Subject:
Hemophilia Products – Factor VIII (Hemophilia A, Congenital and Acquired): Advate, Adynovate, Afstyla, Eloctate, Helixate FS, Hemofil M, Koate-DVI, Kogenate FS, Kovaltry, Monoclate-P, Novoeight, NUWIQ, Recombinate, Xyntha, Obizur, Jivi and Esperoct
Description:
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IMPORTANT NOTE:
The purpose of this policy is to provide general information applicable to the administration of health benefits that Horizon Blue Cross Blue Shield of New Jersey and Horizon Healthcare of New Jersey, Inc. (collectively “Horizon BCBSNJ”) insures or administers. If the member’s contract benefits differ from the medical policy, the contract prevails. Although a service, supply or procedure may be medically necessary, it may be subject to limitations and/or exclusions under a member’s benefit plan. If a service, supply or procedure is not covered and the member proceeds to obtain the service, supply or procedure, the member may be responsible for the cost. Decisions regarding treatment and treatment plans are the responsibility of the physician. This policy is not intended to direct the course of clinical care a physician provides to a member, and it does not replace a physician’s independent professional clinical judgment or duty to exercise special knowledge and skill in the treatment of Horizon BCBSNJ members. Horizon BCBSNJ is not responsible for, does not provide, and does not hold itself out as a provider of medical care. The physician remains responsible for the quality and type of health care services provided to a Horizon BCBSNJ member.
Horizon BCBSNJ medical policies do not constitute medical advice, authorization, certification, approval, explanation of benefits, offer of coverage, contract or guarantee of payment.
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Hemophilia is a rare disorder in which the blood does not clot normally. It is a medical condition in which the ability of the blood to clot is severely reduced, causing the sufferer to bleed severely from even a slight injury. The condition is typically caused by a hereditary lack of a coagulation factor, most often factor VIII. Hemophilia A and B are X-linked recessive diseases that present in male children of carrier females. However, hemophilia must sometimes be differentiated from other bleeding disorders when the family history is negative or unknown. Differentiation between hemophilia and other conditions, such as some types of von Willebrand disease or acquired factor inhibitors, and distinction between hemophilia A and B are crucial for appropriate management.
Hemophilia typically refers to an inherited bleeding disorder caused by deficiency of coagulation factor VIII (hemophilia A), factor IX (hemophilia B), or factor XI (hemophilia C). Hemophilia A – Inherited deficiency of factor VIII; is an X-linked recessive disorder. Hemophilia A is more common than hemophilia B, representing 80-85% of the total hemophilia population. Hemophilia A can also be an acquired which is a bleeding disorder that is caused by autoantibodies that affect clotting factor VIII activity. The main reason for production of factor VIII autoantibodies in a particular individual are not clear, but may involve the presence of certain gene polymorphisms (e.g, HLA, CTLA4) and/or autoreactive CD4+ T lymphocytes.
Clinical manifestations of hemophilia relate to bleeding from impaired hemostasis, sequelae from bleeding, or complications of coagulation factor infusion. Patients with moderate hemophilia often bleed in response to minor intercurrent injury and invasive procedures. Bleeding is less frequent than in severe hemophilia and typically occurs four to six times yearly. However, more frequent bleeding may occur if a target joint develops. Some patients with moderate hemophilia may express a more severe phenotype requiring the use of prophylactic treatment regimens. In contrast, individuals with mild hemophilia generally only have bleeding in response to injury/trauma or surgery, and bleeding may not become clinically apparent until later in life. Delayed bleeding can occur after minor surgical procedures such as tooth extraction, even in patients with mild disease.
The diagnostic evaluation in cases of suspected hemophilia typically begins with a thorough review of the patient's personal bleeding history and family history. Screening tests are then performed and the diagnosis is confirmed with a specific clotting factor activity measurement(s) and/or genetic testing. Laboratory testing is similar for the majority of patients with hemophilia. Initial testing includes screening tests of hemostasis, including prothrombin time (PT), activated partial thromboplastin time (aPTT), and platelet count. Mixing studies for the aPTT assay are performed if the aPTT is prolonged. If mixing studies show correction, consistent with a factor deficiency rather than an inhibitor, factor activity levels are then measured. An exception is diagnosis in a male neonate with a positive family history, in which factor levels are often measured directly on cord blood.
