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Hemophilia Products - Factor VIII Prior Authorization Program Summary
Policy Number: PH-0340
Intravenous
Last Review Date: 06/04/2024
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, 09/2018, 10/2018, 03/2019, 10/2019, 02/2020, 09/2020, 06/2022, 04/2023, 06/2023, 09/2023, 06/2024
- Length of Authorization
Coverage is provided for 3 months and may be renewed thereafter, unless otherwise specified*.
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
- Quantity Limit (max daily dose) [NDC unit]:
- N/A
- Max Units (per dose and over time) [HCPCS Unit]:
- Advate: 64,400 billable units per 28-day supply
- Adynovate: 46,000 billable units per 28-day supply
- Afstyla: 69,000 billable units per 28-day supply
- Eloctate: 74,750 billable units per 30-day supply
- Kogenate: 64,400 billable units per 28-day supply
- Kovaltry: 55,200 billable units per 28-day supply
- Novoeight: 69,000 billable units per 28-day supply
- Nuwiq: 64,400 billable units per 28-day supply
- Hemofil M: 55,200 billable units per 28-day supply
- Koate DVI: 55,200 billable units per 28-day supply
- Recombinate: 64,400 billable units per 28-day supply
- Xyntha/Xyntha Solofuse: 41,400 billable units per 28-day supply
- Obizur: 115,000 billable units per 90-day supply
- Jivi: 41,400 billable units per 30-day supply
- Esperoct: 40,250 billable units per 28-day supply
- Altuviiio: 23,000 billable units per 28-day supply
- Initial Approval Criteria 1-17,22,23
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:
- Advate, Eloctate Ф, Hemofil M, Koate/Koate DVI, Kogenate FS Ф, Novoeight, Recombinate, Xyntha/Xyntha Solofuse Ф, Nuwiq, Adynovate, Kovaltry, Afstyla, Jivi, Esperoct, Altuviiio Ф
Hemophilia A (congenital factor VIII deficiency) †
- Diagnosis of congenital factor VIII deficiency has been confirmed by blood coagulation testing; AND
- If the request is for Jivi, patient must be at least 12 years of age; AND
- Will not be used for the treatment of von Willebrand’s disease; AND
- Used as treatment in at least one of the following:
- On-demand treatment and control of bleeding episodes OR
- Perioperative management (*Authorizations valid for 1 month); OR
- Routine prophylaxis; AND
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- Used to reduce the frequency of bleeding episodes; OR
- Used to reduce the frequency of bleeding episodes and reduce the risk of joint damage in children without pre-existing joint damage (Kogenate-FS ONLY); AND
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- Used as primary prophylaxis in patients with severe factor VIII deficiency (factor VIII level of <1%); OR
- Used as secondary prophylaxis in patients with at least TWO documented episodes of spontaneous bleeding into joints; OR
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- Patient was previously treated with valoctocogene roxaparvovec and factor VIII activity levels decreased and/or bleeding was not controlled
Hemophilia Management Program |
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- Obizur 9
Acquired Hemophilia A (acquired factor VIII deficiency) † Ф
- Patient is at least 18 years of age; AND
- Diagnosis of acquired factor VIII deficiency has been confirmed by blood coagulation testing; AND
- Used as on-demand treatment and control of bleeding episodes; AND
- Is NOT being used for congenital Hemophilia A OR von Willebrand disease; AND
- Patient does not have baseline anti-porcine factor VIII inhibitor titer >20 Bethesda Units (BU)
Hemophilia Management Program |
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† FDA Approved Indication(s); ‡ Compendia Recommended Indication(s); Ф Orphan Drug
- 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:
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- 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.
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- 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 1-17,22,23
Coverage can be renewed based upon the following criteria:
- Patient continues to meet the indication-specific relevant criteria identified in section III; AND
- Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: anaphylaxis and hypersensitivity reactions (e.g., angioedema, chest tightness, dyspnea, wheezing, urticaria, pruritus, hypotension, etc.), thromboembolic events (thromboembolism, pulmonary embolism), development of neutralizing antibodies (inhibitors), etc.; 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
On-demand treatment of bleeding episodes and control of bleeding episodes
- Renewals will be approved for a 6-month authorization period.
