Clinical Policy: (Antihemophiliac Factor/von Willebrand Factor Complex Human) Reference Number: CP.MP.404 Effective Date: January 2008 Last Review Date: 12/16 See Important Reminder at the end of this policy for important regulatory and legal information. Coding Implications Revision Log Description, is a lyophilized concentrate of purified von Willebrand factor (VWF) and blood clotting factor VIII as well as other plasma proteins including fibrinogen and albumin was originally approved by the Food and Drug Administration for use in adult patients to treat and prevent bleeding from hemophilia A. Later the approval was expanded to treat spontaneous and traumatic bleeding for severe Von Willebrand s disease (VWD) and for mild and moderate VWD when desmopressin use is known or suspected to be inadequate. Policy/Criteria I. It is the policy of Health Net of California that is considered medically necessary in adult patients for treatment and prevention of bleeding in hemophilia A (classic hemophilia). II. It is the policy of Health Net of California that is considered medically necessary in adult and pediatric patients with severe VWD and either of the following indications: A. Treatment of spontaneous and trauma-induced bleeding episodes; B. Prevention of excessive bleeding during and post- surgery. III. It is the policy of Health Net of California that is considered medically necessary in adult and pediatric patients with mild to moderate VWD when the use of desmospressin is known or suspected to be inadequate or contraindicated and meet either of the following indications: A. Treatment of spontaneous and trauma-induced bleeding; B. Prevention of excessive bleeding during and post- surgery. Background Inherited bleeding disorders include abnormalities of coagulation factors and platelet function. Hemophilia A, also known as factor VIII deficiency or classic hemophilia, is the most common type of hemophilia. Hemophilia A is a genetic disorder caused by missing or defective factor VIII. Treatment of hemophilia A is replacement therapy with clotting factor VIII. Desmopressin, the synthetic version of a natural hormone vasopressin, is used to treat people with mild to moderate hemophilia A. Desmopressin stimulates the release of stored factor VIII and VWF and increases the level of these proteins in the blood. VWF, a clotting protein, carries and binds factor VIII, which then can stay in the bloodstream longer. Page 1 of 5
VWD is an inherited bleeding disorder that is caused by deficiency or dysfunction of VWF, a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and also binds and stabilizes blood clotting factor VIII (FVIII) in the circulation. Therefore, defects in VWF can cause bleeding by impairing platelet adhesion or by reducing the concentration of FVIII.. VWD is the most common bleeding disorder, affecting up to 1% of the US population. Treatment for VWD depends on the diagnosis and severity. The mainstay of treatment is Desmopressin, which stimulates the release of VWF from cells, which also increases FVIII. Desmopressin is effective in almost all patients with mild or moderate type 1 disease. In comparison, a high proportion of those with severe bleeding symptoms and marked prolongation of the bleeding time do not respond sufficiently to desmopressin. Desmopressin is also ineffective in the rare patients with type 1 disease who have normal or decreased levels of plasma VWF accompanied by absent stores of VWF in platelets. Desmopressin can be used to treat bleeding complications or to prepare patients with VWD for surgery. Patients with type 3 VWD and those with more severe type 2 disease often require replacement therapy with VWF. Replacement therapy is also indicated in some patients with type 1 VWD who have a more severe decrease of VWF, particularly in more serious bleeding situations when other measures have failed, or in those who may need prolonged treatment (e.g, post-surgery)., is purified from pooled human plasma from many donors and contains the clotting proteins deficient or defective in VWD, factor VIII and VWF. It is pasteurized by heat treatment and administered by the intravenous route. The FDA based its approval of use of in patients with VWD on results from two clinical trials - a U.S. clinical study of 35 VWD patients undergoing surgery and another study in Europe of an additional 27 subjects. Across the studies, 44 of the 62 patients underwent surgeries that could be classified as major (including joint replacement, intracranial surgery, and hysterectomy). In 61 subjects, investigators classified the efficacy of as either good or excellent. 