Subject:
Hemophilia Products – Factor IX (Hemophilia B): Alphanine SD, Alprolix, Bebulin, Benefix, Idelvion, Ixinity, Mononine, Profilnine, Rebinyn, and Rixubis
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 B – Inherited deficiency of factor IX; also called Christmas disease; is an X-linked recessive disorder. Hemophilia B is much less common and tends to be less severe than Hemophilia A. It occurs in approximately 1 in 30,000 live male births – about half have severe disease (i.e, factor IX activity <1% of normal).
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 Bebulin in September 2018 and the last manufactured product had an expiration of November 2018. This decision was based on the reduced demand and is not connected with any new safety or efficacy findings in relation to the therapy.
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. AlphaNine SD, Bebulin, BeneFIX, Profilnine SD, Mononine, Rebinyn, Rixubis, IXINITY, Alprolix, and Idelvion are considered medically necessary for any of the following FDA-approved conditions:
1. Hemophilia B (congenital factor IX deficiency aka Christmas disease)
· Diagnosis of congenital factor IX deficiency has been confirmed by blood coagulation testing; 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 (excluding Rebinyn); OR
· Patient must have severe hemophilia B (factor IX level of <1%) OR
· Patient has at least two documented episodes of spontaneous bleeding in the joints
· Perioperative management; AND
· If the request is for prophylaxis and the requested dose exceeds a total weekly dose of 160 IU/kg in members <12 years or 120 IU/kg in members ≥12 years, a half-life study should be performed to determine the appropriate dose and dosing interval.
· If the request is for Alprolix, Idelvion, or Rebinyn, a half-life study should be performed to determine the appropriate dose and dosing interval
· For Alprolix, 50 IU/kg every 7 days is the preferred dosing regimen. To obtain 100 IU every 10 days, a half-life study must be submitted showing a significant clinical benefit over 50 IU/kg every 7 days.
· Prior to switching to Alprolix, Idelvion, or Rebinyn, a half-life study should also be performed on current non-EHL factor VIII product to ensure that a clinical benefit will be achieved.
· 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 (yearly for the mild and moderate patients, semi-annually for the severe patients)
· Therapy NOT used for induction of immune tolerance in patients with Hemophilia B for ONLY the following products:
o Rixubis
o Ixinity
o Rebinyn
o Alprolix
o Idelvion
o AlphaNine SD
o BeneFIX
o Mononine
· Requests for the drug Bebulin will only be reviewed for retrospective requests, not prospective requests as the drug is discontinued and not currently available.
III. When medically necessary, Alphanine SD, Alprolix, Bebulin, BeneFIX, Idelvion, IXINITY, Mononine, Profilnine, Rebinyn, and Rixubis 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 acute treatment will be 6 months and may be renewed.
1. 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 (excluding Rebinyn)
i. Unless otherwise specified, the initial authorization for prophylaxis treatment will be 12 months and may be renewed.
c. Perioperative management
i. Unless otherwise specified, the authorization is valid for 1 month
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.
Alprolix
| Indication | Dose |
| Control and prevention of bleeding episodes Hemophilia B | One unit per kilogram body weight increases the circulating Factor IX level by 1% (IU/dL). Estimate the required dose or the expected in vivo peak increase in Factor IX level expressed as IU/dL (or % of normal) using the following: IU/dL (or % of normal) = [Total Dose (IU)/Body Weight (kg)] x Recovery (IU/dL per IU/kg)
Minor and Moderate
Circulating Factor IX required (% of normal) = 30-60 IU/dL -Repeat every 48 hours as needed
Major
Circulating Factor IX required (% of normal) = 80-100 IU/dL - Consider repeat dose after 6-10 hours, then every 24 hours for 3 days, then every 48 hours until healing achieved. |
| Perioperative management Hemophilia B | Minor
Circulating Factor IX required (% of normal) = 50-80 IU/dL -Repeat every 24-48 hours as needed, until bleeding stops and healing is achieved.
