Monday, December 5, 2011

A Month of Miracles

It has been a little over a month since Will's surgery, and Will still remains seizure free.  Developmentally, he is doing great.  We continue to witness miracles each and every day.

Will has been working very hard in all of his therapy sessions.  He is seeing a total of 6 different therapists for physical, occupational, and speech therapy.  Sometimes he gets a bit exhausted, but he really enjoys all the attention he gets during his sessions and all of his hard work is really paying off!  He has been signing for "more" when playing and eating.  His gross motor skills remain strong, and he is slowly gaining better fine motor control.  He is enjoying scribbling with crayons, and loves to play with the Magnadoodle.  His favorite game is hiding in the dining room curtains and playing "peek a boo".

Communication still remains very weak, and Will has a long road ahead of him before he will be able to talk, but his therapists are optimistic that someday speech will come.  Today, he had a HUGE breakthrough as he learned how to request things by pointing at the object he wants.  Kelly cried tears of joy to watch our little Buster, as this is one of the basic building blocks of communication that a month ago we weren't sure if he would ever be able to do.  Of course, he is already being a little stinker and pointing at things that he isn't supposed to have because he knows we are so proud of him for doing it that it is tough to tell him no.  Another thing he is doing is standing in front of our fireplace at home and saying "Ha, Ha" - clearly trying to tell us that it is hot.  A few days ago, Kelly left the room and Will started to cry, saying "MaMaMaMaMa".  Not quite "Ma-Ma", but we'll take it. It was music to our ears.

People ask us questions about his surgery all the time, and the questions usually start out with "I know this is a silly question..." or "You probably don't want to talk about this, but...", so we thought it might be helpful to answer a few of the most common questions on here.  And, if you happen to see us and have more questions, ask away.  We really don't mind talking about it.

What happened to the space in his head where his temporal lobe used to be? Did they put something in there?
Nope, no prosthetic brain, no temporal lobe shaped piece of styrofoam. There is just a big empty space in his head now that is filled with fluid.

Does he have to wear a helmet?  How long will it take for his skull to heal?
No, he doesn't have to wear a helmet, but we do have to be very careful to make sure he doesn't fall down on his head.  The section of his skull that was removed was put back in using titanium screws, so it is intact, but it will take about 6 weeks for the bone to fuse back together, and a full 3 months until the skull will be completely healed.  The screws will remain in his head, and they won't show up on metal detectors so he won't have any problems going through airport security.

Are there any activities Will won't be able to do now that he had brain surgery?
Will won't be able to participate in contact sports like football, wrestling or hockey, although wrestling around on the living room floor and a game of touch football in the backyard will be fine.  His surgeon also said that he won't be able to ride rollercoasters.

How did the surgery impact his vision?
Although they did not have to sever his optic nerve, there are some branches of the optic nerve that runs through the temporal lobe which were removed.  As a result, Will lost the upper right quadrant of vision in both eyes, but because he is so young his body seems to have already adjusted for it and this should not have any effect on his future.  As long as his seizures are controlled, he will be able to drive a car.

What was with those fiducial markers they put on his head before the surgery?
During Will's surgery, the neurosurgeon used state of the art technology called frameless stereotactic imaging.  Will had an MRI the day before his surgery, and they placed fiducial markers on his head that looked like white lifesavers.  These showed up on the MRI, and they remained on his head during the surgery.  They were actually stuck on so good that they took the hair off with them when they were removed - he has bald spots where each of them were placed!  During surgery, they used a special positioning device that was actually screwed into his skull to keep his head completely motionless.  He has a tiny scar in the middle of his forehead and on the back of his head from this.  Now here's where it gets high tech:  Once his head was demobilized, the stereotactic imaging device matched up the image of Will's brain on the MRI the fiducial markers on his head, essentially projecting the image of the MRI onto the outside of his head.  A computer with the MRI image guided surgeons so that they were able to remove exactly the right section.

When will he have another EEG?  
Unless we see seizure activity, Will won't get another EEG until 6 months following his surgery.  During surgery, EEG leads were placed directly on his brain and these showed normal brain activity following the resection.

So, is he "cured" now?  Will his seizures come back?
Unfortunately, there are no guarantees, but we celebrate each and every seizure free day for the miracle that it is.  In order to be declared "seizure free", Will needs to go at least 2 years without a seizure.  He continues to take a low dose of Keppra as a maintenance medication in hopes of preventing a relapse.  Infantile spasms is a catastrophic form of epilepsy, meaning that the abnormal brain activity that characterizes IS can cause permanent damage to a developing brain.  We will only learn in time what damage, if any, was done to Will's brain as a result of 10 months of uncontrolled spasms.  For this, we can only pray, pray, pray.

Can he eat anything he wants to?  
Will is finally allowed to eat a normal diet.  We can't really blame him, but he really only seems to want to eat carbs right now, so he is eating a lot of Cheerios, pretzels and Goldfish crackers.  He needs to make up for lost time!

How is Jack doing with all of this?
Jack is doing awesome.  He is happy that his brother isn't having any more seizures, and when we pray together he says that this is what he is thankful for.  The two are interacting and genuinely enjoying each other.  Sure, there are still times where Will starts taking apart Jack's trainset as he plays with it, and there are times when Jack rips a toy out of Will's hands, but this is what brothers do!  We also see them play chase, play peekaboo, wrestle, laugh, and continue to interact rather than just coexist.  There were countless times when "medicated Will" really frustrated or hurt Jack, especially when he was on some of the nasty medications.  A hidden burden in all of this had been the lump in our throats as we would try to explain to Jack that his brother didn't mean to hurt him, it was because of the medicine that he was taking.  It is such a happy feeling for us to let the two of them have fun and just stand back, watch, and smile.

Sunday, November 20, 2011

Pressing Play

Things have been kind of crazy since we got home.  We are gradually trying to get life back to some kind of normalcy.  It took Will a while to realize that he was safe here, and no one was going to poke him or try to draw blood here at home.  But, each day he has cried a little bit less and played a little bit more to where now, 2 weeks later, we finally feel like his is able to relax and is getting back to life as a toddler.  We were actually able to go to church as a family this morning!

Since surgery, Will has been on a special steroid medication to keep his brain from swelling at the surgery site.  He is also still on Keppra to keep the seizures at bay, and Pepcid to combat the reflux caused by the steroids. Just 10 days after surgery he was off of all pain medication.  He is one tough little boy!

So far, Will has not had any seizures.  That is a miracle in and of itself!  He had a look on his face a few times shortly after we got home that made us nervous, however we think it might have been a side effect of the steroids.  We are thrilled to be living life without any seizures right now, and we couldn't ask for things to be going any better with him. We know the seizures could come back at any moment, but we are thanking God for every blissful day without them.

The capabilities of Will's brain essentially froze back in January when his Infantile Spasms started.  He was just beginning to learn to wave and clap back then, and those abilities disappeared once the spasms (seizures) began.  At that time, his mental abilities slowed to a crawl.  We tried and tried to get him to wave or clap again, figure out new things, focus on children's books and pictures, say any words or make sounds, but he would just look at us and smile.  That beautiful smile that managed to take away some of the sting of watching your child and knowing that he had a very long road ahead of him. It was like someone took his brain and just hit "pause". As parents, it was one of the most painful and terrifying things you could ever imagine.

