Download Primary Biliary Cirrhosis Name Change Initiative

Primary Biliary Cirrhosis Name Change Initiative
Part 1
Supported by the PBCers Organization
As most of you know, the name Primary Biliary Cirrhosis (PBC) is very deceiving. For years PBC wasn't diagnosed
until a late disease stage and the patient had developed cirrhosis and in some patients liver failure. The past eighteen
years have brought many improvements for the PBC patient by way of early diagnosis and medications which slow the
disease’s progress. As treatments improve, most patients diagnosed with primary biliary cirrhosis may never reach
cirrhosis, need a liver transplant or be at risk of dying.
Changing the name of PBC has been discussed for years among both patients and the medical community. In April, at
the 2014 EASL Conference held in London, changing the name of PBC became more than just a quick point of
discussion. A panel of patient advocates and medical experts met and discussed the need to change the name not only
for the sake of the patients, but also to assist the providers treating PBC patients. The name change discussion continued
at the Milan European Association for the Study of the Liver (EASL) Symposium in May 2014, and in July this year the
PBCers Las Vegas PBC Conference.
Since April, the “Name Change Initiative” has taken on a life of it’s own with the help of medical professionals such as
Doctors Robert Gish, Eric Gershwin, Keith Lindor, John Vierling and many more. PBC organizations and support groups
have joined hands with medical professionals and facilities around the World, hoping to change the name of primary
biliary cirrhosis. The PBC name change is a global initiative with a goal of changing the name to accurately describe what
the diagnosis of “PBC” means for patients.
You may ask why change the name now? Dr. Robert Gish said “this couldn’t happen at a better time since 80-85% of
PBC patients do not have cirrhosis and may never develop it with further advances in therapy”. At recent medical
conferences, various reasons were discussed why the PBC name needs to be changed. The two most important are “to
correct the inaccuracy” of the disease name and “removing the cirrhosis stigma” which will help PBC patients, especially
those newly diagnosed. Also, the time frame couldn’t be better for the name change initiative. For the first time, the World
Health Organization (WHO) is asking medical professionals to help, online, in the revision process of the diagnosis and
symptom codes (ICD-11).
Two possible names being considered are primary biliary cholangitis and primary biliary cholestasis, most medical
professionals prefer cholangitis. The deadline for submissions is October 15, 2015, and the new ICD-11 codes will go into
affect sometime in 2017.
The proposed name primary biliary cholangitis construction is as follows:
Primary – not secondary to another cause
Biliary – bile ducts
Cholangitis – inflammation of the bile ducts
Since our conference in July, there have been numerous name change discussions among the PBCers. Some mentioned
we shouldn’t spend the organization’s money on this project, and continue promoting our education programs. The name
change initiative is not affecting the PBCers Organization financially in any way at this time. What it is costing is the
time and energy of volunteers who are helping to promote the name change. Any expenses the organization incurs will
be small. If there are any major expenses that come up, Dr. Robert Gish has pledged $5,000 to cover those expenses.
We sincerely thank Dr. Gish for his generosity.
In regard to our education programs, we will continue as we always have. Offering education, help and support to PBC
patients, their family members and friends. Promoting PBC awareness to the general public and hosting educational
fundraising events to fund PBC research. No matter what the name, PBC is a rare disease and we need to continue
making the general public aware of PBC and the need for research.
At the 2014 PBC Conference in Las Vegas, most of the guest speakers mentioned the name change initiative in their
presentations. Dr. John Vierling, who is well known in the medical community, spoke in depth on the topic. Dr. Vierling is
Professor of Medicine and Surgery at Baylor College of Medicine in Houston. He also serves as Director of Baylor Liver
Health and Chief of Hepatology. Listen to Dr. Vierling’s PBC conference video:
http://www.youtube.com/watch?v=WMFy90Zq4pI&feature=youtu.be
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Dr. Vierling said “There is a widespread misunderstanding about PBC and a need for greater awareness. Since cirrhosis
occurs only in the late stage, the name primary biliary cirrhosis is actually a misnomer for patients in the earlier
stages of the illness. The more technically correct and ponderous term for PBC is chronic non-supportive destructive
cholangitis. Changing the name to primary biliary cholangitis will better serve patients and the medical community
worldwide. The name proposal is now gaining momentum to formally change the medical name of the disease from
primary biliary cirrhosis to primary biliary cholangitis. This change does not affect the acronym PBC, but would eliminate
the stigma, confusion and fear caused by inclusion of the word “cirrhosis” in the name.”