Major
Circulating Factor IX required (% of normal) = 60-100 IU/dL (initial level) - Consider repeat dose after 6-10 hours, then every 24 hours for 3 days, then every 48 hours until bleeding stops and healing achieved. |
| Routine prophylaxis Hemophilia B | 50 IU/kg once weekly or 100 IU/kg once every 10 days. Adjust dosing regimen based on individual response. |
AlphaNine SD
| Indication | Dose |
| Control and prevention of bleeding episodes Hemophilia B | One unit per kilogram body weight increases the circulating Factor IX level by 1% (IU/dL). Number of Factor IX IU required = body wt (kg) x Desired increase in Plasma Factor IX(percent) x 1.0 IU/kg
Minor
Circulating Factor IX required (20-30 % of normal) = 20-30 IU/dL -Repeat every 12-24 hours as needed for 1-2 days
Moderate
Circulating Factor IX required (25-50 % of normal) = 25-50 IU/dL -Repeat every 12-24 hours as needed for 2-7 days
Major
Circulating Factor IX required (50 % of normal) = 50-100 IU/dL - Consider repeat dose after 12 hours as needed for 3-5 days. Following this treatment period, FIX levels should be maintained at 20% (20 IU FIX/kg/twice daily) until healing has been achieved. Major hemorrhages may require treatment for up to 10 days. |
| Routine prophylaxis Hemophilia B | 25-40 IU/kg two times weekly or 15-30 IU/kg two times weekly. Adjust dosing regimen based on individual response. |
| Perioperative management Hemophilia B | Prior to surgery, FIX should be brought to 50-100% of normal (50-100 IU/kg repeat every 12 hours). For the next 7 to 10 days, or until healing has been achieved, the patient should be maintained at 50-100%FIX levels (50-100 IU/kg every 12 hours). |
BeneFIX
| Indication | Dose |
| Control and prevention of bleeding episodes Hemophilia B, and Perioperative management Hemophilia B | One unit per kilogram body weight increases the circulating Factor IX level by 1% (IU/dL).
ADULT: Number of Factor IX IU required = body wt (kg) x Desired increase in Plasma Factor IX(percent) x 1.3 IU/kg;
CHILD (<15 years) Number of Factor IX IU required = body wt (kg) x Desired increase in Plasma Factor IX(percent) x 1.4 IU/kg
Minor
Circulating Factor IX required (% of normal) = 20-30 IU/dL -Repeat every 12-24 hours as needed for 1-2 days
Moderate
Circulating Factor IX required (% of normal) = 25-50 IU/dL -Repeat every 12-24 hours as needed for 2-7 days
Major
Circulating Factor IX required (% of normal) = 50-100 IU/dL - Consider repeat dose after 12-24 hours as needed for 7- 10 days. |
| Routine prophylaxis Hemophilia B | 25-40 IU/kg two times weekly or 15-30 IU/kg two times weekly. Adjust dosing regimen based on individual response. |
Bebulin (product currently discontinued; only reviewed for retrospective cases)
| Indication | Dose |
| Control and prevention of bleeding episodes Hemophilia B | One unit per kilogram body weight increases the circulating Factor IX level by 1% (IU/dL). Number of Factor IX IU required = body wt (kg) x Desired increase in Plasma Factor IX(percent) x 1.2 IU/kg
Minor
Circulating Factor IX required (% of normal) (20%)= 25-35 IU/dL -Repeat every 24 hours as needed until adequate wound healing
Moderate
Circulating Factor IX required (% of normal) (40%)= 50-65 IU/dL -Repeat every 24 hours as needed for 2 days or until adequate wound healing
Major
Circulating Factor IX required (% of normal)(>60%) = 75-90 IU/dL - Consider repeat dose after 24 hours as needed for 2-3 days or until adequate wound healing. |
| Routine prophylaxis Hemophilia B | 25-40 IU/kg two times weekly or 15-30 IU/kg two times weekly. Adjust dosing regimen based on individual response. |
Perioperative management
Hemophilia B | Minor
Circulating Factor IX required (% of normal) (40-60%)= 50-75 IU/dL given 1 hour prior to surgery, repeat every 12 hours, and continue replacement therapy over 1 to 2 weeks postop until adequate wound healing is achieved.
Major
Circulating Factor IX required (% of normal) (>60%)= 75-90 IU/dL given 1 hour prior to surgery, repeat every 12 hours, and continue replacement therapy over for up to 2 weeks postop. If treatment is required beyond 2 weeks post-up, then dosing interval can be adjusted to every 24 hours and continued until adequate wound healing is achieved. |
Idelvion
| Indication | Dose |
| Control and prevention of bleeding episodes | · One IU of IDELVION per kg body weight is expected to increase the circulating activity of Factor IX as follows:
o Adolescents and adults: 1.3 IU/dL per IU/kg (2.1)
o Pediatrics (<12 years): 1 IU/dL per IU/kg (2.1)
· Administer intravenously. Do not exceed infusion rate of 10 mL per minute.
· Dosage and duration of treatment with IDELVION depends on the severity of the Factor IX deficiency, the location and extent of bleeding, and the patient’s clinical condition, age and recovery of Factor IX.
· Determine the initial dose using the following formula:
o Required Dose (IU) = Body Weight (kg) x Desired Factor IX rise (% of normal or IU/dL) x (reciprocal of recovery (IU/kg per IU/dL))
· Adjust dose based on the patient’s clinical condition and response.