Suddenly, he is now very focused and able to perform new tasks.  Most of them are very small things that only we would notice, like how he now goes the opposite direction around the kitchen table to catch Dan when they are playing chase.  Some of them are bigger things. He is waving consistently. He has just begun clapping again, and is very proud of himself for doing it.  He is making sounds on command:  Da, Ta, Pa, Sss, Eee.  He mimics the way we pat our dog Duke on the back.  He initiates playing with us.  All of these are things that he couldn't do 2 weeks ago.  At dinner, he began to imitate the sign for "more", something we have been trying in vain to teach him for over a year.  He seems like his development is picking up right where he left off back in January, as if the the surgeons took his brain and finally pressed "play".  We can't wait to see what he does tomorrow!  

Will has an incredible appetite right now.  One night this week he ate 1 1/2 full chicken breasts for dinner along with a lot of noodles, bread, and cookies.  The poor guy was food deprived for so long when he was on the Ketogenic diet that he is now making up for lost time.  Of course, the steroids have contributed to his voracious appetite as well.  The size of his belly and the way it sticks out almost defies gravity.  Believe it or not we really are trying to pace him and his eating habits!

Will's stitches were removed last week at the Clinic.  He knew the building instantly, and when the 2 nurses walked into the room he was immediately uncomfortable.  He has learned that he is ALWAYS the center of attention in a doctor's office, and he would prefer to be invisible.  Imagine every time you are taken to a room and the door closes, and all you know is that the people who just walked in are looking at you and you know they are going to hurt you.  It took 3 of us to hold him down, and another to remove the stitches.  After what felt like an eternity but was really only about 30 minutes, his stitches are now out and his incision is healing well.  It is still very visible and he still fusses with it from time to time, but eventually it will heal and his hair will grow out and hopefully cover most of it.  

This past week, Will had a physical therapy evaluation, and did very well.  We were both there, and that seemed to give him a level of comfort.  He played with the toys that they had, and threw a ball to Dan, which was a huge surprise.  The therapist was very surprised with his ability level, as she sees a lot of kids who have recently had brain surgery and said he was doing excellent.  He had speech therapy this week too, and for the next several week he has a full slate of physical, occupational and speech therapy.  Even though Will is doing well, he is still very delayed developmentally and has a lot of hard work ahead of him.

It is truly miraculous to watch Will learning new things each and every day.  To watch him, it seems unbelievable that barely over 2 weeks ago he had a large section of his brain removed.  He did not lose a single skill, his personality is exactly the same, and he is surprising us all the time with his amazing recovery.  Everyday is a little bit better than the day before.  

Wednesday, November 9, 2011

It's Good to be Home

This would have been posted a lot sooner today, but Will has suddenly found a real interest in the laptop, and every time it was out he had to sit on our lap and peck away at the keyboard - a great sign!

We got home yesterday afternoon, and Will slowly let his guard down and is now happy to be home.  We picked up some burgers and fries on the way home, and when we sat Will in his chair and put some fries in front of him, he actually fed himself!  This was the first time since surgery that he wasn't clamoring for one of us to hold him, and he suddenly had one heck of an appetite.

His stitches are holding nicely, he is handling the pain pretty well, and he is only on ibuprofen and tylenol for painkillers now.  And best of all, still no seizures!

It was great to see the view from our living room window last night rather than the view from the hospital window, and it was a beautiful evening as well!

After we got home, he was pretty clingy to the two of us.  Sleeping is still an extreme challenge.  He basically demanded that one of us hold him while the other be next to him, otherwise he would cry.  Also he is quite startled by loud noises, even loud multiple conversations have made him pretty upset.  We hope it is because he has clarity in his head like he's never had before, and things like this bother him now that the irregular brain activity has been removed.

During the course of the day today he really seems to have let his guard down, and it appears that he has begun his progress.  He seems very aware of his surroundings, and he is playing with his toys on the floor without either of us nearby.  He is engaging his toys as much as before (if not even more so), and seems to be really enjoying himself.  He waved several times today, not a great wave but very deliberate, which we haven't seen in a long time.  He still has moments where he suddenly looks around and realizes that we are farther away than he would like, and moves quickly to get to us.

Now is where the real test begins.  We need to start watching his development and helping him as much as possible, wherever possible.  We will now find out whether his delays are solely because of the bad brain causing seizures that was removed, or if there are other issues in his brain that are causing this as well, and there may be nothing that we can do about them (scary thought).  It would be so great if he could start talking.  We have to approach this as if the seizures are coming back, so we can't waste any time in trying to get his mental development moving.  Who knows- the seizures might come back tomorrow, and freeze his development again.  As soon as we can push him to learn things we need to do so.

It's good to be home!

Tuesday, November 8, 2011

Free at Last!

We are in the room, all packed up and ready to go!  They have pulled all of the needles out, bandages are off, and the last of the torture is done.
Will is sleeping on Kelly's lap.  All of the doctors and specialists have given their final blessings and we are just waiting on some discharge paperwork and prescriptions and we are GOING HOME!!!!

Monday, November 7, 2011


Will and his very tired looking Mama
We are sitting in the hospital room, and for the first time since his surgery...Will is showing interest in a toy! He has pretty much done nothing but cry or sleep for the past few days, so this is an awesome change.  We are starting to see glimpses of our little boy again, and that is a wondrous thing.

Clinically, Will is progressing very well.  He is eating and drinking again, his incision is healing well, and his swelling seems to be somewhat stabilized.  All of this adds up to a very good thing...if all goes well, we are going home tomorrow (Tuesday)!  The doctors all agree that Will is just not going to be happy until he gets out of here.  The poor kid stiffens up in fear everytime a nurse walks past our room, let alone comes in.  Not that we can blame him.  He has had so many blood draws and IV lines run that the nurses are having a hard time finding an "unused" vein.  His arms and legs are bruised and purple.  The poor kid needs a break.  We will find out more on rehab therapy tomorrow, but they going to be setting it up on an outpatient basis, which means we are all going to get to sleep in our own beds again.

We continue to be humbled by the outpouring of support that we have received for our little Buster Beans. We have gotten so many emails, texts, messages, wall posts and cards that it is impossible to respond to them all.  Will has people from around the globe praying for him, from close friends and family to total strangers, all who hear his story and want to do their part.  In the past week, Buster's Blog logged almost 4,000 hits!  Unbelievable.  From the bottom of our heart, we thank all of you!

And, perhaps the most important update of far, so good.  No seizures!

According to Plan

Yesterday (Sunday) was day #3 post-surgery.  Will experienced a lot of discomfort during the course of the day, and it seemed like a long wait every time he needed more Codeine.  He is not exactly happy, although he is absolutely not giving up.  They had to do another blood draw yesterday, and boy did he put up a fight!  As tough as it is to watch them pin him down (and even tougher to participate in), it is great to see how much strength he has when he needs it.  It's amazing that a 23 pound boy needs 4 people to pin him down to get a blood draw.

During the surgery, they had to sever some of his jaw muscles when they made their incisions.  Because of this, we were told that chewing will be very painful, and it will contribute to headaches.  So we have been taking it easy with feeding him and trying to choose softer foods from the hospital menu.  Yesterday his appetite finally showed up - he ate a full plate of Macaroni & Cheese and a Salisbury Steak.  He also drank more fluids yesterday than we have seen him drink since the surgery.  These are signs that he is traveling farther down the road to recovery.  His mood is sort of sad, he just sits and cuddles with us and looks at the TV or whatever else he feels like looking at.  We have gotten a few very faint grins but not the glowing smiles that we are used to.  Hopefully as his jaw heals the smiles will come back.

