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Diagnosis Agenda
March 5-6, 2019
Part 2
Welcome and announcements Donna Pickett, MPH, RHIA Co-Chair, ICD-10 Coordination and Maintenance Committee
Diagnosis Topics:
Contents
Babesiosis ........................................................................................................................................... 10
David, Berglund, MD Mikhail Menis, PharmD, MS Epi, MS PHSR Epidemiologist Office of Biostatistics and Epidemiology CBER/FDA C3 Glomerulopathy .......................................................................................................................... 13
David Berglund, MD Richard J. Hamburger, M.D. Professor Emeritus of Medicine, Indiana University Renal Physicians Association
Eosinophilic Gastrointestinal Diseases............................................................................................ 18
David Berglund, MD Bruce Bochner, MD Samuel M. Feinberg Professor of Medicine Northwestern University Feinberg School of Medicine and President, International Eosinophil Society
March 5-6, 2019
Food Insecurity.................................................................................................................................. 20
Donna Pickett
Glut1 Deficiency ................................................................................................................................ 23
David Berglund, MD
Hepatic Fibrosis ................................................................................................................................ 25
David Berglund, MD
Hypereosinophilic Syndromes ......................................................................................................... 27
David Berglund, MD Bruce Bochner, MD Samuel M. Feinberg Professor of Medicine Northwestern University Feinberg School of Medicine and President, International Eosinophil Society
Intracranial Hypotension ................................................................................................................. 30
David Berglund, MD Other Eosinophil Diseases ................................................................................................................ 35
David Berglund, MD Pulmonary Eosinophilic Diseases .................................................................................................... 38
David Berglund, MD Bruce Bochner, MD Samuel M. Feinberg Professor of Medicine Northwestern University Feinberg School of Medicine and President, International Eosinophil Society
Social Determinants of Health ......................................................................................................... 44
Donna Pickett Efrem Castillo, MC, CPE Chief Medical Officer, UnitedHealthcare Temperature-Sensitive Acquired Autoimmune Hemolytic Anemias........................................... 47
David Berglund, MD Jaime Morales, MD, FAAP Global Head for Complement Disorders Medical Affairs, Sanofi Genzyme ICD-10-CM TABULAR OF DISEASES - PROPOSED ADDENDA .......................................... 49
Traci Ramirez
ICD-10-CM INDEX OF DISEASES - PROPOSED ADDENDA ................................................. 54
Traci Ramirez
March 5-6, 2019
Coordination and Maintenance Committee meetings for implementation on October 1, 2019.
April 2019 Notice of Proposed Rulemaking to be published in the Federal Register as mandated by Public Law 99-509. This notice will include references to the finalized FY 2020 ICD-10-CM diagnosis and ICD- 10-PCS procedure codes to date. It will also include proposed revisions to the MS-DRG system based on ICD-10-CM/PCS codes on which the public may comment. The proposed rule can be accessed at:
http://www.cms.gov/Medicare/Medicare-Fee-for-Service- Payment/AcuteInpatientPPS/index.html?redirect=/AcuteInpatientPPS/ IPPS/list.asp
May 10, 2019 Deadline for receipt of public comments on proposed new diagnoses codes and revisions discussed at the March 5-6, 2019 ICD-10 Coordination and Maintenance Committee meetings for implementation on October 1, 2020.
June 2019 Final addendum posted on web pages as follows:
Diagnosis addendum - http://www.cdc.gov/nchs/icd/icd10cm.htm
Procedure addendum - http://cms.hhs.gov/Medicare/Coding/ICD10/index.html
June 14, 2019 Deadline for requestors: Those members of the public requesting that topics be discussed at the September 10-11, 2019 ICD- Coordination and Maintenance Committee meeting must have their requests submitted to CMS for procedures and NCHS for diagnoses.
August 1, 2019 Hospital Inpatient Prospective Payment System final rule to be published in the Federal Register as mandated by Public Law 99-509. This rule will also include links to all the final codes to be implemented on October 1, 2019.
