or maybe lamictal isn't to blame and he does need the surgery. Either way, we need to test and see because Bertrand deserves this chance.

I've spent the morning fighting for my boy, calling in the "big guns". Wish me luck.

UPDATE:

This afternoon, I spoke with Bertrand's Pharmacist (the one who once opened the pharmacy on a Sunday just for us, after we forgot to pick up his medication). According to him, Lamictal can cause dry mouth and other ocular issues, but "dry eyes" as a symptom were not in his literature. He recommended some eye ointments/drops that were new to the market. He also recommended a prescription about which we should speak to Bertrand's ophthalmologist.

From my own research, I learned that Lamictal is a (modest) inhibitor of the enzyme dihydrofolate reductase, which completes the two-step activation of dietary folate into the bioactive form, tetrahydrofolate. A B-12 deficiency would increase the adverse effect potential of the Lamictal. Dry eyes can be a symptom of B-12 deficiency...

So, TONIGHT, Bertrand starts some new sublingual vitamin B supplementation (THANKS, AMY!) and new eye drops. ;) Bertrand will see his pediatrician about all of this on Thursday. His neurology appointment was moved up a little earlier to September 1st. Grr.

A (tiny) happy dance.

Bertrand working out with his "personal trainer".

Bertrand's gastroenterology appointment this morning was just the good news I've been needing to hear. Starting with Bertrand's stats, he measured:

• 39.5 inches (just above the 50th percentile for height)
• 36 pounds (just below the 75th percentile for weight)

I could've happy danced for these numbers alone! All of our hard work with his diet and exercise (standing, gait trainer, horseback riding, etc.) have paid off. We have only a few more inches taller (or pounds smaller) to go to reach normal weight-for-height!

Then, his Doctor came in and said that, looking at Bertrand's most recent liver enzyme levels drawn in May, if Bertrand had been a new patient he would've wondered why B would need to see a GI doc! His liver appears almost entirely normal!

We shared a laugh that Bertrand is probably the first person in history to have their liver enzymes DECREASE while on depakote. But, due to the depakote, the GI Doctor would like to continue vitamin D and carnitine supplementation to protect the liver.

However, he now feels that we can wean Bertrand's prevacid! (While watching for signs of reflux.) And, in 6 months, we can begin a cautious actigall wean as well! (While monitoring his liver enzymes.)

That may not seem like a lot, but 2 fewer medications (administered a total of 4 times daily) is 1 step closer to a more enjoyable childhood for my precious boy. And that's worth celebrating!

Medication Changes

Bertrand had an appointment with his neurologist today. We updated her on everything including the changes in Bertrand, both positive (mood, interactivity) and negative ("weird looping seizures"). The neurologist is pleased with his progress and believes that Bertrand's new seizure type is actually a kind of partial seizure, since he retains some consciousness and it affects him asymmetrically.

Bertrand's blood draw from last week had his depakote *just* in effective range and his lamictal high in the effective range. We will be increasing his depakote to 250mg twice a day and lowering his lamictal (since depakote raises lamictal levels) to 25mg twice a day. Another blood draw will be taken next week and then in a month. If Bertrand's seizures have improved by then, we can begin a SLOW wean from keppra (800mg will take 8 months).

It is worth noting that Bertrand's liver enzymes have remained steady or have even fallen while on depakote and lamictal, both of which are metabolized in the liver. Depakote is known to be particularly harsh on the organ. Not resting on our laurels, we are upping Bertrand's carnitine supplement (Carnitor SF) to 4.5mL twice a day, which should continue to protect the liver. And, since depakote binds with vitamin D, Bertrand will be getting plenty of sunshine this summer along with his 1000IU vitamin D supplement. :)

If Bertrand's seizures do not improve on this current drug cocktail, we've already begun discussion on the addition of another drug: topamax. Topamax is known to work well on multifocal seizures, which are the kind Bertrand has. We know that there is an very small chance that a 4th antiepileptic drug will help, but of course we have to try. Bertrand has little to lose from another failure but so much to gain if we succeed! We will never give up.