Table 1: The 2nd edition World Federation of Hemophilia (WFH) guidelines for management of hemophilia screening tests for hemophilia
Interpretation of screening tests
| Possible diagnosis | PT (prothrombin time) | APTT* (activated partial thromboplastin time) | BT (bleeding time) | Platelet Count |
| Normal | Normal | Normal | Normal | Normal |
| Hemophilia A or B** | Normal | Prolonged* | Normal | Normal |
*Results of APTT measurements are highly dependent on the laboratory method used for analysis
**The same pattern can occur in presence of FXI, FXII, prekallikrein, or high molecular weight kininogen deficiencies
Table 2: The 2nd edition World Federation of Hemophilia (WFH) guidelines for management of hemophilia severity classification criteria for hemophilia
Relationship of bleeding severity to clotting factor level
| Severity | Clotting Factor Level | Bleeding Episodes |
| Severe | <1 IU/dl (<0.01 IU/ml) or <1% of normal | Spontaneous bleeding into joints or muscles, predominantly in the absence of identifiable hemostatic challenge |
| Moderate | 1-5 IU/dl (0.01-0.05 IU/ml) or 1-5% of normal | Occasional spontaneous bleeding; prolongs bleeding with minor trauma or surgery |
| Mild | 5-40 IU/dl (0.05-0.40 IU/ml) or 5-<40% of normal | Severe bleeding with major trauma or surgery. Spontaneous bleeding is rare. |
The manufacturer discontinued the manufacturing and distribution of Monoclate-P in March 2018 and supply was available through early 2019. This was due to low demand for plasma derived FVIII products versus recombinant products as per the manufacturer.
Policy:
(NOTE: For Medicare Advantage, please refer to the Medicare Coverage Section below for coverage guidance.)
I. When anti-hemophilia products are considered medically necessary, initial therapy will be approved based on the following:
1. The provider must provide the actual prescribed dose with ALL of the following supporting documentation:
a. Patient’s age
b. Patient’s weight
c. Severity of the factor deficiency
i. (i.e., severe = <1% factor activity, moderate = ≥1 to ≤5% factor activity, mild = >5 to 40% factory activity)
d. Bleed history
e. Inhibitor status
f. Intended use/regimen: prophylaxis, on-demand, peri-operative
II. Advate, Eloctate, Helixate FS, Hemofil M, Koate-DVI, Kogenate FS, Monoclate-P, Novoeight, Recombinate, Xyntha, NUWIQ, Koate, Kovaltry, Adynovate, Afstyla, Jivi, and Esperoct are considered medically necessary for any of the following FDA-approved conditions:
1. 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 of 12 years of age and older; AND
· Used as treatment in at least one of the following:
· Control and prevention of acute bleeding episodes (hemorrhage); OR
· Routine prophylaxis to prevent or reduce the frequency of bleeding episodes; OR
· Patient must have severe hemopohilia A (factor VIII level of <1%); OR
· Patient has at least two documented episodes of spontaneous bleeding into joints
· Perioperative management; OR
· Routine prophylaxis 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)
· Patient must have severe hemopohilia A (factor VIII level of <1%); OR
· Patient has at least two documented episodes of spontaneous bleeding into joints
· If the request is for routine prophylaxis and the requested dose exceeds a total weekly dose of 200 IU/kg in members <12 years or 160 IU/kg in members ≥12 years, a half-life study should be performed to determine the appropriate dose and dosing interval.
