ph-0339 - Medical Policies - Alabama
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Hemophilia Products - Factor IX: Alphanine SD, Alprolix, BeneFIX, Idelvion, Ixinity, Mononine, Profilnine, Rebinyn, and Rixubis
Policy Number: PH-0339
Intravenous
Last Review Date: 08/04/2020
Date of Origin: 12/16/2014
Dates Reviewed: 12/2014, 04/2015, 05/2015, 09/2015, 12/2015, 03/2016, 06/2016, 12/2016, 06/2017, 09/2017, 11/2017, 11/2018, 03/2019, 02/2020, 08/2020
- Length of Authorization
Unless otherwise specified*, the initial authorization will be provided for 3 months and may be renewed.
Note: The cumulative amount of medication the patient has on-hand will be taken into account for authorizations. Up to 5 ‘on-hand’ doses for the treatment of acute bleeding episodes will be permitted at the time of the authorization request.
* Initial and renewal authorization periods may vary by specific covered indication
- Dosing Limits
A. Quantity Limit (max daily dose) [NDC unit]:
- N/A
B. Max Units (per dose and over time) [HCPCS Unit]:
Alprolix, Rebinyn |
23,000 billable units per 28-day supply |
Idelvion |
25,300 billable units per 28-day supply |
AlphaNine SD, Ixinity, Profilnine, Mononine |
36,800 billable units per 28-day supply |
BeneFIX |
46,000 billable units per 28-day supply |
Rixubis |
73,600 billable units per 28-day supply |
- Initial Approval Criteria1-9,10,11,15
Hemophilia Management Program |
Requirements for half-life study and inhibitor tests are a part of the hemophilia management program. This information is not meant to replace clinical decision making when initiating or modifying medication therapy and should only be used as a guide. |
Coverage is provided in the following conditions:
Universal Criteria
- Diagnosis of congenital factor IX deficiency has been confirmed by blood coagulation testing; AND
- Therapy NOT used for induction of immune tolerance in patients with Hemophilia B [ONLY the following products]:
-
-
- Alprolix
- Rixubis
- Ixinity
- Idelvion
- Rebinyn
- AlphaNine SD
- Mononine
- BeneFIX; AND
-
-
Hemophilia B (congenital factor IX deficiency aka Christmas disease) †
- Used as treatment in at least one of the following:
- Control and prevention of acute bleeding episodes (episodic treatment of acute hemorrhage); OR
- Perioperative management (*Authorizations valid for 1 month); OR
- Routine prophylaxis to prevent or reduce the frequency of bleeding episodes (excluding Rebinyn); AND
-
-
- Patient must have severe hemophilia B (factor IX level of <1%); OR
- Patient has at least two documented episodes of spontaneous bleeding into joints
-
-
Hemophilia Management Program |
|
† FDA Approved Indication(s)
- Dispensing Requirements for Rendering Providers (Hemophilia Management Program)
- Prescriptions cannot be filled without an expressed need from the patient, caregiver or prescribing practitioner. Auto-filling is not allowed.
- Monthly, rendering provider must submit for authorization of dispensing quantity before delivering factor product. Information submitted must include:
-
- Original prescription information, requested amount to be dispensed, vial sizes available to be ordered from the manufacturer, and patient clinical history (including patient product inventory and bleed history)
- Factor dose should not exceed +1% of the prescribed dose and a maximum of three vials may be dispensed per dose. If unable to provide factor dosing within the required threshold, below the required threshold, the lowest possible dose able to be achieved above +1% should be dispensed. Prescribed dose should not be increased to meet assay management requirements.
-
- The cumulative amount of medication(s) the patient has on-hand should be taken into account when dispensing factor product. Patients should not have more than 5 extra doses on-hand for the treatment of acute bleeding episodes.
- Dispensing requirements for renderings providers are a part of the hemophilia management program. This information is not meant to replace clinical decision making when initiating or modifying medication therapy and should only be used as a guide.