PS - I am very proud to say that Bertrand was congratulated on his weight AND his weight-bearing!

Medication Update (& Baby Watch)

Bertrand & Mama cuddling on the couch.

The lap space may be getting tight but there will always be room for Bertrand!

Medication Update:

• While Bertrand's depakote levels were barely in effective range at their trough, his lamictal level was 9 at trough with an effective range of 3-14. This means we can't raise his lamictal.
• Bertrand's depakote was raised to 375mg (125mg AM, 250mg PM) and resulted in a night owl! The medication is supposed to make him sleepy, but fewer seizures make him more conscious and interactive. Once the level evens-out in his blood, this should resolve.
• Bertrand will get his all of his blood levels (including medications, liver, vitamin D, carnatine and others) drawn in another 1-2 weeks. There is concern for his increasing liver values.
• He will then have another blood draw on May 17th after seeing his neurologist. She says that depending on his seizure & liver activity, we will consider raising Bertrand's keppra next.

Baby Watch:

Regular readers of this blog know that Bertrand's little sister is due today! Plenty of signs indicate that she will be joining us soon. We will keep you all posted. :)

Hallelujah! Finally!

The response from Bertrand's neurologist:

I asked our nurse to help with ordering labs: lamictal, trough depakote (1-2 hours before his dose of depakote ideally), CBC, CMP, and GGT (to make sure his liver function abnormalities are liver related). If these are fine, I will ask to increase his depakote further to 1 pill in am and 2 pills in pm or 375mg/day. If labs are not good, will have to discuss with you how to help with the myoclonus--these can be tricky to treat.

As for the carnitor, I agree with keeping him on it while on depakote. A multivitamin with good vitamin D source would also be good.

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

Bertrand was sent home from school today for "wheezing". His oxygen saturation is fine. And, of course, he has been happy and playful since he got home. The little faker. ;) Better safe than sorry, though!

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UPDATE: So, Bertrand wasn't faking the cold. According to the blood work from this afternoon, he is definitely sick if cheerful. Because of that, I'm not putting much stock in his CBC numbers.

Disappointingly, Bertrand's liver enzymes were all elevated. ALT 82 (normal range: 5-45), AST 79 (normal range: 20-60) & GGT was 64 (normal range: 4-15). Granted, this is the lowest his GGT has ever been, but it breaks my heart to see his ALT & AST values creep up again.

Bertrand's trough depakote level was 57.7. Effective range is 50.0-125.0, so it is just barely in the effective range and definitely needs to be increased. This could lead to even more elevated liver values. Only time will tell, but all the more reason to keep monitoring him!

A rant on medication...

Bertrand's seizure medication levels are as follows:

• Lamictal level is 10.6 (effective range: 3.0-14.0) @75mg.
• I would like the Lamictal held steady.
• Depakote level is 28.3 (effective range: 50.0-125.0) @125mg. Now @250mg.
• I would like an additional 125mg before bed to decrease sleep-related clusters.
• Keppra level is 26.9 (effective range: 5.0-45.0) @800mg.
• I would like to eventually wean Keppra to decrease sleepiness and rage.

Bertrand's neurologist made a BIG DEAL about the caution needed with medications such as lamictal and depokote, which are metabolized in the liver. In fact for close 2 years, Bertrand wasn't put on these first-line epilepsy drugs because of this overwhelming concern for his liver.

His doctor emphasized the need for frequent lab draws and supplementation with carnitine (protects the liver) and vitamin D (because it binds with depakote/valproic acid) as a HUGE must, if we insisted on taking the risk of putting Bertrand on these medications!

So basically it is MY fault if anything goes wrong. Okay, fine. I'm risking my child. I am confident enough in my research and second opinion from Cleveland to take this risk. BUT, as far as the required precautions go, I CAN'T ORDER LABS OR WRITE PRESCRIPTIONS!!!

The past month has been nothing but radio silence from his neurologist. She is not responding to calls or emails.

Bertrand's labs need to be checked SOON! (It's been a month!) And he needs his depakote prescription increased! (He's having myoclonic clusters!) And his carnitine prescription is about to run out! (His liver! Remember!?)

