Dr Mommy, MD*

Ever the scientist, I have decided to conduct an experiment while our doctor is on vacation. Keep the AEDs (anti-epileptic drugs) stable, and get rid of the multi-vitamin powder and Carnitor. hypothesis: Maybe these two "non-critical" drugs are actually impacting seizure control.

Cole has been on Carnitor since the introduction of the ketogenic diet in June 2010. We were told that it would help protect his liver and it would give his metabolism a boost with all of the fat in his diet.

I asked if it had any contraindications, (just like I do with any drug we go on). They said no and the literature would suggest that. It is not contraindicated with any other drug. They also said: It is practically impossible to overdose on and might cause diarrhea. Okay, diarrhea is the least of our problems, and we dismissed this "safe" drug and focused on the life-changing implementation of the diet. We had much bigger things on our plate. Time passed (almost a year) and we slowly dropped the diet. However, we chose to remain on Carnitor because it couldn't hurt to give his liver extra protection if he was on Depakote, right? The doctor totally agreed. So we stayed on it. Had blood tests, things were peachy.

Here is where I messed up.

I asked questions, like I should, and took the answers given by a medical professional to be the complete answers. Remember, as awesome as they are, doctors practice medicine. I think I scanned the prescription leaflet once when we first got it, but did not see anything that stuck out to me that would be worrisome.

This is what the fine print says on the Carnitor website (which I didn't look at until recently).

Before using this medication, tell your doctor or pharmacist your medical history, especially of: kidney disease, seizures. Um, yes. Dilated kidneys in utero, the diabetes insipidous! It may be the Carnitor that is putting pressure on his kidneys? What?! NO one thought that it could be the Carnitor and we have seen 4 different doctors regarding this issue.

Seizures have been reported to occur in patients with or without pre-existing seizure activity receiving either oral or intravenous levocarnitine. In patients with pre-existing seizure activity, an increase in seizure frequency and/or severity has been reported. NO one ever mentioned that Carnitor could increase seizure activity. Even the people in Miami. I don't remember it being in the info packet from the pharmacy, but there it is...in black and white.

Fast forward to the beginning of the week.

I am constantly trying to re-asses things and see if I can make them better. I don't really make any serious moves without Dr's approval, but I am usually the one to suggest a new route of medicating. I am the instigator. Not the norm, but I have a relationship with the doctors that is trusting and when I suggest something, they will tell me if they feel it is a good idea or not. Neither the neurologist or pediatrician has the ego where they believe that only they know what is right for my child. I'm lucky. They understand that I have dedicated my life to making a better quality of life for my son and I usually have done plenty of research before I make a suggestion.

Like I mentioned, the doctor is out. I've done stuff like this before with sugar, instigating Modified Atkins by myself, removing extra Magnesium, etc. Although I would not suggest this for anyone else. I have had 30+ years of experience with epilepsy and have worked in the medical field for a long time. I never do something like this without careful thought. Always consult with your doctor before making changes, especially with prescriptions...disclaimer over :) I thought, "Let's just hold these two and see if there is a difference". The multi-vitamin, while hypoallergenic and pricey, has to have some sort of bonding agent to keep all of those vitamins together, plus flavoring, etc. There could be something in there that doesn't agree with Cole. The Carnitor, we're off the diet, on a teeny dose of Depakote, do we really need to "protect his liver", like we have been assuming all along it was doing? I knew that we could have results, or things would stay the same. It was worth a shot.

Cole has been without his multi-vitamin and Carnitor for three days. His seizures have dramatically decreased. They are still present, but he can go occasionally go a solid 10 minutes without any type of noticeable activity. Miracle.

