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Hemophilia Products - Factor VIII Prior Authorization Program Summary

Policy Number: PH-00340

Esperoct (Intravenous)

Document Number: IC-0340

Last Review Date: 09/01/2020

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, 09/2018, 10/2018, 03/2019, 10/2019, 02/2020, 09/2020

  1. 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

  1. Dosing Limits
  1. Quantity Limit (max daily dose) [NDC unit]:

N/A

  1. Max Units (per dose and over time) [HCPCS Unit]:
  • Advate: 73,600 billable units per 28 day supply
  • Adynovate: 36,800 billable units per 28 day supply  
  • Afstyla: 69,000 billable units per 28 day supply
  • Eloctate: 40,250 billable units per 30 day supply
  • Kogenate: 43,125 billable units per 30 day supply
  • Kovaltry: 86,250 billable units per 30 day supply
  • Novoeight: 82,800 billable units per 28 day supply
  • Nuwiq: 86,250 billable units per 30 day supply
  • Hemofil M: 55,200 billable units per 28 day supply
  • Koate DVI: 55,200 billable units per 28 day supply
  • Recombinate: 55,200 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 units per 28 days
  1. Initial Approval Criteria 1-14,15,16,21

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

  1. Advate, Eloctate, Hemofil M, Koate-DVI, Kogenate FS, Novoeight, Recombinate, Xyntha/ Xyntha Solofuse, Nuwiq, Adynovate, Kovaltry, Afstyla, Jivi, Esperoct

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 12 years of age and older; AND
  • Will not be used for the treatment of von Willebrand’s disease; AND
  • 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
  • Used for routine prophylaxis: AND
      • Used to prevent or reduce the frequency of bleeding episodes; OR
      • Used to prevent or reduce the frequency of bleeding episodes and reduce the risk of joint damage in children without pre-existing joint damage (Kogenate-FS ONLY); AND
              • Patient must have severe hemophilia A (factor VIII level of <1%); OR
              • Patient has at least two documented episodes of spontaneous bleeding into joints.

Hemophilia Management Program
  • If the request is for routine prophylaxis and the requested dose exceeds dosing limits under part II or if member BMI≥ 30, a half-life study should be performed to determine the appropriate dose and dosing interval.
  • If the request is for Eloctate, Adynovate, Jivi, or Esperoct, the following criteria should be met:
    • Patient is not a suitable candidate for a standard non- EHL factor VIII product.
    • A half-life study must be scheduled to determine the appropriate dose and dosing interval of the EHL product when initiated.
    • Prior to switching to Eloctate, Adynovate, Jivi, or Esperoct a half-life study should also be performed on current non- EHL factor VIII product to ensure that a clinical benefit will be achieved.
    • If the request exceeds any of the following dosing limits, documentation must be submitted specifying why the member is not a suitable candidate for Hemlibra and alternative EHL factor VIII products.
      • 50 IU/kg every 4 days (total weekly dose of 87.5 IU/kg) for Eloctate
      • 40 IU/kg twice weekly (total weekly dose of 80 IU/kg) for Adynovate
      • 60 IU/kg every 5 days (total weekly dose of 84 IU/kg) for Jivi
      • 50 IU/kg every 4 days (total weekly dose of 87.5 IU/kg) for Esperoct
  • For minimally treated patients (< 50 exposure days to factor products) previously receiving a different factor product, inhibitor testing is required at baseline, then at every comprehensive care visit (yearly for the mild and moderate patients, semi-annually for the severe patients)
  1. Obizur 10

Acquired Hemophilia A (acquired factor VIII deficiency) †

  • Diagnosis of acquired factor VIII deficiency has been confirmed by blood coagulation testing; AND
  • Used as treatment of bleeding episodes; AND
  • Is NOT being used for congenital Hemophilia A OR von Willebrand disease

Hemophilia Management Program
  • For members with a BMI ≥ 30, a half-life study should be performed to determine the appropriate dose and dosing interval.
  • For minimally treated patients (< 50 exposure days to factor products) previously receiving a different factor product, inhibitor testing is required at baseline, then at every comprehensive care visit (yearly for the mild and moderate patients, semi-annually for the severe patients)

† FDA Approved Indication(s)

  1. 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.
  1. Renewal Criteria 1-14,15,16,21

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
  1. Dosage/Administration1-16

Advate

Indication

Dose

Control and prevention of bleeding 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 under age of 6).  Continue until the bleeding episode is resolved (as indicated by relief of pain) or healing is achieved (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 under age of 6).  Continue until the bleeding episode is resolved (as indicated by relief of pain) or healing is achieved (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 under age 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.

Perioperative management Congenital Hemophilia A

Minor

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 under age of 6).  Duration of  therapy depends on the desired level of FVIII

Adynovate

Indication

Dose

Control and prevention of bleeding 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

Minor

Target Factor VIII required (% of normal) (60-100%) = 30-50 IU/ kg –Single dose within one hour of 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 of the operation to achieve 100% activity. Repeat dose every 8-24 hours (every 6 to 24 hours for patients under age of 12).  Duration of therapy 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

Treatment and control of bleeding 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 the bleeding episode is resolved

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.

