CMS Approves New, Specific Diagnosis Code for Friedreich’s Ataxia

The Centers of Medicare & Medicaid Services (CMS) has approved a new, specific diagnosis code for Friedreich’s ataxia (FA) to be used by clinicians in medical records and examined by health insurance companies to determine coverage.

“We believe the new FA specific ICD-10 code G11.11 will lead to fewer rejected health insurance claims, help monitor outcomes of clinical management guidelines, and enhance ability to evaluate symptom progression and track disease prevalence,” Jennifer Farmer, the CEO of Friedreich’s Ataxia Research Alliance (FARA), said in a press release.

The International Classification of Diseases (ICD) coding system, established by the World Health Organization, is used by more than 100 countries to report diseases, injuries, symptoms, abnormal findings, social circumstances, external causes of disease, and cause of death.

The system allows easy access and analysis of health information, including death and disease rates, as well as data comparison between hospitals, regions, and countries, and across different time periods.

However, each country is responsible for approving new ICD codes. The U.S. agency in charge of that is the CMS, which administers the country’s main healthcare programs, including Medicare, Medicaid, and the Children’s Health Insurance Program.

In the previous ICD-9 system there was a specific diagnosis code for FA (334.0). When the U.S. moved to the ICD-10 system in 2015, the code for FA (G11.1) became less specific as it also covered a group of conditions described as early-onset cerebellar ataxias.

According to FARA’s educational video about the importance of the new FA code, from the 1,276 patients previously assigned the ICD-9’s specific FA code, only 558 (44%) were transitioned to the ICD-10 code that included FA, and 404 (32%) ended up being coded incorrectly.

FARA, along with physicians in the Collaborative Clinical Research Network in FA, recognized the need for greater code specificity and, in a 2019 meeting with the CMS, requested that FA would be removed from the G11.1 code and assigned its own specific code.

The new code is expected to facilitate clinical management, resulting in fewer rejections of health insurance claims, and expediting payer coverage approvals without the need for direct access to patients’ medical records for review of medical necessity.

It also will help track FA’s frequency, symptom onset and progression, medical outcomes over time (with current care and with future approved therapies), and hospitalizations; all likely contributing to health policy decision-making.

The new code was effective Oct. 1, and FARA recommends that every FA patient ask their clinician to update their ICD-10 diagnosis code from G11.1 to G11.11.

More information regarding the new code’s use is available here.