Perioperative management of bleeding
- Coverage may NOT be renewed
Routine prophylaxis
- Renewals will be approved for a 12-month authorization period; AND
- Patient has demonstrated a beneficial response to therapy (i.e., the frequency of bleeding episodes has decreased from pre-treatment baseline)
- Dosage/Administration 1-16,22
Advate
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU/kg) = desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor Circulating Factor VIII required (% of normal) (20-40%) = 10-20 IU/ kg - Repeat every 12-24 hours as needed (every 8 to 24 hours for patients underage of 6). Continue until the bleeding episode is resolved (approximately 1 to 3 days). Moderate Circulating Factor VIII required (% of normal) (30-60%) = 15-30 IU/ kg - Repeat every 12-24 hours as needed (every 8 to 24 hours for patients underage of 6). Continue until the bleeding episode is resolved (approximately 3 days or more). Major Circulating Factor VIII required (% of normal) (60-100%) = 30-50 IU/ kg - Repeat every 8-24 hours as needed (every 6 to 12 hours for patients underage of 6). Continue until the bleeding episode is resolved. |
Routine prophylaxis Congenital Hemophilia A |
For prophylaxis regimen to prevent or reduce frequency of bleeding episodes, dose between 20 to 40 IU per kg every other day (3 to 4 times weekly). Alternatively, an every third day dosing regimen targeted to maintain FVIII trough levels ≥ 1% may be employed. Adjust dose based on the patient’s clinical response. |
Perioperative management Congenital Hemophilia A |
Dose (IU/kg) = desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Circulating Factor VIII required (% of normal) (60-100%) = 30-50 IU/ kg – Single dose within one hour of the operation. Repeat after 12- 24 hours for optional additional dosing as needed to control bleeding. Major Circulating Factor VIII required (% of normal) (80-120%) = Preoperative: 40-60 IU/ kg to achieve 100% activity. Followed by a repeat dose every 8-24 hours (every 6 to 24 hours for patients underage of 6) postoperatively until healing is complete. |
Adynovate
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU) = Body Weight (kg) x Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor Target Factor VIII level (IU/dL or % of normal) (20-40%) = 10-20 IU/kg - Repeat every 12-24 hours until the bleeding episode is resolved Moderate Target Factor VIII level (IU/dL or % of normal) (30-60%) = 15-30 IU/kg - Repeat every 12-24 hours until the bleeding episode is resolved Major Target Factor VIII level (IU/dL or % of normal) (60-100%) = 30-50 IU/kg - Repeat every 8-24 hours until the bleeding episode is resolved. |
Perioperative management Congenital Hemophilia A |
Dose (IU) = Body Weight (kg) × Desired factor VIII Rise (IU/dL or % of Normal) × 0.5 (IU/kg per IU/dL) Target Factor VIII required (% of normal) (60-100%) = 30-50 IU/ kg – Single dose within one hour before the operation. Repeat after 24 hours, if necessary, single dose or repeat as needed until bleeding is resolved. Major Target Factor VIII required (% of normal) (80-120%) (pre- and post- operative) = 40-60 IU/ kg within 1 hour before the operation to achieve 100% activity. Repeat dose every 8-24 hours (every 6 to 24 hours for patients under age of 12) to maintain FVIII activity within the target range and continue until adequate wound healing. |
Routine prophylaxis Congenital Hemophilia A |
Administer 40-50 IU per kg body weight 2 times per week in children and adults (12 years and older). Administer 55 IU per kg body weight 2 times per week in children (<12 years) with a maximum of 70 IU per kg. Adjust the dose based on the patient’s clinical response. |
Afstyla
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU) = Body Weight (kg) x Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor Target Factor VIII level (IU/dL or % of normal) 20-40% - Repeat every 12-24 hours until the bleeding episode is resolved. Moderate Target Factor VIII level (IU/dL or % of normal) 30-60% - Repeat every 12-24 hours until the bleeding episode is resolved. Major Target Factor VIII level (IU/dL or % of normal) 60-100% - Repeat every 8-24 hours until the bleeding episode is resolved. |
Perioperative management Congenital Hemophilia A |
