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.


  1. I hope you know our thoughts and prayers are with you. You are showing incredible strength. Thank you for keeping us informed.

  2. Praying for you, but heavy-hearted. I was hoping for more agreement between the doctors. It does sound like they all agree on the immediate course of action, though, which helps. We will continue to pray for you. Hope you do start to go places as a family again! :)