Dravet syndrome is a rare form of severe epilepsy that causes frequent and often prolonged seizures. It typically begins in the first year of life — and is a life altering condition for children with Dravet syndrome and their families.

After years of advocacy efforts led by the Dravet Syndrome Foundation (DSF), during the most recent round of revisions the Centers for Disease Control and Prevention (CDC) and the Centers for Medicare & Medicaid Services (CMS) created new International Classification of Disease codes — known as ICD-10 codes — for Dravet syndrome.

“ICD-10 codes are used in the medical system, both for [health insurance] billing and for epidemiological [disease tracking and control] purposes,” Veronica Hood, PhD, DSF’s scientific director, told Healthline.

“Local experts, state, federal, and even all the way up to the World Health Organization use ICD-10 codes to track the incidence [likelihood] and prevalence [actual occurrence] of different disorders — and that can really help inform policies and implementation of public health outreach efforts,” she said.

The new codes may help families get insurance reimbursement for the latest and most promising Dravet syndrome treatments. They’ll also make it easier for scientists to track and study the condition, which may lead to new treatments in the future.

Read on to learn more about the role that ICD-10 codes play in the medical management and study of Dravet syndrome — and how the new codes may benefit families and scientists.

Multiple countries around the world use ICD-10 codes to document and classify medical diagnoses. ICD-10 represents the 10th major revision of the coding system.

When ICD-10 was first issued, it included no designated code for Dravet syndrome. The condition was instead lumped under the code for other epilepsy and recurrent seizures.

“That code’s pretty nonspecific,” said Hood. “It could be applied to a variety of different epilepsy syndromes.”

Now, thanks to advocacy efforts from DSF and members of its medical advisory board, the following ICD-10 codes have been adopted in the United States:

  • G40.83 Dravet syndrome
  • G40.833 Dravet syndrome, intractable, with status epilepticus
  • G40.834 Dravet syndrome, intractable, without status epilepticus

“I’m really proud of the work that DSF did in order to make this happen,” said Dr. Ian Miller, a pediatric neurologist and former member of DSF’s medical advisory board who played a key role in advocating for the new codes.

“It’s a huge improvement in terms of the steps that have been taken,” he added.

Hood hopes that the new ICD-10 codes will help families get insurance reimbursement for their children’s prescribed treatments.

“Having a code that specifically says ‘Dravet syndrome’ helps insurance companies to understand more about the specific epilepsy that the patient has,” she said.

“That’s particularly important with this rare disease because there are certain medications that are contraindicated that can actually make seizures worse in Dravet syndrome,” she continued.

These medications include a common class of antiseizure drugs known as sodium channel blockers. They work well for treating many other types of epilepsy, but taking them on a regular basis can worsen seizures in people with Dravet syndrome.

The Food and Drug Administration (FDA) has recently approved the following antiseizure medications to reduce the frequency of seizures in people with Dravet syndrome:

  • cannabidiol (Epidiolex)
  • fenfluramine (Fintepla)
  • stiripentol (Diacomit)

Under the older ICD-10 codes, families may have found it difficult to obtain reimbursement from insurance providers for newly approved medications.

“Some of these medications are more expensive than other seizure prevention options, and that can sometimes create pushback from insurance companies that want to make sure patients have tried other options first,” Hood said. “Having a specific code is important for insurance companies to understand why this particular patient with epilepsy needs this particular medication.”

The creation of new ICD-10 codes may also help scientists study Dravet syndrome — and push for the development and approval of new treatments.

Researchers use ICD-10 codes to track the number of people affected by specific health conditions. To do this effectively, they need disease-specific codes.

The new codes may help scientists gain a clearer understanding of how many people live with Dravet syndrome.

They may also help researchers quantify the high costs of medical care for Dravet syndrome. This may help them advocate for the approval of treatments that may seem unduly expensive for managing other types of epilepsy but are more cost effective for managing Dravet syndrome.

“These community members have a very severe form of epilepsy, and they are going to be high [healthcare] utilizers relative to other more benign forms of epilepsy,” said Miller.

“If you can’t tease apart that these patients are utilizing lots and lots of resources at baseline, it won’t make sense to approve a new expensive medication,” he continued.

Researchers may also use the new ICD-10 codes to identify and recruit people with Dravet syndrome to take part in clinical trials. These studies are essential for developing new treatments, which could help improve the health and quality of life of people with this condition.

The new ICD-10 codes have only been adopted in the United States. However, DSF hopes that the World Health Organization will incorporate the new codes into future ICD revisions issued to countries around the world.

In the meantime, healthcare systems across the United States are updating their databases with the new codes.

To raise awareness of the codes, DSF has created business cards and a downloadable flyer that families can share with healthcare professionals. This may help ensure that their children’s medical records are updated with the new codes.

“Ideally, clinicians would just be aware of the change in codes, and their system would alert them, and this would all happens seamlessly,” said Hood.

“But in practice, we know that things can get missed,” she continued, “so DSF has tried to make families aware that these codes are now in place and has encouraged them to tell their healthcare providers.

New ICD-10 codes for Dravet syndrome may help families coping with this condition get insurance reimbursement for the latest and most effective treatments.

The new codes may also help scientists study the disease to learn how many people are affected by it, how much those people spend on medical care, and more.

The codes may help researchers identify and recruit eligible participants for clinical trials, which may ultimately help them develop new treatments for the disease.

Parents of children with Dravet syndrome can visit the DSF website to order business cards or download flyers with information about the new codes to share with their child’s doctors, nurses, and therapists.