Minor Target Factor VIII level (IU/dL or % of normal) 30-60%- Repeat every 24 hours, for at least one day, until healing is achieved. Major Target Factor VIII level (IU/dL or % of normal) 80-100%- Repeat every 8-24 hours until adequate wound healing, then continue for at least another 7 days to maintain a Factor VIII activity of 30-60% (IU/dL). |
Routine prophylaxis Congenital Hemophilia A |
Adults and adolescents (≥12yrs old): Administer 20-50 IU per kg body weight 2 to 3 times per week. Adjust the dose based on the patient’s clinical response. Children (<12 yrs old): Administer 30-50 IU per kg body weight 2 to 3 times per week. Adjust the dose based on the patient’s clinical response. |
Altuviiio
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Minor/Moderate Single dose of 50 IU/kg. For minor and moderate bleeding episodes occurring within 2 to 3 days after a prophylactic dose, a lower dose of 30 IU/kg dose may be used. Additional doses of 30 or 50 IU/kg every 2 to 3 days may be considered. Major Single dose of 50 IU/kg. Additional doses of 30 or 50 IU/kg every 2 to 3 days can be considered. Note: For resumption of prophylaxis (if applicable) after treatment of a bleed, it is recommended to allow an interval of at least 72 hours between the last 50 IU/kg dose for treatment of a bleed and resuming prophylaxis dosing. Thereafter, prophylaxis can be continued as usual on the patient’s regular schedule. |
Perioperative management Congenital Hemophilia A |
Minor Single dose of 50 IU/kg. An additional dose of 30 or 50 IU/kg after 2 to 3 days may be considered. Major Single dose of 50 IU/kg. Additional doses of 30 or 50 IU/kg every 2 to 3 days may be administered as clinically needed for perioperative management. |
Routine prophylaxis Congenital Hemophilia A |
The recommended dosing for routine prophylaxis for adults and children is 50 IU/kg of Altuviiio administered once weekly. |
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Eloctate
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor and Moderate Circulating Factor VIII required (% of normal) (40-60%) = 20-30 IU/ kg - Repeat every 24-48 hours as needed (every 12 to 24 hours for patients under age of 6). Continue until the bleeding episode is resolved. Major Circulating Factor VIII required (% of normal) (80-100%) = 40-50 IU/ kg - Repeat every 12-24 hours as needed (every 8 to 24 hours for patients under age of 6). Continue until the bleeding episode is resolved (approximately 7-10 days). |
Routine prophylaxis Congenital Hemophilia A |
Adults and adolescents ≥ 6: The recommended starting regimen is 50 IU/kg administered every 4 days. The regimen may be adjusted based on patient response with dosing in the range of 25-65 IU/kg at 3–5-day intervals. Children < 6 years of age: The recommended starting regimen is 50 IU/kg administered twice weekly. The regimen may be adjusted based on patient response with dosing in the range of 25-65 IU/kg at 3–5-day intervals. More frequent or higher doses up to 80 IU/kg may be required. |
Perioperative management Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Circulating Factor VIII required (% of normal) (50-80%) = 25-40 IU/ kg - Repeat every 24 hours as needed (every 12 to 24 hours for patients underage of 6). Continue at least 1 day until healing is achieved. Major Circulating Factor VIII required (% of normal) (80-120%) = Preoperative: 40-60 IU/ kg – Followed by a repeat dose of 40-50 IU/kg after 8-24 hours (6 to 24 hours for patients under age of 6). Continue every 24 hours until adequate wound healing; then continue therapy for at least 7 days to maintain FVII activity within the target range. |
Esperoct
Indication |
Dose |
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On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
One IU of Factor VIII activity corresponds to the quantity of Factor VIII in one milliliter of normal human plasma. The calculation of the required dosage of Factor VIII is based on the empirical finding that one IU of Factor VIII per kg body weight raises the plasma Factor VIII activity by two IU/dL. To achieve a specific target Factor VIII activity level, use the following formula: Dosage (IU) = Body Weight (kg) × Desired Factor VIII Increase (IU/dL or % normal) × 0.5 ; OR
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Routine prophylaxis Congenital Hemophilia A |
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Perioperative management Congenital Hemophilia A |
