Hemophilia Clotting Factors (L31078)

Contractor Information

Contractor Name
Wisconsin Physicians Service Insurance Corporation		 Contractor Number
00951, 00952, 00953, 00954, 52280, 05101, 05201, 05301, 05401, 05102, 05202, 05302, 05402		 Contractor Type
Carrier - FI - MAC

LCD Information

Document Information
LCD ID Number
L31078

LCD Title
Hemophilia Clotting Factors

Contractor's Determination Number
INJ-003

AMA CPT/ADA CDT Copyright Statement
CPT codes, descriptions and other data only are copyright 2011 American Medical Association (or such other date of publication of CPT). All Rights Reserved. Applicable FARS/DFARS Clauses Apply. Current Dental Terminology, (CDT) (including procedure codes, nomenclature, descriptors and other data contained therein) is copyright by the American Dental Association. © 2002, 2004 American Dental Association. All rights reserved. Applicable FARS/DFARS apply.

Primary Geographic Jurisdiction


Oversight Region

Original Determination Effective Date
For services performed on or after 11/15/2010

Original Determination Ending Date


Revision Effective Date
For services performed on or after 01/01/2012

Revision Ending Date


CMS National Coverage Policy
Title XVIII of the Social Security Act section 1862 (a)(1)(A). This section allows coverage and payment of those services that are considered to be medically reasonable and necessary.

42 CFR 410.10(q) - Hemophilia clotting factors (The limitation contained in 42 CFR 410.10(q) states that this applies to hemophilia patients competent to use these factors without supervision)

Section 1861(s)(2)(I) of the Act provides Medicare coverage of blood clotting factors for hemophilia patients competent to use such factors to control bleeding without medical supervision, and items related to the administration of such factors.

The Medicare Modernization Act section 303(e)(1) added section 1842(o)(5)(C) of the Social Security Act which requires that, beginning January 1, 2005, a furnishing fee will be paid for items and services associated with clotting factor.

The Center for Medicare and Medicaid Services (CMS) includes the clotting factor furnishing fee in the published national payment limits for clotting factor billing codes. When the national payment limit for a clotting factor is not included on the Average Sales Price (ASP) Medicare Part B Drug Pricing File or the Not Otherwise Classified (NOC) Pricing File, the contractor must make payment for the clotting factor as well as make payment for the furnishing fee.

Payment for Blood Clotting Factor Administered to Hemophilia Inpatients 20.7.3 -
Section 6011 of Public Law (P.L.) 101-239 amended §1886(a)(4) of the Social Security Act (the Act) to provide that prospective payment system (PPS) hospitals receive an additional payment for the costs of administering blood clotting factor to Medicare hemophiliacs who are hospital inpatients.

Section 6011(b) of P.L. 101.239 specified that the payment be based on a predetermined price per unit of clotting factor multiplied by the number of units provided. This add-on payment originally was effective for blood clotting factors furnished on or after June 19, 1990, and before December 19, 1991.
Section 13505 of P. L. 103-66 amended §6011 (d) of P.L. 101-239 to extend the period covered by the add-on payment for blood clotting factors administered to Medicare inpatients with hemophilia through September 30, 1994.
Section 4452 of P.L. 105-33 amended §6011(d) of P.L. 101-239 to reinstate the add-on payment for the costs of administering blood-clotting factor to Medicare beneficiaries who have hemophilia and who are hospital inpatients for discharges occurring on or after October 1, 1998.

National Coverage Decision 45-24 - Anti-inhibitor Coagulant Complex (AICC)

Medicare Benefit Policy Manual Chapter 15 - Covered Medical and Other Health Services

50.5.5 - Hemophilia Clotting Factors; (Rev. 1, 10-01-03); A3-3112.4.B.2, HO-230.4.B.2

Medicare Claims Processing Manual Chapter 17 - Drugs and Biologicals
80.4 - Billing for Hemophilia Clotting Factors

Pub 100-04 Medicare Claims Processing Manual - Transmittal 521 - Date: April 8, 2005
Change Request 3755

Physician Fee Schedule Database Quarterly Update (April, July, Oct. only) 03/20/09
CR 6484

