I suck. Seriously, life is moving and I find it hard to post updates here about Jenelle because everything is status quo. So, since my last update in April, 2018, a LOT has changed with Jenelle. The good news is that we have a new hope.
As mentioned, back in April we were hoping to wean Jenelle off some medications that she was taking that would exclude her from a new drug trial at CHOC. I didn't want to give specifics about it in case it kept us out of the trial. We successfully weaned Jenelle off Onfi, Banzel and lowered her Risperdone from 3mg a day to 1 mg three times a day. The Risperdone wean was torture as we had a summer of grumpy, cranky Jenelle. We finally decided to stop the wean and keep it at a lowered dose. Unfortunately, we didn't wean fast enough and we missed the window for that drug trial. At this point, Jenelle was only taking Felbamate and Risperdone for seizures and behavior, and we had increased the VNS settings "Rapid Stim" meaning it will send something to her brain continually. This helped a bit.
Then, on June 25, 2018, everything changed. The FDA approved it's first cannabis drug, a drug called Epidiolex
specifically created for difficult to treat Epilepsy. Brett and I were inundated with calls, text messages, e-mails and such asking us if this was something that could help Jenelle. I had heard about Epidiolex, but had also read that the trials were done on children with Dravet Syndrome, something Jenelle does not have. However, this was good news. And then, I learned that Epediolex was not only approved for Dravet, but also for patients with Lennox Gastaut Syndrome. Finally, some hope!
If you recall back in 2017, Jenelle was regressing so much, and we had exhausted almost all the medications known for Lennox Gastaut Syndrome that our only options remaining was the new drug trial, a drug called Fycompa that had some nasty behavior side effects that we were hesitant to try and possible surgery or medical marijuana. Brett and I have always had marijuana or CBD oil as a last resort because neither of us have ever used marijuana, and we just didn't know what we were getting ourselves into. We were almost out of options, and just waiting for science to catch up! And then... it did!
In late August, I sent an e-mail to Dr. Tran about Epidiolex. She said she was still waiting for the FDA to decide how it was going to be distributed, but that Jenelle was top on her list for the drug. Funny thing, when the FDA first categorized Epidiolex, they put it in the same category as Heroine. Something that would have made it impossible for our doctor to prescribe. Dr. Tran was working with others and the FDA to fix this classification. Once that was fixed, The FDA then had to decide how to dispense it. Was this something we could only get at the doctor's office, or at a specialty pharmacy, or at our local CVS/Target?
Today was the magic day. I got a call from CVS Specialty Pharmacy and we've been approved by insurance. This morning, Dr. Tran e-mailed the titration schedule, and as soon as we have it in hand, we are ready to go! This could not come soon enough. Because we weaned Jenelle off all but one seizure medication (and the VNS) Jenelle's seizures have gotten worse. We debated with Dr. Tran about increasing her Felbamate, but decided to wait it out. In the last month, we've used Diastat three times and I was worried we couldn't wait much longer. Knowing it could arrive any day makes the worry go away some.
So, will this be a miracle drug? Maybe. In my Lennox Gastaut support groups, I've read update from families that were on the Epidiolex trial. They studied this drug for 4 years! Many LGS families have reported amazing results - less seizures, more comprehension by their child, more vocalization as non-verbal children now having actual words in their vocabulary! And some families who didn't see much improvement. For the most part, the results are good, so we are hopeful and will keep our fingers crossed. Any improvement has to be better than where we are now.
Brett asked me the other day if I thought this could really be a big change for Jenelle. Honestly, I'm more scared that it might actually work for Jenelle than I am of it failing her. As always, I'll keep you posted.
In other news, or girl turned sweet 16 in October. This school year has been crazy with big brother Jack applying to colleges and Jenelle a sophomore at the rival high school. We're enjoying life for now and hopeful more in the future. Here are some photos from the summer and Jenelle's birthday. Thank you for the continued thoughts and prayers.
|Grumpy Jenelle on Mother's Day|
|Grumpy Jenelle on 4th of July!|
|Bored Jenelle at JD's scrimmage.|
|First day of School - our new Senior and Sophomore.|
|Grumpy Jenelle getting a kiss from Daddy.|
|Senior Photo day.|
|Happy Jenelle at the Angels Challenger Day Games celebrating her 16th Birthday with her friends!|
|A special play date with her former aide Gerri.|
We are still in the holding pattern for Epidiolex for Mira - fingers crossed we will be able to try it within the next few months. Please post how Jenelle is doing on it!
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