This rule can be accessed at:
March 5-6, 2019
http://www.cms.gov/Medicare/Medicare-Fee-for-Service- Payment/AcuteInpatientPPS/index.html?redirect=/AcuteInpatientPPS/ IPPS/list.asp
August 2019 Tentative agenda for the Procedure part of the September 10-11, 2019 ICD-10 Coordination and Maintenance Committee meeting will be posted on the CMS webpage at –
https://www.cms.gov/Medicare/Coding/ICD10/C-and-M-Meeting- Materials.html
Tentative agenda for the Diagnosis part of the September 10-11, 2019 ICD-10 Coordination and Maintenance Committee meeting will be posted on the NCHS webpage at - http://www.cdc.gov/nchs/icd/icd10cm_maintenance.htm
Federal Register notice for the September 10-11, 2019 ICD- Coordination and Maintenance Committee meeting will be published. This will include the tentative agenda.
August 2, 2019 On-line registration opens for the September 10-11, 2019 ICD- Coordination and Maintenance Committee meeting at: https://www.cms.gov/apps/events/default.asp
September 2, 2019 Because of increased security requirements, those wishing to attend the September 10-11, 2019 ICD-10 Coordination and Maintenance Committee meeting must register for the meeting online at:
https://www.cms.gov/apps/events/default.asp
Attendees must register online by September 2, 2019; failure to do so may result in lack of access to the meeting.
September 10-11, 2019 ICD-10 Coordination and Maintenance Committee Meeting.
March 5-6, 2019
- Day 1: March 5, 2019: The meeting will begin at 9:00 AM ET and will end promptly at 1:
PM ET. There will not be a lunch break for this session. The meeting will be webcast via CMS at http://www.cms.gov/live/.
- Day 2: March 6, 2019: The meeting will begin at 9:00 AM ET and will end at 5:00 PM ET. Lunch will be held from 11:30 AM ET to 1:00 PM ET. The meeting will be webcast via CMS at http://www.cms.gov/live/.
- Toll-free dial-in access is available for listen-only participants who cannot join the webcast: Day 1-March 5, 2019: Phone: 1-877-267-1577; Meeting 990 668 147. Day 2-March 6, 2019: Phone: 1-877-267-1577; Meeting 990 668 147. We encourage you to join early, as the number of phone lines is limited.
March 5-6, 2019
Contact Information Mailing address:
National Center for Health Statistics ICD-9-CM Coordination and Maintenance Committee 3311 Toledo Road Hyattsville, Maryland 20782 Fax: (301) 458-
Comments on the diagnosis proposals presented at the ICD Coordination and Maintenance Committee meeting should be sent to the following email address: nchsicd10CM@cdc.gov
Donna Pickett (301) 458-
David Berglund (301) 458-
Cheryl Bullock (301) 458-
Shannon McConnell-Lamptey (301) 458-
Traci Ramirez (301) 458-
March 5-6, 2019
Babesiosis
Human babesiosis is a tick-borne zoonosis caused by intra-erythrocytic protozoa of the genus Babesia. Babesiosis can also be transmitted by transfusion of blood and blood components collected from an infected donor. Although the majority of U.S. babesiosis cases are caused by B. microti , which is prevalent in the Northeast and upper Midwest, other Babesia species such as B. duncani, B. divergens, and other species have been implicated in transmission in multiple U.S. states. This proposal is based on a request for new specific codes for multiple species of Babesia, received from Office of Biostatistics and Epidemiology, Center for Biologics Evaluation and Research (CBER), of the Food and Drug Adminstration (FDA).
As the majority of Babesia infections are presumed to be asymptomatic and may never be diagnosed, especially in younger persons, babesiosis may persist undetected. Transfusion of blood and blood components collected from asymptomatic donors may result in transfusion-transmitted babesiosis (TTB), leading to potentially fatal clinical illness in blood transfusion recipients. It has been also determined by FDA that babesiosis is a transfusion-transmitted infection as the disease agent was identified to be fatal or life-threatening and is transmissible by blood or blood components.