Hemophilia Clotting Factor Indicator on ASP Drug Pricing File 03/20/09; CR 6402

*Pub 100-04 Medicare Claims Processing Manual Transmittal 2223, Date: May 20, 2011
Change Request 7430; SUBJECT: July Update to the CY 2011 Medicare Physician Fee Schedule Database (MPFSDB) EFFECTIVE DATE: January 1, 2011, IMPLEMENTATION DATE: July 5, 2011, Wilate - Effective Date: July 1, 2011


Formerly:
MCM 2050.5, 5245
CIM 45-24
PM AB-98-3, 01/29/98; PM AB-99-75, 10/99

Indications and Limitations of Coverage and/or Medical Necessity
Discussion
Hemophilia is a hereditary blood disease characterized by greatly prolonged coagulation time. The blood fails to clot and abnormal bleeding occurs. It is a sex-linked hereditary trait transmitted by normal heterozygous females who carry the recessive gene. It occurs almost exclusively in males. For purposes of Medicare coverage, hemophilia encompasses Factor VIII deficiency (classic hemophilia, hemophilia A), Factor IX deficiency (hemophilia B, Christmas disease, plasma thromboplastin component), and von Willebrand's disease. Approximately 80% of those with hemophilia have type A.

Epidemiology:
Hemophilia
Affects 1 in 10,000 males worldwide
Hemophilia A represents 80% of all cases.
Ethnicity: affects all ethnic groups
Hemophilia A
Prevalence in general population: 1 in 5000
Incidence: 1 in 10,000 male births
Sex: almost exclusively male
Hemophilia B
Prevalence in general population: 1 in 30,000
Incidence: 1 in 100,000 male births
Sex: almost exclusively male
Vitamin K deficiency
Rare in adults; common in infants
Complicates liver disease, malabsorption

The frequency and severity of hemorrhagic events induced by hemophilia are related to the amount of coagulation factor in the blood. Those with mild hemophilia, (defined as having from 5% to 40% of normal coagulation factor activity), experience complications only after having undergone surgery or experiencing a major physical trauma. Those with moderate hemophilia (from 1% to 5% of coagulation factor activity) experience some spontaneous hemorrhage but normally exhibit bleeding provoked by trauma. Those with severe hemophilia (less than 1% of coagulation factor activity) exhibit spontaneous hemarthrosis and bleeding.

Short-term prophylactic treatment is given to patients before they undergo surgical procedures or engage in activities that carry a high risk of provoking a bleed. It may also be given to break the cycle of frequent bleeding into specific joints (target joints).

Treatment for these patients is dependent on the severity of the disease and may include the administration of blood clotting factors such as Factor VIII, Factor IX, Factor VIIa and, Anti-inhibitors to control the bleeding. Medicare provides coverage of self-administered blood clotting factors for hemophilia patients who are competent to use such factors to control bleeding without medical supervision. Medicare covers blood clotting factors for the following conditions:
- Factor VIII deficiency (classic hemophilia, hemophilia A).
- Factor IX deficiency (hemophilia B, Christmas disease, plasma thromboplastin component).
- von Willebrand's disease.

Antihemophilic factor is usually indicated for hemophilia when a bleeding episode arises (demand treatment) or when bleeding is anticipated or likely (prophylactic treatment). Primary prophylaxis is highly recommended. The strategy is to maintain the missing clotting factor at levels ~1% or higher on a regular basis in order to prevent bleeds, especially the onset of hemarthroses.

Inhibitors
Inhibitors are antibodies that neutralize factor VIII and can render replacement therapy ineffective. They are found more commonly in patients with moderate to severe hemophilia (up to 30% of those with severe disease) who have received significant amounts of replacement therapy.

Immune tolerance strategies in those with identified inhibitors also have been successful. Assuming no anamnestic response, low-titer inhibitors occasionally can be overcome with high doses of factor VIII. Recombinant human coagulation factor VIIa (rFVIIa) is indicated for the treatment of patients with bleeding episodes and for the prevention of bleeding in surgical interventions or invasive procedures in patients with hemophilia A or B with inhibitors to factor VIII or factor IX. High-titer inhibitors have been treated with variable success using porcine factor VIII, factor IX complex concentrates, recombinant factor VIII, and exchange plasma pheresis.