To achieve a specific target Factor VIII activity level, use the following formula: Dosage (IU) = Body Weight (kg) × Desired Factor VIII Increase (IU/dL or % normal) × 0.5 ; OR
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Hemofil M
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Early hemarthrosis or muscle bleed or oral bleed Circulating Factor VIII required (% of normal) (20-40%) = Begin infusion every 12 to 24 hours for one-three days until the bleeding episode as indicated by pain is resolved or healing is achieved. More extensive hemarthrosis, muscle bleed, or hematoma Circulating Factor VIII required (% of normal) (30-60%) = Repeat every 12-24 hours for usually three days or more until pain and disability are resolved. Life threatening bleeds such as head injury, throat bleed, severe abdominal pain Circulating Factor VIII Required (% of normal) (60-100%) = Repeat every 8-24 hours until the bleeding threat is resolved. |
Perioperative management Congenital Hemophilia A |
Minor Circulating Factor VIII required (% of normal) (60-80%) A single infusion plus oral antifibrinolytic therapy within one hour is sufficient in approximately 70% of cases. Major Circulating Factor VIII required (% of normal) (80-100% pre- and post-operative): Repeat dose every 8-24 hours depending on state of healing. |
Jivi
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x reciprocal of expected recovery (or observed recovery, if available) (e.g., 0.5 for a recovery of 2 IU/dL per IU/kg) Minor Circulating Factor VIII required (% of normal) (20-40%) – 10-20IU/kg repeat dose every 24-48 hours until bleed resolves Moderate Circulating Factor VIII required (% of normal) (30-60%) – 15-30IU/kg repeat dose every 24-48 hours until bleed resolves Major Circulating Factor VIII Required (% of normal) (60-100%) – 30-50IU/kg repeat dose every 8-24 hours until bleed resolves |
Perioperative management Congenital Hemophilia A |
Minor Circulating Factor VIII required (% of normal) (30-60%) – 15-30IU/kg repeat dose every 24 hours for at least 1 day until healing is achieved Major Circulating Factor VIII required (% of normal) (80-100%) – 40-50IU/kg repeat dose every 12-24 hours until adequate wound healing is complete, then continue therapy for at least another 7 days to maintain Factor VIII activity of 30–60% (IU/dL) |
Routine prophylaxis Congenital Hemophilia A |
The recommended initial regimen is 30–40 IU/kg twice weekly. Based on the bleeding episodes, the regimen may be adjusted to 45–60 IU/kg every 5 days or may be further individually adjusted to less or more frequent dosing. |
Koate/Koate DVI
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor Circulating Factor VIII required (% of normal) (30%) = 15 IU/kg repeat dose every 12 hours until hemorrhage stops and healing has been achieved. Moderate Circulating Factor VIII required (% of normal) (50%) = 25 IU/kg repeat dose every 12 hours until healing has been achieved. Major Circulating Factor VIII Required (% of normal) (80-100%) = Initial: 40-50 IU/kg. Maintenance dose 25 IU/kg. Repeat every 12 hours for at least 3 – 5 days until healing has been achieved for up to 10 days. |
Routine prophylaxis Hemophilia A § |
25-40 IU/kg three times weekly or 15-30 IU/kg three times weekly. Adjust dosing regimen based on individual response. |
Perioperative management Congenital Hemophilia A |
Prior to surgery Circulating Factor VIII Required (% of normal) (80-100%) = 40-50 IU/kg for one dose prior to surgery. After surgery Circulating Factor VIII Required (% of normal) (60-100%) = 30-50 IU/kg repeat dose every 12 hours for the next 7 – 10 days or until healing has been achieved. |
Kogenate FS
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor Circulating Factor VIII required (% of normal) (20-40%) = 10-20 IU/ kg - Repeat dose if there is evidence of further bleeding and continue until the bleeding episode is resolved. Moderate Circulating Factor VIII required (% of normal) (30-60%) = 15-30 IU/ kg - Repeat every 12-24 hours as needed. Continue until the bleeding episode is resolved. Major Circulating Factor VIII Required (% of normal) (80-100%) = Initial: 40-50 IU/ kg; Repeat 20-25 IU/kg every 8-12 hours until the bleeding episode is resolved. |
Routine prophylaxis Congenital Hemophilia A |
Routine Prophylaxis in Adults 25 units per kg of body weight three times per week. Routine Prophylaxis in Children 25 IU/kg of body weight every other day. |
