Parents of a child or adult with Dravet syndrome are acutely aware of the need for rescue medications due to the frequency of prolonged seizures and/or seizure clusters that are experienced by the patient community. Rescue medications work quickly to stop atypical seizures and help to avoid emergency situations. They are prescribed with the intent to be used as needed, rather than on a daily basis as the other anti-epileptic drugs the patient takes.
When a patient is prescribed a rescue medication, they should also receive an emergency protocol from the prescribing neurologist detailing what to do in the case of a prolonged seizure or a cluster of seizures. This protocol will include medication dosing, when to seek additional treatment, patient information, and contact information for the parent and physician. As rescue medications are typically central nervous system suppressants, it is important to remember that the use of a rescue medication doesn’t always prevent a trip to the ER and it is important to seek emergency treatment as needed. DSF offers an example of an emergency protocol. A copy of the emergency protocol should be kept with the patient, along with the rescue medication, at all times.
Nicole Villas, DSF Board President and Scientific Director, has put together the summary below on current rescue medications used in the United States for Dravet syndrome, in response to questions from the community about what is available. This way they can discuss with their child’s neurologist what might be the best option for their loved one.
What rescue meds do doctors prescribe for status?
There are two main at-home rescue medications doctors typically prescribe for prolonged seizures. Both medications, diazepam and midazolam, are members of the chemical family called benzodiazepines, and are central nervous system suppressants. Diazepam is most often prescribed as a rectal gel, either under the brand name Diastat or generic diazepam. There are other forms of diazepam, including oral tablets (sometimes referred to as Valium) and IV injectable solutions, but none are appropriate for at-home use during prolonged seizures. Valtoco, a nasal diazepam spray manufactured by Neurelis, is currently under FDA review in 2019 for treatment of cluster seizures.
Midazolam, the second most commonly prescribed rescue medication, is sometimes referred to by the brand name Versed. Midazolam is often used in IV format in hospital for stopping prolonged seizures, and that same IV solution has been used at home by members of our community, either administered nasally with an atomizer (a sponge-like attachment to the syringe that separates the liquid into small droplets for faster absorption) or deposited inside the patient’s cheek, but was not FDA approved for at-home use until 2018. Two brands of midazolam are currently available by prescription for prolonged seizures: Seizalam is a solution approved as an intramuscular injection for stopping status seizures approved in 2018, and Nayzilam is an intranasal spray approved in 2019 for treatment of cluster seizures.
A third member of the benzodiazepine family, lorazepam (brand name Ativan), is sometimes used via IV in the hospital for status seizures. However, because it takes longer to act on seizures and the at-home formulation is a tablet, it is not often used for prolonged convulsive seizures in the home. Some families use lorazepam for clusters of seizures or other seizure types during which the patient is more aware and can swallow a tablet.
Finally, clonazepam (Klonopin), another benzodiazepine that takes a bit longer to act on seizures, has been available in the past in thin wafers that disintegrate when placed in the patient’s mouth as well as tablet form, but the wafers have been discontinued, not due to safety or efficacy.
Diazepam and Midazolam, the first two mentioned, remain most physicians’ and patients’ preferred rescue medications for prolonged convulsive seizures due to their quick mechanism of action, wide availability, ease of administration, and efficacy.
What other options are out there?
Although benzodiazepines are the most studied and effective at-home treatments for prolonged convulsive seizures, sometimes patients with Dravet syndrome experience other less life-threatening seizure types that may respond to other treatments. Benzodiazepines are addictive, so frequent use may reduce their effectiveness or induce withdrawal symptoms when they are discontinued. As such, our community often looks to other types of medications for clusters of seizures or periods of increased seizure activity. Some patients respond well to larger doses of their current or former daily medications including sodium valproate (Depakote), levetiracetam (Keppra), or topiramate (Topamax). However, this type of treatment for cluster seizures has not been well studied and any attempt to treat at home should be discussed in advance with and ordered by the patient’s neurologist.
Some patients with Dravet syndrome have had a Vagal Nerve Stimulator (VNS) device implanted. This device sends frequent electrical charges to the vagus nerve in the neck to prevent seizures and can be activated by a magnet to send stronger signals with longer duration in an attempt to end a longer seizure. Caregivers of patients with Dravet syndrome report varied responses to treatment, and its efficacy in stopping status epilepticus in Dravet syndrome has not been well characterized in medical literature.