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.

Eloctate

Indication

Dose

Control and prevention of bleeding 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

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. More frequent or higher doses up to 80 IU/kg may be required in children less than 6 years of age.

Perioperative management Congenital Hemophilia A

Minor

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 under age 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 (every 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 to maintain FVII activity within the target range.

Esperoct

Indication

Dose

Control and prevention of bleeding 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

Type of bleeding

Adolescents/Adults

≥12 years

Dose (IU/kg)

Children

<12 years

Dose (IU/kg)

Additional doses

Minor

Early hemarthrosis, mild muscle bleeding, or oral bleeding

40

65

One dose should be sufficient

Moderate

More extensive hemarthrosis, muscle bleeding, or hematoma

40

65

An additional dose may be administered after 24 hours

Major

Life- or limb-threatening hemorrhages, gastro- intestinal bleeding, intracranial, intra-abdominal or intrathoracic bleeding, fractures

50

65

Additional dose(s) may be administered approximately every 24 hours

Routine Prophylaxis Congenital Hemophilia A
  • Adults and adolescents (≥ 12 years): The recommended starting dose is 50 IU per kg body weight every 4 days. This regimen may be individually adjusted to less or more frequent dosing based on bleeding episodes.
  • Children (< 12 years): A dose of 65 IU per kg body weight twice weekly. This regimen may be individually adjusted to less or more frequent dosing based on bleeding episodes.

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

Type of surgery

Adolescents/Adults

≥12 years

Dose (IU/kg)

Children

<12 years

Dose (IU/kg)

Additional doses

Minor

Including tooth extraction

50

65

Additional dose(s) can be given  after 24 hours if necessary

Major

Intracranial, intra-abdominal, intrathoracic, or joint replacement surgery

50

65

Additional doses can be given  every 24 hours for the first week and then approximately every 48 hours until wound healing has occurred

Hemofil M

Indication

Dose

Control and prevention of bleeding 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

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.

Koate DVI

Indication

Dose

Control and prevention of bleeding Congenital Hemophilia A

Dose (IU/kg) = Desired factor VIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL)

Mild

Circulating Factor VIII required (% of normal) (20%) = 10 IU/kg- Therapy need not be repeated unless there is evidence of further bleeding.

Moderate

Circulating Factor VIII required (% of normal) (30-50%) = 15-25 IU/kg - If further therapy is required, repeated doses of 10-15 IU per kg every 8-12 hours may be given.

Severe

Circulating Factor VIII Required (% of normal) (80-100%) =40-50 IU/kg – followed by a maintenance dose of 20-25 IU per kg every 8-12 hours.

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

For major surgical procedures, the Factor VIII level should be raised to approximately 100% by giving a preoperative dose of 50 IU/kg.  The Factor VIII level should be checked to assure that the expected level is achieved before the patient goes to surgery. In order to maintain hemostatic levels, repeat infusions may be necessary every 6 to 12 hours initially, and for a total of 10 to 14 days until healing is complete. The intensity of Factor VIII replacement therapy required depends on the type of surgery and postoperative regimen employed.  For minor surgical procedures, less intensive treatment schedules may provide adequate hemostasis.

Kogenate FS

Indication

Dose

Control and prevention of bleeding 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

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

Control and prevention of bleeding Congenital Hemophilia A

  • Required dose (IU) = body weight (kg) x desired Factor VIII rise (% of normal or IU/dL) x reciprocal of expected/observed recovery (e.g., 0.5 for a recovery of 2 IU/dL per IU/kg)
  • Estimated Increment of Factor VIII (IU/dL or % of normal) = [Total Dose (IU)/body weight (kg)] x 2 (IU/dL per IU/kg)

Minor

(Early hemarthrosis, minor muscle, oral bleeds)

Factor VIII level required (IU/dL or % of normal): 20-40 – repeat every 12-24 hours 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:

  • Adults and adolescents: 20 to 40 IU of KOVALTRY per kg of body weight two or three times per week.
  • Children ≤12 years old: 25 to 50 IU of KOVALTRY per kg body weight twice weekly, three times weekly, or every other day according to individual requirements

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 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 – 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

Control and prevention of bleeding 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%), at least

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)

Prophylaxis to prevent or reduce the frequency of bleeding episodes

Hemophilia A

Adults and adolescents (>12 yrs):
20-50 IU/kg three times weekly OR
20-40 IU/kg every other day
Children (<12 yrs):
25-60 IU/kg three times weekly OR

25-50 IU/kg every other day

NUWIQ

Indication

Dose

Control and prevention of bleeding 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

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(acute), then 50-100 IU/dL (after acute bleed is controlled) every 4 to 12 hours

Recombinate

Indication

Dose

Control and prevention of bleeding 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

Control and prevention of bleeding 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

  1. 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:

  • Outpatient Facility
  • Physician office

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

Advate

Baxalta US Inc

J7192

1 IU

250 units

00944-3051-02

500 units

00944-3052-02

1000 units

00944-3053-02

1500 units

00944-3054-02

2000 units

00944-3045-10

3000 units

00944-3046-10

4000 units

0944-3047-10

Kogenate FS

Bayer HealthCare LLC

J7192

1 IU

250 units

00026-3782-25

500 units

00026-3783-35

1000 units

00026-3785-55

2000 units

00026-3786-65

3000 units

00026-3787-75

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

Biogen Idec Inc

J7205

1 IU

250 units

64406-0801-01

500 units

64406-0802-01

750 units

64406-0803-01

1000 units

64406-0804-01

1500 units

64406-0805-01

2000 units

64406-0806-01

3000 units

64406-0807-01

4000 units

64406-0808-01

5000 units

64406-0809-01

6000 units

64406-0810-01

Koate-DVI

Grifols Therapeutics Inc

J7190

1 IU

250 units

76125-0250-20

76125-0253-25

500 units

76125-0667-30 76125-0662-50

1000 units

76125-0672-50

76125-0674-10

Hemofil M

Baxalta US Inc

J7190

1 IU

250 units

00944-3940-02

500 units

00944-3942-02

1700 units

00944-3946-02

1000 units

00944-3944-02

Novoeight

Novo Nordisk

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

2000 units

68982-0146-01

Obizur

Baxter Healthcare

J7188

1 IU

500 units

00944-5001-xx

Xyntha/Xyntha Solofuse

Wyeth Biopharma

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

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

J7199, J7204 (effective 7/1/2020)

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
  1. References
  1. Advate [package insert].  Westlake Village, CA; Baxalta US Inc. December 2018. Accessed January 2020.
  2. Afstyla [package insert]. Kankakee, IL; CSL Behring, LLC; September 2017. Accessed January 2020.
  3. Eloctate [package insert]. Cambridge, MA; Biogen Idec Inc.; September 2019. Accessed January 2020.
  4. Hemofil M [package insert]. Westlake Village, CA; Baxalta US Inc. June 2018. Accessed January 2020.
  5. Koate DVI [package insert]. Research Triangle Park, NC; Grifols Therapeutics Inc.; August 2012.  Accessed January 2020.
  6. Kogenate FS [package insert].  Whippany, NJ. Bayer HealthCare LLC; May 2016. Accessed January 2020.
  7. Novoeight [package insert]. Bagsvaerd, Denmark; Novo Nordisk; November 2018.  Accessed January 2020.
  8. NUWIQ [package insert]. Elersvagen, Sweden; Octapharma AB; July 2017. Accessed January 2020.
  9. Obizur [package insert].  Westlake Village, CA; Baxter Healthcare. December 2019. Accessed January 2020.
  10. Recombinate [package insert].  Westlake Village, CA; Baxalta US Inc. June 2018. Accessed January 2020.
  11. Xyntha & Xyntha Solofuse [package insert].  Philadelphia, PA; Wyeth Biopharma; August 2020. Accessed August 2020.
  12. Adynovate [package insert]. Westlake Village, CA; Baxalta US Inc.; May 2018. Accessed January 2020.
  13. Kovaltry [package insert]. Whippany, NJ; Bayer HealthCare LLC; March 2016. Accessed January 2020.
  14. Jivi [package insert]. Whippany, NJ; Bayer HealthCare LLC; Augusts 2018. Accessed January 2020.
  15. 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 January 2019.
  16. 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.
  17. Annual Review of Factor Replacement Products. Oklahoma Health Care Authority Review Board. Updated April 2016. Access January 2019.
  18. Graham A1, Jaworski K. Pharmacokinetic analysis of anti-hemophilic factor in the obese patient. Haemophilia. 2014 Mar;20(2):226-9.
  19. Croteau SE1, Neufeld EJ. Transition considerations for extended half-life factor products. Haemophilia. 2015 May;21(3):285-8.
  20. 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).
  21. MASAC RECOMMENDATION CONCERNING PROPHYLAXIS. 2016 National Hemophilia Foundation.  MASAC Document #241; February 2016.  Available at: http://www.hemophilia.org.   Accessed January 2019.
  22. 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.
  23. 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.
  24. 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

Obizur

ICD-10

ICD-10 Description

D68.311

Acquired hemophilia

Advate, Eloctate, Hemofil M, Koate-DVI, Kogenate FS, Recombinate, Xyntha/ Xyntha Solofuse, Novoeight. NUWIQ, Adynovate, Kovaltry, Afstyla, and Jivi

ICD-10

ICD-10 Description

D66

Hereditary factor VIII 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) 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/LCD):

Jurisdiction(s): N

NCD/LCD Document (s): A56482

https://www.cms.gov/medicare-coverage-database/search/article-date-search.aspx?DocID=A56482&bc=gAAAAAAAAAAA

Jurisdiction(s): J,M

NCD/LCD Document (s): A56065

https://www.cms.gov/medicare-coverage-database/search/article-date-search.aspx?DocID=A56065&bc=gAAAAAAAAAAA

Jurisdiction(s): H,L

NCD/LCD Document (s): A56433

https://www.cms.gov/medicare-coverage-database/search/article-date-search.aspx?DocID=A56433&bc=gAAAAAAAAAAA

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