He has been a lot more calm and his attention span has been lengthened-CARNITOR® (levocarnitine) is a naturally occurring substance required in mammalian energy metabolism. It has been shown to facilitate long-chain fatty acid entry into cellular mitochondria, thereby delivering substrate for oxidation and subsequent energy production. Fatty acids are utilized as an energy substrate in all tissues except the brain. In skeletal and cardiac muscle, fatty acids are the main substrate for energy production. So, adding energy to a child that already has attention issues and hyperactivity. I totally see how this is another reason that it was prescribed for keto, since most children can become lethargic, taking all of the carbs out of their diet. He has been nicer and more cooperative since its removal.

Even though he is having seizure activity, most of the clustering is concentrated around falling asleep, waking up and fatigue. I even did another experiment and took him to the zoo yesterday. Without pre-loading him with drugs. We were in the sun, he got in and out of the stroller multiple times, it was incredible! Usually, once he gets out of the stroller, there is no getting back in. He paid attention to the animals and exhibits and occasionally asked simple questions. It was like I had another child! We got through 3 hours at the zoo and a long time in the car (gotta love traffic) with no extra pharmacological intervention.Awful pic, sans animals, but it was the only one I got of him actually looking. Things aren't completely revolutionized :) It was a wonderful, almost "normal" day. Yes, we had a nurse, hat, glasses, oxygen and a really heavy bag full of medication, etc., but we did it!

So here is a warning to all of you. Don't take the answers you get from medical professionals as complete information. Look up the drug on it's own website, read the entire thing. Trust the professionals, but do your research! We have had this happen to us more than a couple of times now. It has usually been in relation to some sort of AED. Most doctors will titrate to the highest dose of an AED to see if it will be effective. Usually, with Cole, the higher the dose, the worse things get. Who knows if Carnitor really did protect his liver during the diet? His lab values were always normal. I can't beat myself up about it. We have been on this drug for over a year and no one has suggested stopping it. I do know that I will be suggesting that we take him off of it, after completing my week-long experiment and having proof.

Now to figure out if that multi-vitamin is really helping. Always some other experiment/adventure to embark on. You are your child's greatest advocate. Sometimes, Mommy doctors really do know best.

*UPDATE: After polling the masses, it turns out that Nano VM has indeed been the cause of more seizures in Dravet kids. Bye-bye multi-vitamin! I LOVE my social network buddies. Ask a question, and multiple people will give you the answer. I just wish that I would have asked earlier. I need to start paying more attention to the online threads, I usually just scan them when I have the time. I could have had this important information without my trial and error experiment. Que sera sera. All of these other Mommy Doctors are invaluable to me! It is so strange how our kids are each so different, yet so the same.

Passage

I was looking at these the other day. I admit, I shed a tear.

When did that little munchkin become the big boy who is going to get on the bus (hopefully)?

His cheeks are not as chubby now, he has all of his teeth. His eyelashes are still the same though, gorgeous. So interesting to reflect on the passing of time. Occasionally, it feels like he has been around forever and I can not remember life before him. Other times, it seems like we just brought him home from the hospital, in a blizzard.

Anyone that has a newborn, you might want to avoid me. I could possibly become a little aggressive and not return your baby. :)

Natural Disaster

It has been more than a little crazy around here this week.
First, a 5.9 earthquake. It literally scared my nurse to the point she thought the world was ending. She ran outside and was screaming. I had to yell at her and tell her to come back in the house and worry about her safety instead of concentrating on Cole. Really reassuring! She was very shook up through out the rest of the day and I tried to give her the remaining part of her shift off. She thought she was going to die. Me, not so much. I must say, since I have felt other earthquakes, this one was the longest I have ever felt, but not the strongest. The fault lines are so different out here. So, aside from our whole house shaking and rattling, no damage done. Brian got sent home and felt more swaying than we did as he was in a high rise.

Now, a hurricane is headed our way!

I never thought that moving to DC would mean earthquakes and hurricanes. It's a great thing that we have a resident emergency management guru. We dug through our 72 hour kits just to be sure and yep, it's all there. SO-if you live in the area and need food, water, electricity or shelter...come on over! Remember, I am married to the man who got a generator and a camp stove for his 30th birthday. We have a bunch of devices that could come in useful during an emergency, not including our food storage and thank goodness it is all organized. So, after our dig through, where we got rid of a few things that expired, we refreshed our battery supply, got more gas for the generator, bought a new lantern and are ready to hunker down if we need to.