Over the past decade, there have been a growing number of reported cases in the United States, including TTB cases. Babesiosis infections have also been spreading to non-endemic states. In response, efforts are being made to mitigate the risk of human babesiosis infections, including the development of donor screening tests and testing strategies. On March 6, 2018, FDA licensed two independent assays for screening donors for B. microti: the Imugen Babesia microti Arrayed Fluorescent Immunoassay (AFIA) for the detection of B. microti -specific antibodies and the Imugen Babesia microti Nucleic Acid Test (NAT) for the detection of DNA of B. microti. On January 24, 2019, FDA approved a nucleic acid amplification test for donor screening, to detect specific Babesia species: B. microti , B. duncani , B. divergens , and B. venatorum
Creation of new codes for species of Babesia would improve Babesia infection coding granularity, to allow physicians to code the Babesia spp. accurately and to help FDA monitor safety of the blood supply for different Babesia spp. The addition of species-specific Babesia coding granularity will also allow FDA to more easily provide appropriate donor testing recommendations depending on geographic distribution and spread of different Babesia spp. in various regions of the country. Specifically, the improved coding granularity will enable the FDA to monitor spread of different Babesia species in the U.S. by states and counties of residence over time as well as from disease- endemic areas to non-endemic areas, thus allowing FDA to recommend appropriate donor testing strategies to prevent transfusion transmission of different Babesia species as tests for babesiosis are currently species-specific (i.e. no cross-reactivity between species). The improved Babesia coding granularity will also stimulate development of new Babesia species-specific donor tests and tests that can detect multiple Babesia species, which will further improve detection of blood donors positive for Babesia spp. and prevent transfusion-transmission. In summary, the added Babesia coding granularity will allow FDA to assess distribution of different Babesia spp. in the United States using large databases as required by FDAAA of 2007, make suitable recommendations for appropriate donor testing, stimulate development of species-specific testing, and prevent transfusion- transmitted Babesia infections and donor loss, thus helping to assure a safe and adequate blood
March 5-6, 2019
supply. Finally, the improved coding granularity will enhance Babesia coding accuracy and level of detail allowing physicians to make species-specific diagnosis.
References
Conrad, P.A., et al., Description of Babesia duncani n.sp. (Apicomplexa: Babesiidae) from humans and its differentiation from other piroplasms. Int J Parasitol, 2006. 36(7):779-789. https://doi.org/10.1016/j.ijpara.2006.03.
FDA. Recommendation for reducing the risk of transfusion-transmitted babesiosis: draft guidance for industry [Internet]. Silver Spring (MD): US Food and Drug Administration;2018 [cited 2018 Aug 24]. Available from: https://www.fda.gov/downloads/BiologicsBloodVaccines/GuidanceComplianceRegulatoryInformation/Guidances/B lood/UCM614734.pdf
Gubernot DM, Lucey CT, Lee KC, Conley GB, Holness LG, Wise RP. Babesia infection through blood transfusions: reports received by the U.S. Food and Drug Administration, 1997–2007. Clin Inf Dis 2009;48:25–30. https://doi.org/10.1086/
Gubernot DM, Nakhasi HL, Mied PA, Asher DM, Epstein JS, Kumar S. Transfusion-transmitted babesiosis in the United States: summary of a workshop. Transfusion 2009 Dec;49(12):2759-71. https://doi.org/10.1111/j.1537- 2995.2009.02429.x
Herwaldt, B.L., et al., Transfusion-associated babesiosis in the United States: a description of cases. Ann Intern Med,
- 155(8):509-519. https://doi.org/10.7326/0003-4819-155-8-201110180-
Krause, P.J., et al., Increasing health burden of human babesiosis in endemic sites. Am J Trop Med Hyg, 2003. 68(4):431-436. Retrieved from http://www.ajtmh.org/cgi/pmidlookup?view=long&pmid=
Menis M, et. al. Babesiosis Occurrence among the Elderly in the United States, as Recorded in Large Medicare Databases during 2006-2013. PLoS One. 2015 Oct 15;10(10):e0140332. https://doi.org/10.1371/journal.pone.