Will's brother Jack saw him (and us) last night for the first time since before surgery.  It was so great to see his big grin and to get a running hug from him, which neither of us will ever forget.  We spent a lot of time preparing Jack for surgery so he would be ready to see his brother afterwards and not be affected by it too badly.  He came into the room, looked Will over for about a minute, and went back to playing with his cousins down the hall.  It couldn't have gone better.

So, it must be very obvious to our visitors how tired we are, based on the multiple "Wow, you look tired" comments.  We have some awesome family members who got us a room at the Intercontinental Hotel which is connected to the hospital.  This has given us an opportunity to escape to the room and shower and get some uninterrupted sleep.  Dan went to the room last night and came back at about 6:30 this morning, and then Kelly went over to the room until about noon today.

The doctors are pleased with where he is in his recovery, as well as his level of function now that they removed a part of his brain.  There have been no setbacks or surprises, and none of the dreaded "S" word, as one of the nurses calls it.  So far everything is going according to plan, and hopefully will continue that way.  Now that we are rested and cleaned up we are trying our best to help get our little Buster out of here.  We think he'll really perk up if we could just get him home.

Saturday, November 5, 2011

Day #2 Done

Will had a pretty rough day.  He just wasn't happy with anything.  He cried a lot, and felt like he got tortured by the wonderful nurses here who are doing their absolute best to care for him.  He really hates seeing anyone wearing scrubs walk in the door.  He was uncomfortable all day, and kept switching back and forth between Dan and Kelly every few minutes, hoping that one or the other would provide him with the relief or comfort that he was desperately looking for.

He only drank about 5 ounces of apple juice today, and barely ate anything.  He just wasn't interested and seemed like he did not have an appetite.  We got him to eat about a 10 spoonfuls of applesauce but he wasn't happy about it.  So, they gave him an IV bag.  After he had this bag, his Codeine dose, and some Tylenol, he perked up tonight.  He ate a small pack of graham crackers and drank some Gatorade, and then got comfortable on Kelly's lap and gazed at her for a while.  For the moment he was content, and he fell asleep.

He had his good moments today as well, looking at books, pointing at things in the books, and making some of the same noises and sounds that he made before he had the surgery, which makes us wonder if his brain had already moved on and began using other areas to begin his speech development. Hopefully, if nothing else, the absence of the left temporal lobe will mean that the seizures are done and the static in his brain is gone.

The Bandages are Off

Will is continuing to improve.  We had a bit of a rough morning because he had to get poked and prodded several times, but he is now resting comfortably.  He has some swelling, which we expect to get worse before it gets better, but right now it is not terrible.  The doctors removed his bandages this morning so we are now able to see his incision for the first time.  It is a bit larger than anticipated, but it looks like it is almost all in his hairline.  They didn't have to shave much of his hair, so most of his little blonde curls are still there.  It is great to get to kiss that little head again!

Friday, November 4, 2011

Recovery Continues

Just a quick update...Will has now been moved out of the P-ICU and into the Pediatric Epilepsy Monitoring Unit, which is a good sign that he is recovering well.  We were so happy to see everyone and they were happy to see us.

All of the wires, tubes, and needles that were connected to Will are gone, except for one IV in his foot just in case he needs something quick.  This was great because it meant we could put clothes on him, and it also makes it so much easier to hold and comfort him.  He has progressed from Morphine through IV to Codeine orally.  He has been drinking apple juice, but hasn't been able to eat much other than a few Cheerios and a few spoonfuls of Chocolate pudding.  He actually walked a few steps from Kelly to Dan earlier tonight, and we were happy to see that he has the ability and the strength to do so.  Also, since he is out of P-ICU, they take vitals every 4 hours instead of every hour on him.  He never likes getting vitals taken.

Will's Post-Op MRI
The post-op MRI came back great.  All remaining brain tissue looks healthy, and there is no sign of stroke, hemorrhage, etc...just a little post-operative swelling.  The MRI image now shows the section of brain which has been removed and it just looks like a dark empty space.  The surgeons were very pleased with what it showed, and they feel that everything that needed to be removed was removed.  In the end, they had to take his entire left temporal lobe, including the hippocampus, but doctors are optimistic that Will should be able to regain all function using his right side.

Now that the drain has been removed from his head, and they are beginning to taper off the anti-inflammatory drugs, the swelling could get pretty bad.  Our nurse cautioned us that day 3 could be the worst day for swelling because of the timing of these things.  We are already seeing the swelling moving forward across Will's temple and towards his left eye.  We'll see how he is in the morning.

As of right now he has been laying in bed cuddled up with Kelly, sleeping mostly.  He wakes occasionally and looks around, whimpers a little bit and goes back to sleep.  We'll see what tomorrow brings, but as of now everything is still going as planned!

One Miserable little guy

During the night, Will pulled out 2 of his IV's and came close to pulling the drain tube out of his head.  They took mercy on him and pulled out his catheter before he pulled it out for them.  He is not sedated, but is being given a steady stream of Morphine every 4 hours.  After about 3 1/2 hours it becomes apparent that the Morphine is wearing off and that last half hour seems like an eternity.  They came to the room late this morning and removed his drain tube from his head, and it took 4 of us to hold him in place for the tube to be pulled out and 2 stitches put in place.  Needless to say, he is getting a reputation in the ICU as a tough little fighter.

Will is sleeping for the most part, and has spent a lot of time on Kelly's lap all snuggled up and doing his fair share of crying.  We can only imagine what kind of a headache he must have, and the confusion that is going on in his little head right now.  He is still gaining his color back, and is looking better all the time.  The swelling is not too bad yet, but we have been cautioned that after up to 48 hours of surgery he might look like a boxer after a fight.

We are going to be following him to a post-op MRI shortly, and after that we will be moved back to the Pediatric Epilepsy Monitoring Unit, which is where we have spent all of our other stays while at the Clinic.  We are looking forward to seeing all of our old friends in that unit!

Lots of doctors, nurses, and specialists have been rolling in and out of here day and night.  Lots of questions and lots of readings, blood samples, etc.. to make sure he is OK.  Despite all the difficulties with him right now, it is a great thing that he is so full of fight, and is resisting us every step of the way.  We might be pretty scared if he just laid there and let them stick him with needles, etc...

So far, so good!

Thursday, November 3, 2011


We are now in the room with Will, holding him in our arms and rocking him.  He is pretty out of it, is very pale and has a lot of tubes coming out of him but he is able to move all of his limbs and his vital signs are good.  We are rejoicing that he made it through surgery and is now on the road to recovery.

The surgeon said that the surgery went more or less according to plan.  It was no surprise to all of us to hear that our little warrior was strong throughout the procedure.  The surgeon removed the areas that they anticipated, along with an additional section of the temporal lobe to the posterior of his brain.  During surgery, the intraoperative monitoring showed a fairly large amount of abnormal spiking brain waves coming from the posterior of the left temporal lobe. After the area was removed, the abnormal spiking stopped.  This is a VERY good sign, and definitely gives us hope that the surgery could be a success.  He also said that the brain tissue that was removed felt very dense and rubbery, signs that show him that there was cortical dysplasia, or a malformation, present.  Although we will need to wait for the pathology reports to know for sure, we were able to breathe a huge sigh of relief, as we feel like given what they found inside Will's brain, we know in our hearts that proceeding to brain surgery was the right decision.