Sure, Bertrand's liver has shown improvement as of late, but given that his neurologist hasn't even looked at his lab results SHE DOESN'T KNOW THAT! (Also, strong precautions, such as checking LFTs, CBCs, medication levels, carnatine & vitamin D levels, etc., are taken with patients whose livers are completely NORMAL!)

Hormones aside, I feel I am justified in being furious. My calls & emails go into a void when my child's life is at risk! And, every seizure he has could be exacerbating his brain damage.

I've been escalating my communication efforts, and trying different channels to reach her. If I don't hear anything on Monday, I may very well show up in person next week!

A good news day!

Bertrand calmly reads books with his Nana while waiting on the anesthesiologist.

Bertrand has been on the longest streak of good--or at least "not bad"--news that he's ever had! I've been "knocking on wood" all day. Thursday's procedures started with the easiest time Bertrand has ever spent NPO (Nil Per Os meaning without oral food and fluids). He went into sedation without any tantrums or issues.

His "dental restoration" consisted of x-rays, a cleaning, and getting his molars sealed. According to the dentist his teeth actually look great for a special needs kid! There was zero sign of nerve damage! In the future, any time that Bertrand is put under anesthesia, the dentist recommends another thorough dental exam and cleaning.

His esophagogastroduodenoscopy (EGD) involved his gastroenterologist looking at his esophagus, stomach and part of the intestine along with a stomach biopsy. Everything looked "fantastic" according to the doctor (no ulcers or other visible damage) and the biopsy confirmed that everything is normal (no sign of celiac, allergies, or other issues)! Bertrand will stay on 15mg of prevacid for now and we will discuss removal again in a year.

Bertrand had blood drawn while he was sedated for his usual panels--Comprehensive Metabolic Panel (CMP) & Complete Blood Count (CBC)--along with his Lamictal and Depakote levels. His CMP showed that... *drum roll* ALT was 72 (normal range 5-45) and AST was 58 (normal range 20-60)!!! Both of these values are the lowest Bertrand's liver values have EVER been and the AST is now in the NORMAL RANGE! Yippee!

Bertrand gets the CBC drawn mainly as a way of checking his platelets--if the liver isn't functioning well then platelet counts drop and one experiences difficulties clotting. Well, the test was invalid because his sample clotted, so I think that answers the question.

The last results to come in were the medication levels. Bertrand's lamictal levels still aren't back, but his depakote level was 28.3mcg/mL. Effective range for depakote is 50-125mcg/mL, which means that the depakote in his system (a dose of 125mg once a day) isn't high enough to see any effect yet! This is good news because we hadn't really seen any changes in him--seizures, mood, or otherwise--while on the depakote. We're keeping our fingers crossed to see what upping his depakote to 250mg per day does next week!

I hope the "good news" streak continues for Bertrand's genetics appointment on Monday! :)

Depakote Begins

Today, Bertrand had a seizure-y day at school and at home. He was still in high spirits, smiley, and very awake, however, a particularly long cluster of myoclonus was the last straw. Rather than wait until tomorrow, I started his 125mg of depakote this evening. We'll be monitoring him closely over the next several weeks and his blood levels will be drawn in a week. Our fingers & toes are crossed.

Yo-yo

Bertrand displays his preference for music over toys.

PAST WEEK

The last few weeks have been tough as we've dealt with Bertrand's medication changes in an attempt to achieve further seizure control.

After a 25mg increase in Lamictal, Bertrand's seizures increased. Reducing the dose--in preparation for the addition of Depakote--brought seizure numbers down a bit. (Paradoxical seizures perhaps?)

This Thursday, we will be starting Depakote (valproic acid) in the hopes that it will nix some of Bertrand's nasty remaining seizures. We won't see it's full effects until late March.

Through it all, Bertrand's continued smiles and laughter have powered us and pushed us. We don't want to lose our happy, sweet, little trickster to the fog of seizures again.

TODAY

In this morning's occupational therapy session, Bertrand successfully played with switches, sensory bins, and other toys. He demonstrated his improved reach (gross motor) and finger usage (fine motor), along with developing memory and the comprehension of cause and effect!

