Dravet syndrome is a genetic disorder characterized by severe seizures that begin in infancy. Knowing how to respond to the seizures is essential in managing the disease.
Many types of seizure medications exist, with some taken daily as a preventative measure and others only as needed in cases of emergency. Typically, these two types are taken alongside each other, as rescue medicines are not intended to replace regular, prescribed Dravet treatment.
What are rescue medicines?
Rescue medicines are fast-acting anti-epileptic drugs that help to stop a seizure quickly before it progresses to a medical emergency, such as status epilepticus. Life-threatening, status epilepticus is defined as a seizure lasting more than five minutes, cases where a second seizure occurs without the patient regaining consciousness, or repeated seizures occurring for 30 minutes or more.
Rescue medicines are commonly a type of benzodiazepine. They act quickly as a sedative, reducing brain activity (overactive nerve cells) in the patient with the aim of ending the seizure. They can be vital in preventing the patient from sustaining further damage, and are considered a first-line treatment of status epilepticus.
If a patient is prescribed a rescue medicine, he or she should ensure that the medication is kept on or close at hand at all times. Information on what emergency medication is recommended for the patient, and when and how it should be used, should be included in an emergency seizure management plan approved by the patient’s doctor.
When are rescue medicines used?
Rescue medicines are only given as needed in a seizure emergency. This may vary depending on the patient and the situation. For example, they may be recommended if the patient experiences unusually prolonged or repeated seizures, a seizure that is outside of a normal pattern, or if the seizure occurs at a high-risk time, such as when the patient is sick.
Most seizures end by themselves without needing treatment. However, those that last for more than five minutes can become more difficult to stop and stand a chance of progressing to status epilepticus. Rescue medicines are likely to be recommended in seizures known to be atypical for a given patient.
Generalized tonic-clonic seizures are the most likely to progress to status epilepticus, and can be fatal. Emergency rescue medicines are almost always recommended for tonic-clonic seizures lasting longer than five minutes.
Types of rescue medicines
It can be a challenge to safely give a rescue medicine to a person during a seizure. Various formulations are available that offer different routes of administration, depending on the patient, the type of seizure, and who is available to give the medication.
Oral pills will clearly not help people unable to swallow. So formulations allowing medication to be placed under the tongue (sublingual) or between the cheek and gum (buccal) exist. If it is difficult to place anything in the mouth of a person having a seizure, a rectally administered diazepam gel, called Diastat, can be used.
Clinical trials are now assessing the safety and efficacy of rescue medicines delivered as a nasal spray, such as the midazolam intranasal spray, USL261.
In a hospital, rescue medicines may be administered as an injection. But in general, this type of delivery of a seizure treatment is not advised for those who are not medical professionals.
Family members and caregivers of Dravet patients are strongly recommended to receive training, from a healthcare professional, in when to administer and how to safely administer emergency rescue medicines.
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