Brain surgery is not for the faint-hearted
By the time we were able to get into the P-ICU to see him, it had been a very long day.  We were up at 3:00AM, and as you can imagine, stress levels were fairly high throughout the day.  It was no surprise that Kelly's brother Andy felt faint and had to lay down on the couch in Will's room.  Just a few minutes later, Kelly started to pass out and had to join him.  Not sure they will ever hear the end of this one, and of course everyone was ready with cameras to capture the moment.

It sounds like we are in for a long night.  They need to check his vitals every hour throughout the night, and they told us that we should take turns sleeping and watching over Will, so it doesn't sound like there will be much rest for the weary.  In the morning, Will is going to get sedated again (for the 3rd day in a row) for another MRI.  They are hopeful after that is over that we will get to move out of P-ICU and into the regular epilepsy monitoring unit.

Thank you again, everyone, for all of your thoughts, prayers and well wishes.  While we may not be able to respond to every message, we have read all of them and it has helped to keep us strong throughout all of this.  We are truly blessed.

Surgery Complete!

Just got an update (12:31) that the surgery is complete, and everything went according to plan.  We are waiting to talk with the surgeon and will hopefully be able to see Will in another hour or two.

Surgery Update

Update from the operating room (11:58):  Closing, Will is still doing great.

This is great news because it means that they didn't have to remove more tissue that what was planned!

Guardian Angels

Still waiting for an update...the hours are passing slowly around here.

Will is blessed with several guardian angels in the operating room with him today.  Back when Will was on ACTH, the home health nurse who came to our house on many occasions to do Will's blood pressure checks just happened to also be the surgical nurse for Dr. Bingaman.  It was such a welcome sight to see her face this morning and know that she was going to be in the OR with Will.

Next, we have Gabrielle, our EEG tech who is wonderful and has been with us through every step of the way on this journey...every inpatient admission and then some.  She just sent us a text to let us know that one of the epilepsy nurses who has children our age is assigned to Will's case went into the OR to let everyone in there know how special he is!  

Will has an amazing team caring for him today, between all of those here at the Clinic, our family sitting vigil with us, the amazing doctors that are caring for him and all of our prayer warriors who are caring for him on the outside.  Thank you everyone!

Beginning Resection

Update from the operating room (10:34AM):  Beginning the resection now, Will is doing great!

The resection is the actual removal of Will's brain tissue.

Halloween Pictures

A few days late, but here are a few pictures of the boys in their Halloween costumes.  Jack was a fish and Will was a cowboy.  Need to lighten the mood a bit!

Will is still in surgery, and we just got a visit from Gabrielle (our FAVORITE EEG tech) who popped in to the operating room to check on Will and watch over him.  Thank you, Gabby!

Surgery Began

Surgery began at 8:43 am

Kissing Goodbye

We just left the operating room where we kissed our little Buster goodbye.  He flashed Kelly one of his big, beautiful smiles just before he fell asleep.

Wednesday, November 2, 2011

Final Checks

We went to the Clinic today for some more pre-surgery checks, a pre-surgery physical, more bloodwork and an MRI.  Will was sedated, and came through it just fine.  His cold seems to be clearing up which is good news.  He now has some special fiducial markers glued to his head that they will be using during the surgery tomorrow.  They are essentially used as "landmarks" that show up on the MRI so the surgeon knows where these are located on the outside of his head and how that corresponds to what is inside of his head.

We are to be at the Clinic at 5:30 tomorrow morning, surgery is scheduled to begin at 7:00 AM.  They will be giving us periodic updates via pager.  We need to be prepared that the surgeon may contact us during the surgery and let us know that the intraoperative monitoring or the texture of Will's brain tissue indicates that more needs to come out.

We still have some major bumps in the road ahead of us, and a lot of work in the course of Will's rehab.  We will be trying to periodically update the blog during the course of all of this in the course of the next few days.  The posts may be short, vague, or worse, but we'll do the best we can.

Over the past few days, we have been overwhelmed with support, prayers, and messages of all kinds.  We are so touched to know how that so many people love our little boy.  We will never be able to thank everyone for all of your support!

Sunday, October 30, 2011

Countdown to 11.3.11

Will stuffing his face with Cheerios
We are counting down the days until Will's surgery.  It is so difficult to believe that in just a few short days we are going to be removing approximately 10% of our little Buster's brain.  We have been very busy getting everything lined up with Will's doctors for the surgery, and making sure that all of our plans are set.  Most important, we have been trying to spend as much time as we can with our two boys before the big day.

Will is now completely off of the Ketogenic diet.  We started by gradually reducing his ratio over a 2 week time period, and then slowly began introducing "normal" food.  We gave him a few McDonald's french fries, and looked it up in KetoCalculator to see how far off his ratio it was.  For months, every bite we put into Will's mouth was at a ratio of 4:1 (4 grams of fat to every 1 gram of everything else) or 3:1.  We were shocked to see that a McDonald's french fry has a ratio of only 0.4:1...meaning that every meal we have been giving Will while on the diet had TEN times the fat of a McDonald's french fry! Yikes.

Will can now eat anything he wants, and he can have as much as he wants.  Surprisingly, Will became so used to his limited meal selection that all of this new food with texture and taste is very different to him. He is refusing some things that we thought he would definitely eat.  We took him out for ice cream as a special treat, and he spit it right out.  We got him a smoothie instead which he loved, and he even had fun sharing it with his brother.

Will enjoying his treat
As part of Will's pre-surgical appointments, we met with his neurosurgeon, who went over the course of the surgery in detail. We were pleasantly surprised to find out that they want to remove less brain than we originally thought. After they reviewed all of his MRI images in detail (by the day of the surgery he will have had 4), they are optimistic that they may be able to spare a portion of Will's left temporal lobe that appears to be healthy brain tissue called the hippocampus.  The hippocampus plays a key role in short term memory, a function that is obviously important for daily life, so we were thrilled to hear that they might not have to remove it.

Will is going to need to have a blood transfusion during the surgery, and his surgical team asked for 2 units of blood.  He is too little to bank his own blood, and unfortunately neither of us were a match, as Will has a fairly rare blood type.  Luckily, Aunt Faith and Aunt Mary are both a match for his blood type and have already had their blood taken by the Red Cross and it is ready to go.  We think it might be a sign of all the prayers being lifted up for Will that he is getting the blood of FAITH and MARY.  Thank you both so much, and thank you to everyone who was willing to donate but was not a match!

Will is now off of all drugs except for Keppra. We are happy with this because Keppra is not a severe drug like many of the others that he has taken, and it has very little negative side effects for Will.  If the surgery works, he will likely need to remain on this as a "maintenance medication" for one year following his surgery, to prevent a relapse of seizures.  We are prepared to put Will back on the Ketogenic Diet after surgery if we have to, but Will's doctors are optimistic that if the surgery is successful we won't need to.

Will has a lot of pre-surgical testing ahead of him this week, including an additional MRI.  Right now he is fighting a cold that we are hopeful will pass soon so surgery will not need to be postponed.  The actual surgery on Thursday (November 3) is most likely going to be first thing in the morning, so we will need to report to the Clinic at around 5:30 AM. They expect that the surgery will last approximately 5-6 hours.  He will stay in intensive care for about 2-3 day, followed by 3-5 days in the pediatric epilepsy monitoring unit, followed by up to 3 weeks of rehab.  We won't know until after surgery what type of rehab therapy Will is going to need, which might require a longer hospital stay, although we are hoping that we will be able to be able to do it on an outpatient basis.