Dr. Vierling further discussed the following needs to change the PBC name.
o Support: Patient support will be enhanced with family members and friends understanding that most patients don’t
have cirrhosis. So when the word cirrhosis is used, the diagnosis and prognosis will be correct for that patient.
o Anti-discrimination: Patients will be correctly diagnosed with primary biliary cholangitis and not be incorrectly
labeled with cirrhosis. Also there won’t be that implied connection, most commonly with alcohol and or drug use.
Hopefully removing some of the discrimination in patients, it will be easier purchasing life and health insurance
(various countries), mortgage real estate insurance (UK) and travel insurance (various countries).
o Research Donations: Most large research donors want to donate to liver diseases that have not reached the
cirrhosis stage. The focus of research concerns preventing cirrhosis and its complications. Even after educating the
donor about PBC, many will still donate to other diseases. Cirrhosis in the name may have kept PBC research from
receiving many large donations.
o Simplicity: Make it simple for both patients and doctors to understand that the disease is focused on the biliary
system, not cirrhosis since stages 1, 2 & 3 do not have cirrhosis.
o Clarity for Tests and Procedures: The type of tests and procedures vary with the PBC disease stage. Some tests
need to be performed prior to the cirrhosis stage, while other more extensive testing when cirrhosis is present. The
new global coding will help make sure patients have the proper tests and procedures for their disease stage. Also,
that it is covered by their insurance company, instead of increased or high out-of-pocket costs to patient.
o Accuracy: this follows the correct clinical, biochemical and the histological staging of this disease and non-invasive
techniques of staging the disease ultimately may be used in most patients. The name change will help achieve the
goals of a correct disease description, and stop people from feeling isolated with their disease due to an incorrect
diagnosis, and the despair often associated with the term cirrhosis.
o Rules & Metrics: With the anticipation of new compliance rules and metrics for different diagnoses and disease
stages there are expected rewards and penalties for test performance. The implementation of quality metrics will be
part of electronic health records and be part of budgeting and cost containment. Current quality metric examples
include testing for hepatitis C in the birth cohort and colon cancer screening in at risk age groups. Soon there will be
metrics on how patients with cirrhosis of any kind are tested and surveyed. If a patient does not have cirrhosis, they
should not be labeled as cirrhosis and “over tested”.
Some reasons the medical community would like the name changed:
o Inaccurate for everyone since the majority of patients do not have cirrhosis.
o Negative connotations of “cirrhosis” among:
o Patients and families (end-stage disease, inevitable need for transplantation or death).
o Lay people (associate with alcohol, drugs, viral hepatitis, and terminal disease).
o Potential research donors prefer to invest in preventing cirrhosis.
Time for a global change:
o Emerging consensus of support.
o Little support for the status quo to keep calling PBC primary biliary cirrhosis.
o ICD 10 code can incorporate new name since all changes must be submitted by Oct, 2015 (See ICD Codes Part 2).
If changing the PBC name will give more accurate information in the patient’s medical chart, and newly diagnosed
patients will not have to feel the cirrhosis stigma, then we have done what is right by promoting the PBC name change
initiative.
In summary, the name change initiative is consistent with our position of advocacy, preventing discrimination, and
providing the best of care for patients with PBC. The proposed name change also refines the clarity of disease state and
stage and ultimately allows patients, providers and advocates to more actively participate in the next major advances
expected in managing PBC.
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Part 2 ICD Codex System
The International Statistical Classification of Diseases and Related Health Problems, is usually called by the short-form
name International Classification of Diseases (ICD). It is designed as a health care classification system, providing a
system of diagnostic codes for classifying diseases, including nuanced classifications of a wide variety of signs,
symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or disease. The
International Classification of Diseases is published by the World Health Organization (WHO) and used worldwide for
morbidity and mortality statistics, reimbursement systems, and automated decision support in health care. In the United
States, the National Center for Health Statistics (NCHS), part of the Centers for Medicare and Medicaid Services
(CMS) oversees all changes and modifications to the ICD codes, in cooperation with WHO.