Perioperative management Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor Circulating Factor VIII required (% of normal) (30-60%) = 15-30 IU/ kg – Repeat every 12- 24 hours until bleeding is resolved. Major Circulating Factor VIII required (% of normal) (100%) = Preoperative: 50 IU/ kg to achieve 100% activity. Followed by a repeat dose every 6-12 hours to keep FVIII activity in desired range. Continue until healing is complete. |
Kovaltry
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Minor (Early hemarthrosis, minor muscle, oral bleeds) Factor VIII level required (IU/dL or % of normal): 20-40 – repeat every 12-24 hours for at least 1 day, until bleeding episode as indicated by pain is resolved or healing is achieved. Moderate (More extensive hemarthrosis, muscle bleeding, or hematoma) Factor VIII level required (IU/dL or % of normal): 30-60 – repeat every 12-24 hours for 3 to 4 days or more until pain and acute disability are resolved. Major (Intracranial, intra-abdominal or intrathoracic hemorrhages, gastrointestinal bleeding, central nervous system bleeding, bleeding in the retropharyngeal or retroperitoneal spaces, or iliopsoas sheath, life or limb threatening hemorrhage) Factor VIII level required (IU/dL or % of normal): 60-100 – repeat every 8-24 hours until bleeding is resolved. |
Routine prophylaxis Congenital Hemophilia A |
Individualize the patient’s dose based on clinical response:
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Perioperative management Congenital Hemophilia A |
Minor (Such as tooth extraction) Factor VIII level required (IU/dL or % of normal): 30-60 (pre- and post-operative) – repeat every 24 hours for at least 1 day until healing is achieved. Major (Such as intracranial, intraabdominal, intrathoracic, or joint replacement surgery) Factor VIII level required (IU/dL or % of normal): 80-100 (pre- and post-operative) – repeat every 8-24 hours until adequate wound healing is complete, then continue therapy for at least another 7 days to maintain Factor VIII activity of 30-60% (IU/dL). |
Novoeight
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor Circulating Factor VIII required (% of normal) (20-40%), every 12 – 24 hours for at least 1 day until the bleeding episode is resolved Moderate Circulating Factor VIII required (% of normal) (30-60%), every 12 – 24 hours until pain and acute disability are resolved, approximately 3-4 days Major Circulating Factor VIII Required (% of normal) (60-100%), every 8 – 24 hours until resolution of bleed, approximately 7-10 days. |
Perioperative management Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor Circulating Factor VIII required (% of normal) (30-60%) every 24 hours for at least 1 day until healing is achieved. Major Circulating Factor VIII required (% of normal) (80-100%) every 8 – 24 hours until adequate wound healing, then continue therapy for at least 7 days to maintain a factor VIII activity of 30 – 60% (IU/dL) |
Routine prophylaxis Hemophilia A |
Adults and adolescents (≥12 yrs): 25-50 IU/kg every other day |
NUWIQ
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose Required IU = body weight (kg) x desired Factor VIII rise (%) (IU/dL) x 0.5 (IU/kg per IU/dL) Expected Factor VIII rise (% of normal) = 2 x administered IU/body weight (kg) Minor Required peak post-infusion Factor VIII activity (% of normal or IU/dL): 20-40 every 12 – 24 hours for at least 1 day, until the bleeding episode is resolved Moderate to Major Required peak post-infusion Factor VIII activity (% of normal or IU/dL): 30-60 every 12 – 24 hours for 3-4 days or more until the bleeding episode is resolved Life-threatening Required peak post-infusion Factor VIII activity (% of normal or IU/dL): 60-100 every 8 – 24 hours bleeding risk is resolved |
Routine prophylaxis Congenital Hemophilia A |
Dose Required IU = body weight (kg) x desired Factor VIII rise (%) (IU/dL) x 0.5 (IU/kg per IU/dL) Expected Factor VIII rise (% of normal) = 2 x administered IU/body weight (kg) Adolescents (12-17 years) and adults 30 – 40 IU/kg every other day Children (2-11 years) 30 – 50 IU/kg every other day or three times per week |
Perioperative management Congenital Hemophilia A |
