Poor little Jenelle is a turnip - As in you can't draw blood from a turnip!
Dr. Shields predicted that Jenelle would let us know if weaning her from Vigabatrin was a good thing. Last Thursday was Jenelle's last day on Vigabatrin, and the first time we ever used her Diastat (emergency rectal valium to stop out of control seizures.) As we were getting ready for daycare that morning, I knew she was having many seizures. On the drive to daycare, I realized that whenever Jenelle was quiet, she was seizing. Then I also realized that she had had 5 seizures lasting over 5 minutes in one hour. As I was explaining everything to her daycare provider, we realized that during our 5 minute conversation, she had also been seizing the whole time (and hadn't stopped), so I administered her Diastat and called UCLA. The Diastat did its job and kept us from going to the emergency as it gave Jenelle some much needed seizure relief. It didn't stop her seizures, but they were lasting mere seconds as opposed to minutes.
Dr. Wu was the Neurologist that returned my call as was the same doctor that treated Jenelle in the hospital last April, and had written the report on her last EEG. Dr. Shields had already left for a huge Epilepsy conference that apparently "everyone" was going to attend! Dr. Wu pulled Jenelle's records and had Dr. Shields notes in front of her as well as the last EEG. I explained that Jenelle was having many 5 minute "myoclonic" seizures. Dr. Wu asked me to describe what Jenelle was doing during the seizure, and I explained what I was seeing. Dr. Wu then explained that what I was describing did not sound like a "myoclonic", but rather like an "atonic" seizure. OK - Here is your "crash course" in seizures types: "Myoclonic" seizures are "jerk" type seizures (literally when holding Jenelle, you'll feel her body jerk when having a myoclonic), "Atonic" seizures are considered "drop" seizures and usually the body "drops" like with a head drop, and finally, "Absence" are what used to be called Petite Mal seizures, and it is where the person becomes limp and unresponsive. Apparently, I've been mis-categorizing Jenelle's seizures when talking to Dr. Shields. After the clarification, I began to realize that we really haven't seen the myoclonic jerks since Jenelle reached the max dose of Topamax, and that mostly her seizures now are Atonic. Dr. Wu said that Dr. Shields had written down a drug he wanted to start, but that with our clarification, she felt the drug Dr. Shields wanted to use was not appropriate. She then recommended we start a drug called Felbatol.
Felbatol is what they call a "black box" drug, and was once removed from pharmacy shelves because there were some side effects reported, including death. The major side effect from Felbatol is bone marrow suppression or more commonly know as Aplastic Anemia (where the body stops producing blood.) Before we could start Jenelle on this drug, we had to get a baseline CBC count and liver panel. She will need blood tests every two weeks while on this drug until they determine that she is tolerating it. From emails to other families with LGS, I'm hearing very good things about Felbatol, and it seems to be one of the only drugs that works best with patients like Jenelle, will little cognitive side effects.
First thing first, we had to get a blood test before starting this new drug! This was not as easy as it sounds! Amazingly, Jenelle didn't make a peep during the many attempts to draw blood - which also could have been the Diastat/Valium taking effect. Once all was done, Jenelle was more stable and I went back to the office. I was expecting a horrible night of uncontrolled seizures last Thursday, but to our surprise, Jenelle fell asleep early and slept through the night. Friday morning, Jenelle was much more stable, so I took her to daycare and went to work. Just as I was settling into my day, the doctor's office called and the blood taken on Thursday had clotted and was un-usable. I was going to have to return to get Jenelle for another blood test! To make a long story short - 4 1/2 hours, 4 Phlebotomists and 2 lab locations later and they finally had enough blood to run the test. Poor Jenelle was in more pain this time as I noticed was stamping her foot with our last attempt as three of us held her down to get the blood. Poor thing is a turnip - no doubt about it! Unfortunately, Jenelle's veins are stretchy from her seizure meds thus making her blood draws difficult, to say the least.
Two hours after getting blood, the doctor called with the "all clear" to start the Felbatol. Jenelle has been taking it since Friday night and we will be weaning her up to the max dose over the next 4 weeks. When she is finally at the max dose, assuming there are no side effects, Jenelle will only be on two medications: Topamax and Felbatol. She did well this weekend but still had some seizures. Fortunately they are no where near the length they were on Thursday.
Like Dr. Shields predicted - Jenelle let us know that things weren't right. I'm grateful Dr. Wu was available and took her time to understand the situation to help us choose the most appropriate next step. Of course, I feel horrible about mis-categorizing her seizure type all this time, but I also realize we are still learning, and this is all apart of the learning curve of Epilepsy. We are still waiting on insurance to hear about the Keto Diet and about the doctor in San Diego - in fact, I think it is my turn to give them a call! I forgot to mention in my last update about the comment the insurance girl made to me on the phone (Brett really wanted me to include this so you all would now exactly what we are up against in dealing with our HMO.) When I first called in the appeal for Jenelle to start the Ketogenic Diet, the young girl from the insurance company who was taking down my information interrupted me and said, "And why does your two year old daughter need to lose weight?" ... get it? Ketogenic Diet = Lose weight? Instead of laughing and crying at this question, I simply explained that the diet was to help stop her seizures, and not for weight loss. I amaze myself sometimes - See what we are up against? ;)
Thanks for the continued prayers. Please pray that Jenelle tolerates this new medication without side effects and that it is the needle in the haystack we've been looking for! I'll keep you posted!