Sharing with Jack
All of this for what his doctors estimate is a 60%-70% chance of success.  That is the most difficult part in all of this...we don't even know if it is going to work.  A year ago, we would have never imagined that we would be surgically removing a portion of little Will's brain, but we feel that we owe it to him to give him the best chance at a normal life as possible.  We are so thankful that he is too young to know what is about to happen to him.  He doesn't know to be scared.  Unfortunately for us as parents, we do, and we are terrified.  Please pray extra hard this week for our little Buster Beans that the surgery will work, and pray that we can maintain the presence of mind we are going to need to get through this.

Just a note for those of you who receive email subscriptions to this blog - the blog emails can only be scheduled to go out once a day, and we plan on trying to post updates throughout the day on Thursday.  If you want to see those updates when we post them, you will need to either check the site manually from time to time, or else friend request Kelly on Facebook as we will try post the updates there.

Friday, October 7, 2011

In Search of a Crystal Ball

Will at 3 months, long before we had ever heard of Infantile Spasms
This week we traveled to Baltimore to meet with the head of the Johns Hopkins pediatric epilepsy department to get another opinion prior to Will's surgery.  It was an exhausting few days, but we actually were able to enjoy a few highlights of the city, and had a good appointment as well.  We did ask him if he had considered practicing in Aruba or maybe Hawaii to give us an exotic destination to travel to, and his fellow said he would much prefer that as well.  We might be on to something, although we must admit Baltimore is a very nice, clean city with a really awesome harbor and great historic architecture.

Dr. Baltimore's opinion (we don't like to use their real names on the blog) was somewhat in line with the opinions that we already have.  Surprisingly, he said that he can see a subtle left temporal lobe focal point for Will's seizures on the EEG, but felt that Will's MRI looked clear and he did not see any malformation.  This is a little different from what the neurologist in Detroit said, as Dr. Detroit said he could clearly see a malformation in the left temporal lobe on the MRI images, but he was unable to detect a focus on the EEG.  Of course, Will's doctors at the Clinic have said that they see a focus on the EEG pattern as well as a malformation on the MRI images in the left temporal lobe, but all doctors agree that whatever is there is very subtle and not glaringly obvious.

Essentially, we have three of the top specialists in the world for Infantile Spasms, all stating that they see some type of abnormality in Will's left temporal lobe, but they differ in how they view the abnormality.  All agree that something just doesn't seem right in that area of the brain.  This makes sense to us, as the left temporal lobe is the area of the brain responsible for speech, which is the most pronounced area of Will's developmental delays.

Seizures can be generalized (coming from all over the brain) or they can be focal (coming from a specific location in the brain).  However, sometimes it can be hard to detect a seizure's focus, because often although the abnormal brain activity may originate in one area of the brain, the abnormal activity spreads throughout the entire brain as a result.  Dr. Baltimore said that even though Will has significant delays, Will is doing very well when compared with other IS children his age, a blessing that we are thankful for each and every day.  Many infantile spasms kids cannot walk, sit up, or even hold up their heads.  To him, he felt that this would indicate that Will has a small structural brain abnormality that is causing Will's seizures.  In other words, if the seizures were generalized and coming from all areas of the brain, Will would not be doing as well as he is because that would indicate that there are abnormalities throughout.  In his opinion, it is much more likely that there is some small area that is the focus of Will's seizures, which appears to be the left temporal lobe.  Dr. Detroit essentially told us the same thing...he said that he would be willing to bet that Will has a focal point to his seizures, because Will is very highly functioning when you consider his diagnosis and his EEG pattern.  However, as you may recall, Dr. Detroit recommended holding off on surgery until a more clear focus point was seen on EEG.

Dr. Baltimore agreed with the Clinic that surgery to remove the left temporal lobe would be a good next step for Will, and he also agreed that we should not remove the occipital and parietal lobes at this time if we can avoid it.  In his opinion it was a question of timing, as to when we felt ready to proceed with surgery, or whether we want to try additional medications or the Ketogenic Diet for a longer time period.  Since he is in charge of the Ketogenic Diet program at Johns Hopkins, the hospital that pioneered the diet, he said that he generally would keep a child on the diet for a while longer (6 months or even a year) before proceeding to surgery.  However, he felt that while we may be able to modify the Ketogenic Diet to attempt to further reduce Will's seizures, he did not feel that Will is likely achieve seizure freedom from diet alone.

Dr. Baltimore again confirmed that the Clinic has been very thorough in the battery of tests that Will has undergone thus far, and felt that we have tried virtually everything he would recommend as well.  He also agreed with the approach outlined by the Clinic as to pre-surgical testing, which was comforting.

All in all, it was a very good appointment and we are glad that we went.  If we combine the opinions of all of the neurologists we have seen, even though they are not all in complete agreement, we actually feel we are making a well informed and appropriate decision to have surgery to remove Will's left temporal lobe.  Regardless, we have a lot to think about in the coming month, and we will continue to explore and research other options to feel confident we are making the right choice.  We just wish we could find a crystal ball that would tell us if it will work.

Tuesday, September 27, 2011

Decisions, Decisions

Last week, we made the most difficult decision of our lives, but it was a choice that we had to make.  Our little Buster Beans is going to have the left temporal lobe of his brain removed on November 3rd, at the Cleveland Clinic.

Deciding to have surgery was a very tough decision.  Deciding what part of his brain to remove was agonizing.  We want to make sure enough is removed to stop the seizures, but we also know that once brain matter is removed, you can't put it back.  The current plan is that during the surgery, they will do continuous brain monitoring, and if it appears necessary, there is a chance that the surgeon may also have to remove the left parietal and occipital lobes as well. This would likely be done either the same day or in an additional surgery a few days later.  We are praying that won't be necessary, as removal of the occipital lobe means that they will have to sever Will's optic nerve, permanently reducing his field of vision by 50% in both eyes.  Stopping the spasms is, unquestionably, our number one priority, but we are hoping to spare his vision if at all possible.

We are still traveling next week to Johns Hopkins in Baltimore for another opinion, and are hoping to get additional answers as to how much of Will's brain should be removed, but we really don't feel that it is in Will's best interest to postpone the surgery any longer.  His seizures have been pretty bad lately, both in duration and in intensity, and his development still remains stagnant.  Nothing we try seems to work, and it is absolutely heartbreaking.  We started weaning Depakote last week, and we will also wean him off of the Ketogenic diet prior to surgery so that we don't have to worry about his blood sugar and can give him IV fluids during surgery.  We are really looking forward to seeing him eat a big meal to his heart's content-he will be so happy!

As you can imagine, we have been completely overwhelmed trying to make arrangements and prepare ourselves for what lies ahead.  We were told that Will will likely spend 5-7 days in the hospital following surgery, followed by up to 3 weeks in the hospital for rehabilitation and therapy.  The most critical time period for his brain and skull to heal is the first 6 weeks, so Kelly is going to be taking a leave of absence to care for him until he has recovered.  Thankfully, we have a lot of family and friends who will be there to help support our family during this difficult time.

We honestly have no idea how we are going to get through this.  His happy little spirit has been incredible.  No matter what he is going through, whatever treatment we have made the decision to put him on, he still smiles at us like we are his entire world.  For now, we are just cherishing and kissing his little blonde curls as much as we possibly can.

