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Support Letter for ICD-10
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Dear Doctors and Supporters of the Lipoprotein(a) Foundation, 
 
As you know the Lipoprotein(a) Foundation was formed with a vision to live in a world where high Lipoprotein(a) is routinely diagnosed, treated and family screened.  Our mission is to reveal this inherited lipid risk for premature cardiovascular disease and death; educate and empower patients and save lives.   With that vision in mind, I am writing to share our important accomplishment in the press release Lipoprotein Foundation Proposes New ICD-10 Diagnostic-Code, and also a request for your support, as clinician/researcher/patient who understands both the science of Lp(a), and the importance to patient’s lives of increasing recognition of this potentially lethal risk factor. 
 
Please cut and paste the email below supporting a new ICD-10 Diagnostic-Code for high Lp(a) (please feel free to customize and edit) and send to  nchsicd10CM@cdc.gov by November 13, 2017.  Please also share with your colleagues, friends and physician/research organizations and ask them to do this too!
 
 
 
David Berglund, MD, MPH
Medical Officer / Classification and Public Health Data Standards
National Center for Health Statistics, Mailstop P08
Metro IV, 2nd floor, Rm. 2534
3311 Toledo Rd. 
Hyattsville, MD 20782
 
Dear Dr. Berglund and Members of the ICD-10 Coordination and Maintenance Committee Meeting:
ICD-10 Codes for Elevated Lipoprotein(a)  
On behalf of the Lipoprotein(a) Foundation and the more than 63 Million Americans living with or at risk for cardiovascular disease due to elevated lipoprotein(a), we write today in support of the ICD-10 Elevated Lipoprotein(a) Code Proposal.   
The Lipoprotein(a) Foundation is a scientific evidence-based organization.  The proposal attached is based on the most up-to-date research currently available.   
Elevated Lipoprotein(a) [Lp(a)] is a highly prevalent, codominant genetic lipid disorder and risk factor for cardiovascular disease (CVD) including heart attack, stroke and peripheral arterial disease as well as calcific aortic valve stenosis (CAVS). Blood levels of Lp(a) span a wide range (<0.1 mg/dL to over 200 mg/dL), with median levels globally of 10-15 mg/dL. Elevated Lp(a) level (>30 mg/dL or >75 nmol/L) affects about 20 to 30% of the global population, and is causally linked to increased atherothrombotic events and CAVS. High Lp(a) is one of the most common hereditary disorders, although many who have it have not been recognized.
Lp(a) is a large particle, with two large linked components, one that is like low-density lipoprotein (LDL), termed apolipoprotein B (apoB), and another that is similar to plasminogen, termed apolipoprotein(a) (apo(a)). The apo(a) part varies widely in form, particularly between different individuals, giving rise to over 40 different forms of different sizes. Smaller and denser forms are associated with higher cardiac risk, in a fashion that is determined by genetics, and cannot be controlled by diet or exercise. Lp(a) may affect cardiac risk in more than one way. The apo(a) being structurally like plasminogen, but without its effects, may interfere with fibrinolysis, and thus promote thrombosis. Also, the apoB part of Lp(a) may promote atherosclerosis similarly to LDL cholesterol.
Elevated Lp(a) is usually silent, without signs or symptoms, and not able to be detected by history or physical. Elevated Lp(a) is measured by a blood test. Recent genome-wide association and Mendelian randomization studies indicate that Lp(a) is a causal and independent risk factor for CVD. Thus, the first sign of elevated Lp(a) can be sudden death from myocardial infarction or stroke. There has been development of selective and potent Lp(a)-lowering agents, which has re-stimulated interest in Lp(a). Levels of Lp(a) may also be lowered by apheresis. Further understanding of Lp(a) pathophysiology and its clinical importance in the treatment of CVD may help reduce the residual risk present following current standard therapy. Lp(a) testing is recommended for those at intermediate or high CVD risk, a strong family history, recurrent CVD, premature CVD, and those unresponsive to guideline recommended therapies. Measurement of Lp(a) levels in patients with at least intermediate CVD risk are supported by several recent guidelines from medical societies, including the National Lipid Association, the Canadian Cardiovascular Society Guidelines for the Management of Dyslipidemia for the Prevention of Cardiovascular Disease in the Adult, and from the Task Force for the Management of Dyslipidaemias of the European Society of Cardiology (ESC) and European Atherosclerosis Society (EAS), the 2016 ESC/EAS Guidelines for the Management of Dyslipidaemias. 
An elevated level of Lp(a) is the only identifiable risk factor in many patients with CVD, particularly younger (<65 years old), reflecting its genetic contribution to CVD risk. In others, it compounds the risk from other risk factors. Finding a high level of Lp(a) in an asymptomatic patient would indicate the need for more aggressive treatment of other cardiovascular risk factors.
To enable tracking individuals and their families with high Lp(a), the Lipoprotein(a) Foundation and its scientific advisory board have proposed to create a specific ICD-10-CM code for elevated Lp(a). This will enable clinicians to more easily convey the etiology of CVD and CAVS risk, and to tailor preventative and treatment strategies for this, as well as providing a basis for data collection, for research into the CVD impact of this lipid.
References
 
Anderson TJ, Grégoire J, Pearson GJ. 2016 Canadian Cardiovascular Society Guidelines for the Management of Dyslipidemia for the Prevention of Cardiovascular Disease in the Adult. Can J Cardiol. 2016 Nov;32(11):1263-1282. Epub 2016 Jul 25.
 
Catapano AL, Graham I, De Backer G, et al. 2016 ESC/EAS Guidelines for the Management of Dyslipidaemias. Eur Heart J. 2016 Oct 14;37(39):2999-3058. Epub 2016 Aug 27.
 
Kronenberg F1, Utermann G. Lipoprotein(a): resurrected by genetics. J Intern Med. 2013 Jan;273(1):6-30. Epub 2012 Nov 12. 
 
Tsimikas S. A Test in Context: Lipoprotein(a): Diagnosis, Prognosis, Controversies, and Emerging Therapies. J Am Coll Cardiol. 2017 Feb 14;69(6):692-711. 
 
Tsimikas S. Lp(a) as a new target for reduction of risk of cardiovascular disease and emergence of novel therapies to lower Lp(a). Curr Opin Endocrinol Diabetes Obes. 2016 Apr; 23(2): 157–164. 
 

 
 
TABULAR MODIFICATIONS
 
                        E78      Disorders of lipoprotein metabolism and other lipidemias
 
                                    E78.4   Other hyperlipidemia
Delete                                    Familial combined hyperlipidemia
 
New code                               E78.41  Elevated Lipoprotein(a)
 
New code                                E78.49  Other hyperlipidemia
                                                             Familial combined hyperlipidemia
 
                        Z83      Family history of other specific disorders
 
                                   Z83.4    Family history of other endocrine, nutritional and metabolic diseases
 New sub-subcategory            Z83.43  Family history of other disorder of lipoprotein metabolism and other lipidemias
 
 New code                                             Z83.430   Family history of elevated lipoprotein(a)
 New code                                             Z83.438   Family history of other disorder of lipoprotein metabolism and other lipdemias                                                                                Family history of familial combined hyperlipidemia
 
 
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