ScientiaCME Best practices and opportunities for improvement in the medical management of Dravet syndrome: damn it, Dravet – what to do

Details

In this online, self-learning activity:

Dravet syndrome (DS) is a rare form of early-onset epilepsy syndrome affecting between 1:16,000 and 1:46,000 and is associated with pleomorphic seizure activity, cognitive decline, motor, and behavior abnormalities. Sudden unexpected death in epilepsy is the cause of death in nearly half of the deaths in patients with DS, with the mean age of death 8.7 years old and 73% of deaths occurring before age 10. Seizures typically begin in the first year of age, with most occurring between months 5 and 8, and it is usually a prolonged, tonic-clonic (accounting for 52% of first seizures) or hemiclonic (35%) seizure. Environmental triggers or events, such as fever, acute stress, and physical exercise may precipitate seizures, and at least 85% of those who are clinically diagnosed with DS have variations in the SCN1A gene. Unfortunately, diagnosis is not uncommonly delayed until a patient 3 years of age or older, prior to which antiseizure medication selection may be suboptimal or ineffective, which may lead to seizure exacerbation, an increased risk of status epilepticus, and worse cognitive outcomes.

Topics Covered

Faculty Introductions, disclosures

Background: review of DS

Epidemiology
Etiology
Clinical presentations
Prognosis
Challenges in diagnosis

Best practices and updates in management

Trigger avoidance
Goals of therapy
The role of pharmacological agents
Valproic acid
Fenfluramine
Pharmaceutical cannabidiol
Topiramate
Stiripentol
Clobazam
First-line, assessment of response, and options for failure of initial therapy
Beyond
Medications to avoid
Investigational agents (e.g., soticlestat)
Nonpharmacologic approaches
Diet (ketogenic)
Role of vagus nerve stimulation
Pediatric vs adult care
Individualizing treatments
Transition of care
· Patient case(s)

Summary, conclusions, and best practice recap

Learning Objectives

By the end of the session the participant will be able to:

• Summarize the presenting signs and symptoms of Dravet syndrome (DS) as well as the health care burden unique to patients with DS over time.
• Recall the present and emerging therapies for DS.
• Formulate a treatment plan for a patient with DS.

Target Audience

HCPs including but not limited to: neurologists, primary care physicians, and pediatric specialists; physician assistants, nurse practitioners, and pharmacists who practice in neurology; and any other clinicians who commonly encounter potential patients with DS.

Additional credit info

ACCME Activity #202948388

ScientiaCME is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.

Credit Designation: ScientiaCME designates this educational activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™ toward the AMA Physician’s Recognition Award. Physicians should only claim credit commensurate with the extent of their participation in the activity.

CME-MOC_badge

ABIM MOC Recognition Statement: Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.0 MOC points in the American Board of Internal Medicine’s (ABIM) Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit.

ABIM MOC Credit Type: Medical Knowledge

ABP MOC Recognition Statement: Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.0 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit participant completion information to ACCME for the purpose of granting ABP MOC credit.

ABP MOC Credit Type: Lifelong Learning and Self-Assessment

Physicians: For maintenance of certification (MOC) points, you must enter your board certification ID # and birth date correctly. It is the learner’s responsibility to provide this information completely and accurately at the completion of the activity. Without providing it, the learner will NOT receive MOC points for this activity. By providing this data, you acknowledge that it will be shared with ACCME and the applicable certifying board. Please note: Not all activities on this site provide MOC points. If this activity does not specify that it provides MOC points in this section, then it does NOT provide MOC points. This activity provides MOC points only for ABIM and ABP.

Pharmacists

ScientiaCME is accredited by the Accreditation Council for Pharmacy Education (ACPE) as a provider of continuing pharmacy education. This activity is approved for 1.0 contact hours of continuing pharmacy education credit. Proof of participation will be posted to your NABP CPE profile within 4 to 6 weeks to participants who have successfully completed the post-test. Participants must participate in the entire presentation and complete the course evaluation to receive continuing pharmacy education credit. ACPE #0574-0000-25-008-H01-P. This is an Application (A)-type activity.

Pharmacists: You must enter your NABP # and birth date correctly so that proof of participation can be posted to your NABP CPE profile. It is the learner’s responsibility to provide this information completely and accurately at the completion of the activity. Without providing it, the learner will NOT receive CPE credit for this activity.

Nurses: The American Nurses Credentialing Center (ANCC) accepts AMA PRA Category 1 Credit(s)™ from organizations accredited by the ACCME. This activity is designated for up to 1.0 AMA PRA Category 1 Credit(s)™.

Physician Assistants: The American Academy of Physician Assistants (AAPA) accepts AMA PRA Category 1 Credit™ assigned by organizations accredited by the ACCME as satisfying Category 1 CME for National Commission on Certification of Physician Assistants (NCCPA) national certification maintenance. This activity is designated for up to 1.0 AMA PRA Category 1 Credit(s)™.

Nurse Practitioners: The American Academy of Nurse Practitioners Certification Board (AANPCB) states that continuing education providers accredited by the ACCME may provide acceptable, accredited Advanced Practice Provider content. This activity is designated for up to 1.0 AMA PRA Category 1 Credit(s)™.