For the first time WHO is calling on experts and users to participate in the revision process for ICD-11 through a webbased platform. The outcome will be a classification that is based on user input and needs. Users include physicians,
nurses, other providers, researchers, health information managers and coders, health information technology workers,
policy-makers, insurers and patient organizations. Deadline for submission is October 15, 2015 and the final ICD-11
should be released in 2017. This comes at just the right time in the Primary Biliary Cirrhosis Name Change Initiative. It
gives additional time to submit requests and present updated PBC information, promote the inaccuracy of the PBC name
and patient discrimination.
How do the ICD codes affect us as patients: ICD Codes are found on patient paperwork, including hospital records,
physician records, and death certificates. ICD codes are used for all diagnosed illnesses and diseases (short or long term)
and symptoms. The ICD codes are used when patient providers submit bills to insurance companies for services. They
match the ICD codes with the Current Procedural Terminology (CPT) codes. They are then used by insurers to
determine the amount of reimbursement that a provider will receive by an insurer for services performed. Since everyone
uses the same codes to mean the same thing, they ensure uniformity. CPT codes are developed, maintained and
copyrighted by the American Medical Association (AMA). Medicare uses the Healthcare Common Procedure Coding
System (HCPCS) which are an adjusted form of CPT code.
If the diagnosis is for something acute, something that goes away with treatment like a rash or the flu, then the ICD
code will be less important to us. Because the illness or condition will go away, the code will stay on our record, but won't
affect future care. However, if we are diagnosed with a chronic or lifelong problem, like heart disease or diabetes,
then the ICD code will follow us for most of our medical care, and will help our healthcare providers make determinations
about our care. As electronic medical records are implemented across the world, these codes will affect our care more
and more including being linked to quality measures such as surveillance for liver cancer that will include additional blood
and imaging tests for patients with chronic liver diseases. Proper coding will help with determining the correct testing to
take place for a given patient to prevent over utilization and avoid errors in best practice algorithms.
The List of ICD-9 codes 520–579: diseases of the digestive system.
Liver
• (570) Acute and subacute necrosis of liver
o (570.0) Hepatic failure, acute
• (571) Chronic liver disease and cirrhosis
o (571.0) Fatty liver, alcoholic
o (571.2) Cirrhosis, liver, alcoholic
o (571.4) Hepatitis, chronic, unspec.
o (571.5) Cirrhosis, NOS
o (571.6) Primary biliary cirrhosis
o (571.9) Liver disease, chronic, unspec.
• (572) Liver abscess and sequelae of chronic liver disease
o (572.2) Coma, hepatic
o (572.4) Hepatorenal syndrome
• (573) Other disorders of liver
o (573.3) Hepatitis, toxic
ICD-9 will continue to be required until September 30, 2015.
The List of ICD-10 codes 1.0 K00 – K93: diseases of the digestive system.