- Renewal Criteria 1-9,10,11,15
Coverage can be renewed based upon the following criteria:
- Patient continues to meet universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in section III; AND
- Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following: symptoms of allergic-anaphylactic reactions (anaphylaxis, dyspnea, rash), thromboembolic events (thromboembolism, pulmonary embolism), and development of neutralizing antibodies (inhibitors); AND
- Any increases in dose must be supported by an acceptable clinical rationale (i.e. weight gain, half-life study results, increase in breakthrough bleeding when patient is fully adherent to therapy, etc.); AND
- The cumulative amount of medication(s) the patient has on-hand will be taken into account when authorizing. The authorization will allow up to 5 doses on-hand for the treatment of acute bleeding episodes as needed for the duration of the authorization; AND
Treatment of acute bleeding episodes/Treatment of Spontaneous and trauma-induced bleeding episodes/On-demand treatment of bleeding episodes
- Renewals will be approved for a 6 month authorization period
Prevention of acute bleeding episodes/Routine prophylaxis to prevent or reduce the frequency of bleeding episode
- Renewals will be approved for a 12 month authorization period
- Dosage/Administration1-9
Alprolix
Indication |
Dose |
Control and prevention of bleeding episodes Hemophilia B |
One unit per kilogram body weight increases the circulating Factor IX level by 1% (IU/dL). Estimate the required dose or the expected in vivo peak increase in Factor IX level expressed as IU/dL (or % of normal) using the following: IU/dL (or % of normal) = [Total Dose (IU)/Body Weight (kg)] x Recovery (IU/dL per IU/kg) Minor and Moderate Circulating Factor IX required (% of normal) = 30-60 IU/dL -Repeat every 48 hours as needed Major Circulating Factor IX required (% of normal) = 80-100 IU/dL - Consider repeat dose after 6-10 hours, then every 24 hours for 3 days, then every 48 hours until healing achieved. |
Perioperative management Hemophilia B |
Minor Circulating Factor IX required (% of normal) = 50-80 IU/dL -Repeat every 24-48 hours as needed, until bleeding stops and healing is achieved. Major Circulating Factor IX required (% of normal) = 60-100 IU/dL (initial level) - Consider repeat dose after 6-10 hours, then every 24 hours for 3 days, then every 48 hours until bleeding stops and healing achieved. |
Routine prophylaxis Hemophilia B |
50 IU/kg once weekly or 100 IU/kg once every 10 days. Adjust dosing regimen based on individual response. |
AlphaNine SD
Indication |
Dose |
Control and prevention of bleeding episodes Hemophilia B |
One unit per kilogram body weight increases the circulating Factor IX level by 1% (IU/dL). Number of Factor IX IU required = body wt (kg) x Desired increase in Plasma Factor IX(percent) x 1.0 IU/kg Minor Circulating Factor IX required (20 – 30 % of normal) = 20-30 IU/kg -Repeat every 12 hours as needed for 1-2 days Moderate Circulating Factor IX required (25 - 50% of normal) = 25-50 IU/kg -Repeat every 12 hours as needed for 2-7 days Major Circulating Factor IX required (50% of normal) = 50-100 IU/kg - Consider repeat dose after 12 hours as needed for 3-5 days. Following this treatment period, FIX levels should be maintained at 20% (20 IU FIX/kg/twice daily) until healing has been achieved. Major hemorrhages may require treatment for up to 10 days |
Routine prophylaxis Hemophilia B § |
25-40 IU/kg two times weekly or 15-30 IU/kg two times weekly. Adjust dosing regimen based on individual response. |
Perioperative management Hemophilia B |
Prior to surgery, FIX should be brought to 50-100% of normal (50-100 IU/kg repeat every 12 hours). For the next 7 to 10 days, or until healing has been achieved, the patient should be maintained at 50-100%FIX levels (50-100 IU/kg every 12 hours). |
BeneFIX
Indication |
Dose |
Control and prevention of bleeding episodes Hemophilia B
And
Perioperative management of Hemophilia B |
Calculating the Initial Dose One unit per kilogram body weight increases the circulating Factor IX level by 1% (IU/dL).
