Paperwork – oh the paperwork!

Each time you visit a physician’s office or hospital, we leave with piles of forms and papers detailing the visit. The providers seen, the prescriptions written, results from tests conducted, instructions for follow up, and more. And each of those pieces of paper includes numerous codes that allow for tracking across the health systems that we are a part of.

One of the most important codes on those forms is called an ‘ICD code’. Or the International Classification of Diseases.

Used worldwide, there are millions of ICD codes.

Let’s say for example you are pregnant and you go to the doctor for a routine pre-natal visit. The physician will code their notes of the encounter form with all of the conditions and diagnoses that arose during that visit.

Pregnant? Check – there’s a code for that.
Perhaps have high blood pressure? There’s a code for hypertension.
Gestational diabetes? You guessed it. There’s a distinct code.
Experiencing Braxton hicks? Yep. Another code.
So… You’re getting the gist.

Any one physician visit or clinical encounter can yield multiple codes reflecting the complexity of the conditions that are being addressed.

But What Are These Codes Actually Used For?

The International Classification of Diseases (ICD) is the foundation for the identification of health trends and statistics, and the international standard for reporting diseases and health conditions. Owned, developed and published by the World Health Organization (WHO), it is the diagnostic classification standard for all clinical and research purposes.

The healthcare industry, clinical providers, IT professionals, data administrators, insurance providers, government agencies and many other stakeholders utilize ICD codes to properly populate electronic health records, track epidemiological trends, and support medical reimbursement decisions. Uses of the ICD codes also include retrieval and analysis of health information for evidence-based decision making; monitoring of the incidence and prevalence of diseases; observing reimbursements trends; and tracking of longitudinal outcomes and adherence to care guidelines.

ICD Code Refinement – Duchenne/Becker Muscular Dystrophy

In short, there is no code for Duchenne or Becker MD.

That means, when you see your physician, the code you receive is a broad Muscular Dystrophy category that includes most of the muscular dystrophy subtypes.

Relevance to Duchenne

The passage of the MD-CARE Act in 2001 set in motion a ground-swell of infrastructure development in Duchenne/Becker muscular dystrophy:

• MD STARnet Surveillance was initiated at the CDC in 2002
• The CDC Care Considerations for DBMD were published in the Lancet in 2010 (and the updated Care Considerations have been submitted to Lancet for review and publication)
• CINRG’s natural history study and international data collection
• PPMD’s DuchenneConnect Registry
• The establishment of operationalized care standards that became the basis for PPMD’s criteria for certification of the national Certified Duchenne Care Centers
• PCORI funding of PPMD’s DuchenneConnect Registry and the OneFlorida consortium
• And so much more

The lack of an ICD code specific to Duchenne has proven a barrier to diagnosis, care, surveillance, research, and access:

• While we have established surveillance, we have also had to develop costly and time consuming systems to discern Duchenne cases from those abstracted using the 359.1 (ICD-9) and G71.0 (ICD-10) code
• Payer decisions are being impacted as over-estimates of economic impacts are made when applying the calculations to their datasets
• And while care standards have been established, CDC has been unable to assess whether those standards are being implemented

So PPMD – with funding support from the Foundation to Eradicate Duchenne (FED) – and close collaboration with our colleagues at the Centers for Disease Control and Prevention (CDC) set out to change this. For the last two years we have been working to engage the federal Committee that oversees ICD-10 Code refinement and to build the evidence necessary to support the refinement of the ICD-10 code for MD.

On September 13, 2017 Annie Kennedy of PPMD and Dr. Kathryn Wagner of Kennedy Krieger Institute appeared before the ICD-10 Coordination & Maintenance Committee on behalf of PPMD, FED, the FSH Society, and our global MD community to together move forward a nomination for greater specificity in coding.

While there will ultimately be many benefits to the overall health system once we have greater coding specificity, it is our hope that a specific ICD code for Duchenne or Becker MD will allow for payers to better understand how investments into therapeutic interventions can impact cost utilization long-term. We are also eager to be able to assess how care standards are being implemented.