· For members with a 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, a half-life study should be performed to determine the appropriate dose and dosing interval.
o Prior to switching to Eloctate Adynovate, Jivi, or Esperoct, a half-life study should be performed on current non-extended half life (EHL) factor VIII product to ensure that a clinical benefit will be achieved.
o 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:
- For Eloctate: 50 IU/kg every 4 days (total weekly dose of 87.5 IU/kg)
- For Adynovate: 40 IU/kg twice weekly (total weekly dose of 80 IU/kg)
- For Jivi: 60 IU/kg every 5 days (total weekly dose of 84IU/kg)
- For Esperoct: 65 IU/kg twice weekly (total weekly dose of 130 IU/kg)
o Member does not have inhibitors to factor VIII
· For minimally treated patients (< 50 exposure days to factor products) previously receiving a different factor product, inhibitor testing is required at baseline in any patient that has received factor, then at every comprehensive care visit (at least once a year).
· Requests for the drug Monoclate-P will only be reviewed for retrospective requests, not prospective requests as the drug is discontinued and not currently available.
III. Obizur is considered medically necessary for any of the following FDA-approved conditions:
1. 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
· Is NOT being used in patients with a baseline anti-porcine factor VIII inhibitor titer of greater than 20 BU (Bethesda units).
· 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 in any patient that has received factor, then at every comprehensive care visit (at least once a year).
IV. When medically necessary, Advate, Eloctate, Helixate FS, Hemofil M, Koate-DVI, Kogenate FS, Monoclate-P, Novoeight, Recombinate, Xyntha, NUWIQ, Koate, Kovaltry, Adynovate, Afstyla, Jivi, and Esperoct will be approved based on the following FDA approved dosing (see tables below).
1) Initial authorization periods vary by specific indication:
a. Control and prevention of acute bleeding episodes (hemorrhage)
i. Unless otherwise specified, the initial authorization for an acute treatment, short-acting product will be 6 months and may be renewed.
- 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.
b. Routine prophylaxis to prevent or reduce the frequency of bleeding episodes
i. Unless otherwise specified, the initial authorization for prophylaxis treatment product will be 12 months and may be renewed.
c. Perioperative management
i. Unless otherwise specified, the authorization is valid for 1 month
d. Routine prophylaxis 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)
i. Unless otherwise specified, the initial authorization for prophylaxis treatment product will be 12 months and may be renewed.
2) For patients whom PK test is recommended, initial approval will be for 3 months and renewal will be based upon completion of the PK test.
[INFORMATIONAL NOTE: Requirements for half-life study 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. ]
3) Dispensing Requirements for Renderings Providers
a. Prescriptions cannot be filled without an expressed need from the patient, caregiver or prescribing practitioner. Auto-filling is not allowed.
b. 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 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 required range of prescribed dose, then rendering provider must provide proof of all available vial sizes from the manufacturer prior to dispensing factor product and the lowest possible dose able to be achieved above +1% should be dispensed. Prescribed dose should not be increased to meet assay management requirements.
c. The prescriber should discuss with the patient that a treatment log should be maintained and a copy should be submitted (via prescriber or pharmacy) upon request for renewal purposes.
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 6 to 12 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 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%) – 40-65 IU/kg single dose should be sufficient
Moderate
Circulating Factor VIII required (% of normal) (30-60%) – 40-65 IU/kg, repeat an additional dose after 24 hours if needed
Major
Circulating Factor VIII Required (% of normal) (60-100%) – 50-65 IU/kg, repeat additional dose(s) every 24 hours as needed |
| Perioperative management Congenital Hemophilia A | Minor
Circulating Factor VIII required (% of normal) (30-60%) – 50-65 IU/kg repeat additional dose(s) every 24 hours if necessary
Major
Circulating Factor VIII required (% of normal) (80-100%) – 50-65 IU/kg repeat additional dose(s) approximately every 24 hours for the first week if necessary and then every 48 hours until wound healing has occurred |
| Routine prophylaxis Congenital Hemophilia A | Routine Prophylaxis in Adults and Adolescents (≥12 years)
The recommended starting dose for routine prophylaxis is 50 units per kg of body weight every 4 days. The regiment may be individually adjusted to less or more frequent dosing based on bleeding episodes.
Routine Prophylaxis in Children <12 years)
The recommended dose for routine prophylaxis is 65 IU/kg of body weight twice weekly. The regimen may be individually adjusted to less or more frequent dosing based on bleeding episodes. |
Helixate 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
The recommended dose for routine prophylaxis is 25 units per kg of body weight three times per week.