Minor Circulating Factor IX required (% of normal) = 20-30 IU/dL - Repeat every 12-24 hours as needed for 1-2 days Moderate Circulating Factor IX required (% of normal) = 25-50 IU/dL - Repeat every 12-24 hours as needed for 2-7 days Major Circulating Factor IX required (% of normal) = 50-100 IU/dL - Consider repeat dose after 12-24 hours as needed for 7- 10 days. |
Routine prophylaxis Hemophilia B |
For long term prophylaxis against bleeding, the recommended regimen is 100 IU/kg once weekly.
|
Idelvion
Indication |
Dose |
Control and prevention of bleeding episodes |
Minor/Moderate Desired peak Factor IX Level (% of normal or IU/dL): 30-60, dosed every 48-72 hours for at least 1 day until healing is achieved Major Desired peak Factor IX Level (% of normal or IU/dL): 60-100, dosed every 48-72 hours for 7-14 days until bleeding stops. Maintenance dose is weekly. |
Perioperative management Hemophilia B |
Minor Desired peak Factor IX Level (% of normal or IU/dL): 50-80, dosed every 48-72 hours for at least 1 day until healing is achieved Major Desired peak Factor IX Level (% of normal or IU/dL): 60-100, dosed every 48-72 hours for 7-14 days until bleeding stops. Repeat dose every 48-72 hours for the first week or until healing is achieved. Maintenance dose is once or twice weekly. |
Routine prophylaxis Hemophilia B |
Patients ≥12 years of age: 25-40 IU/kg body weight every 7 days. Patients who are well-controlled on this regimen may be switched to a 14-day interval at 50-75 IU/kg body weight. Patients <12 years of age: 40-55 IU/kg body weight every 7 days. |
Ixinity
Indication |
Dose |
Control and prevention of bleeding episodes Congenital Hemophilia B |
One IU per kg body weight increases the circulating activity of factor IX by 0.698 IU/dL Initial dose: Required factor IX units (IU) = body weight (kg) x desired factor IX increase (% of normal of IU/dL) x reciprocal of observed recovery (IU/kg per IU/dL) Maintenance dose: Depends upon the type of bleed or surgery, clinical response, and the severity of the underlying factor IX deficiency Minor Desired peak Factor IX Level (% of normal or IU/dL): 30-60, dosed every 24 hours on days 1-3 until healing is achieved Moderate Desired peak Factor IX Level (% of normal or IU/dL): 40-60, dosed every 24 hours on days 2-7 until healing is achieved Major or Life threatening Desired peak Factor IX Level (% of normal or IU/dL): 60-100, dosed every 12 – 24 hours on days 2-14 until healing is achieved |
Perioperative management Congenital Hemophilia B |
Minor Pre-op: Desired peak Factor IX Level (% of normal or IU/dL) 50-80 Post-op: Desired peak Factor IX Level (% of normal or IU/dL) 30-80, dosed every 24 hours on days 1-5, depending on type of procedure Major Pre-op: Desired peak Factor IX Level (% of normal or IU/dL)60-80 Post-op: Desired peak Factor IX Level (% of normal or IU/dL) 40-60, dosed every 8 – 24 hours on days 1-3, or 30-50 dosed every 8 – 24 hours on days 4-6, or 20-40 dosed every 8 -24 hours on days 7-14 |
Mononine
Indication |
Dose |
Control and prevention of bleeding episodes and perioperative management Hemophilia B |
One unit per kilogram body weight increases the circulating Factor IX level by 1% (IU/dL). Estimate the required dose with the following formula: Number of Factor IX IU required (IU) = Body Weight (in kg) x desired Factor IX increase (% or IU/dL normal) x 1.0 IU/kg [per IU/dL] Minor Spontaneous Hemorrhage Prophylaxis Circulating Factor IX required (% of normal)(15-25%) = up to 20-30IU/kg for one dose. Repeat in 24 hours if necessary. Major Trauma or Surgery Circulating Factor IX required (% of normal)(25-50%) = up to 75 IU/kg Dosed every 18-30 hours depending on T1/2 and measured Factor IX levels. Continue for up to 10 days depending upon nature of insult. |