Menis M, Anderson SA, Izurieta HS, Kumar S, Burwen DR, Gibbs J, et al. Babesiosis among elderly Medicare beneficiaries, United States, 2006–2008. Emerg Infect Dis. 2012 Jan;18(1):128-31. https://doi.org/10.3201/eid1801.
Persing, D.H., et al., Infection with a babesia-like organism in northern California. N Engl J Med, 1995. 332(5):298-303. https://doi.org/10.1056/NEJM
Vannier EG, Diuk-Wasser MA, Ben Mamoun C, Krause PJ. Babesiosis. Infect Dis Clin North Am. 2015 Jun;29(2):357-
- https://doi.org/10.1016/j.idc.2015.02.
Vannier, E. and P.J. Krause, Human babesiosis. N Engl J Med, 2012. 366(25): 2397-2407. https://doi.org/10.1056/NEJMra
March 5-6, 2019
C3 Glomerulopathy
The Renal Physicians Association (RPA) has requested new ICD-10-CM codes for C glomerulopathy (C3G), a newly classified, uncommon kidney disorder characterized by the deposition of complement component 3 (C3) within the glomeruli. C3G is comprised of two distinct clinical subtypes: C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). While DDD has specific ICD-10-CM codes, there are no specific codes for C3G or the subtype C3GN.
Glomerulonephritis (GN) involves inflammation of kidney glomeruli, which are responsible for filtering the blood. This enables the kidneys to maintain electrolyte balance, remove waste, and control fluid homeostasis. The etiology of GN results from different sources of inflammation, with autoimmune disorders identified as a leading cause. GN is classified into pathogenic types, which have been defined by the classification forms seen in renal biopsy. One of these types is C glomerulopathy.
Membranoproliferative glomerulonephritis (MPGN) has a histopathological pattern of renal injury characterized by the thickening of capillary walls and mesangial enlargement secondary to increased cellularity and matrix deposition. The traditional classification of MPGN was based on the pattern and location of deposits visible via electron microscopy rather than the specific etiology of those deposits. MPGN type 1 has mesangial and subendothelial electron dense deposits, MPGN type 2 has electron dense material in the glomerular basement membrane, and MPGN type 3 has subepithelial deposits with basement membrane spikes. However, with that basis, the current codes for glomerulonephritis (GN) do not include a specific diagnosis code for C3GN.
Specific coding for the C3G two subtypes DDD and C3GN is critical for identifying appropriate disease etiology, patient segmentation, and therapeutic selection. More importantly, this coding aligns with clinical consensus reached by a group of experts in renal pathology, nephrology, complement biology, and complement therapeutics.
References
Bomback AS, Appel GB. Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol. 2012 Nov;8(11):634-642. https://doi.org/10.1038/nrneph.2012.
Pickering, MC., et al. C3 glomerulopathy: consensus report. Kidney International , 84(6):1079-1089, 2013. https://dx.doi.org/10.1038%2Fki.2013.
Sethi S, et al. Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN. J Am Soc Nephrol. 27:1278-1284, 2016. https://doi.org/10.1681/ASN.