Dose Required IU = body weight (kg) x desired Factor VIII rise (%) (IU/dL) x 0.5 (IU/kg per IU/dL) Expected Factor VIII rise (% of normal) = 2 x administered IU/body weight (kg) Minor Required peak post-infusion Factor VIII activity (% of normal or IU/dL): 30-60 (pre- and post-operative) every 24 hours for at least 1 day until healing is achieved Major Required peak post-infusion Factor VIII activity (% of normal or IU/dL): 80-100 (pre- and post-operative) every 8 - 24 hours until adequate wound healing, then continue therapy for at least another 7 days to maintain Factor VIII activity of 30% to 60% (IU/dL) |
Obizur
Indication |
Dose |
On-demand treatment and control of bleeding episodes Acquired Hemophilia A |
Minor and Moderate Loading dose: 200IU/kg; Maintenance dose: Titrate to maintain recommended FVIII trough levels at 50-100 IU/dL every 4 to 12 hours Major Loading dose: 200 IU/kg; Maintenance dose: Titrate to maintain recommended FVIII trough levels at 100-200 (to treat an acute bleed), then 50-100 IU/dL (after acute bleed is controlled) every 4 to 12 hours |
Recombinate
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Early hemarthrosis or muscle bleed or oral bleed Circulating Factor VIII required (% of normal) (20-40%) - Begin infusion every 12 to 24 hours for one-three days until the bleeding episode as indicated by pain is resolved or healing is achieved. More extensive hemarthrosis, muscle bleed, or hematoma Circulating Factor VIII required (% of normal) (30-60%) - Repeat every 12-24 hours for usually three days or more until pain and disability are resolved. Life threatening bleeds such as head injury, throat bleed, severe abdominal pain Circulating Factor VIII Required (% of normal) (60-100%) - Repeat every 8-24 hours until the bleeding threat is resolved. |
Routine prophylaxis Hemophilia A § |
25-40 IU/kg three times weekly or 15-30 IU/kg three times weekly. Adjust dosing regimen based on individual response. |
Perioperative management Congenital Hemophilia A |
Minor Circulating Factor VIII required (% of normal) (60-80%) - A single infusion plus oral antifibrinolytic therapy within one hour is sufficient in approximately 70% of cases. Major Circulating Factor VIII required (% of normal) (80-100% pre- and post-operative) - Repeat dose every 8-24 hours depending on state of healing. |
Xyntha/Xyntha Solofuse
Indication |
Dose |
On-demand treatment and control of bleeding episodes Congenital Hemophilia A |
Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL) Minor Circulating Factor VIII required (% of normal) (20-40%) - Repeat dose every 12- 24 hours for least 1 day, depending upon the severity of the bleeding episode. Moderate Circulating Factor VIII required (% of normal) (30-60%) - Repeat every 12-24 hours as needed. Continue for 3-4 days or until adequate local hemostasis is achieved. Major Circulating Factor VIII Required (% of normal) (60-100%) - Repeat every 8-24 hours until bleeding is resolved. |
Perioperative management Congenital Hemophilia A |
Minor Circulating Factor VIII required (% of normal) (30-60%) - Repeat every 12- 24 hours. Continue for 3-4 days or until adequate local hemostasis is achieved. For tooth extraction, a single infusion plus oral antifibrinolytic therapy within 1 hour may be sufficient. Major Circulating Factor VIII required (% of normal) (60-100%) - Repeat every 8-24 hours. Continue until threat is resolved, or in the case of surgery, until adequate local hemostasis and wound healing are achieved. |
Routine prophylaxis Hemophilia A |
Adults and adolescents (≥12 years): The recommended starting regimen is 30 IU/kg of Xyntha administered 3 times weekly. Children (<12 years): The recommended starting regimen is 25 IU/kg of Xyntha administered every other day. More frequent or higher doses may be required in children <12 years of age to account for the higher clearance in this age group. Note: Adjust the dosing regimen (dose or frequency) based on the patient’s clinical response. |
§ Utrecht and/or Malmö protocols used as basis for dosing
- Billing Code/Availability Information
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 |
HCPCS Codes |
1 Billable Unit Equiv. |
Vial Size |
NDC |
Advate |
Baxalta US Inc |
J7192 |
1 IU |
250 units |
00944-3051-02 |
500 units |
00944-3052-02 |
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1000 units |
00944-3053-02 |
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1500 units |
00944-3054-02 |
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2000 units |
00944-3045-10 |
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3000 units |
00944-3046-10 |