Profilnine
Indication |
Dose |
Control and prevention of bleeding episodes Hemophilia B |
One unit per kilogram body weight increases the circulating Factor IX level by 1% (IU/dL). Number of Factor IX IU required = body wt (kg) x Desired increase in Plasma Factor IX(percent) x 1.0 IU/kg Mild to Moderate Single dose of product sufficient to raise plasma factor IX levels to 20-30% of normal. 20-30 IU/kg every 16-24 hours until hemorrhage stops and healing is achieved. For minor, may repeat for 1-2 days, for moderate, may repeat for 2-7 days. Major Single dose of product sufficient to raise plasma factor IX levels to 30-50% of normal. Daily infusions up to 3-10 days are generally required. |
Routine prophylaxis Hemophilia B § |
25-40 IU/kg two times weekly or 15-30 IU/kg two times weekly. Adjust dosing regimen based on individual response. |
Perioperative management Hemophilia B |
Surgery associated with bleeding in factor IX deficient patients requires factor IX levels of 30-50%, 30-50 IU/kg every 16-24 hours for 7-10 days until healing is achieved. For dental extractions, the factor IX level should be raised to 50% immediately prior to procedure; additional factor IX complex may be given if bleeding recurs. |
Rebinyn
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia B |
Minor and Moderate 40 IU/kg of actual body weight. A single dose should be sufficient for minor and moderate bleeds. Additional doses of 40 IU/kg can be given. Major 80 IU/kg of actual body weight. Additional doses of 40 IU/kg can be given. |
Perioperative management of bleeding Congenital Hemophilia B |
Minor Pre-op: 40 IU/kg of actual body weight (single pre-op dose should be sufficient) Post-op: Additional doses can be given if required Major Pre-op: 80 IU/kg of actual body weight Peri/Post-op: 40 IU/kg of actual body weight. As clinically needed for the perioperative management of bleeding, repeated doses of 40 IU/kg (in 1-3 day intervals) within the first week after major surgery may be administered. Due to the long half-life the frequency of dosing in the post-surgical setting may be extended to once weekly after the first week until bleeding stops and healing is achieved. |
Rixubis
Indication |
Dose |
Control and prevention of bleeding episodes Hemophilia B |
One IU per kilogram body weight increases the circulating activity of Factor IX by 0.7 IU/dL for patients <12 years of age and 0.9 IU/dL for patients ≥ 12 years of age. Initial dose = body wt (kg) x desired factor IX increase (percent of normal or IU/dL) x reciprocal of observed recovery (IU/kg per IU/dL) Minor Circulating Factor IX level required (% or IU/dL) = 20-30 every 12 - 24 hours for at least 1 day, until healing is achieved Moderate Circulating Factor IX level required (% or IU/dL) = 25-50 every 12 - 24 hours for 2 – 7 days, until bleeding stops and healing is achieved Major Circulating Factor IX level required (% or IU/dL) = 50-100 every 12 - 24 hours for 7 – 10 days, until bleeding stops and healing is achieved |
Routine prophylaxis Hemophilia B |
Dosing for previously treated patients (PTPs): Patients <12 years of age 60 – 80 IU/kg twice weekly Patients ≥ 12 years of age 40 – 60 IU/kg twice weekly Adjust the dose based on the individual patient’s age, bleeding pattern, and physical activity. |
Perioperative management Hemophilia B |
Minor Circulating Factor IX level required (% or IU/dL) = 30-60 every 24 hours for at least 1 day, until healing is achieved Major Circulating Factor IX level required (% or IU/dL) = 80-100 every 8 - 24 hours for 7 – 10 days, until bleeding stops and healing is achieved |