All of this comes in the middle of an already-interesting week. I mentioned previously that we are going through the more meds/less meds debate. We took Cole off of the Clonazepam doses. Then off of Depakote for 24 hours. His seizures did decrease, but they are still there and too many. Plus, he has been having tonic-clonics almost every day. However, his eyes are brighter and he is happier. SO, the entire week has been on pins and needles, but it has nothing to do with Richter Scales or inclement weather. We put him back on half of a depakote dose at night, and are going to keep it that way for 2 weeks to let it slowly work out of his system. The Dr is going on vacation for a week, how dare he! ;), which he totally deserves...it is just complicated timing. So, we'll be in a holding pattern next week and have the go-ahead to do what we believe is best and to consult with the new doc that joined the Brain Institute in Miami if we need to.

When you have a child with special needs, something like a power outage can be life-threatening. So, we have updated our emergency plan, gotten refills on all of our prescriptions and considered where we will go if we can't meet Cole's needs at home. All of this planning is a breeze compared to the fear that has struck in our hearts if we can't control this new cycle of seizures. It seems like our next move will have to be Stiripentol and clobabzam. We might try Verapamil (a blood pressure med) first, since Stiripentol works best when in combination with other medications. Those are Topamax (which Cole does not do great on) and clobazam (which acts a lot like clonazepam, also which does not do well with Cole). We are not totally writing off the clobazam, we just know that other drugs that "act" like it have not worked for Cole in the past and Stiripentol needs another drug to make it the most effective. Usually, the "Magic 3" are Stiripentol, Depakote and clobazam. Right now, I can't even think straight to write out our action plan. We do have one though. Just like our action plan for a disaster. I'm not freaking out about 80 mile an hour winds right now. I grew up where the wind blows so hard, our trees were all permanently leaning. I'm more worried about the future of my little boy.

Dravet, a natural disaster.

Cookies For Cole

It's a bake sale! Coming to your neighborhood, September 17th.

If you have been reading Warriors for any length of time and want to know how you can help, here is a great way to do just that.

I am not a professional pastry chef like my sister, but most of my friends and family would say that I can bake with the best of them. However, you don't need to necessarily know your way around the kitchen for this. This is something that anyone can get involved in, no matter where you live, any time or any place. Even if you only make one batch and sell it to your neighbors, every dollar counts.

Here's what you do: bake something, sell it and donate all of the proceeds to Cole's Cause. It can be really small and simple, like one batch of cookies, or you can recruit your friends and it can have lots of variety and involve lots of people getting something delicious. So many of the people we love live far away from us, so this is a way to come together at the same time and do something great.

It doesn't just have to be cookies. Cakes, pies, eclairs, brownies, popcorn, any treat will do! We are also going to have sugar-free, gluten-free, and keto-friendly goodies at ours :)

If you want the file to print out your own awesome poster or flyers, just e-mail me epilepsywarriors@gmail.com

Every little bit helps.

Drugged

Dravet is a very complex disorder. Things are constantly changing, especially in the first 5 years or so of life. Cole is a true testament to, "Never Get Comfortable".

We had a great honeymoon with Depakote. You may recall my love letter. His long seizures were less frequent, they were shorter. He seemed to be able to handle overstimulation better and started exploring the world outside of our four walls. There were still plenty of seizures, but most of them were small. Then the small seizures increased to the point that I had to use extra medication to stop the clusters. Almost every day. So we decreased the dose and added Topamax. We changed things around, lowering the dose twice and things seemed to even out again where I was only using extra rescue meds a couple of times a week instead of every day. That lasted for a week or so and the myoclonics were back, with a vengeance. It seemed that he couldn't even go 5 full minutes without some sort of seizure activity. So we changed things again, and they got worse...not better. So we doubled one med and they got just plain awful. Cole was only having 45 seconds to a minute of respite in between clusters and he also started having a lot of myoclonic eye flutters, something he has only had a handful of times in his life so far.

