Is There Something Else Wrong With Your Kid?

Most of our family members, friends, and new acquaintances really don't know much about epilepsy.  We appreciate that others feel comfortable enough to ask us questions about his condition, and happily answer them to the best of our ability.  However, I do feel especially alert and protective when asked certain things.  Questions or comments that induce the Momma Bear in me may include:

"Your son is always unhappy, that's not normal."

"Have you had him assessed by a Psychiatrist?"

"Does your son have Autism?"

I am also often offered advice of how they would deal with misbehavior.

Now don't get me wrong, I am comfortable with these types of questions when I feel it's given with sincere concern.  I will admit it could be possible there are other conditions contributing to his behavior, but none we know of with certainty.  On the other hand, I'm not too happy when I feel these types of questions are posed with a judgmental and somewhat critical air. 

How to tell the difference?  Well, it's not simple.  I have to stop my mind from reacting instinctively and do my best to give the person the benefit of the doubt.  Sometimes it's not so easy to be generous in thinking the best of other's intentions. 

In attempting to answer some of these questions myself, it only takes a glance at our son's seizure history and the amount of medication he is taking, and has taken in the past, to see it's amazing he's doing as well as he is.  Wouldn't you be prone to mood swings and depression if you were that frequently sleep deprived or disoriented from seizures, and add onto that massive amounts of medication???

The topic of our son's behavior and cognitive abilities comes up frequently in our discussions with our Neurologist, and he has a word that completely sums up what our son is going through....Encephalopathy.

I turned to MedicineNet.com to describe what encephalopathy is and it's symptoms.

What is encephalopathy?
Encephalopathy is a term that means brain disease, damage or malfunction.  Encephalopathy can present a very broad spectrum of symptoms that range from mild, such as some memory loss or subtle personality changes, to severe, such as dementia, seizures, coma, or death.  In general, encephalopathy is manifested by an altered mental state that is sometimes accompanied by physical manifestations (for example, poor coordination of limb movements).

The term encephalopathy is very broad and in most cases, is preceded by various terms that describe the reason, cause, or special conditions of the patient that leads to brain malfunction.

The cause of encephalopathy in our son is clearly Epilepsy.

What are the symptoms of encephalopathy?
Despite the numerous and varied causes, at least one symptom in all cases is an altered mental state.  The altered mental state may be subtle and develop slowly over years or be profoundly obvious and develop rapidly.  Often, symptoms of altered mental status can present as inattentiveness, poor judgement, or poor coordination of movements.

Other symptoms that my occur include:lethargy, dementia, seizures...

Often the severity and type of symptoms are related to the severity and cause of the brain disease or damage

So to answer the questions mentioned at the beginning of this post, our son's behavior and mood is directed related to his epilepsy. 

Epilepsy Statistics

I just came across a very informative article on SheKnows.com intended to create some awareness regarding epilepsy.

It included some incredible numbers like the fact that epilepsy and seizures affect 3 million Americans of all ages.  That's double the number of people known to be affected by autism.

Go to SheKnows to read more.

Topamax

The most recent addition to our son's med cocktail is a drug called Topamax (Topiramate).

The improvements to our son's health have been amazing!! He has gone from having 2-8 tonic clonic seizures per day along with many absence seizures, to only 2-4 per week!

Now I don't want to get my hopes too high regarding this drug, seeing as we need to get past the honeymoon phase.  However, I can't help but feel grateful for any improvement to our son's health.  He is able to communicate better, wants to play with friends more often, and is regaining a desire to pursue his interests.  He is even picking on his little brother with renewed enthusiasm.

The recognition of any side effects from Topamax is still up in the air.  It is particularly difficult to tell what drug is causing "what" because he is on 3 different medications, but I'm keeping a watchful eye and hoping we don't see anything too severe.

Another possible treatment we are looking into, and has been received by others with success is the Ketogenic Diet.  We have several friends who have found the diet to work miracles in their children's seizure control and their overall well being. They are a wealth of knowledge and support if we do decide to go down that route.  For now, we are in discussions to with our Neurologist and Dietician to possibly start the diet after we've had time to monitor Topamax.