§ Utrecht and/or Malmö protocols used as basis for dosing
Hemophilia products are covered under the prescription drug benefits of a member’s plan. Claims for hemophilia products submitted for payment under any benefit section of the member’s plan (other than prescription drug benefits) will be denied as non-covered benefits. The only exceptions to this are claims for hemophilia products used in an inpatient facility or for emergency use, accidents or surgery (Type Services A, S, or 2) in the following settings:
If home health nursing assistance is needed for drug administration, the hemophilia product should be accessed and paid through the member’s prescription benefit coverage. Nursing services should be billed only for the administration of the hemophilia product under the member’s home health benefits. |
HCPCS code & NDC:
Drug |
Manufacturer |
J-Code |
1 Billable Unit Equiv. |
Vial Size |
NDC |
AlphaNine SD |
Grifols Biologicals Inc |
J7193 |
1 IU |
500 units |
|
1000 units |
|
||||
1500 units |
|
||||
Mononine |
CSL Behring LLC |
J7193 |
1 IU |
500 units |
00053-6232 |
1000 units |
00053-6233 |
||||
Alprolix |
Biogen Idec, Inc |
J7201 |
1 IU |
250 units |
64406-0966 |
500 units |
64406-0911 |
||||
1000 units |
64406-0922 |
||||
2000 units |
64406-0933 |
||||
3000 units |
64406-0944 |
||||
4000 units |
64406-0977 |
||||
Profilnine |
Grifols Biologicals Inc |
J7194 |
1 IU |
500 units |
|
1000 units |
|
||||
1500 units |
|
||||
BeneFIX |
Wyeth Biopharma |
J7195 |
1 IU |
250 units |
58394-0633 |
500 units |
58394-0634 |
||||
1000 units |
58394-0635 |
||||
2000 units |
58394-0636 |
||||
3000 units |
58394-0637 |
||||
Ixinity |
Cangene Corp |
J7195 |
1 IU |
250 units |
70504-0287 |
500 units |
|
||||
1000 units |
|
||||
1500 units |
|
||||
2000 units |
70504-0288 |
||||
3000 units |
70504-0289 |
||||
Rixubis |
Baxalta US Inc |
J7200 |
1 IU |
250 units |
00944-3026 |
500 units |
00944-3028 |
||||
1000 units |
00944-3030 |
||||
2000 units |
00944-3032 |
||||
3000 units |
00944-3034 |
||||
Idelvion |
Novozymes Biopharma A/S |
J7202 |
1 IU |
250 units |
69911-0864 |
500 units |
69911-0865 |
||||
1000 units |
69911-0866 |
||||
2000 units |
69911-0867 |
||||
3500 units |
69911-0869 |
||||
Rebinyn |
Novo Nordisk Inc |
J7203 |
1 IU N/A |
500 units |
00169-7905 |
1000 units |
00169-7901 |
||||
2000 units |
00169-7902 |
- References
- AlphaNine SD [package insert]. Los Angeles, CA; Grifols Biologicals Inc.; June 2020. Accessed June 2020.
- Alprolix [package insert]. Cambridge, MA; Biogen Idec, Inc.; November 2019. Accessed January 2020.
- BeneFIX [package insert]. Philadelphia, PA; Wyeth Biopharma; July 2019. Accessed January 2020.
- Ixinity [package insert]. Winnipeg, Manitoba, Canada. Cangene Corporation; December 2018. Accessed January 2020.
- Mononine [package insert]. Kankakee, IL; CSL Behring LLC; December 2018. Accessed January 2020.
- Profilnine [package insert]. Los Angeles, CA; Grifols Biologicals Inc.; December 2018. Accessed January 2020.
- Rebinyn [package insert]. Plainsboro, NJ; Novo Nordisk Inc.; May 2017. Accessed January 2020.
- Rixubis [package insert]. Westlake Village, CA; Baxalta US Inc.; December 2019; Accessed January 2020.
- Idelvion [package insert]. Bagsvaerd, Denmark; Novozymes Biopharma A/S; October 2019. Accessed January 2020.
- MASAC RECOMMENDATIONS CONCERNING PRODUCTS LICENSED FOR THE TREATMENT OF HEMOPHILIA AND OTHER BLEEDING DISORDERS. 2016 National Hemophilia Foundation. MASAC Document #249; October 2016. Available at: http://www.hemophilia.org. Accessed June 2017.