SO-what do we do? More meds=more seizures. Less meds=more seizures. We can't go back on the diet or he will stop eating completely. Neurofeedback didn't work. Homeopathy didn't work. SPIO and other special equipment is not enough. Is it time for stiripentol already? I'm not sure what to do since we have tried Plan A, B and C. Is it time for Plan D so soon?

To stop the cycling, today we gave him a loading dose of clonazepam, "To throw water on the fire". We've done this with other meds before, but never with a benzodiazepine. The doctor reassured me that it would make him very tired and told me to make sure he got a good nap. We've used clonazepam before as a rescue drug (0.25 mg) and I knew it worked well for Cole to stop small seizures. So, with a quadruple dose in his system (basically 4 pills equaling 1 mg), plus 3 other seizure medications, he should be knocked out cold, right? Um, no...if you recall, my son has the nickname "Mini Hulk". He can withstand incredibly high doses of medication and has a very high pain threshold. Remember when I won the Mother of The Year Award when I broke his foot during therapy? Yeah, high tolerance for pain and drugs is an understatement. I actually chuckled when I read the new protocol this morning.

Instead of sleeping, like a regular person would if they had this amount of benzos in them would, he is a total chatterbox. Right now, he is alternating between talking like a pirate, singing Old MacDonald, and having conversations with inanimate objects. You can totally tell he is drugged, especially if you know him. Yet, hardly a seizure since the big dose. Oh, it is such a balance. Seizures or being strung out? All in the day of the life of Cole.

Cole's Cause

Hi, this is Brian. I know it’s not often I write on the blog. Let’s be honest, I’m more of a reader just like you. I’m grateful Niki spends the time to record our journey with epilepsy. It’s amazing to look back a year ago and see how far we have come. In fact, it was almost one year ago we got Cole's official Dravet diagnosis and started the fundraising effort for his seizure dog. Thanks to our families, friends, friends of friends, co-workers, strangers, and blog readers around the country, together we raised enough money to begin the training process for Cole’s seizure alert service dog. Cole’s service dog will soon be a reality – all because of you. Words will never be enough to show how grateful we are for your generosity.

I say ‘begin the process’ because we won’t actually get to meet Cole’s dog until the end of October. For the last several months, we have been sending Cole’s shirts to the trainers so his dog can know his scent after he has a seizure. Though we never like to see Cole have a seizure, it will help his dog become familiar with his smell and hopefully alert us before he has a seizure.

We are counting the days (as you can see to the right on the blog) until we meet the newest member of our family. We won’t know which dog will be Cole’s until just before we leave for training at 4 Paws For Ability.

In the meantime, we have a few hurdles ahead that really have us worried. Unfortunately, they are three major financial hurdles: the 2 week dog training trip to Ohio, a portable FAA approved oxygen concentrator, and a return visit to Miami Children’s Medical Center.

The trip to Ohio
Even though we raised enough money to train Cole’s dog (thank you again!!), we came up short for the trip. We’re driving so we don’t have the airline ticket or rental car expenses but we’ll have other expenses. We’ll spend about 2 weeks in a hotel so that will be the largest expense along with gas for the car, food, and dog supplies. We have a hotel reserved where most of the other families in our training class are staying since the hotel has been very accommodating to prior groups. We figure with the hotel, gas, food, and dog supplies, we’re looking at about $3000. The hotel is in the Hilton family. We’re open to people that are willing to donate some points. There’s probably nobody out there with 2 weeks worth of points, but we’d be more than happy and extremely grateful for any contribution toward our trip to get Cole’s dog.