Thursday, August 25, 2011

The Rollercoaster Ride

Will loves to go down the slide!
Well, our last post reported 7 consecutive days seizure free...a miracle in and of itself.  Will actually made it 10 days without a seizure, then BAM. He had a short minor seizure, but it was a seizure and the party was over. For us, those 10 days felt like Christmas every day. We thought we FINALLY had it, and we were actually starting to imagine what our lives - Will's life - could be like without seizures.  We so badly wanted Ketogenic Diet to be Will's miracle, but now we are back on the emotional rollercoaster ride again.

Since those ten days, he has been in a backslide, and little Buster Beans is back to daily seizures.  He is still having less seizures than he did prior to the diet, but they are lasting longer.  This past weekend, he had a seizure that lasted 27 minutes.  Think of holding your child for 27 minutes while he just continues to seize and seize, and maybe you can imagine how heartbroken we felt.  We are trying anything and everything we can to get back to where we were before, to no avail.  They have even decreased Will's calories back down to only 900 per day so that he gets even less carbs, but we haven't seen any improvement.

Reluctantly, we gave Will his first dose of Depakote, another medication, last night.  We were very hesitant to do this, because even though he is still having regular seizures, Will has been so happy and alert since coming out of the medicine fog caused by some of the heavy hitting meds he was on just a short time ago.  Depakote carries with it a risk of liver problems, and increases the risk of kidney stones from the Ketogenic Diet, although it is a fairly mainstream antiepileptic medication and its side effects are much less than others we have tried.

Things definitely haven't been all bad.  A few weeks ago at one of Will's therapy sessions at the Clinic, Will showed interest in the therapist's iPad.  We borrowed Grammy and Grandpa's iPad to try it out with Will and we were amazed at how much interest he showed in it.  After 3 nights of working with him, our little Buster Beans was actually pointing at the screen with his index finger!  We have spent HOURS in therapy with Will trying to teach him to point, with little success.  Three nights of the iPad, and he was doing it!  Since then he has also started making a much wider range of sounds when he opens his mouth. We have definitely heard him say "ca ca" and "ki ca" every time that one of the iPad programs shows a cat. He is still a long way away from talking or even saying his first word, but for the first time, we are seeing evidence that he is capable of forming language.  This is very exciting for us!  Needless to say, we bought our own iPad, which was promptly dropped on the ground destroying it less than 2 days later.  Luckily, Apple has amazing customer service and gave us a new one with no questions asked!

Will's favorite place to climb
Will's neurologist estimates that the Depakote trial will take approximately two months, although we have learned that nothing in the course of his treatment moves at the anticipated pace.  This is the last medication that we are planning to try before resorting to brain surgery, which means that we could be proceeding to surgery as early as the end of this year or the beginning of next year.

We have decided make the trek to Baltimore to Johns Hopkins for yet another opinion prior to surgery.  Johns Hopkins is not only one of the top pediatric neurological centers in the world, but Johns Hopkins also pioneered the Ketogenic Diet.  They had a cancellation and we were able to get Will in for a consultation in early October...just in time for our 10th wedding anniversary. We are seriously considering sending a memo to the next neurological conference asking the best of them to please start practicing in the Caribbean or maybe even Hawaii.  Johns Hopkins is lining up appointments for Will for another EEG, a meeting with one of their top epileptologists and the head of their ketogenic diet center, as well as a meeting with the head of their neurosurgery department.  As you can imagine, Will has a pretty thick medical file at this point, so just coordinating getting all of his records sent out has been rather time consuming, but we are hoping this will give us peace of mind to know we are doing what is best for our little Buster Beans.

Sunday, July 31, 2011

Keeping our Fingers Crossed!

As of about 4 AM Sunday morning, Will has officially gone SEVEN DAYS seizure free!!!!

We continue to see him move in a positive direction.  He is very happy, interactive, and energetic.  He is exploring and studying things like we haven't seen before, and advancing with very subtle little things that only those who have been watching him through a microscope would notice.  We still don't know if these things are the result of only the Ketogenic diet or a combination of the diet and him not being on mind-altering drugs, but at this point we think that there is no denying that the diet is stopping the seizures.  We'd love to know what an EEG would look like now. Is it clean, or is it still scrambled but we aren't seeing seizures?

Of all treatments to have success with (and we would have taken any of them), we are so thrilled that the one that worked is one that has very little risk to him, and does not have any nasty side effects.  We are trying not to look too far ahead, and just be happy that the seizures seem to be under control.  We still have the worry that there could be an underlying issue with his brain, however having seizure control is a huge accomplishment.  For right now, we are going to be happy for our little Buster!

Sunday, July 24, 2011

On the Bubble

We have great news to report!  We are now 5 weeks into the Ketogenic Diet, and we have seen Will's seizures slowly become less and less frequent.  Right now he is averaging one seizure every two to three days.  At first, he was having them every day, then every other day, then every 3rd day...  most recently he went about 6 hours short of going a full FIVE DAYS without a seizure!  Also, we have completed weaning the Vigabatrin, which is a great thing.  The side effects on his personality as well as the risk of vision loss from Vigabatrin were very tough.  Will has also completed the full course of the Amoxicillin as treatment for Lyme Disease, so now the only drug we are giving him is Keppra twice per day, which hasn't ever really done anything for Will...nothing good, but nothing bad either.

We have seen great changes in Will over the past week.  His alertness, energy, mood, and focus have all improved.  We don't know if this is the result of the drug weans, the implementation of the diet, the Lyme disease treatment ending, or any combination of these.  For the moment we are excited and optimistic that the diet is improving Will's condition, and we pray for complete seizure freedom someday.  It would truly be a miracle if we are able to treat him through diet!  For the first time in months, we feel like we actually have HOPE.

Will has become much more verbal and babbles more than we have ever heard him do before.  He is still not saying words - mostly just "Da, Da, Da" and "Ga, Ga, Ga", but he's working on it more than he had since the seizures started in January.  Even more, he is learning to wave, which is a huge deal!  He waved to Dan's sister and her family the other day as they were in their car ready to leave our house, and also waved for Marie (our babysitter) on Wednesday.  His wave is very labored, he holds his hand out and moves his fingers one at a time but it is very obvious what he is trying to do.  We keep working on it.
Will enjoying the sprinkler in the backyard

We had an appointment at the Clinic earlier this week, and the main reason for this appointment was to discuss Will's progress on the Ketogenic diet, and whether or not we were ready to proceed to surgery.  A few days prior to his appointment, we hadn't seen much improvement yet and Will had a bad seizure so we were feeling ready to schedule his surgery.  Then, as if on cue, Will began to show improvement so we knew that we needed to give the diet more time.  Surgery is definitely still a possibility, however his neurologist was encouraged by the success that he is having on the diet.  We all agreed that the diet is showing huge progress and we have the luxury of trying this for a while longer before needing to make a decision on surgery...a decision we have dreaded.  Will's neurologist described 3 types of surgical candidates - those where the need for brain surgery is obvious, those where brain surgery is not an option for one reason or another, and finally, those where brain surgery is an option but there are no guarantees that it will work.  Will's case falls into the third category, but right now he is, in the words of his neurologist, "on the bubble" as to whether surgery is the right thing to do.  Anything is possible at this point.

We know that there is still an underlying issue in his brain, however if he is able to work through it while not suffering from seizures, that would be incredible.  There have been some cases where the Ketogenic diet actually heals the brain abnormalities that cause infantile spasms.  Of course, if Ketogenic diet doesn't work, we are keeping the surgery option on the table.  Hard work, perseverence, patience, faith, and luck all are key ingredients.  The diet isn't easy, and is extremely time consuming.  We remain optimistic that the diet is showing great signs of improvement, and hopefully it will continue so that we don't have to think about surgery in the future.