Anti-inhibitor coagulant complex (AICC) is a drug used to treat hemophilia in patients with Factor VIII inhibitor antibodies. AICC has been shown to be safe and effective and is covered by Medicare when furnished to patients with hemophilia A and inhibitor antibodies to Factor VIII who have major bleeding episodes and who fail to respond to other less expensive therapies.

Immune tolerance induction
Immune tolerance induction is designed to overcome the effects of antihemophilic factor or Factor IX inhibitors in certain hemophiliac patients, thus restoring effectiveness of antihemophilic factor or Factor IX therapy to resolve active bleeding in these patients. It consists of administration of very high doses of anti-hemophilic factor or Factor IX over an extended period of time.

A. Self-administered blood clotting factors for hemophilia patients and items related to the administration of such factors are covered under Part B when the following criteria exist:

1. The factor is used to control bleeding associated with hemophilia.

2. The patient is competent to use such factors without medical or other supervision; and

3. 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 by the carrier for each patient. Changes in a patient's medical needs over a period of time may require adjustments in the profile. It is expected that the treating source; e.g., a family physician or Comprehensive Hemophilia Diagnostic and Treatment Center, will have such information.


Coding Information

Bill Type Codes:

Contractors may specify Bill Types to help providers identify those Bill Types typically used to report this service. Absence of a Bill Type does not guarantee that the policy does not apply to that Bill Type. Complete absence of all Bill Types indicates that coverage is not influenced by Bill Type and the policy should be assumed to apply equally to all claims.

011x Hospital Inpatient (Including Medicare Part A)
012x Hospital Inpatient (Medicare Part B only)
013x Hospital Outpatient
018x Hospital - Swing Beds
021x Skilled Nursing - Inpatient (Including Medicare Part A)
022x Skilled Nursing - Inpatient (Medicare Part B only)
023x Skilled Nursing - Outpatient
071x Clinic - Rural Health
072x Clinic - Hospital Based or Independent Renal Dialysis Center
073x Clinic - Freestanding
074x Clinic - Outpatient Rehabilitation Facility (ORF)
075x Clinic - Comprehensive Outpatient Rehabilitation Facility (CORF)
077x Clinic - Federally Qualified Health Center (FQHC)
083x Ambulatory Surgery Center
085x Critical Access Hospital

Revenue Codes:

Contractors may specify Revenue Codes to help providers identify those Revenue Codes typically used to report this service. In most instances Revenue Codes are purely advisory; unless specified in the policy services reported under other Revenue Codes are equally subject to this coverage determination. Complete absence of all Revenue Codes indicates that coverage is not influenced by Revenue Code and the policy should be assumed to apply equally to all Revenue Codes.

Contractors may specify Revenue Codes to help providers identify those Revenue Codes typically used to report this service. In most instances Revenue Codes are purely advisory; unless specified in the policy services reported under other Revenue Codes are equally subject to this coverage determination.
Complete absence of all Revenue Codes indicates that coverage is not influenced by Revenue Code and the policy should be assumed to apply equally to all Revenue Codes.
TOB Note (above): Code 73X end-dated for Medicare use March 31, 2010; code 77X effective for dates of service on or after April 1, 2010.

Note: WPS Medicare has identified the Type of Bill (TOB) and Revenue Center (RC) codes applicable for use with CPT/HCPCS codes included in this LCD. Providers are reminded that not all CPT/HCPCS codes listed can be billed with all TOB and/or RC codes listed. CPT/HCPCS codes are required to be billed with specific TOB and RC codes. Providers are encouraged to refer to the CMS Internet-Only Manual (IOM) Pub. 100-04 Claims Processing Manual for further guidance.
0250 Pharmacy - General Classification
0636 Pharmacy - Drugs Requiring Detailed Coding

