Blood clotting factors (J7183-J7199) for hemophilia patients with any of the following diagnoses may be covered to control bleeding:

• Factor VIII deficiency (classic hemophilia);
• Factor IX deficiency (also termed plasma thromboplastin component (PTC) or Christmas factor deficiency);
• Von Willebrand's disease.

Prophylactic therapy (regular administration of clotting factor to prevent bleeding) is covered for individuals with severe hemophilia A or B. Severe hemophilia A or B defined as factor VIII or factor IX less than 1 percent.

Prophylactic therapy should be instituted early (prior to the onset of frequent bleeding), with the aim of keeping the trough FVIII or FIX level above 1 percent between doses. This can usually be accomplished by giving 25-50 FVIII units/kg 3 times per week or every other day, or 40-100 FIX units/kg 2 to 3 times weekly.

It is also recommended that individuals on prophylaxis have regular follow-up visits to evaluate joint status, to document any complications, and to record any bleeding episodes that occur during prophylaxis.

When using NovoSeven® Coagulation Factor VIIa (Recombinant)(J7189), special consideration needs to be given as the Food and Drug Administration (FDA) approved indications are limited to the following:

• treatment of bleeding episodes in hemophilia A or B patients with inhibitors to Factor VIII or Factor IX
• prevention of bleeding in surgical interventions or invasive procedures in hemophilia A or B patients with inhibitors to Factor VIII or Factor IX
• treatment of bleeding episodes in patients with congenital FVII deficiency
• prevention of bleeding in surgical interventions or invasive procedures in patients with congenital FVII deficiency

The use of blood clotting factors other than the FDA labeled indications is considered experimental/investigational and therefore, not covered. A participating, preferred, or network provider can bill the member for the non-covered service.

The amount of clotting factors determined to be necessary to have on hand and thus covered under this provision will be based on the historical utilization pattern or profile developed for each patient. It is expected that the treating source, e.g., a family physician or Comprehensive Hemophilia Diagnostic and Treatment Center, will have such information. Unanticipated occurrences involving extraordinary events such as automobile accidents, inpatient hospital stays, etc., will change this base line data and will be appropriately considered. In addition, changes in a patient's medical needs over a period of time require adjustments in the profile.

A patient profile form is required for each patient receiving these factors. The information must be updated and available upon request. This information must include the following:

• Patient's name, address, phone number, date of birth, height, and weight
• Allergies, insurance ID number, patient's diagnosis, and appropriate diagnosis code
• Factor prescribed (include manufacturer, brand name, and procedure code)
• Percentage level, inhibitor present, approximate number of units per dose, and doses per month

Incomplete information will result in a denial of services. Additionally the treating physician's name, provider number, date, and the provider's signature, must accompany the profile and prescription. (NOTE: It is not appropriate for the profile to state "signature on file.") The prescription must be rewritten yearly.

NOTE:

Coverage for blood clotting factors is determined according to individual or group customer benefits. Blood clotting factors are not reimbursable under the prescription benefit.

Place of Service: Inpatient/Outpatient

The infusion of hemophilia clotting factor is typically an outpatient procedure which is only eligible for coverage as an inpatient procedure when uncontrolled by outpatient treatment for one of the following:  severe abdominal pain, acute neurological changes such as severe headache or altered mental status, protracted/severe bleeding, or hemarthrosis.

Description

Hemophilia, a blood disorder characterized by prolonged coagulation time, is caused by a deficiency of a factor in the plasma necessary for blood to clot.

NOTE:

This policy is designed to address medical guidelines that are appropriate for the majority of individuals with a particular disease, illness, or condition. Each person's unique clinical circumstances may warrant individual consideration, based on review of applicable medical records.

Under the Federal Employee Program, all services that utilize FDA-approved drugs, devices, or biological products are eligible when intended for the treatment of a serious or life-threatening condition and when medically necessary and appropriate for the patient’s condition.

Highmark Medicare Services MPB N-108, Anti-Inhibitor Coagulant Complex (AICC) - NCD 110.3

Kase CS. Hemostatic Treatment in the Early Stage of Intracerebral Hemorrhage. The Recombinant Factor VIIa Experience. Stroke. 2005;36:2321-2322.

Sugg RM, Gonzales NR, Matherne DE, et al. Myocardial Injury in Patients with Intracerebral Hemorrhage Treated with Recombinant Factor VIIa. Neurology. 2006;67:1053-1055.

Juvela S, Kase CS. Advances in Intracerebral Hemorrhage Management. Stroke. 2006;37:301-304.

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Manco-Johnson MJ, Abshire TC, Shapiro AD, et al. Prophylaxis versus Episodic Treatment to Prevent Joint Disease in Boys with Severe Hemophilia, New Engl J Med. 2007;357(6):535-544.