March 5-6, 2019
TABULAR MODIFICATIONS
N00 Acute nephritic syndrome
N00.5 Acute nephritic syndrome with diffuse mesangiocapillary glomerulonephritis Add Excludes1: Acute nephritic syndrome with C3 glomerulopathy (N00.A) Add Acute nephritic syndrome with C3 glomerulonephritis (N00.A)
N00.6 Acute nephritic syndrome with dense deposit disease Add Acute nephritic syndrome with C3 glomerulopathy with dense deposit disease Revise Acute nephritic syndrome with membranoproliferative glomerulonephritis, type_
New code N00.A Acute nephritic syndrome with C3 glomerulonephritis Add Acute nephritic syndrome with C3 glomerulopathy, NOS Add Excludes1: Acute nephritic syndrome (with C3 glomerulopathy) with dense deposit disease (N00.6)
N01 Rapidly progressive nephritic syndrome
N01.5 Rapidly progressive nephritic syndrome with diffuse mesangiocapillary glomerulonephritis Add Excludes1: Rapidly progressive nephritic syndrome with C3 glomerulopathy (N01.A) Add Rapidly progressive nephritic syndrome with C glomerulonephritis (N01.A)
N01.6 Rapidly progressive nephritic syndrome with dense deposit disease Add Rapidly progressive nephritic syndrome with C3 glomerulopathy with dense deposit disease
New code N01.A Rapidly progressive nephritic syndrome with C3 glomerulonephritis Add Rapidly progressive nephritic syndrome with C3 glomerulopathy, NOS Add Excludes1: Rapidly progressive nephritic syndrome (with C3 glomerulopathy) with dense deposit disease (N01.6)
March 5-6, 2019
New code N04.A Nephrotic syndrome with C3 glomerulonephritis Add Nephrotic syndrome with C3 glomerulopathy Add Excludes1: Nephrotic syndrome (with C3 glomerulopathy) with dense deposit disease (N04.6)
N05 Unspecified nephritic syndrome
N05.5 Unspecified nephritic syndrome with diffuse mesangiocapillary glomerulonephritis Add Excludes1: Unspecified nephritic syndrome with C3 glomerulopathy (N05.A) Add Unspecified nephritic syndrome with C3 glomerulonephritis (N05.A)
N05.6 Unspecified nephritic syndrome with dense deposit disease Add Unspecified nephritic syndrome with C3 glomerulopathy with dense deposit disease
New code N05.A Unspecified nephritic syndrome with C3 glomerulonephritis Add Unspecified nephritic syndrome with C3 glomerulopathy Add Excludes1: Unspecified nephritic syndrome (with C3 glomerulopathy) with dense deposit disease (N05.6)
N06 Isolated proteinuria with specified morphological lesion
N06.5 Isolated proteinuria with diffuse mesangiocapillary glomerulonephritis Add Excludes1: Isolated proteinuria with C3 glomerulopathy (N06.A) Add Isolated proteinuria with C3 glomerulonephritis (N06.A)
N06.6 Isolated proteinuria with dense deposit disease Add Isolated proteinuria with C3 glomerulopathy with dense deposit disease
New code N06.A Isolated proteinuria with C3 glomerulonephritis Add Isolated proteinuria with C3 glomerulopathy Add Excludes1: Isolated proteinuria (with C3 glomerulopathy) with dense deposit disease (N06.6)
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N07 Hereditary nephropathy, not elsewhere classified
N07.5 Hereditary nephropathy, not elsewhere classified with diffuse mesangiocapillary glomerulonephritis Add Excludes1: Hereditary nephropathy, not elsewhere classified with C glomerulopathy (N07.A) Add Hereditary nephropathy, not elsewhere classified with C glomerulonephritis (N07.A)
N07.6 Hereditary nephropathy, not elsewhere classified with dense deposit disease Add Hereditary nephropathy, not elsewhere classified with C glomerulopathy with dense deposit disease
New code N07.A Hereditary nephropathy, not elsewhere classified with C glomerulonephritis Add Hereditary nephropathy, not elsewhere classified with C glomerulopathy Add Excludes1: Hereditary nephropathy, not elsewhere classified (with C glomerulopathy) with dense deposit disease (N07.6)
March 5-6, 2019
TABULAR MODIFICATIONS
K52 Other and unspecified noninfective gastroenteritis and colitis
K52.2 Allergic and dietetic gastroenteritis and colitis Delete Excludes2: food protein-induced proctocolitis (K52.82)
New code K52.23 Milk protein-induced proctocolitis
New code K52.24 Allergic proctocolitis Food-induced eosinophilic proctocolitis Food protein-induced proctocolitis