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4000 units |
00944-3047-10 |
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Kogenate FS |
Bayer HealthCare LLC |
J7192 |
1 IU |
250 units |
00026-3782-25 |
500 units |
00026-3783-35 |
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1000 units |
00026-3785-55 |
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2000 units |
00026-3786-65 |
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3000 units |
00026-3787-75 |
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Recombinate |
Baxalta US Inc. |
J7192 |
1 IU |
220-400 units |
00944-2841-10 |
401-800 units |
00944-2842-10 |
||||
801-1240 units |
00944-2843-10 |
||||
1241-1800 units |
00944-2844-10 |
||||
1801-2400 units |
00944-2845-10 |
||||
Kovaltry |
Bayer HealthCare LLC |
J7211 |
1 IU |
250 units |
00026-3821-25 |
500 units |
00026-3822-25 |
||||
1000 units |
00026-3824-25 |
||||
2000 units |
00026-3826-50 |
||||
3000 units |
00026-3828-50 |
||||
Eloctate |
Bioverativ Therapeutics Inc. |
J7205 |
1 IU |
250 units |
71104-0801-01 |
500 units |
71104-0802-01 |
||||
750 units |
71104-0803-01 |
||||
1000 units |
71104-0804-01 |
||||
1500 units |
71104-0805-01 |
||||
2000 units |
71104-0806-01 |
||||
3000 units |
71104-0807-01 |
||||
4000 units |
71104-0808-01 |
||||
5000 units |
71104-0809-01 |
||||
6000 units |
71104-0810-01 |
||||
Koate/Koate DVI |
Grifols Therapeutics Inc |
J7190 |
1 IU |
250 units |
76125-0250-20 76125-0253-25 76125-0256-20 76125-0257-25 76125-0258-02 76125-0259-02 |
500 units |
76125-0661-02 76125-0662-50 76125-0663-50 76125-0665-02 76125-0667-30 76125-0668-30 |
||||
1000 units |
76125-0672-50 76125-0674-10 76125-0675-12 76125-0676-50 76125-0678-10 76125-0679-12 |
||||
Hemofil M |
Takeda Pharmaceuticals USA, Inc. |
J7190 |
1 IU |
250 units |
00944-3940-02 |
500 units |
00944-3942-02 |
||||
1000 units |
00944-3944-02 |
||||
1700 units |
00944-3946-02 |
||||
Novoeight |
Novo Nordisk Inc. |
J7182 |
1 IU |
250 units |
00169-7825-01 |
500 units |
00169-7850-01 |
||||
1000 units |
00169-7810-01 |
||||
1500 units |
00169-7815-01 |
||||
2000 units |
00169-7820-01 |
||||
3000 units |
00169-7830-01 |
||||
Nuwiq |
Octapharma AB |
J7209 |
1 IU |
250 units |
68982-0140-01 |
500 units |
68982-0142-01 |
||||
1000 units |
68982-0144-01 |
||||
1500 units |
68982-0154-01 |
||||
2000 units |
68982-0146-01 |
||||
2500 units |
68982-0148-01 |
||||
3000 units |
68982-0150-01 |
||||
4000 units |
68982-0152-01 |
||||
Obizur |
Baxalta US Inc. |
J7188 |
1 IU |
500 units |
00944-5001-xx |
Xyntha/Xyntha Solofuse |
Wyeth Pharmaceuticals LLC |
J7185 |
1 IU |
250 units |
58394-0012-01 58394-0022-03 |
500 units |
58394-0013-01 58394-0023-03 |
||||
1000 units |
58394-0014-01 58394-0024-03 |
||||
2000 units |
58394-0015-01 58394-0025-03 |
||||
3000 units |
58394-0016-03 |
||||
Afstyla |
CSL Behring, LLC |
J7210 |
1 IU |
250 units |
69911-0474-02 |
500 units |
69911-0475-02 |
||||
1000 units |
69911-0476-02 |
||||
1500 units |
69911-0480-02 |
||||
2000 units |
69911-0477-02 |
||||
2500 units |
69911-0481-02 |
||||
3000 units |
69911-0478-02 |
||||
Adynovate |
Baxalta US Inc. |
J7207 |
1 IU |
250 units |
00944-4622-01 |
500 units |
00944-4623-01 |
||||
750 units |
00944-4626-01 |
||||
1000 units |
00944-4624-01 |
||||
1500 units |
00944-4627-01 |
||||
2000 units |
00944-4625-01 |
||||
3000 units |
00944-4628-01 |
||||
Jivi |
Bayer HealthCare LLC |
J7208 |
1 IU |
500 units |
00026-3942-25 |
1000 units |
00026-3944-25 |
||||
2000 units |
00026-3946-25 |
||||
3000 units |
00026-3948-25 |
||||
Esperoct |
Novo Nordisk Inc. |
J7204 |
1 IU |
500 units |
00169-8500-01 |
1000 units |
00169-8100-01 |
||||
1500 units |
00169-8150-01 |
||||
2000 units |
00169-8200-01 |
||||
3000 units |
00169-8300-01 |
||||
Altuviiio |
Bioverativ Therapeutics Inc. |
J7214 |
1 IU |
250 units |
71104-0978-01 |
500 units |
71104-0979-01 |
||||
1000 units |
71104-0981-01 |
||||
2000 units |
71104-0982-01 |
||||
3000 units |
71104-0983-01 |
||||
4000 units |
71104-0984-01 |
- References
- Advate [package insert]. Lexington, MA; Baxalta US Inc. March 2023. Accessed May 2024.
- Afstyla [package insert]. Kankakee, IL; CSL Behring, LLC; June 2023. Accessed May 2024.
- Eloctate [package insert]. Waltham, MA; Bioverativ Therapeutics Inc.; May 2023. Accessed May 2024.
- Hemofil M [package insert]. Lexington, MA; Takeda Pharmaceuticals USA, Inc. March 2023. Accessed May 2024.
