Specific National Code for PWS Expected to Facilitate Research, Improve Patient Care

Prader-Willi syndrome (PWS) has been given its own unique code by the National Center for Health Statistics (NCHS), which will make it easier to track accurate data on medical care and clinical outcomes of people with the disease.

The NCHS is responsible for assigning different diseases with ICD-10 (International Classification of Diseases, Tenth Revision) codes; this is critical for tracking data about particular diseases.

Until recently, PWS has been under the ICD-10 code Q87.1, which covers a number of rare genetic disorders that are associated with short stature. The code has also included at least eight other conditions, such as Cockayne syndrome, Aarskog syndrome, Noonan syndrome, and Seckel syndrome.

However, apart from the relatively arbitrary symptom of short stature, the conditions included in the Q87.1 code are quite different from each other. They have different genetic causes, lead to different medical manifestations, and typically require different types of treatment.

The fact that PWS has been so far grouped together with other diseases has made it difficult for researchers to obtain useful and accurate statistics about the disease itself.

Starting on Oct. 1, the ICD-10-CM code for PWS is Q87.11 Prader-Willi syndrome.

“We’re thrilled to have this unique ICD-10 code for PWS, which will facilitate research studies and improve patient care,” Theresa Strong, director of research programs at the Foundation for Prader-Willi Research, said in news release.

“The new ICD-10 code will allow scientists to conduct epidemiologic research on PWS, determine true mortality rates, recruit patients for clinical trials, track outcomes of clinical interventions, and develop protocols for standard of care,” Strong added.

The NCHS is the principal health statistics agency in the U.S. Its main mission is to provide statistics and data to guide public policies and action, with the ultimate goal of improving the health of Americans.

Getting the agency to designate a unique code for PWS was a group effort, said Strong.

“We’re grateful to the Prader-Willi Syndrome Association (USA) and the International Prader-Willi Syndrome Organisation who partnered with us to advance the ICD-10 proposal, to the many PWS experts who contributed to the development of the proposal, and to Dr. Dan Driscoll, who presented the proposal to the ICD-10-CM Coordination and Maintenance Committee,” she said.