Routine Prophylaxis in Children
The recommended dose for routine prophylaxis is 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. |
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%) – 10-20 IU/kg repeat dose every 24 to 48 hours until bleed resolves
Moderate
Circulating Factor VIII required (% of normal) (30-60%) – 15-30 IU/kg repeat every dose every 24-48 hours until bleed resolves
Major
Circulating Factor VIII Required (% of normal) (60-100%) – 30-50 IU/kg repeat every 8-24 hours until bleed resolves |
| Perioperative management Congenital Hemophilia A | Minor
Circulating Factor VIII required (% of normal) (30-60%) – 15-30 IU/kg repeat dose every 24 hours for at least 1 day until healing is achieved
Major
Circulating Factor VIII required (% of normal) (80-100%) – 40-50 IU/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. |
| 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. |
| 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. |
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). |
Monoclate P (product currently discontinued; only reviewed for retrospective cases)
| Indication | Dose |
| Control and prevention of bleeding and 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)
Mild Hemorrhages
Minor hemorrhagic episodes will generally subside with a single infusion if a level of 30% or more is attained.
Moderate Hemorrhage and Minor Surgery
For more serious hemorrhages and minor surgical procedures, the patient’s Factor VIII level should be raised to 30‑50% of normal, which usually requires an initial dose of 15‑25 I.U. per kg. If further therapy is required a maintenance dose is 10‑15 I.U. per kg every 8‑12 hours.
Severe Hemorrhage
In hemorrhages near vital organs (neck, throat, subperitoneal) it may be desirable to raise the Factor VIII level to 80‑100% of normal which can be achieved with an initial dose of 40‑50 I.U. per kg and a maintenance dose of 20‑25 I.U. per kg every 8‑12 hours.
Major Surgery
For surgical procedures a dose of AHF sufficient to achieve a level 80‑100% of normal should be given an hour prior to surgery. A second dose, half the size of the priming dose, should be given five hours after the first dose. Factor VIII levels should be maintained at a daily minimum of at least 30% for a period of 10‑14 days postoperatively. Close laboratory control to maintain AHF plasma levels deemed appropriate to maintain hemostasis is recommended. |
| 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. |
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) |
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 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 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
| 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. |
V. When medically necessary, Obizur will be approved based on the following FDA approved dosing (see table below).
1) Initial authorization period is based on the indication:
a. Treatment of acute bleeding episodes (hemorrhage)
i. Unless otherwise specified, the initial authorization for acute treatment will be 6 months and may be renewed.
a. 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.
2) For patients whom PK test is recommended, initial approval will be for 3 months and renewal will be based upon completion of the PK test.
[INFORMATIONAL NOTE: Requirements for half-life study 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. ]
3) Dispensing Requirements for Renderings Providers
a. Prescriptions cannot be filled without an expressed need from the patient, caregiver or prescribing practitioner. Auto-filling is not allowed.
b. 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 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 required range of prescribed dose, then rendering provider must provide proof of all available vial sizes from the manufacturer prior to dispensing factor product and the lowest possible dose able to be achieved above +1% should be dispensed. Prescribed dose should not be increased to meet assay management requirements.
c. The prescriber should discuss with the patient that a treatment log should be maintained and a copy should be submitted (via prescriber or pharmacy) upon request for renewal purposes.
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 |
VI. For continuing therapy, Advate, Eloctate, Helixate FS, Hemofil M, Koate-DVI, Kogenate FS, Monoclate-P, Novoeight, Recombinate, Xyntha, NUWIQ, Koate, Kovaltry, Adynovate, Afstyla, Jivi, and Esperoct are considered medically necessary and approved when ALL of the following criteria are met:
· Patient continues to meet criteria identified in section I and II and dosing identified in section IV; AND
· Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following:
· Symptoms of allergic-anaphylactic reactions (anaphylaxis, dyspnea, rash);
· Development of neutralizing antibodies (inhibitors); AND
o To determine if factor VIII inhibitors are present, perform a Bethesda assay. The presence of inhibitors should be suspected if the expected factor VIII activity concentrations in plasma are not attained or if bleeding is not controlled with the recommended dose. Monitor all patients regularly for the development of inhibitors by appropriate clinical observations and laboratory tests
· 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.).