FAA approved portable oxygen concentrator
Cole has to have oxygen available at all times. Sometimes his seizures cause him to stop breathing and he will quickly turn blue. It can happen very fast and some seizures are worse than others and sometimes he needs more oxygen than other times. It’s very random and completely unpredictable. The one thing we do know – he needs it ready and available always. We take it with us swimming, to the store, or anywhere else we go. It is located in two different places in our house and we also have it in our car. This is an added cost to us, even with insurance. We really run into problems if we are going to travel. Cole sees a team of specialists at Miami Children’s Hospital in Florida every six months. His first visit was a 5-day hospital stay in May. Each time he gets on a plane, there is the chance that he will have a seizure and need oxygen. You can only take special FAA approved concentrators on airplanes, and Cole's has to be continuous flow, because he stops breathing. There are only two models that do this. Cole needs this concentrator. His doctor has approved it, written a letter and we have been battling with the insurance to try and get it paid for. Their response is to pay for it out of pocket, and then they we can try and submit a claim for reimbursement – not likely to get approved. We do not have the funds to pay out of pocket for a concentrator, so it is costing us $500 or more each time we travel, just for special oxygen since we can’t take our own. We spent $1,000 in two months on travel oxygen, to move out to DC and to take him to the doctors in Florida. The travel oxygen rental fee ends up being far more than the price of an airline ticket. In order to buy an FAA approved, continuous flow, portable oxygen concentrator, it’s about $3800 – but it would save us in the end from all the rental fees.

Return visit to Miami Children’s
Since we’ve moved to Virginia, we are now able to take Cole to a specialized team of doctors at Miami Children’s Hospital. His first visit was in May - a 5 day hospital stay - and he needs to return every six months. We are so grateful to the team at Miami Children’s. We have been so impressed with their level of care, knowledge, anxious concern, prompt responses, and the list goes on. These guys are the best for Cole. But just as a regular check up with your doctor is good, so is one with your neurologist – especially in Cole’s case since his seizures are always changing. We’re set to take Cole back to Miami in December. Three airline tickets, food, and rental oxygen add up. Thankfully we have health insurance that helped cover a majority of Cole’s very expensive first visit. But we still had some expensive co-payments since his visit was so long, he saw several specialists, had lots of tests, and much more.

It’s difficult writing this. It’s hard to ask for your help, especially since so many of our family and friends and others have already donated. We couldn’t have made it this far without you. Cole's care is complicated and ever-changing. It’s expensive, even with insurance. But we do know we’re in the right place. We are where we supposed to be. More has happened here in our first six months than in the last three years with Cole’s care. Since we moved, Cole has a nurse during the day; has been accepted to the school district’s special education pre-school program –he starts next month, he’s having fewer long major seizures, and we’re able to get out a little more (but armed with rescue medication and oxygen).

We’re trying to do all we can to give Cole the very best to fight for a better day, every day. It's hard to see him suffer on a daily basis but his smiles, laughs, and hugs make it all worth it. I’m holding down one job and looking for another, hopefully teaching part time in the evenings. If nothing happens, I’ll keep searching and looking, even working at Home Depot if I need to.

Above all, thank you. Thanks to all of you who have helped us. Cole would hug every one of you if he could, and so would we. Thank you again to those of you who have been generous. Thank you for your sacrifice in our behalf. We recognize and appreciate our loved ones and others who have already given so much. We have learned some amazing life lessons along the way in our journey together. Our eyes have been opened to the goodness of people’s hearts. It’s humbling to see how many people really do care. It’s sometimes harder being served by others than giving the service. I’d rather give, give, and give. And we hope to give back as much as we can for the rest our lives and pay it forward. If you or someone you know is able to contribute to Cole’s cause, thank you so very much! You can use the “donate” button at the top right of the side bar, you do not have to have a PayPal account. Or if you have another way you’d like to donate, please email us: epilepsywarriors@gmail.com.

Even if you’re not able to donate or are one of our many previous generous donors, please keep visiting us on our blog. Thanks for your interest, concern, and encouragement. You are part of Cole's success and happiness. Thanks for your prayers. We feel it and need them every day. Those are things money can’t buy and we’ll be forever grateful for.

