What is an ICD-10 Code?

Developed by the World Health Organization (WHO), this is the tenth version of the International Classification of Diseases (ICD), known as ICD-10. It is a system used worldwide by doctors, hospitals, insurance companies, and health organizations to classify and code all diagnoses, symptoms, and procedures. Each code is unique and provides specific information about a particular condition or disease.

The KCNQ2-related epilepsy ICD-10 codes will go into effect on October 1, 2024.

Why Are These Codes Important?

• Better Recognition of KCNQ2-related disorders: With KCNQ2-related epilepsy ICD-10 codes, KCNQ2-related epilepsy will now be more easily recognized by healthcare providers and insurers. This ensures that medical records accurately reflect the KCNQ2 diagnosis a patient has, rather than being categorized under other broader conditions.
• Improved Access to Care and Resources: These codes can make it easier for patients to obtain the medical care and therapies they need. Insurance companies often require an ICD-10 code for approval of treatments. With these new codes, there will be fewer barriers to getting the right care for your loved ones.
• Enhanced Research Opportunities: Researchers can now more accurately track and study KCNQ2-related epilepsy, which will help understand the disorder's full spectrum. This can lead to better treatments and, hopefully, a cure in the future.
• Increased Awareness and Advocacy: Specific codes raise the visibility of KCNQ2-related epilepsy within the medical community and among policymakers. This can lead to increased funding for research and support for our community.

Four new KCNQ2-Related Epilepsy ICD-10 codes

Here are the new ICD-10 codes, along with a brief explanation of each:

• G40.841 - KCNQ2-related epilepsy, not intractable, with status epilepticus
  This code is used when a patient has KCNQ2-related epilepsy that can be controlled with treatment (not intractable), but they have experienced status epilepticus. Status epilepticus is a prolonged seizure or a series of seizures without recovery in between, requiring urgent medical treatment.
• G40.842 - KCNQ2-related epilepsy, not intractable, without status epilepticus
  This code applies to patients whose KCNQ2-related epilepsy is controllable with treatment (not intractable) and who have not experienced status epilepticus.
• G40.843 - KCNQ2-related epilepsy, intractable, with status epilepticus
  This code is for patients whose KCNQ2-related epilepsy is difficult or impossible to control with treatment (intractable) and who have experienced status epilepticus.
• G40.844 - KCNQ2-related epilepsy, intractable, without status epilepticus
  This code refers to patients with KCNQ2-related epilepsy that is difficult to control with treatment (intractable) but who have not experienced status epilepticus.

What Do These Terms Mean?

• Not Intractable: This means that epilepsy can be controlled with treatment, such as medications or other therapies. It doesn't mean the condition is cured, but it can be managed.
• Intractable: This refers to epilepsy that is difficult to control despite treatment. Patients with intractable (or drug-resistant) epilepsy is considered unmanageable after at least two appropriate medicine trials.
• Status Epilepticus: This is a condition in which a seizure lasts longer than five minutes or when one seizure follows another without the person regaining consciousness in between.

Appreciation for Support During this Multi-Year Process

Gaining this recognition for KCNQ2 as an important and distinct disease was a major undertaking over the past few years. We could not have achieved this important milestone without the support of several key partners.

A special thanks to Xenon Pharmaceuticals for their unwavering support throughout this process. Their partnership has been invaluable in helping us bring attention to the needs of the KCNQ2 community.

We also want to express our deepest gratitude to our dedicated team of medical experts. Dr. John Millichap, in particular, spearheaded the effort and presented on our behalf to the Centers for Disease Control (CDC). His expertise and dedication have been instrumental in securing these new ICD-10 codes.

What Happens Next?

While this is a major step forward, there is still work to be done. We need to ensure that these new codes are widely adopted and used by healthcare providers and insurers so that the impact of KCNQ2-related epilepsy is fully recognized. We encourage all families and caregivers to inform their healthcare providers about these new ICD-10 codes, which go into effect October 1, 2024.

This achievement is a testament to the hard work and dedication of everyone in the KCNQ2 community. Together, we are making progress toward better care, understanding, and treatment for those affected by KCNQ2.