· Plasma factor VIII activity should be monitored by performing the one-stage clotting assay to confirm adequate concentrations of factor VII have been achieved and maintained.
· 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 duration of the authorization.
· For the following disease specific criteria, renewal will be approved for a 6 month authorization period:
· Treatment of acute bleeding episodes
· Treatment of spontaneous and trauma-induced bleeding episodes
· On-demand treatment of bleeding episodes
· For the following disease specific criteria, renewal will be approved for a12 month authorization period:
· Prevention of acute bleeding episodes
· Route prophylaxis to prevent or reduce the frequency of bleeding episodes
· Dispensing Requirements for Renderings Providers
a. Prescriptions cannot be filled without an expressed need from the patient, caregiver or prescribing practitioner. Auto-filling is not allowed.
b. 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 and patient clinical history (including patient product inventory and bleed history)
o 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 required range of prescribed dose, then rendering provider must provide proof of all available vial sizes from the manufacturer prior to dispensing factor product and the lowest possible dose able to be achieved above +1% should be dispensed. Prescribed dose should not be increased to meet assay management requirements
c. The prescriber should discuss with the patient that a treatment log should be maintained and a copy should be submitted (via prescriber or pharmacy) upon request for renewal purposes.
· Requests for the drug Monoclate-P will only be reviewed for retrospective requests, not prospective requests as the drug is discontinued and not currently available.
VII. For continuing therapy, Obizur is considered medically necessary and approved when ALL of the following criteria are met:
· Patient continues to meet criteria identified in section I and III and dosing identified in section V; AND
· Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following:
· Symptoms of allergic-anaphylactic reactions (anaphylaxis, dyspnea, rash);
· Development of neutralizing antibodies (inhibitors); AND
o To determine if factor VIII inhibitors are present, perform a Bethesda assay. The presence of inhibitors should be suspected if the expected factor VIII activity concentrations in plasma are not attained or if bleeding is not controlled with the recommended dose. Monitor all patients regularly for the development of inhibitors by appropriate clinical observations and laboratory tests
· 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.).
· Plasma factor VIII activity should be monitored by performing the one-stage clotting assay to confirm adequate concentrations of factor VII have been achieved and maintained.
· 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 duration of the authorization.
· For the following disease specific criteria, renewal will be approved for a 6 month authorization period:
· Treatment of acute bleeding episodes
· Treatment of spontaneous and trauma-induced bleeding episodes
· On-demand treatment of bleeding episodes
· Dispensing Requirements for Renderings Providers
a. Prescriptions cannot be filled without an expressed need from the patient, caregiver or prescribing practitioner. Auto-filling is not allowed.
b. 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 and patient clinical history (including patient product inventory and bleed history)
o 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 required range of prescribed dose, then rendering provider must provide proof of all available vial sizes from the manufacturer prior to dispensing factor product and the lowest possible dose able to be achieved above +1% should be dispensed. Prescribed dose should not be increased to meet assay management requirements
c. The prescriber has discussed with the patient that a treatment log must be maintained and a copy of the log has been submitted (via prescriber or pharmacy) for renewal purposes.
VII. All other uses of Advate, Adynovate, Afstyla, Eloctate, Helixate FS, Hemofil M, Koate-DVI, Kogenate FS, Kovaltry, Monoclate-P, Novoeight, NUWIQ, Recombinate, Xyntha, Jivi, Esperoct and Obizur are considered investigational.
Medicare Coverage:
Hemophilia Products for Factor VIII, (Hemophilia A, Congenital and Acquired) are covered when NCD 110.3 and LCD L35111 criteria is met. For additional information and eligibility, refer to NCD 110.3 and LCD L35111.
National Coverage Determination (NCD) for Anti-Inhibitor Coagulant Complex (AICC) (110.3). Available to be accessed at CMS National Coverage Determinations (NCDs) Alphabetical Index search page: https://www.cms.gov/medicare-coverage-database/indexes/ncd-alphabetical-index.aspx.