1 National Heart, Lung and Blood Institute Humate- P is indicated for use in adult and pediatric patients for treatment of spontaneous and trauma-induced bleeding when use of DDAVP is thought or known to be inadequate or contraindicated. has received FDA approval for use in prophylactic management of surgery and invasive procedures in patients with VWD. 2 Coding Implications This clinical policy references Current Procedural Terminology (CPT ). CPT is a registered trademark of the American Medical Association. All CPT codes and descriptions are copyrighted 2015, American Medical Association. All rights reserved. CPT codes and CPT descriptions are from the current manuals and those included herein are not intended to be all-inclusive and are included for informational purposes only. Codes referenced in this clinical policy are for informational purposes only. Inclusion or exclusion of any codes does not guarantee coverage. Providers should reference the most up-to-date sources of professional coding guidance prior to the submission of claims for reimbursement of covered services. Page 2 of 5
CPT Description Codes 99601 Home infusion/specialty drug administration, per visit (up to 2 hours) 99602 Home infusion/specialty drug administration, each additional hour HCPCS Codes J7187 S9345 Description Injection, von Willebrand Factor Complex, (), per Iu VWF-RCO Home infusion therapy, anti-hemophilic agent infusion therapy (e.g., factor VIII); administrative services, professional pharmacy services, care coordination, and all necessary supplies and equipment (drugs and nursing visits coded separately), per diem ICD-10-CM Diagnosis Codes that Support Coverage Criteria ICD-10-CM Description Code D66 Hereditary factor VIII deficiency D68.0 Von Willebrand s disease Reviews, Revisions, and Approvals Date Approval Date Policy adopted from Health Net NMP 404 (Antihemophiliac Factor/von Willebrand Factor Complex Human) 12/16 12/16 References 1. U.S. Food and Drug Administration. Antihemophilic Factor/von Willebrand Factor Complex (Human), Dried, Pasteurized.. Oct 2007 2. National Heart, Lung and Blood Institute. The Diagnosis, Evaluation and Management of von Willebrand Disease. Aug 2016. Available at: https://www.nhlbi.nih.gov/healthpro/guidelines/current/von-willebrand-guidelines 3. Mannuccio Mannucci P, Kyrle PA, Schulman S, Di Paola J, et al.. Prophylactic efficacy and pharmacokinetically guided dosing of a von Willebrand factor/factor VIII concentrate in adults and children with von Willebrand's disease undergoing elective surgery: a pooled and comparative analysis of data from USA and European Union clinical trials. Blood Transfus. 2013 Oct;11(4):533-40. 4. Auerswald G, Kreuz W. Haemate P/ for the treatment of von Willebrand disease: considerations for use and clinical experience. Haemophilia. 2008 Nov;14 Suppl 5:39-46 5. Berntorp E. Haemate P/: a systematic review. Thromb Res. 2009 Nov;124 Suppl 1:S11-4 6. Carter NJ, Scott LJ. Human plasma von Willebrand factor/factor VIII complex (Haemate P/): in von Willebrand disease and haemophilia A. Drugs. 2007;67(10):1513-9 Page 3 of 5
7. Eckhardt CL, Mauser-Bunschoten EP, Peters M, et al. Inhibitor incidence after intensive FVIII replacement for surgery in mild and moderate haemophilia A: a prospective national study in the Netherlands. Br J Haematol. 2012 Jun;157(6):747-52 8. Castaman G, Coppola A, Zanon E, et al. Efficacy and safety during formulation switch of a pasteurized VWF/FVIII concentrate: results from an Italian prospective observational study in patients with von Willebrand disease. Haemophilia. 2012 Sep 7. 9. Rothschild C, D'oiron R, Borel-Derlon A, et al. Use of Haemate P as immune tolerance induction in patients with severe haemophilia A, who have failed previous induction attempts: a multicentre observational study. Haemophilia. 2012 Oct 8. 