First Day

Hey, sorry we dropped off there for a little bit. We have been more than a little hectic around here. To add to the list of natural disasters, there is now severe flooding and school has been canceled for the first time ever due to excessive rain. Here is Cole's first day of getting on the bus...it is his favorite part of the day.The mandatory first day of school picture. Isn't he cute?
We hope it lasts. We've had a couple of hiccups this week regarding the school, nursing, etc. For a moment or two I considered home school (which is what they wanted). I got my fight back and we'll see what happens next week!

Dog Shopping

I have been trying to get Cole to understand that he is getting a new doggy. He doesn't have the ability to understand the concept of a service dog, or what this one will do, or that it will go every where with him.

I've just been trying to get him to realize that he is getting a new buddy, and get him excited about it. This has been going on for a long time.

I have had him look at pictures of dogs. I have tried to talk to him about our former family members "The Girls", even showing him pictures and videos of them. He can not remember them. Only recognizes himself in the pictures. Leilani and Lily are a thing of the past for him without constant re-enforcement.

When I ask if he wants a dog, he exclaims, "Yes!" Then continues by saying, "Let's get in the car. Go buy one at the store!" You get everything at the store, silly.

Funny story, as I mentioned, according to him you can get anything by going to the store with monies, even a boat. He told his dad the other day that he wanted a boat. Brian said, "Oh, I wish! I would really like to get a boat too, buddy". Cole answered, "Let's go to store and get one!", totally matter-of-fact. If it were only that easy :)

To say the least, the excitement about the timeline for going to Ohio getting smaller and smaller has been reserved for Brian and I. We are counting down the days. We have even got coordinating Halloween costumes for Cole and his buddy!

Yesterday, I tried again to show Cole the Dogs In Training page to see if it would spark interest. It is like sitting down to a Mail Order Bride website, trying to scan only pictures and a brief profile, to choose a partner for life. Even though technically it is not our choice, it is still kind of fun, and weird all at the same time! Scrolling through the pictures and imagining which dog is going to change your world. I tried this before and he just looks and says, "Doggie!" and runs away to do something else. This time, he looked at every dog. I asked, "Which dog do you like?" He went down the entire page and pointed to every single one, making a comment. He repeated over and over, "I like that one!" or "I want that one". If I scrolled too fast, he would stop me and make me go back. He asked again this morning, "See dogs?" His concentration has improved since being off of the Nano VM and Carnitor. The seizures have started to increase again, but I really think that is because of the Depakote and Topamax. He still doesn't "get" that one of these dogs could be his, but at least he is looking!

Now I have been looking at this page for months hoping and dreaming about our new partner. I have my favorites, but I know that 4 Paws will choose the perfect dog for Cole.

So, every dog on that page is good enough for me :)

After the last few days being from you-know-where, this was a pleasant reprieve.

Which one do you like?

Stories Of Hope

Cole's story (a piece of it) is now being shared on Dravet.org!
You can go here to see it along with some of our other friend's stories.

Dravet.org, Dravet Syndrome Foundation and ICE Alliance are great resources for parents, doctors, families and patients. There are links to all of them on the sidebar of our blog.

We are so grateful to be a part of all of these networks.

The OTHER Cole

Right now, our good buddy Cole K is having brain surgery.

He is in great hands with Dr Bingaman at Clevland Clinic, who also performed our friend John's surgery last year.

He has been diagnosed with Infantile Spasms and they are taking out his left Temporal and Occipital lobes. This is a very complicated surgery.

Cole and his parents are in our thoughts and prayers today. We've been through some tough stuff together, but this is the most intense. They are SO brave...true warriors! We just want the best for our Cole, and theirs.

You can read the OTHER Cole's story here, or his link is on the sidebar of our blog.

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.