New ICD-10 codes are now in effect for Dravet syndrome. Previously, a patient diagnosed with Dravet syndrome would be entered into the medical coding system under a non-specific epilepsy code that did not fully represent the spectrum of medical needs for an individual with Dravet syndrome. The Dravet Syndrome Foundation worked with our Medical Advisory Board to obtain new specific ICD codes that were approved in 2020.  These three new codes (G40.83, G40.833, and G40.834) now accurately represent the diagnosis of Dravet syndrome.

What are ICD-10 codes? ICD stands for International Classification of Disease, and the “10” represents that ICD-10 is the 10th revision to the ICD codes. The ICD was created in 1893 to begin tracking mortality data in a broad and consistent manner1. The World Health Organization (WHO) took over management of ICD in 1948 during publication of ICD-6, and still oversees the ICD today1. The ICD has evolved over time to include more specificity in coding, broader applications across the world, and updated diagnostic codes1. The eleventh revision, ICD-11, will take effect in 2022 with updates to match the current technology used in medical records1. Several countries, including the US, have modifications of the ICD code to enhance the specificity of diagnostic coding and to improve morbidity reporting1.

Why did Dravet syndrome need unique ICD-10 codes? Dravet syndrome could previously have been coded as “G40.8 Other epilepsy and recurrent seizures.” This diagnostic code is quite vague and could represent a broad population of patients with varying severity of disease and presentation of comorbidities. Having a unique diagnostic code (G40.83 Dravet syndrome) that is further broken down into subcategories (G40.833 intractable, with status epilepticus and G40.834 intractable, without status epilepticus) now provides a distinction that a patient is diagnosed specifically with Dravet syndrome. Dravet syndrome is a unique disorder that has a characteristic presentation of symptoms requiring medical interventions, and Dravet syndrome also has important medical contraindications that would not be attributed to a nonspecific diagnostic code like “other epilepsy.”

Why are ICD-10 codes important? Because ICD codes are used consistently across health care systems, it allows for wide tracking of specific disease-related morbidity and mortality2. Using consistent and succinct medical codes facilitates easier access to retrieval of health information for a particular disease that can assist in informing current treatment approaches1. Public health officials at the local, state, and federal-levels all utilize information from ICD-10 tracking to inform policies and guide implementation of activities2. Public health research can use ICD-10 codes from medical records to track services, treatments, and complications for a specific ICD diagnosis code. This information can help to inform research into the severity of a particular diagnosis and the occurrence of comorbidities2. This information may also be useful in determining the outcomes of a diagnosis in the context of the interventions and treatment approaches. Ultimately these types of studies can inform future determinations of the best course of treatment for an individual patient2. The broad usage of ICD-10 codes allows researchers and policy makers to share and track health data not only across geographical locations but also across time1. Having a unique code for a rare disease like Dravet syndrome is particularly important for increasing understanding of the medical course and best treatment approaches for patients.

In addition to advancing research and increasing knowledge, having a unique ICD-10 diagnosis code may help with medical access to treatments. ICD-10 codes are used in medical billing to determine if interventions are appropriate for a diagnosis and to ensure proper reimbursement2. As new therapeutics continue to be approved specifically for Dravet syndrome, an ICD-10 code that more accurately and immediately represents the appropriate diagnosis may help to expedite access to treatment.

Help promote the accurate use of the new ICD-10 codes! In the coming weeks, those in DSF’s Family Network should be receiving a mailer about the new ICD-10 codes for Dravet syndrome that includes some business cards with the ICD-10 codes printed on the back. Please use these cards when you see your primary care doctors, specialists, and therapists to inform them of the new diagnostic codes for Dravet syndrome. If you need additional materials to share the codes, you can request additional business cards or download a flyer with the ICD-10 information.