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Hemophilia is a rare bleeding disorder in which the blood does not clot normally. Hemophilia is usually inherited and caused by a defect in one of the genes that determine how the body makes blood clotting factor VIII or IX. Rarely, hemophilia can be acquired. This can happen if the body forms antibodies that attack the clotting factors in the bloodstream, preventing the clotting factors from working. People born with hemophilia have little or no clotting factor, which is a protein needed for normal blood clothing. These proteins work with platelets, which are small blood cell fragments that form in the bone marrow, to help the blood clot. When blood vessels are injured, clotting factors help platelets stick together to plug cuts and breaks on the vessels and stop bleeding.
The two main types of hemophilia are A and B. People with hemophilia A, also called Classic hemophilia or Factor VIII deficiency, are missing or have low levels of clotting factor VIII. About 8 out of 10 people with hemophilia have type A. People with hemophilia B, also called Christmas disease or Factor IX deficiency, are missing or have low levels of clotting factor IX.
Factor X (ten) deficiency, also known as Stuart-Prower Factor Deficiency, is a disorder caused by a lack of protein called factor X in the blood. The factor X protein plays an important role in activating the enzymes that help to form a clot. Deficiency of this protein leads to problems with coagulation. The incidence of Factor X deficiency is estimated at 1 in 500,000 to 1 in a million.
Hemophilia can be mild, moderate, or severe, depending on how much clotting factor is in the blood. About 7 out of 10 people who have hemophilia A have the severe form of the disorder. People who do not have hemophilia have a factor VIII activity of 100 percent. People who have severe hemophilia A have a factor VIII activity of less than 1 percent. Hemophilia usually occurs in males. About 1 in 5,000 males are born with hemophilia each year.
The main treatment for hemophilia is called replacement therapy. Concentrates of clotting factor are slowly dripped or injected into a vein. Clotting factor concentrates can be made from human blood or recombinant clotting factors. Replacement therapy can be preventive/prophylactic or used on an as-needed basis called demand therapy.
Generic Name: Factor VIII (human)
Generic Name: Factor VIII (recombinant)
Generic Name: Factor VIII (Recombinant), PEGylated
Generic Name: Factor VIII/VWF
Generic Name: Factor IX (human)
Generic Name: Factor IX (complex)
Generic Name: Factor IX (recombinant)
Generic Name: Coagulation Factor X (human)
Prior authorization is required.
Factor VIII is considered medically necessary for the following disease states:
Factor IX is considered medically necessary for the following disease states:
Factor X is considered medically necessary for the following disease states:
The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.
Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Nervous/Mental Conditions, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:
A. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and
B. appropriate with regard to standards of good medical practice; and
C. not solely for the convenience of the Member, his or her Provider; and
D. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.
For the definition of Medically Necessary, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.
4/1999: Approved by Pharmacy & Therapeutics (P & T) Oncology Committee
1/30/2002: Factor VIII (Human), Factor VIII (Recombinant) and Factor VIII (Porcine) policies combined into one policy titled "Factor VIII"; Prior authorization deleted
4/24/2002: Type of Service and Place of Service deleted. Code Reference section completed
11/6/2002: Koate-DVI® added, Koate-P® and Koate-HP® deleted
8/17/2004: Code Reference section updated, ICD-9 diagnosis code 286.1, 286.4, 286.5 added, HCPCS S9345 added
9/24/2004: Code Reference section updated, CPT code 36440, 85244 deleted
10/29/2006: Factor VIII and Factor IX policies combined
9/12/2007: Added Mississippi Comprehensive Health Insurance Risk Pool Association will no longer provide benefits for antihemophilic factor, factor VIII, factor IX, factor concentrate or factorate products of any kind and services or related supplies received on an outpatient basis effective August 1, 2007, to Policy Exceptions
12/31/2008: Code Reference section updated per 2009 CPT/HCPCS revisions.