Oh there's more...we've added 2 more pieces to our puzzle:
A Naturopath who specializes in epilepsy and
An upcoming Ictal SPECT.

Meds, Meds, Meds...

The last few months have been ridiculous...so many medication changes!
In fact, I've been monitoring our big guy's seizure activity 24/7 for the last 2 months.  I slept in his room, and watched his every move so I could track EVERYTHING and know exactly what these drugs were doing, good or bad.

First off, our Big Guy was taking 5 different medications.

Keppra
Clonazepam
Lamotrigine
Valproic Acid
Hydrocortisone

Good news, he is no longer taking Valproic Acid.  I've talked before about the awful decline our son experienced as a result of this medication.  We're done with it.

Next we weaned him off the Lamotrigine because it didn't seem to help, and actually seemed to increase seizures.  Oh, it was awful.  We were so very close to taking our poor boy to the hospital because we thought he was in Status.

One night I counted 7 Tonic Clonic seizures in his sleep and countless absence, it was extremely upsetting. What was even more frightening was the development of new seizures, which were probably Complex Partials.   Our son would wake from sleeping and stare off into the distance, completely unresponsive and then look terrified of whatever he was staring at.  A few times he screamed in fear.  It was heartbreaking to watch and then hear him talk later about seeing something scary. He would ask, "When is this going to end"?

Since he's stopped taking the Lamotrigine, he's also stopped having his "scary" seizures.  No one misses those ones.

Well, I was feeling pretty good that we were down 2 meds, but there was another that we were leery to begin in the first place...the hydrocortisone. Hello, it's a steroid in an 8 year olds body...not good.  He gained probably 15 lbs within 6-8 weeks,  his blood pressure was increasing along with mood swings. We were working with the assumption that he had an autoimmune condition, and a steroid should have cut seizures down drastically.  It did not.

As much as these medications can do good, they can also do harm.  It is a huge relief to not give them to our son any more.  However, when so many medications are taken away there is usually another to take their place.  Our newest, and possibly last medication option: Topiramate, otherwise known as Topamax.

Famous Epileptics

A friend shared with me an interesting slideshow of famous epileptics featured on the LIFE website.

Take a look!

Family Fun Part 3

Day 3 To Do List:

- Geocaching...of course.
- Visit Prehistoric Creatures...who doesn't in their time off?
- Soak up some overdue sun...this one is permanently on the list.
- Wrap up our mini holiday...sad but true.

Took a brief break from our traveling to pick up a cache.

He had to a photo taken next to the first dinosaur he saw!

Yes! Real Dinosaurs!!

Always a nice performance for the camera.

Cute Baby is definitely more excited about his walking than being surrounded by dinosaurs.

A much deserved rest before heading home.

Close to Home Fun Part 2

Everyone in our family feels more alive in the outdoors, especially in the mountains.  So, naturally, we headed to the mountains on our 2nd day of family time.  We hiked, found geocaches, and had a little picnic where the boys were eager to help out with a camp fire. The day was just full of smiles.

Another Geocache!! 

Cute Baby learns early what to do with rocks when near a body of water.

He feels like his older brothers now!

Our happy hiker.

Geocache #2 for the day.

Happy Hiker turned Pirate.

Geocaching Fun

As mentioned in the last post, our fun plans changed from travelling to the U.S., to having fun close to home.  One of our favourite family activities is geocaching.  The kids love using the GPS to do a little treasure hunting.  And yes we live in Canada, so there is still snow everywhere but that doesn't stop us.  It felt so nice to get outdoors, get fresh air, and forget about the "other" trip.  ;)

On our path trying to find a cache.

Yeah, he likes snow a little too much.

Cute baby wanted to get out of the baby carrier and crawl in the snow like everyone else.

Me loving some snow time with cute baby.

We found it!! The cache was full of playing cards, like pokemon or bakugan, to trade.

Searching for the perfect trade.

Pre-Existing Condition Lesson Learned

The last week of March we had big plans to visit with family and friends in the U.S.  We notified everyone we wanted to be sure to visit...my husband's parents, some siblings, dear friends we haven't seen in years, cousins, and most importantly our Japanese daughter, an international student who lived with us for 3 years, who was leaving the country that week.

