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Dravet Syndrome and Lennox-Gastaut Syndrome: Perspectives from the Patient Journey - Episode 16

Approaching Treatment for LGS

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Kelly Knupp, MD, provides insight on optimizing treatment for patients with Lennox-Gastaut Syndrome.

Joseph E. Sullivan, MD: We do have this long list of medications that are approved for LGS and they go back right topiramate, lamotrigine, felbamate, there are 7, I will see if I can list them all, clobazam. Those are all these and then we have the new ones of cannabidiol and fenfluramine. Which one did I miss? I think I got 6 there. Kelly, these patients come to you as a third opinion. They have already been on 3 of the older – these are not old meds I am just saying older, how do you go about approaching with the family the options in terms of new versus old etc.?

Kelly Knupp, MD: It’s challenging because we don’t have as good guidelines of what’s next and there haven’t been comparison studies. Is felbamate better than valproic acid, I have no idea because it has never been compared. My approach is often to say what are the options that you haven’t tried yet, what are the adverse effect profiles of these, do we have data and Dravet syndrome that allows me to rank these and if you were to have a common adverse effects of this, is this a benefit for you or a problem, and adverse effects sometimes truly are a benefit. If we have a child who is overweight being on something like fenfluramine or felbamate that may decrease appetite may be to their benefit whereas a medicine that may increase appetite such as lamotrigine or oxcarbazepine or valproic acid for another patient maybe a benefit if they are a poor eater. A lot of it is trying to match up adverse effect profiles with the options that haven’t been tried yet. Also exploring things like ketogenic diet just as something that is realistic for a particular family, has a surgical evaluation then done. We can start a treatment while we are doing a surgical evaluation and then also reassessing has anybody looked at etiology. Do we have an underlying etiology and does that guide as in anyway and choosing a medication from one of these medications? I also emphasize to families that this maybe the choice of medication that we are using now but all these things that we are discussing maybe something that you need to think about at some point in time in your child’s life. This is choosing what the appropriate next medication maybe now and not taking any of these options off the table because at some point we may need to circle back to these. And you may decide to take a break, you may decide enough changes for a while. We are going to sit tight. We have found a balance of quality of life and seizures that is manageable for us, and we want to just be able to have a summer without unexpected responses to medication changes so that we can do some family things and come back and revisit this in 6 months knowing that that’s an OK option as well. Most often we end up choosing broad spectrum medications unless we have a child with Lennox Gastaut who is having a lot of focal seizures and then we may look at one of her sodium channel blockers. But we have a whole host of options to choose from and not a lot of guideline about which one might be better than the other, it really is looking at a lot of these factors and particularly looking at quality of life as we are choosing those medications.

Transcript Edited for Clarity