K52.29 Other allergic and dietetic gastroenteritis and colitis Food hypersensitivity gastroenteritis or colitis Immediate gastrointestinal hypersensitivity
K52.8 Other specified noninfective gastroenteritis and colitis
K52.81 Eosinophilic gastritis or gastroenteritis Delete Eosinophilic enteritis
New code K52.810 Eosinophilic gastroenteritis Add Eosinophilic enteritis
New code K52.811 Eosinophilic gastritis
K52.82 Eosinophilic colitis Delete Allergic proctocolitis Delete Food-induced eosinophilic proctocolitis Delete Food protein-induced proctocolitis Delete Milk protein-induced proctocolitis Add Excludes2: Allergic proctocolitis (K52.24) Add Food-induced eosinophilic proctocolitis (K52.24) Add Food protein-induced proctocolitis (K52.24) Add Food protein-induced enterocolitis syndrome (FPIES) (K52.21) Add Milk protein-induced proctocolitis (K52.23)
March 5-6, 2019
Food Insecurity
The Blue Cross Blue Shield of Vermont and the Yale School of Nursing are requesting new ICD- 10-CM codes related to food insecurity. They note that there is broad national consensus that assessing the social determinants of health (SDH) is a key facet of improving both individual and population health outcomes. BCBS of Vermont notes that this is represented first in the policy aims of government programs such as Healthy People 2020 (Secretary’s Advisory Committee on National Health Promotion and Disease Prevention Objectives for 2020, 2010) and the Center for Medicaid and Medicare Services Accountable Health Communities Project (Alley, Asomugha, Conway, & Sanghavi, 2016; Center for Medicare and Medicaid Innovation, 2017).
Within the social determinants, food insecurity is an established concept of concern. The United States Department of Agriculture publishes a yearly report on the state of food insecurity (Coleman-Jensen, Rabbitt, Gregory, & Singh, 2017). Professional organizations such as the American Academy of Family Physicians (AAFP), the American Academy of Pediatrics (AAP) and the Academy of Nutrition and Dietetics have policy statements on the need for social determinant and food insecurity screening and assessment (American Academy of Family Physicians, 2012; American Academy of Pediatrics, 2015; Holben & Marshall, 2017). To aid clinicians in practice, both the AAFP and the AAP have toolkits with specific steps for SDH and food insecurity screening and assessment (American Academy of Family Physicians, 2018; American Academy of Pediatrics & Food Research and Action Center, 2017)
Although ICD-10-CM has a code for food and water insufficiency (Z59.4, Lack of adequate food and drinking water), the concepts are joined, which makes tracking of each individual issue impossible. In addition, Z59.4 does not specifically represent the social and economic conditions that are inherent in the United States Department of Agriculture 2017 conceptual and operational definition of food insecurity. Lastly, there is no code to represent when patients with prescribed dietary needs are unable to follow a dietary regimen because of cost.
The submitter states that as we work toward more sensitive and specific screening and assessment for the social determinants of health in clinical practice, specific language for the mature concept of food insecurity is a necessary step.
The Blue Cross Blue Shield of Vermont and Yale School of Nursing request to aid patient and population care and study, is to divide Z59.4 capture food and water concepts separately, add a specific code at subcategory Z59.4 for the concern of food insecurity, and add a unique code at Z91.1 and Z71.8 to capture lack of compliance to dietary regimen due to financial hardship, a sequela of food insecurity.
This proposal is supported by a number of organizations and individuals including: the Academy of Nutrition and Dietetics, Colorado Prevention Alliance, Connecticut State Medical Society Blue Cross Blue Shield New York, and the Oregon Primary Care Association.
REFERENCES:
Alley, D. E., Asomugha, C. N., Conway, P. H., & Sanghavi, D. M. (2016). Accountable health communities - addressing social needs through medicare and medicaid. New England Journal of Medicine, 374 , 8-11. doi:10.1056/NEJMp