The boys were thrilled to see Grandma, Grandpa and their cousins, who we really only see maybe 1 or 2 times a year.  We had our bags packed, movies, music, and activities picked out for our long drive down.  We were set to go!

Three hours before departure we received some discouraging news.  My husband's travel insurance, which we previously checked to make sure we had coverage, notified us that they would NOT cover any costs relating to our son's "pre-existing" epilepsy if he had any changes to his drugs in the last 3 weeks.

We had just lowered one of his meds that week!!!!!! In fact, there's almost constant changes to his med schedule!!

Desperately we called several different travel insurance companies, looking for someone who might give us pre-existing condition insurance.  We had NO LUCK!!

The best we could find was a company willing to give us the insurance we were looking for, BUT he could not have had any drug changes in the last 7 days.  Again, we didn't qualify.  :(

The insurance agent also stated another condition that would limit our coverage, "if he has had any symptoms in the last 7 days from his undiagnosed condition".  Well, we know he has Refractory Epilepsy but is undiagnosed as to the cause.  I'm sure that little loophole may prevent us from travelling out of country for quite some time.

I feel a little naive about the whole situation.  I guess we've found our insurance extremely dependable at home, and didn't really ask enough questions for travelling across the border.  I shouldn't have been surprised, but it was still disappointing to call our loved ones and cancel our plans.

What did we do instead???
We did not want to make the scenario any more depressing, so we had some family fun close to home!!  Geocaching, hiking, waterfalls, fire pit picnic, dinosaurs here we come!

Disability Tax Credit

We received some fantastic news today! We've been approved for the Disability Tax Credit.  Which means we can claim credits to help reduce our taxes!

It's so nice when disability benefits work out and help to reduce our stress, even just a little bit.

What a Wonderful Couple of Weeks!!

Our son has officially had his best week in a long time.  It's been just over a week since his last IVIg treatment, and he is doing fantastic.  Only 1 seizure the night of his treatment and nothing since!  We're heading toward a record here: 8 days seizure free and counting.

Another amazing part of this week has been his mental functioning.  What a difference it makes when he doesn't have extra meds in his system and not having to continually recover from seizures.  He is so happy!  He's interacting better that he has in a long time with his little brothers, who absolutely adore him.  They're playing imagination games as much as possible, it's a blast to watch.  One day they pretend to be Transformers, the next day brings on another favorite superhero.  He's been talking about school activities more than usual.  The last few days his class has been participating in Mission Impossible in the gymnasium.  I'm not really sure what it is, but he's excited to tell me about the sharks, bugs and different obstacles they have maneuver around. 

I almost burst into tears of joy when I happened upon a little game he created for he and his brother last night.  Again I don't really know what the game was about, maybe like the Mission Impossible at school, but he had drawn pictures on several pieces of paper of different sizes and lined them up on the floor strategically.  They had the lights out, his little brother had on a toque and sunglasses, and they were getting ready to start playing.  If we hadn't been on our way out to our monthly Epilepsy Parent's Support Meeting, I would have jumped in with them.  I'm going to see if he'll explain it to me tonight!

Last week we also witnessed a major childhood milestone in our son's life: 2 lost teeth.

He's was incredibly excited about 1. pulling the teeth out and 2. $$$$.  He pulled the first tooth out, placed it carefully under the pillow and woke up the next morning to $2.  Same thing happened the next night and he received another $2.  What an easy way to make money!!  He's been excitedly telling everyone we meet about his lost teeth and new found venture.  One of his top front teeth is loose now and he can't wait to yank it out!

After loosing Tooth #1:

Day to Day Life with Epilepsy

If I could use one word that describes what everyday life is like with a child who has epilepsy, it would be UNPREDICTABLE.

I think that would be the answer for any mom, of any circumstance, in that the only thing you can really count on is the unexpected.

Let's take yesterday for example, our son woke up with an amazing energy.  He dressed himself, ate his breakfast happily, played with his little brother kindly and with words, not fists, when there was conflict, he was joking, laughing, and being his goofy self.  I loves days like that, they don't appear too often and everyone is happy and laughing along with him.