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Mayer SA, Brun NC,  Begtrup K, et al. Efficacy and Safety of Recombinant Activated Factor VII for Acute Intracerebral Hemorrhage. New Engl J Med. 2008;358(20):2127-2137.

National Hemophilia Foundation, MASAC (Medical and Scientific Advisory Council) Document #182, MASAC Recommendations Concerning the Treatment of Hemophilia and Other Bleeding Disorders, April, 2008.

Logan AC, Goodnough LT. Recombinant factor VIIa: an assessment of evidence regarding its efficacy and safety in the off-label setting. Hematology Am Soc Hematol Educ Program. 2010;2010:153-9.

Lin Y, Stanworth S, Birchall J, Doree C, Hyde C. Use of recombinant factor VIIa for the prevention and treatment of bleeding in patients without hemophilia: a systematic review and meta-analysis. CMAJ. 2011 Jan 11;183(1):E9-19.

Avorn J, Kesselheim A. A hemorrhage of off-label use. Ann Intern Med. 2011 Apr 19;154(8):566-7.

Logan AC, Yank V, Stafford RS. Off-label use of recombinant factor VIIa in U.S. hospitals: analysis of hospital records. Ann Intern Med. 2011 Apr 19; 154(8):516-22.

Yank V, Tuohy CV, Logan AC, Bravata DM, Staudenmayer K, Eisenhut R, Sundaram V, McMahon D, Olkin I, McDonald KM, Owens DK, Stafford RS. Systematic Review: Benefits and Harms of In-Hospital Use of Recombinant Factor VIIa for Off-Label Indications. Ann Intern Med. 2011 Apr 19;154(8):529-40.

InterQual® Level of Care Criteria 2010. Acute Care Adult. McKesson Health Solutions, LLC.

Advate [antihemophilic Factor (Recombinant), Plasma/Albumin-Free method] [package insert]. Baxter Healthcare Corp. Westlake Village, CA, July, 2007.

Alphanate® (antihemophilic Factor/von willebrand Factor Complex [Human]) package insert. Los Angeles, CA: Grifols Biologicals Inc., January, 2007.

AlphaNine SD® (Coagulation Factor IX [Human]). Package insert. Grifols Biologicals, Inc. Los Angeles, CA. January 2005.

Bebulin VH (Coagulation Factor IX [Human]). Package insert. Baxter Healthcare Corporation. Westlake Village, CA. December 2001.

Benefix (Coagulation Factor IX [Human]). Package insert. Wyeth Pharmaceuticals, Inc. Philadelphia, PA December 2008.

Corifact (Factor VIII Concentrate [Human]. Package insert. CSL Behring LLC. Kankakee, IL. February 2011.

Hemofil M® (Antihemophilic Factor [Human], method M, monoclonal purified injection). Package insert. Baxter Healthcare Corporation. Westlake Village, CA. September 2007.

Humate-P® [Antihemophilic Factor/von Willebrand Factor Complex (Human), Dried, Pasteurized]. [package insert]. CSL Behring LLC. Kankakee, IL. October, 2007.

Koate-DVI® [Antihemophilic Factor [Human]). Package insert. Talecric Biotherapeutics Inc. Research Triangle Park, NC. September 2006.

Kogenate®FS antihemophilic factor (recombinant) [package insert]. Bayer HealthCare, LLC. Tarrytown, NY, October, 2008.

Monarc-M® (Antihemophilic Factor [Human], method M, monoclonal purified injection). Package insert. Baxter Healthcare Corporation. Westlake Village, CA. April 2005.

Monoclate-P® (Antihemophilic Factor [Recombinant]). Package insert. CSL Bering. Kankakee, IL. January 2007.

Mononine® (Coagulation Factor IX [Human]). Package insert. CSL Bering. Kankakee, IL. January 2007.

NovoSeven®  Coagulation Factor VIIa (Recombinant) [package insert]. NovoNordisk®. Princeton, NJ, October, 2006.

Profilnine SD (Coagulation Factor IX [Human]). Package insert. Grifols Biologicals, Inc. Los Angeles, CA. January 2004.

Proplex T Coagulation Factor IX [Human]). Package insert. Baxter Healthcare Corporation. Westlake Village, CA. November 2002.

Recombinate (Antihemophilic Factor [Recombinant]). Package insert. Baxter Healthcare LLC. Westlake Village, CA. October2005.

ReFacto® (Antihemophilic Factor [Recombinant]). Package insert. Wyeth Pharmaceuticals Inc. Philadelphia, PA. December 2007.

Xyntha™ [Antihemophilic Factor (Recombinant), Plasma/Albumin-Free][package insert]. Wyeth Pharmaceuticals, Inc. Philadelphia, PA. April, 2008.