01/01/2009: Accredo preferred provider information removed. BCBSMS information added.
6/30/2009: New HCPC code Q2023 added to covered table.
8/26/2009: Policy statement updated to include medically necessary indications for VIII for routine prophylaxis to reduce the frequency of bleeding episodes and the risk of joint damage in children (0-16) with hemophilia A with no pre-existing joint damage.
12/15/2009: Coding Section revised for 2010 CPT4 and HCPCS revisions
03/08/2010: Description section was updated with Humate-P®, Wilate®. Also added brand names (ReFacto®, Xyntha® and Advate®) for Factor VIII (recombinant). Policy Section updated with coverage for Von Willebrand disease for Factor VIII. Policy Exceptions Section updated to remove Risk Pool language. HCPCS code J7185 & J7187 were added to Covered Codes for Factor VIII.
02/28/2011: Added new HCPCS code J7184 for Wilate® to the Code Reference section.
04/01/2014: Policy title changed from "Hemophilia Factor VIII (Human, Recombinant, Porcine) and Factor IX (Human, Complex, Recombinant)" to "Hemophilia Factor VIII Factor IX." Policy description and FDA Approved Indications for Factors VIII and IX were updated. Policy statement updated to include "Factor VIII Deficiency" and "Hemophilia B" in medically necessary statements. Removed deleted HCPCS code Q2023 from the Code Reference section.
12/31/2014: Added the following new 2015 HCPCS code for Factor VIII to the Code Reference section: J7182. Added the following new 2015 HCPCS codes for Factor IX to the Code Reference section: J7200 and J7201. Revised the description of the following HCPCS code: J7195.
05/28/2015: Policy description updated to add the brand name Eloctate® for Factor VIII (recombinant) and Alprolix® for Factor IX (recombinant).
08/28/2015: Code Reference section updated for ICD-10. Extended ICD-9 diagnosis code 286.5 to the fifth digit as 286.52 - 286.59. Removed deleted HCPCS code J7184.
03/01/2016: Policy description updated to add the brand name Nuwiq® for Factor VIII (recombinant) and brand names Rixibus® and Ixinity® for Factor IX (recombinant). Policy statement unchanged. Policy guidelines updated to add medically necessary definition. Sources section updated.
04/04/2016: Policy description updated to add the brand name Alphanate® for Factor VIII/VWF.
05/26/2016: Policy number L.5.01.433 added.
06/30/2016: Policy title updated to include Factor X. Policy description updated regarding Factor X and its indications. Added brand name Adynovate® for Factor VIII (Recombinant). Added policy statement that Factor X is considered medically necessary for Factor X Deficiency/Stuart-Prower Deficiency. Sources section updated.
11/01/2016: Approved by Pharmacy & Therapeutics (P&T) Committee.
Fact and Comparisons, November, 1998
American Hospital Formulary Services 1998
Micromedex, 1998, 2006
BeneFix® Prescribing Information
Helixate® Prescribing Information
Kogenate® Prescribing Information
Wilate® Prescribing Information
National Heart, Lung, and Blood Institute http://www.nhlbi.nih.gov/health/health-topics/topics/hemophilia/
Alphanate® Prescribing Information
Hemofil M® Prescribing Information
Koate DVI® Prescribing Information
Monoclate P® Prescribing Information
Recombinate® Prescribing Information
Xyntha® Prescribing Information
Advate® Prescribing Information
Humate P® Prescribing Information
AlphaNine SD® Prescribing Information
Mononine® Prescribing Information
Bebulin VH® Prescribing Information
Profilnine SD® Prescribing Information
Eloctate® Prescribing Information
Alprolix® Prescribing Information
Nuwiq® Prescribing Information
Rixubis® Prescribing Information
Ixinity® Prescribing Information
Coagadex® Prescribing Information
Adynovate® Prescribing Information
This may not be a comprehensive list of procedure codes applicable to this policy.
The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.
Covered Codes for Factor VIII