April 30, 2018
Ms. Donna Pickett
Co-Chair, ICD-10-CM Coordination and Maintenance Committee
National Center for Health Statistics
ICD-10-CM Coordination and Maintenance Committee
3311 Toledo Road
Hyattsville, Maryland 20782
RE: ICD-10 Code for Dravet Syndrome
Dear Ms. Pickett:
On behalf of the American Epilepsy Society (AES) I am writing to
support the Dravet Syndrome Foundation proposal to create a new
code for Dravet Syndrome (DS). Founded in 1946, the American
Epilepsy Society (AES) is a medical and scientific society whose
members are dedicated to advancing research and education for
preventing, treating and curing epilepsy.
Dravet Syndrome is a genetic encephalopathy that presents in the
first year of life. It is an uncommon disorder that represents about 7%
of all sever epilepsies starting before the age of 3 years. Currently,
there is no unique ICD code to identify patients with DS. In ICD-10-
CM, Dravet syndrome is currently being reported inconsistently.
Dravet Syndrome has a unique and distinctive phenotypic spectrum
and AES shares the view that it is important to differentiate properly
identify and code DS. While SCN1A mutations are often seen in
children with DS, not all children with SCN1A mutations have an
encephalopathy /DS, and not all children with the Dravet phenotype
harbor a SCN1A mutation.
Specific ICD codes for DS are important for ensuring appropriate and
safe treatment as DS has specific pharmacological sensitivities and prognosis. Effective ICD
coding also contributes to effective clinical research. Specific ICD codes are essential to be
able to better identify patients with DS, measure outcomes, and understand the
epidemiology of the disease, and to help direct treatment.
For these reasons, AES supports establishment of the proposed new code to identify this
condition for research and reporting, and also supports the broader need to align ICD10
codes with other epileptic encephalopathies and genetic disorders from a neurologic and
epilepsy perspective to keep pace with continuing advances in gene discovery and
modifications in clinical phenotyping of the pediatric epilepsies.
Thank you for the opportunity to comment on the proposed ICD-10-CM codes for Dravet
Syndrome and epileptic encephalopathies. Please feel free to contact me with questions.
Shlomo Shinnar, MD, PhD
American Epilepsy Society
Tim Welty, M.A., Pharm.D, FCCP
Council on Clinical Activities
American Epilepsy Society
G. U. Martz, MD; Chair, Practice Management Committee
J. M. Parent, MD
E. Murray, AES Executive Director
M. Meskis, Dravet Foundation Executive Director
N. Villas, Dravet Foundation