Liver (K70–K77) Diseases of liver
• (K70) Alcoholic liver disease
o (K70.0) Alcoholic fatty liver
o (K70.1) Alcoholic hepatitis
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o (K70.2) Alcoholic fibrosis and sclerosis of liver
o (K70.3) Alcoholic cirrhosis of liver
o (K70.4) Alcoholic hepatic failure
o (K70.9) Alcoholic liver disease, unspecified
• (K71) Toxic liver disease
o (K71.0) Toxic liver disease with cholestasis
o (K71.1) Toxic liver disease with hepatic necrosis
o (K71.2) Toxic liver disease with acute hepatitis
o (K71.3) Toxic liver disease with chronic persistent hepatitis
o (K71.4) Toxic liver disease with chronic lobular hepatitis
o (K71.5) Toxic liver disease with chronic active hepatitis
o (K71.6) Toxic liver disease with hepatitis, not elsewhere classified
o (K71.7) Toxic liver disease with fibrosis and cirrhosis of liver
o (K71.8) Toxic liver disease with other disorders of liver
o (K71.9) Toxic liver disease, unspecified
• (K72) Hepatic failure, not elsewhere classified
o Hepatic encephalopathy
• (K73) Chronic hepatitis, not elsewhere classified
• (K74) Fibrosis and cirrhosis of liver
o (K74.0) Hepatic fibrosis
o (K74.1) Hepatic sclerosis
o (K74.2) Hepatic fibrosis with hepatic sclerosis
o (K74.3) Primary biliary cirrhosis
o (K74.4) Secondary biliary cirrhosis
o (K74.5) Biliary cirrhosis, unspecified
o (K74.6) Other and unspecified cirrhosis of liver
• (K75) Other inflammatory liver diseases
o (K75.0) Abscess of liver
o (K75.1) Phlebitis of portal vein
o (K75.2) Nonspecific reactive hepatitis
o (K75.3) Granulomatous hepatitis, not elsewhere classified
o (K75.4) Autoimmune hepatitis
o (K75.8) Other specified inflammatory liver diseases
o (K75.9) Inflammatory liver disease, unspecified
 Hepatitis NOS
• (K76) Other diseases of liver
o (K76.0) Fatty (change of) liver, not elsewhere classified
o (K76.1) Chronic passive congestion of liver
o (K76.2) Central haemorrhagic necrosis of liver
o (K76.3) Infarction of liver
o (K76.4) Peliosis hepatis
o (K76.5) Hepatic veno-occlusive disease
o (K76.6) Portal hypertension
o (K76.7) Hepatorenal syndrome
o (K76.8) Other specified diseases of liver
o (K76.9) Liver disease, unspecified
• (K77) Liver disorders in diseases classified elsewhere
ICD-10 Compliance Deadline Set as October 1, 2015
The Current Procedural Terminology (CPT) code set is a medical code set maintained by the American Medical
Association through the CPT Editorial Panel. The CPT code set describes medical, surgical, and diagnostic services and
is designed to communicate uniform information about medical services and procedures among physicians, coders,
patients, accreditation organizations, and payers for administrative, financial, and analytical purposes.
New editions are released each October. The current version is the CPT 2014.
Information compiled from the following sites:
http://www.ama-assn.org/ama/pub/physician-resources/
http://patients.about.com/od/
http://en.wikipedia.org/
http://www.businessgrouphealth.org/
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Part 3 - Questions & Answers
Robert Gish, MD
Clinical Professor of Medicine, University of Nevada, Las Vegas
Senior Medical Director, St. Joseph’s Hospital and Medical Center, Phoenix
Clinical Professor of Medicine (Consultant), Stanford Hospital and Medical Center, CA
Medical Director, Hepatitis B Foundation - Doylestown, PA
Principal, Robert G Gish Consultants, LLC - San Diego, CA
Sincere thanks to Dr. Gish for all his help and answering our questions.
1. What are your personal thoughts on changing the PBC name?
Patients who are given the diagnosis of cirrhosis are often labeled as “alcoholic” with no knowledge or sensitivity to their
actual diagnosis, or presence or absence of alcohol use. As you know, many liver diseases are linked to genetics and are
not in any way related to a person’s behavior. Unfortunately, the diagnosis of cirrhosis can lead to discrimination in a
number of settings. In a number of countries, once the diagnosis of cirrhosis is made, there is often a denial of life
insurance eligibility and thus the inability to buy real estate/housing (UK). The patient with a cirrhosis diagnosis in their
medical chart can have restrictions on health care access and can have denial of health care insurance. In addition, new
and evolving government regulations are directly linking performance metrics to many diagnoses such as acute
myocardial infarction and congestive heart failure as well as hepatitis C. If the diagnosis is wrong, penalties for the
provider can ensue and inappropriate or excessive testing may occur. This is predicted to be a future risk for providers
who have patients with the incorrect diagnosis of cirrhosis in a patient’s chart.
2. Is the name change initiative for the United States only?
No, the PBC Name Change Initiative is a global effort. It is patient driven, and the clinicians and researchers will support
and absorb the changes.
3. What group or organization will be making the decision for the name change?
The initial submission will be made to the World Health Organization (WHO). It will continue in increments by organization
and country. EASL, AASLD and APASL are all part of the name change dialogue.
4. When is the deadline for the submission of the name change to the WHO and what has been done?
The deadline for submission is October 15, 2015.