He was able to go to school and when I picked him up at the end of the day I was expecting to see him in the same mood.  Instead, I was greeted as always by his aide but along side her was his classroom teacher, which made me a little nervous.  Then I looked at my son.  He was walking with assistance and very pale.  They explained to me that he had just vomited all over his desk, and clearly wasn't feeling well.  I felt awful for him, not the best way to end a day at school.

Well he climbed in our van, we drove across town to pick up his little brother from play school, and then headed straight home.  By the time we got home, he was better.  In fact, he had a quick snack and was ready to pick up where he left off playing with his brother that morning.  I had to check on him a few times to make sure he was ok, but it was as though nothing happened.  The only mention of it was when his dad came home later and he said, "Dad, at school I barfed all over my desk but it's done now".  Well, I guess that's that.

The evening was going pretty smooth until he had an "unexpected" seizure.  It seems to happen like this often where our son is having a fabulous day and WHAM, he hits the floor.  He had left his helmet in his bedroom and didn't have it on when he fell, so the fall resulted in a goose egg on the side of his head and a bleeding nose.  Poor guy slept for the next couple of hours, took his medication, then went back to bed for the rest of the night.

I'd love to say the seizures ended there, but that's rarely the case.  The next day he had a long one lasting over 5 minutes and I had to give him midazolam.  Actually, he had 2 seizures that were much too long and required rescue meds, one in the morning and another in the evening.  Days like that he stays home from school and we all just spend time together.

It would be a fairly safe assumption that these seizure days are hard on the family.  They are difficult.  However, we've been very fortunate to have more good days than bad lately.  Days where I'm playing referee to all of our children, where we can participate in activities as a family, and days where we feel incredibly blessed to have 3 amazing little boys.

IVIg Treatment

We took our son to the hospital this week for his monthly IVIg treatment. Since I'm not really an expert, I'll use Wikipedia's description of what that actually is:


Intravenous immunoglobulin (IVIG) is a blood product administered intravenously. It contains the pooled IgG(immunoglobulin (antibody) G) extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months. It is mainly used as treatment in three major categories:

  Immune deficiencies 

  Inflammartory and autoimmune diseases

  Acute infections.

The Neurologist's theory is that our son possibly has an autoimmune condition.  There has been no definitive confirmation but since our son's medications haven't been enough to keep away any breakthrough seizures, trying the treatment is worth a shot.  It takes 6 months to see if it's helping or not, and he just had his 4th treatment.  So, we're still in the trial phase.

He is surprisingly very calm at these hospital visits.  We take him to the hospital first thing in the morning and are there until mid afternoon.  He's only 7 but seems tougher than the teenagers.  A lot of patients require freezing before the IV is attempted and we've heard the odd cursing at the pain, but not our little guy.  He just breathes right through it, even through the 3-4 attempts it takes to find a good vein.  His strength amazes me.  


On the plus side, he gets to order almost anything he wants for lunch, watches TV, plays Xbox and sometimes gets to play in an attached playroom.  In fact, I think he enjoys being at the hospital all day with so many fun things at his disposal.  It's reassuring too that when the treatment is finished for the day we get to go home.  :)

Valproic Acid Side Effects

As I've mentioned in previous posts, our son has undergone a severe cognitive decline in the last year.  The whole situation has been quite puzzling to everyone involved, our family and the health care professionals. Our son was just not displaying side effects in a typical way.  It was difficult to pin point what was causing his troubles.  Was it as a result of the seizures, the medication, the two combined, or an underlying condition?  We're still not sure what the answer is.  However, during one of our IVIg (intravenous treatment) visits at the Children's Hospital in November, our Neurologist came to us with an interesting theory.

Our Neurologist and Neuropsychologist had just attended a medical conference where a colleague shared his patient's experiences with Valproic Acid.  This doctor had 3-4 cases where he found a direct link between the use of Valproic Acid and Dementia.  Dementia being a serious loss of cognitive ability in a previously unimpaired person.  The Neurologist & Neuropsychologist immediately thought of our son, who was taking 18mL (900mg) of Valproic acid per day.  In fact, he has been on Valproic Acid for half his life.