Thank you, and may God bless you.

Magical Memories

Due to some really hard work, and generosity of others, I was able to sneak away for a few days and get together with some fellow Dravet moms. The paramedics actually made their first house call to our new home while I was away. Another reminder that leaving just can't happen very often.We had a lovely time, with lots of food, laughter and fun. There were some tears involved too. I can not explain the immediate bond you have with another Dravet parent. Our numbers are so small, we immediately understand each other. I got to work on a new pamphlet that I have been a part of for newly diagnosed patients families, talked about my role on the a4cswn committee and realized once again that I can be a resource for others, after having 30+ years of experience with epilepsy. Some one called me wise. What? I'm not wise, but I am totally willing to share whatever information I have. I'm so grateful that these women were willing to share their weekend with me. What a magical place with incredible memories.

Just A Peek

Warriors is getting a facelift

and all without Botox

Here is a little taste of what is to come.

Awesome photography by E Moon

Presents

While trying to convince Cole to create a sample for the nice people in the lab yesterday, I took him around town so we didn't have to go home, and then come right back with the yellow stuff. I don't blame him, who wants to have a bag attached to their body? Still, if you have been reading Warriors for any length of time, you know that he is practically famous for not cooperating when we need him to perform whether it be blood, or any other type of bodily fluid. Yesterday, only two pokes and one blown vein for 7 tubes of blood. Hallelujah! Compared to the multiple sticks and multiple trips last time, this was a cake walk. Now if I could only get him to go on command! TMI.

I took him to a little diner, since I had to wake him up early and these were fasting blood draws. No food, no meds and little sleep makes for an interesting morning, but I didn't want to have to go home and turn right back around. We had a lovely time, just the two of us. He had pancakes with some orange marmalade and I had caramel french toast. Compared to other restaurant nightmares, he was pleasant. Yes, we still had to administer meds while waiting for our food, he still stood on the banquet, was banging on the window, yelled greetings at the other customers (including the ones who were on the other side of the window) and dropped multiple items of silverware. However, he did not lay on the floor or scream bloody murder once. Like I said, it was lovely. What a gift to be able to take him to a restaurant and watch him eat a meal. No major seizure, no special diet, just hanging out with my special boy.

After our little breakfast, he had still not cooperated with his sample for the UA, so I took him to a thrift store. There, with a little digging, I found three precious gems. I have read the entire Mitford series probably 3 times. I always borrowed from my mom, grandma or aunt. However, they live too far away to peruse their library shelves. For $1 each, I found the first three books. Tonight, I am going to curl up with Father Tim and visit a little town that has characters who have become like family over the years. They each have their struggles. Their likes and dislikes. Some of them are downright crazy. Overall, the feeling you get from them is love. It is so nice to just escape to a nice little, charming Carolina town and pull yourself out of the real world; even if it is just for an hour or two.

I finally gave up trying to get Cole to cooperate. We headed home so we could let in the nurse who was arriving for her shift. Wouldn't you know, the minute we got through the door, he went. I should have just come home in the first place! At least I didn't have to pack him back into the car and tote him with me to the lab. It was wonderful to just leave him home with the nurse and not have to worry about putting any additional strain on him. It had already been a long day and it was only noon! He got the privilege of taking a nap, I got the privilege of taking a sample to a lab, alone. Sometimes we all have to give presents to ourselves, even if it is $1 books and 45 minutes of alone time in the car:)

Diagnostics

Have an appointment for Cole today. Something's up, I just don't know what.

Back to being a detective.

What do excessive thirst, interrupted bowel routine, lethargy, poor appetite, constipation, excessive urine output (that is colorless), decreased ability to handle overstimulation, increased myoclonic activity and fever spiking equal??

I don't know either.

Hypercalcemia? Skeqwampus depakote levels? Unbalanced nutrition?

Your guess is as good as mine :)