CPT/HCPCS Codes

Drugs Administered Other Than Oral Method

J7180INJECTION, FACTOR XIII (ANTIHEMOPHILIC FACTOR, HUMAN), 1 I.U.
J7183INJECTION, VON WILLEBRAND FACTOR COMPLEX (HUMAN), WILATE, 1 I.U. VWF:RCO
J7185INJECTION, FACTOR VIII (ANTIHEMOPHILIC FACTOR, RECOMBINANT) (XYNTHA), PER I.U.
J7186INJECTION, ANTIHEMOPHILIC FACTOR VIII/VON WILLEBRAND FACTOR COMPLEX (HUMAN), PER FACTOR VIII I.U.
J7187INJECTION, VON WILLEBRAND FACTOR COMPLEX (HUMATE-P), PER IU VWF:RCO
J7189FACTOR VIIA (ANTIHEMOPHILIC FACTOR, RECOMBINANT), PER 1 MICROGRAM
J7190FACTOR VIII (ANTIHEMOPHILIC FACTOR, HUMAN) PER I.U.
J7191FACTOR VIII (ANTIHEMOPHILIC FACTOR (PORCINE)), PER I.U.
J7192FACTOR VIII (ANTIHEMOPHILIC FACTOR, RECOMBINANT) PER I.U., NOT OTHERWISE SPECIFIED
J7193FACTOR IX (ANTIHEMOPHILIC FACTOR, PURIFIED, NON-RECOMBINANT) PER I.U.
J7194FACTOR IX, COMPLEX, PER I.U.
J7195FACTOR IX (ANTIHEMOPHILIC FACTOR, RECOMBINANT) PER I.U.
J7198ANTI-INHIBITOR, PER I.U.

Not Otherwise Classified (NOC)

J7199HEMOPHILIA CLOTTING FACTOR, NOT OTHERWISE CLASSIFIED

ICD-9 Codes that Support Medical Necessity

286.0CONGENITAL FACTOR VIII DISORDER
286.1CONGENITAL FACTOR IX DISORDER
286.2CONGENITAL FACTOR XI DEFICIENCY
286.3CONGENITAL DEFICIENCY OF OTHER CLOTTING FACTORS
286.4VON WILLEBRAND'S DISEASE
286.52 - 286.59ACQUIRED HEMOPHILIA - OTHER HEMORRHAGIC DISORDER DUE TO INTRINSIC CIRCULATING ANTICOAGULANTS, ANTIBODIES, OR INHIBITORS
286.7ACQUIRED COAGULATION FACTOR DEFICIENCY

Diagnoses that Support Medical Necessity

ICD-9 Codes that DO NOT Support Medical Necessity
Any code not listed above

ICD-9 Codes that DO NOT Support Medical Necessity Asterisk Explanation

Diagnoses that DO NOT Support Medical Necessity
Any diagnosis not listed above

General Information

Documentations Requirements
1. Documentation supporting the medical necessity of this item, such as ICD-9 codes, must be submitted with each claim. Claims submitted without such evidence will be denied as being not medically necessary.
2. Doctors orders and office/hospital progress notes must reflect medical necessity, and be available on request.

Appendices
Utilization Guidelines
Claims for blood clotting factors for hemophilia patients with these diagnoses may be covered if the patient is competent to use such factors without medical supervision.
The amount of clotting factors determined to be necessary to have on hand and thus covered under this provision is based on the historical utilization pattern or profile developed by the contractor for each patient. It is expected that the treating source, e.g., a family physician or comprehensive hemophilia diagnostic and treatment center, have such information. From this data, the contractor is able to anticipate and make reasonable projections concerning the quantity of clotting factors the patient will need over a specific period of time. Unanticipated occurrences involving extraordinary events, such as automobile accidents or inpatient hospital stays, will change this base line data and should be appropriately considered. In addition, changes in a patient's medical needs over a period of time require adjustments in the profile. (50.5.5 - Hemophilia Clotting Factors)

