I told everyone that we were seeing Dr. Shields today (Tuesday) when in actuality, our appointment was for the "7th" which was yesterday. I knew it was the 7th, I just thought the 7th was a Tuesday! LOL Don't worry, we didn't miss the appointment!
Brett and I took Jenelle to UCLA yesterday where we met with Dr. Shields and his resident Dr. Shariti to discuss the recent test results. There is not much new to report since we left UCLA in April - Jenelle is still not a surgical candidate. Her EEG was abnormal (obviously) with no focal point to her seizures, the MRI was "normal" and there were no changes from her MRI in July 2003 (which is really good news), and her PET scan found an abnormality regarding her glucose metabolism in the right frontal lobe, but the results were "too inconclusive" to warrant surgery. Basically, our little girl is one huge mystery! I'll discuss the PET results in detail a little later...
Dr. Shields feels that Jenelle is "transitioning" into Lennox Gastaut Syndrome (LGS), but as he says, "the writing is on the wall, but its not carved in stone yet!" When a child has Infantile Spasms that are symptomatic like Jenelle (i.e. the patient has an "underlying" neurological condition, and in Jenelle's case, her "underlying" condition is undiagnosed) they have a greater possibility of transitioning into LGS, which means their seizures will change their presentation. This is something we have seen in Jenelle. Basically, her seizures have changed from "spasm" like seizures to "myoclonic and tonic/clonic" seizures. This could be the LGS, or simply that her brain has grown, and thus her seizures have changed on us. For now, he is calling it LGS; her ultimate "prognosis" with that is yet to be seen and/or determined. Basically, we were told to expect to deal with her seizures for the rest of her life - and honestly, this wasn't a huge surprise.
Dr. Shields feels we have a "few" more drugs to try before attempting the Ketogenic Diet or a VNS implant. He feels that the Vigabatrin has helped control her Infantile Spasms, and wants to continue with this drug a little longer. He also wants to increase her Topomax a third time to the max dosage to give it a full trial before moving onto another drug called Lamictal. We are slowly increasing Topomax now and will give it another month on the max dose. The Klonopin will stay the same as we don't want to make too many changes at once. He was honest with us and said he felt the other drugs we have yet to try may only have a 10% to 15% chance of successfully stopping her seizures. We asked for more details about the LGS prognosis since we've seen such varying information, and he admitted it was quite grim in that most LGS patients are severely mentally retarded and usually wheelchair bound, but in his experience, very few die. In fact, in his 20+ years of experience, he said he has had 2,000 patients with LGS, and only 2 have died. That is a much better statistic than those we've previously seen. A "shorter" life is still a possibility, and we accept that possibility.
He was very concerned with Jenelle's therapy, and felt our main goal at this time was to provide as much therapy and stimulation as possible so that we can help Jenelle reach her full developmental potential. He felt strongly that in a year or two as her brain grows, we may determine a focal point in the brain to her seizures that can be corrected surgically. It is very important that she progress developmentally prior to any surgery as there is always a risk of losing skills after surgery. The more development, the better the outlook after surgery.
About the PET scan results. We had received a copy of Jenelle's medical records prior to our visit yesterday, so we already knew about the focal point in the Right Frontal Lobe. This was concerning because Jenelle consistently favors and uses her left side of her body better than her right side. We assumed the problem was in the left side of her brain. Usually with a problem in the right side of her brain, it would be more likely that she would have problems using the left side of her body - not that it would be her "best" side. I asked Dr. Shields about this result, and he agreed it was very strange. Because the PET scan results and her physical presentation are confusing and/or conflicting, this is a reason not to support surgery at this time. It is quite possible that if the brain damage is to her right frontal lobe, it happened immediately during her development, and her brain has since taught itself to compensate and make different connections in order for her to use her left side so well. Frankly, they cannot explain the results, but it is something we can watch in the coming years as her brain grows. We are scheduled to see Dr. Shields again in August.
The goal at this point is to help Jenelle progress in her development as much as possible. We realize that seizures are just going to have to be a part of our lives - as much as we may hate them. We hope to find a medication that will provide some amount of seizure control that will help provide Jenelle the most development at this time. I'll continue to update when I have something new - thank you all for your continued prayers and support!