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I'm Aware That I'm Rare: Scott Manaker, MD, PhD (ICD10)

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Dr. Scott Manaker is an actively practicing pulmonary and critical care physician in the Pulmonary and Critical Care Division at the University of Pennsylvania in Philadelphia, where he is also Associate Professor of Medicine and Pharmacology, Vice Chair for Regulatory Affairs for the Department of Medicine and the Physician Advisor to the Penn Medicine Office of Billing Compliance. Dr. Manaker lectures widely across the United States as a physician advocate, speaking on chart documentation, coding, billing and reimbursement topics including the teaching physician regulations, critical care, evaluation and management coding, consultation policies, non-physician providers, and the impact of electronic medical records. In this episode, Dr. Manaker discusses the new and revised ICD-10-CM codes, which creates a marked difference for the world of PH.


I’m Scott Manaker. I’m a pulmonary and critical care physician at the University of Pennsylvania and at Penn Medicine. I’m a professor and the vice chair of the Department of Medicine and I spend a good portion of my time taking care of patients and a good portion of my time performing a lot of committee work focused on diagnosis, coding, documentation and billing.

So, today we’ll talk a little about ICD10 diagnosis coding and the new codes that are specific to your listeners interested in pulmonary hypertension. Let me take a step background and give more of a general introduction to our listeners today. Why are diagnosis codes important at all? Well, for most of the rest of the world outside of the States it’s the standard reasons of public health tracking, epidemiology and important research, because if you have accurate diagnosis codes, you can track patient populations, treatments and look for important effects. In the United States, we have the additional use of diagnosis codes for a variety of payment policies and so it becomes enmeshed in how we get reimbursed and paid for therapies and treatments to patients, including everything from doctor visits to prescriptions.

What are the international classification of diseases, or ICD, diagnosis codes? Well, they began around a century ago, around 1900, for the purposes of indexing hospital records and their use evolved over time to a variety of vital health statistics, including the incidence and prevalence of diseases, morbidity from diseases and mortality or death from disease. These statistics are used in a variety of public health reporting, and I’m sure your listeners are all too familiar with hearing on the radio and the television and reading in the newspaper about evolving health epidemics, the epidemics of obesity, the epidemic of diabetes, and so forth in the United States, and whether specific diseases or disorders are growing in importance or coming under control and effecting fewer and fewer people. These are all important uses of diagnosis codes that would be familiar to our listeners today.

Beginning 1978, the United States adopted the 9th of the international classification of diseases, also known as ICD9, and we used for several decades. Although we planned to move to ICD10 or the 10th edition as early as 1999, it actually did not happen until 2015 and we stopped updating ICD9 or the 9th edition in 2012 in anticipation of moving to the more modern 10th edition, ICD10 in 2015.

For patients with pulmonary hypertension ICD9 was very limited. There were only two appropriate diagnosis codes, basically for primary pulmonary hypertension, and then all other secondary forms of pulmonary hypertension were coarsely lumped into that secondary code. The conversion to ICD10 didn’t really help because those two diagnoses remained with two new ICD10 diagnosis codes, and this was really a problem with patients with pulmonary hypertension, because as our listeners know there were many different forms and many different causes for pulmonary hypertension.

What classification would be ideal for patients with pulmonary hypertension? Well, ironically, the World Health Organization, who also leads the ICD9 and ICD10 and is now hard at work on the 11th edition, or ICD11, they created a whole new classification system for pulmonary hypertension back in the early 2000’s, and that five category classification system was updated and refined as recently as 2013. It divides patients with pulmonary hypertension into idiopathic or unknown cause pulmonary hypertension and a variety of known causes in the first category. The second category pulmonary hypertension caused by left heart disease. The third category is pulmonary hypertension emanating from lung diseases and hypoxemia or low oxygen saturation. The fourth category is chronic thromboembolic disease, and the fifth category or other causes, unclear causes and multi-factorial causes.

Because of this discordance and discrepancy between the World Health Organization classification scheme and the diagnosis codes available to doctors and patients, a coalition made a presentation in September 2016 to the ICD10 coordination and maintenance committee. This is a committee that is part of the Department of Health and Human Services based at CMS, or the Center for Medicare and Medicaid Services. We made a successful presentation to that committee who agreed with most of our requests to both add and expand beyond the outdated primary and secondary pulmonary hypertension diagnosis. They implemented a variety of specific codes to match specific diagnoses, and ideally with known therapies. Examples would be true pulmonary hypertension. Another example would be chronic thromboembolic pulmonary hypertension because, as you and as many of our listeners know, those two specific types of pulmonary hypertension have very specific therapies that are available.

There’s still some work to do further expanding the diagnosis codes for various types of pulmonary hypertension, including an expansion for the specific cardiac malformations that can lead to pulmonary hypertension, and other forms of heart disease besides left heart disease, and also potentially identifying specific codes for a variety of genetic disorders or genetic linkages to pulmonary hypertension, but none-the-less the success of our request and the implementation beginning October 1st of 2015 of these new pulmonary hypertension diagnosis codes is a huge step forward for these patients and providers.

What should patients with pulmonary hypertension do? What should families of patients with pulmonary hypertension do right now? They should simply ask their treating providers, doctors, advanced practice providers, like nurse practitioners and physicians assistants, pharmacists, they should ask all of their treating providers, “Are my pulmonary hypertension diagnoses correct? Has my doctor, has my advanced practitioner identified the correct diagnosis code for my pulmonary hypertension,” because it will have a variety of beneficial effects from the perspectives of public health, of epidemiology and research correctly identifying populations of patients with specific forms of pulmonary hypertension will allow their identification for registries, their identification for important clinical trials, for new therapeutic interventions down the road. It will ensure appropriate payment policies linking the proper drugs to the right patients with the specific forms of pulmonary hypertension, so that they get those right treatments. There’s a variety of wonderful benefits simply from having that conversation between a patient and their family and their providers, “Am I now listed with the proper diagnoses for my type of pulmonary hypertension?”

Many patients will actually see it printed out on the materials that are routinely handed to patients at the conclusion of an office visit, or at their discharge from a hospitalization, and they should use that as an opportunity to ask their providers, “Which diagnoses are the ones representing my pulmonary hypertension and are these the correct diagnoses, the best diagnoses, the most specific diagnoses, or would we all be better off if we chose a different, more accurate and more specific diagnosis to describe my pulmonary hypertension, so that everyone who’s doing research and everyone who’s trying to develop new therapies will know I’m in that correct patient sub group.

I’m Scott Manaker, and I’m aware that I am rare.

Learn more about pulmonary hypertension at Never miss an episode with the phaware® podcast app. Follow us @phaware on facebook, twitter, instagram, youtube & linkedin Engage for a cure: #phaware #phawareMD

Originally published: February 9, 2018
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