Sources of Information and Basis for Decision
Dimitrios A.P., Zaiden, R.A., Saduman, O.; Hemophilia, Overview; emedicine.medscape.com
Updated: Nov. 24, 2009
Sawaf, H., Lorenzana, A., Jardine, L.; Hemophilia A and B; emedicine.medscape.com; Updated: Dec. 2, 2008
Furlong, B. R., Furlong, M.A.; Hemophilia, Type A; emedicine.medscape.com; Updated: Oct. 6, 2008
Furlong, B. R., Furlong, M.A.; Hemophilia, Type B; emedicine.medscape.com; Updated: Oct. 6, 2008
Hedner, U., Kisiel, W.; Use of human factor VIIa in the treatment of two hemophilia A patients with high-titer inhibitors; Clin Invest. 1983 June; 71(6): 1836-1841.
Other contractor policies

Advisory Committee Meeting Notes
This policy does not reflect the sole opinion of the contractor or the Contractor Medical Director(s). Although the final decision rests with the contractor, this policy was developed in cooperation with the Carrier Advisory Committee(s), which include representatives of various medical specialty societies.

Meeting Date:
Wisconsin 06/18/2010
Illinois 05/19/2010
Michigan 05/12/2010
Minnesota 05/06/2010
J5: Iowa, Kansas, Missouri, Nebraska 06/24/2010
Date of the Open Meeting: 04/22/2010

Start Date of Comment Period
06/24/2010
End Date of Comment Period
08/08/2010
Start Date of Notice Period
10/01/2011
Revision History Number
X
Revision History Explanation
04/19/2010-In accordance with Section 911 of the Medicare Modernization Act of 2003, the states of American Somoa, California, Guam, Hawaii, Nevada and Northern Mariana Islands were removed from this LCD because claims processing for those states are transitioning from FI Contractor Wisconsin Physician Services (WPS - 52280) to MAC Part A Contractor  Palmetto.

8/1/2010 - The description for Bill Type Code 11 was changed
8/1/2010 - The description for Bill Type Code 12 was changed
8/1/2010 - The description for Bill Type Code 13 was changed
8/1/2010 - The description for Bill Type Code 18 was changed
8/1/2010 - The description for Bill Type Code 21 was changed
8/1/2010 - The description for Bill Type Code 22 was changed
8/1/2010 - The description for Bill Type Code 23 was changed
8/1/2010 - The description for Bill Type Code 71 was changed
8/1/2010 - The description for Bill Type Code 72 was changed
8/1/2010 - The description for Bill Type Code 73 was changed
8/1/2010 - The description for Bill Type Code 74 was changed
8/1/2010 - The description for Bill Type Code 75 was changed
8/1/2010 - The description for Bill Type Code 83 was changed
8/1/2010 - The description for Bill Type Code 85 was changed

8/1/2010 - The description for Revenue code 0250 was changed
8/1/2010 - The description for Revenue code 0636 was changed

10/18/2010 - In accordance with Section 911 of the Medicare Modernization Act of 2003, the states of Colorado, New Mexico, Oklahoma and Texas were removed from this LCD because claims processing for those states are transitioning from FI Wisconsin Physicians Service (52280) to MAC Part A Trailblazer (04901).

11/21/2010 - The following CPT/HCPCS codes were deleted:
J1785 was deleted from Group 1

01/01/2011 added new HCPCS code J7184 to the policy.

02/21/2011 - In accordance with Section 911 of the Medicare Modernization Act of 2003, the states of Delaware, District of Columbia, Maryland, New Jersey and Pennsylvania were removed from this LCD because claims processing for these states are transitioning from FI Wisconsin Physician Service (WPS 52280) to MAC Part A contractor Highmark (12901).

*07/01/2011, Q2041, Injection, Von Willebrand factor complex (human), Wilate, 1 I.U. will replace
J7184, Injection, von Willebrand factor complex (human), Wilate, per 100 IU effective July 1, 2011 Implementation date July 5,2011. This is based on CR 7430. Note the dosage change in the dosage.;

08/27/2011 - This policy was updated by the ICD-9 2011-2012 Annual Update.

10/01/2011 added ICD-9 codes 286.52-286.59

11/21/2011 - The following CPT/HCPCS codes were deleted:
Q2041 was deleted from Group 1

01/01/2012 Added codes J 1780 and J1783 and retired codeQ2041;
Reason for Change
Related Documents
This LCD has no Related Documents.

LCD Attachments

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Page Last Updated: Wednesday, 04-Jan-2012 15:56:07 CST