- Guidelines for the Management of Hemophilia. 2nd Edition. World Federation of Hemophilia. 2013. Available at: https://www1.wfh.org/publication/files/pdf-1472.pdf. Accessed January 2019.
- Annual Review of Factor Replacement Products. Oklahoma Health Care Authority Review Board. Updated April 2016. Accessed January 2019.
- Graham A1, Jaworski K. Pharmacokinetic analysis of anti-hemophilic factor in the obese patient. Haemophilia. 2014 Mar;20(2):226-9.
- Croteau SE1, Neufeld EJ. Transition considerations for extended half-life factor products. Haemophilia. 2015 May;21(3):285-8.
- Mingot-Castellano, et al. Application of Pharmacokinetics Programs in Optimization of Haemostatic Treatment in Severe Hemophilia a Patients: Changes in Consumption, Clinical Outcomes and Quality of Life. Blood. 2014 December; 124 (21).
- MASAC RECOMMENDATION CONCERNING PROPHYLAXIS. 2016 National Hemophilia Foundation. MASAC Document #241; February 2016. Available at: http://www.hemophilia.org. Accessed January 2019.
- First Coast Service Options, Inc. Local Coverage Article: Hemophilia Clotting Factors (A56482). Centers for Medicare & Medicaid Services Inc. Updated on 12/06/2019 with effective date 07/01/2019. Accessed January 2020.
- Palmetto GBA. Local Coverage Article: Billing and Coding: Guidance for Anti-Inhibitor Coagulant Complex (AICC) National Coverage Determination (NCD) 110.3 (A56065). Centers for Medicare & Medicaid Services Inc. Updated on 10/24/2019 with effective date 10/31/2019. Accessed January 2020.
- Novitas Solutions, Inc. Local Coverage Article: Billing and Coding: Hemophilia Factor Products (A56433). Centers for Medicare & Medicaid Services Inc. Updated on 11/08/2019 with effective date 11/14/2019. Accessed January 2020.
Appendix 1 – Covered Diagnosis Codes
ICD-10 |
ICD-10 Description |
D67 |
Hereditary factor IX deficiency |
Appendix 2 – Centers for Medicare and Medicaid Services (CMS)
Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determination (NCD), Local Coverage Articles (LCAs), and Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. They can be found at: http://www.cms.gov/medicare-coverage-database/search/advanced-search.aspx. Additional indications may be covered at the discretion of the health plan.
Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCA/LCD):
Jurisdiction(s): N |
NCD/LCA/LCD Document (s): A56482 |
Jurisdiction(s): J,M |
NCD/LCA/LCD Document (s): A56065 |
Jurisdiction(s): H,L |
NCD/LCA/LCD Document (s): A56433 |
Medicare Part B Administrative Contractor (MAC) Jurisdictions |
||
Jurisdiction |
Applicable State/US Territory |
Contractor |
E (1) |
CA, HI, NV, AS, GU, CNMI |
Noridian Healthcare Solutions, LLC |
F (2 & 3) |
AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ |
Noridian Healthcare Solutions, LLC |
5 |
KS, NE, IA, MO |
Wisconsin Physicians Service Insurance Corp (WPS) |
6 |
MN, WI, IL |
National Government Services, Inc. (NGS) |
H (4 & 7) |
LA, AR, MS, TX, OK, CO, NM |
Novitas Solutions, Inc. |
8 |
MI, IN |
Wisconsin Physicians Service Insurance Corp (WPS) |
N (9) |
FL, PR, VI |
First Coast Service Options, Inc. |
J (10) |
TN, GA, AL |
Palmetto GBA, LLC |
M (11) |
NC, SC, WV, VA (excluding below) |
Palmetto GBA, LLC |
L (12) |
DE, MD, PA, NJ, DC (includes Arlington & Fairfax counties and the city of Alexandria in VA) |
Novitas Solutions, Inc. |
K (13 & 14) |
NY, CT, MA, RI, VT, ME, NH |
National Government Services, Inc. (NGS) |
15 |
KY, OH |
CGS Administrators, LLC |
FACTOR IX_HEMOPHILIA PRODUCTS - Prior Auth Criteria |
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