Unlocking Codes for Rare Disease Patients

April 21, 2022

What stands between rare cancer patients and life-saving treatment? A 3-to-7-character alpha-numeric code.

ICD Codes’ Importance to Rare Diseases

The global system of ICD codes is crucial for modern health care. It has many uses, but among the most basic is that it allows for precise disease classification. It’s how the world knows how many people are diagnosed with a given condition, the type of specialist diagnosing and managing each patient’s care, and the medications being used.

This information provides welcome insight that can be used to match rare disease patients with the right specialist, a process that can otherwise send them from doctor to doctor searching for a correct diagnosis and an effective treatment plan.

Imprecise Code Problems

Not having precise codes results in incomplete data, which can skew insurers’ coverage decisions and payments to providers for rare conditions like EBV+ post-transplant lymphoproliferative disorder.

Clinicians code information about patients’ conditions and treatments during every visit, and they must choose from available codes – even if the code isn’t as precise as it could be. This leads to imprecise records and underreporting of rare diseases in particular.

Underreporting, in turn, can result in more limited insurance coverage as many payers base such decisions on the number of people who have a particular diagnostic code on record. Another problem is that payment to providers for their care of patients with conditions for which there is no code is inconsistent.

Getting a Code

Securing a code is a protracted process that, in the United States, begins by convincing the ICD-10 Coordination and Maintenance Committee that a code update is necessary.

Even though the process of fully implementing codes in the worldwide system is a years-long process, once a code has been adopted by the U.S. committee, it can be included in clinical guidelines. It’s a critical first step to improving data, especially for ultra-rare conditions like EBV+ PTLD.

While PTLD has an ICD diagnostic code, there aren’t currently codes for the potential medications to treat it. Efforts to change that are underway though. The developer of tab-cel, a drug currently in Phase 3 trials, has taken the necessary first steps for it to be considered for a procedural code for its administration.

The value of precise and updated codes is obvious. Conversely, imprecise or non-existent codes create serious problems, especially for rare disease patients and the providers trying to help them. Other rare disease treatments have gotten new codes in recent years, generating optimism among the EBV+ PTLD community that their turn is coming.

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