We are including some links to some interesting information on the Ketogenic diet, which have also been added under the information tab to the right of the blog.  If you have the time or interest it is worthwhile to take a look.  Who would have known that we would see the most improvement from something having nothing to do with drugs???

Epilepsy's Big Fat Miracle - New York Times

Dateline NBC Special on Ketogenic Diet - Part 1

Dateline NBC Special on Ketogenic Diet - Part 2

For now, we are cautiously optimistic and are happy with what we are seeing in our little Buster - we feel like we are actually starting to have our little boy back!

Saturday, July 2, 2011

Counting Calories

Buster mowing the lawn
The past few weeks have been a bit...overwhelming.  Will's meals take a long time to plan out and prepare, and he eats 4 small meals a day instead of 3, so it seems as though a lot of our time is dedicated to making sure Will is fed and the diet is being administered properly.  We have to check his glucose and blood ketone levels twice daily and we also check his urine several times per day.  All of this keeps us very busy! We have also been very busy trying to get our poor neglected yard into shape again.  It honestly seems hard to find extra time for much of anything lately.

When we first got home from the hospital, things were very rough.  Will was only allowed 900 calories per day, and he was very hungry all of the time.  He often just stood in front of his high chair crying, which absolutely broke our hearts.  We took to eating our meals in the pantry with the door shut because it was just so sad to eat food in front of Will.  Of course, now we have created a monster because after seeing us do that, Jack has started hiding in the pantry and sneaking a LOT of cookies!  After a week, the dietician increased Will's daily calories to 1100 because Will had lost over 3 pounds and gone down a diaper size, and things seem to be much better now.

As our neurologist had predicted, we have been seeing definite improvement in Will since starting the diet but not seizure freedom.  Will has gone from a range of 5 to 10 seizures per day before the diet, to most of the time 1-2 per day, and sometimes they are only occurring every other day.  He has been more aware and interactive with us.  More than anything, he has been very happy, which is absolutely awesome to see.  Although the diet is definitely doing something, we can't conclude it as a success at this point, because in order for his development to move forward we need to completely eliminate the spasms.  So far, that has not yet happened, so we are bracing ourselves for eventual brain surgery but still hoping that things continue to improve.  One other good thing, the blood tests for Will's lactate and pyruvate levels came back completely normal, which means that we can basically rule out mitochondrial disease as a potential cause of his seizures.  This is a huge relief.

Bullseye Rash on Will's Arm
In mid-June we went on a family reunion trip to Pennsylvania right before Will's hospital admission to start the Ketogenic diet.  A great time was had by all, and we spent a lot of time outdoors.  Of course, that also meant we got bit by a lot of bugs while we were there!  We hadn't thought much of it until earlier this week when Will woke up with a rash on his arm that looked just like a bullseye.  Somehow, in addition to everything else, Will ended up with Lyme Disease!  Luckily, we caught it early, so he just needs to take Amoxicillin for 21 days and everything should be fine.  At this point, what is one more drug???  The only problem is that the Amoxicillin (or the Lyme Disease, it is hard to tell) seems to be causing Will to have an increase in seizures again so this will likely delay any decisions we can make about Will's next steps.

Thank you to everyone for following our blog and for all of your continued support.  We hope that everyone enjoys a wonderful 4th of July with their families!

Monday, June 13, 2011

Starting the Ketogenic Diet

Will was admitted to the Clinic earlier today (Monday, 6/13) to start the Ketogenic Diet.  We will likely be here through Thursday.  While we are here, he is also scheduled to have a 3 Tesla MRI and FLAIR study.  A normal MRI is only 1.5 Tesla, which means that this will basically be like an MRI in hi-def.  We are hoping that the 3T MRI will show Will's brain in much greater detail and give the doctors some valuable data.  The FLAIR study was requested by Will's neurosurgeon, as it can sometimes help to identify which areas of the brain need to be removed.

Will's first Ketogenic meal
Will got his first "meal" on the diet tonight.  We were blown away by the small amount of food they gave him!  He basically got a few tiny shot glasses of food, which included straight butter and heavy cream.  When they brought the little portion cups in, we halfway thought they were bringing us Jello Shots, but we were sadly mistaken.  Regardless, when they checked Will's blood levels tonight he was already into "moderate" ketosis, which is a good thing.  We will need to get to "high" levels over the next few days.

We are really praying hard and crossing our fingers that this diet will work, and we hope that everyone reading will do the same.  It would be so amazing if we would be able to treat him without drugs or surgery!

Wednesday, May 25, 2011

No Brainer???

This update has been a difficult one to put together...we have so much information to share, all pieces of a very large puzzle.  At the same time we are trying to keep pushing forward to make sure Will and Jack are getting the day to day care they need, as well as planning and scheduling the next steps in Will's treatment.  

We'll start with the short story - Will has been approved for surgery at the Clinic.  We can do it whenever we want.

Now, for the long story.

Picking up from our last post, Will had an appointment in Detroit on May 6.  The neurologist in Detroit agreed with the Clinic's opinion that there is a subtle malformation in Will’s left temporal lobe that can be seen on the MRI.  This was a relief in a way, because although our doctor at the Clinic is confident that there is something on the MRI, she has mentioned several times to us that her opinion is “controversial” with some of her colleagues at the Clinic.  It was good to hear another one of the few experts on infantile spasms agree with our primary neurologist.  However, the Detroit doctor felt that Will's seizures appear to be too "generalized" (coming from all over the brain) and there was not enough of a "focus" (a localized area where the seizures originate) to justify surgery at this time.  He felt that it is likely Will would need to have surgery in the future, most likely to remove the malformation in the left temporal lobe, and possibly occipital and parietal lobes as well.  He recommended trying the Ketogenic diet (which we were already planning on trying), as well as another medication (Zonegran) if that fails.

So, we left Detroit with mixed emotions.  We were happy that he was as impressed as he was with Will’s development to this point and his abilities.  We were disappointed to hear that he felt surgery was not an option at this point.  We have said this so many times, but right now, we simply don't know what to wish for when it comes to surgery.  If he has brain surgery, he will have a part of his brain removed, and along with that comes some limitations.  If they have to remove the occipital or parietal lobes, they will sever the optic nerve, which means that Will will lose peripheral vision in both eyes.  While he would likely compensate for that because of his young age, he would NEVER be able to obtain a driver’s license.  This is assuming the brain surgery works.  If it doesn’t work, we will have removed a significant portion of Will's brain, and yet not put an end to the onslaught of seizures that plague his development.  Of course, if we don't go the route of brain surgery, then what?  We pray that a medication will work, but if that happens, or if the Ketogenic diet works, there is a strong chance of a relapse.  We have been told that right now he is the best age for surgery to be successful because his brain is still developing, but we want to make sure the choice is right.  Very, very difficult decisions to make.

Two weeks later on May 17, Will's neurologist presented his case to the surgical review board at the Clinic.  This was a team of what we were told was about 40 doctors, nurse practitioners and specialists in a room together, all focusing on Will’s case.  After discussion, Will was approved for surgery at the Clinic!  However, there were definitely some reservations.  It was not an automatic “yes he should have surgery” all around the room, but when it came time to vote, everyone approved it, and nobody said no. We were not allowed to attend, but after the conference we met with our neurologist as well as with the doctor who will perform Will's surgery, if we go that route, to discuss their opinion.

