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.

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.