Nana took Bertrand to his afternoon dance class. Apparently, he protested his new aide and demanded his Ms. E back! --attempting to lure her with eye contact and indignant squeals. Hopefully, his demands were noted and he'll have her back next week. :)

Bertrand's final coup for the day was reaching up and pulling off his Nana's glasses! We were all overjoyed and so proud of Bertrand's reaching & grabbing! We can only hope to lose earrings, break necklaces, and have lots of hair pulled soon! :)

Just because.

I took this video last week. B knew I needed cheering-up. This helped a lot.

Bertrand had ophthalmology and neurology appointments this week.

Ophthalmology:

Bertrand's right eye is still inflamed. There still appears to be some infection. Since it is most likely viral, he was finally taken off the antibiotics and we're hoping his body can fight the rest off on it's own. He has another follow-up in a week.

Neurology:

Bertrand's neurologist was filled-in on our trip to Cleveland, Bertrand's most recent MRI, pending genetic testing, his EGD, dental, ortho, seizures and more. Because of Bertrand's increased seizures, we've agreed to start Bertrand on Depakote sprinkles in a week (in order to lower his Lamictal first since there is interaction between both medications) and then draw medication & liver levels during his sedated EGD procedure. Bertrand's follow-up is in May.

Plan for Better Bones

Bertrand tearing into his Valentine's haul from school.

Bertrand had a fabulous physical therapy session at Shriners and afterward we had an appointment to discuss Bertrand's bone health with his pediatrician. Not only are broken bones painful for Bertrand, but they set him back developmentally--he has yet to recover fully from his broken leg in October. Bertrand broke both his humerus (upper arm) and his femur (upper leg) in 2010, so we want to plan to prevent additional breaks.

The discussion with his pediatrician went fantastically well. We covered topics including: water therapy, weight bearing, vibration therapy, vitamin D, calcium & phosphorous supplementation, bisphosphinate injections, medication interaction (prevacid, depakote), & more.

His pediatrician believes that Bertrand does have low bone density (just like any other child with a neurological disorder would have), so a dexascan wouldn't reveal anything new. However, his breaks have occurred in very typical locations for a child his age and his last bone break may have been due to a growth spurt. Therefore, she is not convinced that his bones are that bad.

Furthermore, Bertrand is still young, he is doing way better than expected (yay!) and there is a lot we can do to build his bones naturally--which would be less brittle than bone created through something like bisphosphinate injections.

Bertrand's Plan for Better Bones:

• Supplementation: We'll do a 3 day food diary to estimate his current calcium, vitamin d, and phosphorus intake. We don't want to overdo the calcium because Bertrand is prone to kidney stones, but we do want to optimize the calcium while bombing his system with vitamin D. We'll adjust his diet accordingly.
• Medication: Along with monitoring levels of vitamin D, calcium, etc., we'll also keep an eye on medication levels and limit those with negative interactions whenever possible. Bertrand will have a Ph test done during his EGD in early March to see if staying on prevacid is necessary now that he is older.
• Sunshine: While sunburns are a big no no, Bertrand will be encouraged to spend lots more time outside with minimal sunscreen to improve vitamin D absorption.
• Water Therapy: To encourage weight bearing with minimal resistance, Bertrand will begin water therapy. This task is being gratefully delegated to Bertrand's Nana, who is a swimmer & enjoys the water. (I detest pools, the beach, and even bathtubs.)
• Stander Time: Bertrand will have his stander re-adjusted at Shriners and we'll become religious about building up his tolerance back up to 2 hours in the stander daily.

PS - Bertrand's pediatrician will take Victoria (Bertrand's sister due in April) on as a new patient even though her office says that she is not taking any more! :) It is such a relief that any early warning signs will be caught!

PPS - Bertrand is back to having horrible drop seizures and myoclonus. We're hoping that he's either sick, or it's the increase in Lamictal, or the drop of the Zonegran, and not his pharmacoresistance rearing its ugly head again. He'll be seeing his neurologist tomorrow. We'll be talking depakote.