With these new confirmed cases in mind, our Neurologist thought it would be best to start weaning our son off the Valproic Acid.  The possibility of the medication causing severe difficulties was pretty alarming since he had been taking it for so long, and so much of it.  Regardless, we were very happy to reduce the medication.  We felt that our son was possibly being over medicated anyway because he really seemed to be in a drug induced stupor all the time.

On the higher doses, our son was extremely tired all day.  He would sleep in so long that I would need to eventually wake him. He'd take long naps and go to bed very early.  Sometimes he would even need to sleep at school because he was so tired.  He had a lot of difficulty getting ready in the morning.  On a typical morning, he would require assistance using the toilet, bathing, and dressing. Each meal was spent coaxing or forcing him to eat, which would sometimes get vomited in resistance.  Reading a book would take an hour, when it used to take maybe 5-10 min.  I could go on, but he basically would need to be carefully walked through each step of his day.  He was also having several absence seizures throughout the day, which wasn't helping his already tired body.

After a month of reducing the Valproic Acid, we've seen a significant improvement.  I wouldn't say he's back to "normal", but we're starting to see more good days than bad.  Maybe 4 days out of 7 he's waking on his own, dressing himself, eating, talking more to us without phasing in and out of seizures, interacting more with kids at school, and his reading isn't as laboured.  There's still a lot of issues we're dealing with in regards to all of these things, but we're just ecstatic that there's any progress at all.

It made me wonder how much influence it had on him when he was 4 or 5 years old.  How much of his development had been impaired because of this drug?  I don't think we'll ever know the answer, and there's probably no point dwelling on it either.

Seizure Control & Medication

Seizure control?  Nope, and we're still searching for answers.  

His typical pattern is to have absence seizures throughout the day, and about 1-2 days a week he'll have a bad cluster day.  These cluster days aren't good.    He'll have multiple tonic clonic (grand mal) seizures, some back to back, some a few hours apart.  Each of these bad days results in rescue meds needing to be given.  We used to give him Ativan to stop the clusters, but I can't stand the side effects.  It takes a few days for this med to work out of his system, and makes him extremely disoriented.  We mainly use midazolam now.  It's quick acting but doesn't last as long in the body, so our son may have a cluster a couple of hours after the previous.

The cluster days always put me on edge.  They are also the main reason we decided to stay home for Christmas.  If our son has too many seizures back to back or if the rescue medication doesn't work, he could require emergency care.  The chances of us having to call an ambulance are high whenever a cluster day appears, so we tend to stay close to home.

All our current maintenance medication:

Valproic Acid

Clonazepam

Keppra

Vitamin B6

Lamictal

Our son also receives an IVIg treatment, which I can explain more in a later post.

We joke about how we feel like a pharmacy, which isn't too far from the truth.  

Severe Cognitive Decline

The last few months have been up and down to say the least.  As mentioned before, school had a rocky start.  We had difficulty getting our son ready for his early school day start.  Often we would need to wake him up by 6am in order to get him alert enough to be at school by 8am.  He would need help showering, dressing, and eating.  He wasn't really responsive and almost always too tired to really be anywhere but home most of the day.

We received more insight into our son's condition when we met with his Neuropsychologist.  She didn't provide any answers as to why he was progressively delayed, but she was able to help us see what his abilities were at the time due to his medical condition.  We knew that his overall functioning had steadily declined, and I knew just from reading with him that he was really struggling.  However, it was worse than we thought.  Our son went from an average 6 year old to an extremely low functioning 7 year old.  Most of the areas they were able to investigate showed that he was functioning much below the 1st percentile.  I was shocked with the realization that he had declined that much cognitively in just 1 year.  These results explained so much about him emotionally as well.  We really needed to simplify things for him; his routine, communication, school, expectations, tasks, everything.

With these assessment results, we, along with his teachers, decided to shorten his school days.  This would allow him to get a couple hours more sleep and enable him to be at school when he's more alert. This modification has been amazing.  Our son appears to have more energy throughout the day, is better able to perform simple tasks to get ready in the morning, is more engaged during class, and is interacting more with other children.  Everyone is so pleased with these changes in our son's abilities.  It gives me hope that maybe we'll keep getting glimpses of how our son used to be.  Maybe he'll continue to pull through all these layers of fatigue, medication and seizures to show us he's still in there.