10. Furlong B, Furlong M. Hemophilia, Type A. emedicine. Last updated January 2007. Available at: http://emedicine.medscape.com/article/779322-overview 11. Geil J. Von Willebrand Disease. emedicine. Last updated February 2007. Available at: http://emedicine.medscape.com/article/959825-overview Important Reminder This clinical policy has been developed by appropriately experienced and licensed health care professionals based on a review and consideration of currently available generally accepted standards of medical practice; peer-reviewed medical literature; government agency/program approval status; evidence-based guidelines and positions of leading national health professional organizations; views of physicians practicing in relevant clinical areas affected by this clinical policy; and other available clinical information. The Health Plan makes no representations and accepts no liability with respect to the content of any external information used or relied upon in developing this clinical policy. This clinical policy is consistent with standards of medical practice current at the time that this clinical policy was approved. Health Plan means a health plan that has adopted this clinical policy and that is operated or administered, in whole or in part, by Centene Management Company, LLC, or any of such health plan s affiliates, as applicable. The purpose of this clinical policy is to provide a guide to medical necessity, which is a component of the guidelines used to assist in making coverage decisions and administering benefits. It does not constitute a contract or guarantee regarding payment or results. Coverage decisions and the administration of benefits are subject to all terms, conditions, exclusions and limitations of the coverage documents (e.g., evidence of coverage, certificate of coverage, policy, contract of insurance, etc.), as well as to state and federal requirements and applicable Health Plan-level administrative policies and procedures. This clinical policy is effective as of the date determined by the Health Plan. The date of posting may not be the effective date of this clinical policy. This clinical policy may be subject to applicable legal and regulatory requirements relating to provider notification. If there is a discrepancy between the effective date of this clinical policy and any applicable legal or regulatory requirement, the requirements of law and regulation shall govern. The Health Plan retains the right to change, amend or withdraw this clinical policy, and additional clinical policies may be developed and adopted as needed, at any time. Page 4 of 5
This clinical policy does not constitute medical advice, medical treatment or medical care. It is not intended to dictate to providers how to practice medicine. Providers are expected to exercise professional medical judgment in providing the most appropriate care, and are solely responsible for the medical advice and treatment of members. This clinical policy is not intended to recommend treatment for members. Members should consult with their treating physician in connection with diagnosis and treatment decisions. Providers referred to in this clinical policy are independent contractors who exercise independent judgment and over whom the Health Plan has no control or right of control. Providers are not agents or employees of the Health Plan. This clinical policy is the property of the Health Plan. Unauthorized copying, use, and distribution of this clinical policy or any information contained herein are strictly prohibited. Providers, members and their representatives are bound to the terms and conditions expressed herein through the terms of their contracts. Where no such contract exists, providers, members and their representatives agree to be bound by such terms and conditions by providing services to members and/or submitting claims for payment for such services. Note: For Medicaid members, when state Medicaid coverage provisions conflict with the coverage provisions in this clinical policy, state Medicaid coverage provisions take precedence. Please refer to the state Medicaid manual for any coverage provisions pertaining to this clinical policy. Note: For Medicare members, to ensure consistency with the Medicare National Coverage Determinations (NCD) and Local Coverage Determinations (LCD), all applicable NCDs, LCDs, and Medicare Coverage Articles should be reviewed prior to applying the criteria set forth in this clinical policy. Refer to the CMS website at http://www.cms.gov for additional information. 2016 Centene Corporation. All rights reserved. All materials are exclusively owned by Centene Corporation and are protected by United States copyright law and international copyright law. No part of this publication may be reproduced, copied, modified, distributed, displayed, stored in a retrieval system, transmitted in any form or by any means, or otherwise published without the prior written permission of Centene Corporation. You may not alter or remove any trademark, copyright or other notice contained herein. Centene and Centene Corporation are registered trademarks exclusively owned by Centene Corporation. Page 5 of 5