Local Coverage Determination (LCD): Hemophilia Factor Products (L35111). Available to be accessed at Novitas Solutions, Inc., Medical Policy Search page: https://www.novitas-solutions.com/webcenter/portal/MedicareJL/LcdSearch?_afrLoop=90769712476969#!%40%40%3F_afrLoop%3D90769712476969%26centerWidth%3D100%2525%26leftWidth%3D0%2525%26rightWidth%3D0%2525%26showFooter%3Dfalse%26showHeader%3Dfalse%26_adf.ctrl-state%3D63y7eftob_46.
Medicaid Coverage:
For Horizon NJ Health members, please follow this link for the corresponding HNJH drug policy https://services3.horizon-bcbsnj.com/ddn/NJhealthWeb.nsf
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Horizon BCBSNJ Medical Policy Development Process:
This Horizon BCBSNJ Medical Policy (the “Medical Policy”) has been developed by Horizon BCBSNJ’s Medical Policy Committee (the “Committee”) consistent with generally accepted standards of medical practice, and reflects Horizon BCBSNJ’s view of the subject health care services, supplies or procedures, and in what circumstances they are deemed to be medically necessary or experimental/ investigational in nature. This Medical Policy also considers whether and to what degree the subject health care services, supplies or procedures are clinically appropriate, in terms of type, frequency, extent, site and duration and if they are considered effective for the illnesses, injuries or diseases discussed. Where relevant, this Medical Policy considers whether the subject health care services, supplies or procedures are being requested primarily for the convenience of the covered person or the health care provider. It may also consider whether the services, supplies or procedures are more costly than an alternative service or sequence of services, supplies or procedures that are at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of the relevant illness, injury or disease. In reaching its conclusion regarding what it considers to be the generally accepted standards of medical practice, the Committee reviews and considers the following: all credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, physician and health care provider specialty society recommendations, the views of physicians and health care providers practicing in relevant clinical areas (including, but not limited to, the prevailing opinion within the appropriate specialty) and any other relevant factor as determined by applicable State and Federal laws and regulations.
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Index:
Hemophilia Products – Factor VIII (Hemophilia A, Congenital and Acquired): Advate, Adynovate, Afstyla, Eloctate, Helixate FS, Hemofil M, Koate-DVI, Kogenate FS, Kovaltry, Monoclate-P, Novoeight, NUWIQ, Recombinate, Xyntha, Obizur, Jivi and Esperoct
Advate
Adynovate
Afstyla
Eloctate
Hemofil M
Hexilate FS
Koate DVI
Kogenate FS
Kovaltry
Monoclate-P
Novoeight
NUWIQ
Obizur
Recombinate
Xyntha
Jivi
Esperoct
Factor VIII, Congenital and Acquired
Hemophilia A, Congenital and Acquired:
References:
1. Adynovate [package insert]. Westlake Village, CA; Baxalta US Inc.; May 2018.
2. Advate [package insert]. Westlake Village, CA; Baxalta US Inc. December 2018.
3. Afstyla [package insert]. Kankakee, IL; CSL Behring, LLC; April 2020.
4. Eloctate [package insert]. Cambridge, MA; Biogen Idec Inc.; September 2019.
5. Helixate FS [package insert]. Kankakee, IL; CSL Behring LLC; May 2016.
6. Hemofil M [package insert]. Westlake Village, CA; Baxalta US Inc. June 2018.
7. Koate DVI [package insert]. Research Triangle Park, NC; Grifols Therapeutics Inc.; August 2012.
8. Kogenate FS [package insert]. Whippany, NJ. Bayer HealthCare LLC; December 2019.
9. Monoclate P [package insert]. Kankakee, IL; CSL Behring LLC; February 2014.
10. Novoeight [package insert]. Bagsvaerd, Denmark; Novo Nordisk; November 2018.
11. NUWIQ [package insert]. Elersvagen, Sweden; Octapharma AB; July 2017.
12. Obizur [package insert]. Westlake Village, CA; Baxter Healthcare. January 2020.
13. Recombinate [package insert]. Westlake Village, CA; Baxalta US Inc. December 2010.
14. Xyntha [package insert]. Philadelphia, PA; Wyeth Biopharma; August 2019.
15. Kovaltry [package insert]. Whippany, NJ; Bayer HealthCare LLC; March 2016.
16. MASAC RECOMMENDATIONS CONCERNING PRODUCTS LICENSED FOR THE TREATMENT OF HEMOPHILIA AND OTHER BLEEDING DISORDERS. 2013 National Hemophilia Foundation. MASAC Document #240; February 2016. Available at: http://www.hemophilia.org. Accessed June 2016.