In addition, the following proposals for the name change have been submitted or in process:
WHO ICD 11 submission filed on line, pending review.
EASL submission done.
PBCers submission done.
AASLD submission pending.
APASL review in process.
Tweets, blogs, posts are all in progress.
Patient PBC organizations & group polls and web pages set up.
5. How likely is it that the name primary biliary cirrhosis will be changed?
With 85% + support, via online surveys, the name change is highly likely.
6. Is it certain the name will be changed to primary biliary cholangitis or is it just one of several names being
suggested?
This is in process, with a very high level of support for the specific name of cholangitis. We are continuing to gather input
and consensus, globally, so this is not a “done deal”. Yet we must recognize the huge momentum we have and the time
and effort to date.
7. I read the term cholangitis means infection. By changing the name to cholangitis, wouldn’t it give false hope
as being curable?
Cholangitis means inflammation of the biliary tree or biliary system, yet it also can mean infection. Importantly, to denote
infection the medical provider must state “infectious” cholangitis.
There are other types of cholangitis that are not infections such as autoimmune cholangitis, a close cousin of primary
biliary cirrhosis (cholangitis).
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Primary – not secondary to another cause
Biliary –bile ducts
Cholangitis – inflammation of the bile ducts
8. Why would providers like the name changed from cirrhosis?
From a provider’s perspective, when a patient does not have cirrhosis, they should not have the term cirrhosis in their
medical records. We need to be accurate as to the disease and disease state to be fair to our patients. Patients often
come into the clinic with the primary biliary cirrhosis diagnosis and think they are dying or need an immediate liver
transplant due to the cirrhosis connotation. The word cirrhosis is inaccurate or not true in 85% of patients and those
85/100 patients should be told about their disease state (lack of cirrhosis) and stage in a fair and transparent fashion.
For that 85% of patients who don’t have cirrhosis at the time of diagnosis it often takes a medical provider 2-3 clinic
sessions to educate the patient. During this time, it puts undue stress on the patient that shouldn’t be there in the first
place. It is important the patient understand they do not actually have cirrhosis, and they may never progress into cirrhosis
or need a transplant.
9. Those in cirrhosis stage and post transplant say it will take away from the severity of their disease, making it
harder to get Social Security Disability and Employer Disability. Is this true?
This is not true. Patients will have a specific code for cirrhosis in their medical chart and have even more details about the
level of cirrhosis and complications with the new ICD 10 and ICD 11 coding. The name change should make the access to
disability easier. Severe fatigue, a complication of primary biliary cholangitis and cirrhosis can be advocated as a reason
for disability in parallel with other discussions.
10. Why are the medical providers willing to promote the name change? Why have they changed their minds
since 2000 when we tried to change the name?
The patients have driven this name change. The providers, clinicians and researchers are supporting the patient
community. Providers also are driven by motivations to have accurate names for the disease states, both for patient care
and research purposes.
11. Doctors already use the ICD coding for PBC with explanations of the disease. Why would the new coding
make it better for PBC?
Yes, codes are already used. The new codes would be much more specific to the disease state and stage. Only those
patients with cirrhosis would have the new code for cirrhosis in their chart. The other 85% would not be falsely labeled
with a disease stage they do not have.
12. When the doctor makes a diagnosis, is this when the ICD code is used in the patient’s medical chart?
Yes, all MDs will have all diagnoses linked to ICD codes for billing, and federal requirements. All payers (insurance
companies) will also use this language.
13. Is this just for diagnosis or does the code include symptoms?
Yes, the ICD codes are for diagnosis and symptoms only.
14. What code is used for tests and procedures?
This is the CPT code system. It is different from ICD 9, 10 or 11 codes.
15. When the doctor’s office sends the bill to the insurance company for payment, is the ICD code used?
Yes. The insurance company then matches the ICD code with their disease or symptom. The CPT code is used for the
procedure or test and has a link to the ICD code to justify the exam, test or procedure or provider time.
16. So when the ICD 11 codes are changed for use in 2017, does this mean the insurance companies will need
new CPT codes to go along with ICD?
I do not know of any plan to change CPT codes. They keep adding new ones as new procedures and changes come up.
END…..
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