We were very grateful to have the knowledge from the visit in Detroit before this day.  Not knowing whether the Clinic would approve or deny surgery, having the discussions with the doctor from Detroit and digested all the possible scenarios in our own minds before the Clinic’s surgical conference was extremely valuable.

We asked the doctors at the Clinic about the lack of focus of Will's seizures on the EEGs, which was the reason why the Detroit doctor said to wait on surgery.  Will's neurologist at the Clinic said that they discussed this, and in their opinion, the cause of the seizures is the left temporal malformation.  She felt that because of his age, it is likely that his seizures won't show a strong focus, and the irregular brain activity from the seizures is giving rise to more abnormal activity which is originating from other areas of the brain.  They feel that if we wait for a more clear focus to appear, we might be waiting for a long time, and because of his age and the risks of brain damage if the spasms continue, we can't wait that long.

No one can know how Will's development will progress once the surgery is complete, IF we choose to do surgery.  We have never read about a child who took steps backwards in development after surgery, and there are a lot of parents we read about whose children who had surgery.  Most marvel at the steps forward in development after surgery.  The doctors at the Clinic feel that he will do well after surgery.  The Detroit doctor felt that surgery would be in Will’s future as well, although he recommended waiting until the exact location becomes more clear.

The path that was suggested by the Cleveland and Detroit doctors alike is that we will increase Vigabatrin (Will’s current med) one more time, although it is not working.  Once that fails, we will try the Ketogenic Diet, which can be described as an extreme version of the Adkins diet, with exact portions each day and no cheating.  Essentially, the Ketogenic diet causes the body to go into ketosis, burning fats instead of carbohydrates.  It has very promising results, showing improvement in seizures in about 30% of patients, and complete seizure freedom in about 10%. It was originally developed in the 1920’s when doctors realized that seizure patients did better following periods of starvation, but then lost popularity to conventional drugs.  Will is going to be hospitalized for 3 days in mid-June to begin the diet.  If this fails, we will have no choice but to seriously consider surgery.  We expect that we will be face to face with that decision at the end of summer.

The challenge with infantile spasms is that no two cases are the same.  There is no standard procedure for a doctor to follow for each case, which makes treatment more challenging, and requires a lot of effort and constant evaluation and re-evaluation by those providing the treatment.  Also, the condition is so rare that there aren't a lot of people around who are dealing with this as well.  We have found the support groups on the internet to be so valuable, because that is the place where all of the parents can exchange notes.

One more good thing - since Will is off of ACTH we were given the green light (well, more like a yellow light) to take him out in public, as long as we are careful to minimize his exposure to germs.  We also finished weaning him off of Topamax, so we are seeing his personality bounce back even more.  Summer is just around the corner, and we are hoping that for the next few weeks we will be able to just be ourselves and enjoy our family, friends and some nice summer weather.

Thursday, May 12, 2011

Last shot of ACTH!

Last shot of ACTH!
Will got his last shot of ACTH today!  Yippee!  Woohoo!  We are so excited to be able to eliminate at least one drug from his arsenal of medications!  We think our insurance company is pretty happy about this too.

This could not have come soon enough, as last week we noticed that he is beginning to grow peachfuzz on his upper lip.  The thought of sending our 22 month old into early puberty is a bit disturbing, to say the least.  Of course, it all would have been worth it IF it worked!
Will's morning meds

Hallelujah!  Praise God!  We are done with this medication and its awful side effects!

Wednesday, May 11, 2011

Throwing Darts at the Wall

Will having fun in his swing
It looks like we are a bit overdue for a blog update...things have been hectic, as you might expect with 2 kids and now that Spring is here, a lawn that needs mowed, soccer games to attend, fish to be caught at the lake, and a whole bunch of doctors appointments to squeeze in in between.  We have been trying to enjoy life as much as possible despite all that is going on around us, and despite the fact that we cannot take Will out in public.  We have had some really nice spring weather this week, which certainly helps!

Dan and Jack at Jack's first soccer game
We met again with Will's metabolic neurologist at the Clinic to follow up on possible causes of Will's optic nerve damage.  He suggested that, although unlikely, it could be possible Will might have a mitochondrial disease.  The only way to test for this is to surgically remove a 1 cm section of Will's thigh muscle and do a biopsy on the tissue, and even after doing this, the test is inaccurate at best.  However, there are some additional blood tests that can be done first which would indicate whether Will has additional factors that would justify performing the more invasive testing.  For now, we have decided to hold off on the muscle biopsy until we have more data, especially because Will simply doesn't have the typical characteristics of mitochondrial disease, such as low energy levels or lack of muscle tone.  The opthalmologist we see stated that the optic nerve damage could also be caused by a malformation of the brain, which seems like a more plausible theory given Will's history.

At the end of last week, we travelled to Detroit to meet with perhaps the most well known specialist on infantile spasms in the world.  He was very upfront and open, and answered our questions in a very straightforward manner.  It was nice to get a "fresh" opinion.  When he walked into the room, he was amazed at how well Will is doing developmentally.  He said that based on his review of Will's PET scan, he wouldn't have expected him to be able to even hold his head up, as his brain activity is so slowed.  Even though it might be a bit paranoid, we have decided to wait to blog-post his opinion on surgery until after we have the opinion from the Clinic, because we don't want to unwittingly bias their decision since we know of several people at the Clinic who do read this blog.

We are starting to understand that not only does Will have a unique disorder, but Will is also a very unique case.  Of course, with infantile spasms, no two cases are alike anyway.  We have met with countless specialists at the Clinic and now a well known neurologist in Detroit who are all extremely experienced and knowledgeable, and yet we feel as though they are all scratching their heads to figure out how to best help our son.  Our past experience with medical issues have always been simple and clear cut.  You get a headache, you take some Advil.  An ear infection, maybe some antibiotics.  There was always a cure.  Dealing with intractible epilepsy is nothing like that, and we feel like we are just throwing darts at the wall and hoping something sticks.  Not a comforting feeling when your son's entire future is at stake.

Will's surgical conference at the Clinic is scheduled for next week, and after that they will give us their opinion as to whether they feel Will is a candidate for surgery.  We have no idea what they are going to say, and we're not entirely sure what to even be praying for.  Obviously, if surgery will help him, then we want to do whatever is best for little Buster Beans, even if that means removing a portion of his brain.  If surgery is not likely to help him, then we don't want to take such an irreversible step.  The problem, of course, is that you can never truly know if brain surgery will work until after it is done.

Buster waking up from a nap
We are still not seeing any results from the Vigabatrin.  Will is having about 10 episodes per day right now, and each episode lasts between 4 and 8 minutes...which is a lot when you consider that each episode contains 30-50 spasms or seizures.  We are going to begin increasing his dose of Vigabatrin this week, are still winding down with the ACTH, and soon we will get to wean Will off Topamax.

We have been doing a lot of reading about a non-drug treatment option called the Ketogenic diet that has promising success rates in infantile spasms patients for whom traditional drug treatments have failed.  Johns Hopkins is now using the Ketogenic diet as a first line treatment for infantile spasms.  It is basically like an extreme version of the Adkins diet, very high in fats and low in carbohydrates and protein.  All food must be carefully prepared and measured, and Will would actually have to be hospitalized to begin the diet.  Given all the drugs that we have tried and failed, the Ketogenic diet has the best chances of success of any non-surgical option available to Will.  We are planning to discuss this with the Clinic at our appointment next week.  Regardless of the decision on surgery, we think this may be worth pursuing.