- Koate/Koate DVI [package insert]. Research Triangle Park, NC; Grifols Therapeutics Inc.; January 2022. Accessed May 2024.
- Kogenate FS [package insert]. Whippany, NJ. Bayer HealthCare LLC; December 2019. Accessed May 2024.
- Novoeight [package insert]. Bagsvaerd, Denmark; Novo Nordisk Inc.; July 2020. Accessed May 2024.
- NUWIQ [package insert]. Elersvagen, Sweden; Octapharma AB; June 2021. Accessed May 2024.
- Obizur [package insert]. Lexington, MA; Baxalta US Inc. March 2023. Accessed May 2024.
- Recombinate [package insert]. Lexington, MA; Baxalta US Inc. March 2023. Accessed May 2024.
- Xyntha & Xyntha Solofuse [package insert]. Philadelphia, PA; Wyeth Pharmaceuticals LLC; July 2022. Accessed May 2024.
- Adynovate [package insert]. Lexington, MA; Baxalta US Inc.; August 2023. Accessed May 2024.
- Kovaltry [package insert]. Whippany, NJ; Bayer HealthCare LLC; December 2022. Accessed May 2024.
- Jivi [package insert]. Whippany, NJ; Bayer HealthCare LLC; August 2018. Accessed May 2024.
- Altuviiio [package insert]. Waltham, MA; Bioverativ Therapeutics Inc.; May 2024. Accessed May 2024.
- MASAC Recommendations Concerning Products Licensed for the Treatment of Hemophilia and Selected Disorders of the Coagulation System. Revised April 11, 2024. National Hemophilia Foundation. MASAC Document #284; August 2020. Available at: http://www.bleeding.org. Accessed May 2024.
- Guidelines for the Management of Hemophilia. 3rd Edition. World Federation of Hemophilia. 2020. Available at: https://www1.wfh.org/publications/files/pdf-1863.pdf. Accessed May 2024.
- Annual Review of Factor Replacement Products. Oklahoma Health Care Authority Review Board. Updated December 2020. Accessed May 2024.
- 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 for Hemophilia A and B with and without Inhibitors. Revised April 27, 2022. National Hemophilia Foundation. MASAC Document #267; April 2022. Available at: http://www.bleeding.org. Accessed May 2024.
- Esperoct [package insert]. Plainsboro, NJ; Novo Nordisk Inc.; February 2024. Accessed May 2024.
- First Coast Service Options, Inc. Local Coverage Article: Billing and Coding: Hemophilia Factor Products (A56482). Centers for Medicare & Medicaid Services Inc. Updated on 09/29/2023 with effective date 10/01/2023. Accessed May 2024.
- 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 11/14/2022 with effective date 11/24/2022. Accessed May 2024.
- Novitas Solutions, Inc. Local Coverage Article: Billing and Coding: Hemophilia Factor Products (A56433). Centers for Medicare & Medicaid Services Inc. Updated on 09/29/2023 with effective date 10/01/2023. Accessed May 2024.
Appendix 1 – Covered Diagnosis Codes
Obizur
ICD-10 |
ICD-10 Description |
D68.311 |
Acquired hemophilia |
Advate, Eloctate, Esperoct, Hemofil M, Koate-DVI, Kogenate FS, Recombinate, Xyntha/ Xyntha Solofuse, Novoeight. NUWIQ, Adynovate, Kovaltry, Afstyla, Jivi, and Altuviiio
ICD-10 |
ICD-10 Description |
D66 |
Hereditary factor VIII deficiency |
Appendix 2 – Centers for Medicare and Medicaid Services (CMS)
The preceding information is intended for non-Medicare coverage determinations. 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 Determinations (NCDs) and/or Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. Local Coverage Articles (LCAs) may also exist for claims payment purposes or to clarify benefit eligibility under Part B for drugs which may be self-administered. The following link may be used to search for NCD, LCD, or LCA documents: https://www.cms.gov/medicare-coverage-database/search.aspx. Additional indications, including any preceding information, may be applied at the discretion of the health plan.
Medicare Part B Covered Diagnosis Codes |
||
Jurisdiction |
NCD/LCA/LCD Document (s) |
Contractor |
N |
A56482 |
First Coast Service Options, Inc. |
J, M |
A56065 |
Palmetto GBA |
H, L |
A56433 |
Novitas Solutions, Inc. |
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 |
M (11) |
NC, SC, WV, VA (excluding below) |
Palmetto GBA |
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 VIII_HEMOPHILIA PRODUCTS - Prior Auth Criteria |
|