We gained a lot of information from Jenelle’s latest EEG; mainly that Jenelle is having a lot of seizures. In fact, not just one seizure type, but three different types of seizures. Basically we confirmed her seizures are getting worse, and while we still have options, those options are growing slim.
Lennox Gastaut Syndrome (LGS) is characterized when the patient has multiple different types of uncontrolled seizures. For Jenelle, her LGS presents with mainly tonic and absence seizures. A known fact with LGS is as the patient reaches their teens, the seizures get very, very difficult to control. While Jenelle’s seizures seem to be getting worse, it may simply be the nature of her epilepsy condition.
Here is what we learned this week:
1. The seizure where her eyebrows twitch ( the “Groucho Marx” seizures) are not complex partial seizures like we thought, but actually “atypical absence” seizures. No matter what we name it, it is still a seizure. This seizure responds well to the Aspire Vagus Nerve Stimulator (VNS) and is not a real emergency for Jenelle unless it goes over 15 minutes.
Last Thursday, before our admission, Jenelle has a 10 minute atypical absence seizure. We used Diastat at 8 minutes, and called 911 for a ride to CHOC Emergency. Her VNS magnet did not stop the seizure, and it was the first time in over a year we had to use Diastat. While we could have waited 15 minutes, we didn't as it had been a while since we were unable to stop a seizure. The ER reported that Jenelle was hypoglycemic, but our neurologist said that probably had little to do in bringing on her seizure and more likely due to her having not eaten. So, blood sugar counts are not really something we will need to monitor.
2. Jenelle is still having “Tonic” seizures (the new one) but they are shorter due to the VNS self activating with the increase in heart rate.
3. Jenelle is now having “epileptic spasms”, which look like she is doing a tummy crunch, then her arms stretch out and she drops her head. Same as the seizures above, this is not an emergency unless it goes longer than 15 minutes. This seizure is similar to the Infantile Spasms, which was Jenelle's diagnosis as a baby. These seizures come in clusters. The good news is that the brain wave activity with “epileptic spams” is not as dangerous or as damaging as Infantile Spasms (i.e. no hypsarrythmia). From our EEG data, Jenelle tends to have clusters of this type of seizure between 4am and 6am. We had no idea because obviously, we are all asleep when this happens.
What we are doing:
1. Our Doctor adjusted the settings on Jenelle's VNS to go off even more frequently and at a higher voltage. This seems to help, but it also means that when we swipe the magnet to activate the VNS on our own during a seizure, we may not get a seizure to stop as the magnet will not activate if the VNS is in a cycle. And of course, because the automatic cycles are more frequent, the magnet may not always work. It seems the higher setting is helping, but we really won't know for a few more weeks.
2. We are decreasing Felbamate. Jenelle's latest labs showed that she was on too high a dosage, so we are back to 900 mg, three times a day.
3. We are increasing Ethosuxamide as it seems the atypical absence seizures returned when we lowered the dosage a month ago.
4. We’ve added a new drug (but not new to Jenelle) called Banzel. Jenelle was on this drug a few years ago under the supervision of Dr. Shields. We stopped it because of insomnia, but felt we need to give it another try to see if it can work in conjunction with Felbamate. She will be on the theraputic dose in four weeks, so we will know more at our next visit.
The doctor wants to see her again at the end of the month, and is going to watch her closely. The EEG confirmed that there is a huge difference in stopping the seizures when using the VNS magnet, so that is definitely helping.
Bottom line, Jenelle’s seizures are getting worse, and we are trying to control them. If Banzel doesn't work with the new cocktail, our next drug to try is Fycompa. Dr. Tran has only used that drug twice, but had very bad behavior side effects with her patients. Because Jenelle is already on a high dose of Felbamate (and Felbamate is also know for bad behavior side effects) we thought Banzel was the better choice for now. After that, unless something new is discovered, we are really out of drug options.
Our next choice is medical cannabis, or brain surgery (corpus callosotomy). If I had a nickel for every time someone asks if we've tried marijuana; I'd be a billionaire! :) We are not against it in any way, but it still is not FDA approved, and it can be very, very expensive. When it comes down to the choice between medical marijuana or surgery, we'll try the medical marijuana. With our luck, when we reach that point there may be studies and/or government approval to help us out. The Laws are always changing! It is something to think about down the road, and we realize that we are getting close to making it a reality.
So, overall - it’s not really news we were hoping to hear. But we've always known that Jenelle is very complex and this is just the nature of her epilepsy. We realize Jenelle will never be seizure free, but we are committed to doing everything possible to make her quality of life the best it can be.
Thank you again for all the prayers. As always, I'll keep you posted.