17. First Coast Service Options, Inc. Local Coverage Determination (LCD): Hemophilia Clotting Factors (L33684). Centers for Medicare & Medicaid Services, Inc. Updated on 01/21/2016 with effective date 01/01/2016. Accessed February 2016.
18. Novitas Solutions, Inc. Local Coverage Determination (LCD): Hemophilia Clotting Factors (L35111). Centers for Medicare & Medicaid Services, Inc. Updated on 01/22/2016 with effective date 01/01/2016. Accessed February 2016.
19. Annual Review of Factor Replacement Products. Oklahoma Health Care Authority Review Board. Updated April 2016. Access June 2016.
20. Graham A1, Jaworski K. Pharmacokinetic analysis of anti-hemophilic factor in the obese patient. Haemophilia. 2014 Mar;20(2):226-9.
21. Croteau SE1, Neufeld EJ. Transition considerations for extended half-life factor products. Haemophilia. 2015 May;21(3):285-8.
22. 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).
23. Blanchette VS, Key NS, Ljung LR, et al. Definitions in hemophilia: communication from the SSC of the ISTH. J Thromb Haemost 2014; 12:1935.
24. Guidelines for the management of hemophilia, 2nd edition. 2012 World Federation of Hemophilia. July 2012. Available at: https://www1.wfh.org/publication/files/pdf-1472.pdf. Accessed March 2018.
25. Collins PW, Chalmers E, Hart DP, et al. UK Haemophilia Centre Doctors. Diagnosis and treatment of factor VIII and IX inhibitors in congenital haemophilia: (4th edition). UK Haemophilia Centre Doctors Organization. Br J Haematol. 2013 Jan; 160(2): 153-70. Available on PubMed.
26. Jivi [package insert]. Whippany, NJ; Bayer HealthCare LLC; August 2018.
27. Hemlibra (emicizumab-kxwh) [package insert]. Genentech, Inc. South San Francisco, CA. October 2018.
28. Espercot [package insert]. Plainsboro, NJ; Novo Nordisk Inc.; October 2019.
29. CSL Behring discontinues Monoclate-P production. March 6, 2018. Available at: http://www.fffenterprises.com/news/articles/article-2018-03-06b.html#:~:text=According%20to%20the%20manufacturer%2C%20%22as,distribution%20of%20Monoclate%2DP.%22
Codes:
(The list of codes is not intended to be all-inclusive and is included below for informational purposes only. Inclusion or exclusion of a procedure, diagnosis, drug or device code(s) does not constitute or imply authorization, certification, approval, offer of coverage or guarantee of payment.)
CPT*
J7182
J7185
J7188
J7190
J7192
J7205
J7207
J7208
J7209
J7210
J7211
J7204
HCPCS
* CPT only copyright 2020 American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.
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Medical policies can be highly technical and are designed for use by the Horizon BCBSNJ professional staff in making coverage determinations. Members referring to this policy should discuss it with their treating physician, and should refer to their specific benefit plan for the terms, conditions, limitations and exclusions of their coverage.
The Horizon BCBSNJ Medical Policy Manual is proprietary. It is to be used only as authorized by Horizon BCBSNJ and its affiliates. The contents of this Medical Policy are not to be copied, reproduced or circulated to other parties without the express written consent of Horizon BCBSNJ. The contents of this Medical Policy may be updated or changed without notice, unless otherwise required by law and/or regulation. However, benefit determinations are made in the context of medical policies existing at the time of the decision and are not subject to later revision as the result of a change in medical policy
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