Minor/Moderate
Desired peak Factor IX Level (% of normal or IU/dL): 30-60, dosed every 48-72 hours for at least 1 day until healing is achieved
Major
Desired peak Factor IX Level (% of normal or IU/dL): 60-100, dosed every 48-72 hours for 7-14 days until bleeding stops. Maintenance dose is weekly. |
Perioperative management
Hemophilia B | Minor
Desired peak Factor IX Level (% of normal or IU/dL): 50-80, dosed every 48-72 hours for at least 1 day until healing is achieved
Major
Desired peak Factor IX Level (% of normal or IU/dL): 60-100, dosed every 48-72 hours for 7-14 days until bleeding stops. Repeat dose every 48-72 hours for the first week or until healing is achieved. Maintenance dose is once or twice weekly. |
| Routine prophylaxis Hemophilia B | Patients ≥12 years of age:
· 25-40 IU/kg body weight every 7 days. (2.1) Patients who are well-controlled on this regimen may be switched to a 14-day interval at 50-75 IU/kg body weight.
Patients <12 years of age:
· 40-55 IU/kg body weight every 7 days. |
Ixinity
| Indication | Dose |
| Control and prevention of bleeding episodes Congenital Hemophilia B | One IU per kg body weight increases the circulating activity of factor IX by 0.698 IU/dL
Initial dose:
Required factor IX units (IU) = body weight (kg) x desired factor IX increase (% of normal of IU/dL) x reciprocal of observed recovery (IU/kg per IU/dL)
Maintenance dose:
Depends upon the type of bleed or surgery, clinical response, and the severity of the underlying factor IX deficiency
Minor
Desired peak Factor IX Level (% of normal or IU/dL): 30-60, dosed every 24 hours ondays 1-3 until healing is achieved
Moderate
Desired peak Factor IX Level (% of normal or IU/dL): 40-60, dosed every 24 hours on days 2-7 until healing is achieved
Major or Life threatening
Desired peak Factor IX Level (% of normal or IU/dL): 60-100, dosed every 12-24 hours on days 2-14 until healing is achieved |
Perioperative management
Congenital Hemophilia B | Minor
Pre-op: Desired peak Factor IX Level (% of normal or IU/dL) 50-80
Post-op: Desired peak Factor IX Level (% of normal or IU/dL)30-80, dosed every 24 hours on days 1-5, depending on type of procedure
Major
Pre-op: Desired peak Factor IX Level (% of normal or IU/dL)60-80
Post-op: Desired peak Factor IX Level (% of normal or IU/dL)40-60, dosed every 8-24 hours on days 1-3, or 30-50 dosed every 8-24 hours on days 4-6, or 20-40 dosed every 8-24 hours on days 7-14 |
Mononine
| Indication | Dose |
Control and prevention
of bleeding episodes
and perioperative
management
Hemophilia B | One unit per kilogram body weight increases the circulating Factor IX level by 1%
(IU/dL). Estimate the required dose with the following formula: Number of Factor IX
IU required (IU) = Body Weight (in kg) x desired Factor IX increase (% or IU/dL
normal) x 1.0 IU/kg [per IU/dL]
Minor Spontaneous Hemorrhage Prophylaxis
Circulating Factor IX required (% of normal)(15-25%) = up to 20-30IU/kg for one
dose. Repeat in 24 hours if necessary.
Major Trauma or Surgery
Circulating Factor IX required (% of normal)(25-50%) = up to 75 IU/kg Dosed every
18-30 hours depending on T1/2 and measured Factor IX levels. Continue for up to
10 days depending upon nature of insult. |
Profilnine SD
| Indication | Dose |
Control and prevention
of bleeding episodes
Hemophilia B | One unit per kilogram body weight increases the circulating Factor IX level by 1%
(IU/dL). Number of Factor IX IU required = body wt (kg) x Desired increase in
Plasma Factor IX(percent) x 1.0 IU/kg
Mild to Moderate
Single dose of product sufficient to raise plasma factor IX levels to 20 to 30 percent of normal. 20-30 IU/kg every 16-24 hours until hemorrhage stops and healing is achieved. For minor, may repeat for 1-2 days, for moderate, may repeat for 2-7 days
Major
Single dose of product sufficient to raise plasma factor IX levels to 30 to 50 percent of normal. Daily infusions are generally required. |
Routine prophylaxis
Hemophilia B | 25-40 IU/kg two times weekly or 15-30 IU/kg two times weekly. Adjust dosing
regimen based on individual response. |
Perioperative
Management
Hemophilia B | Surgery associated with bleeding in factor IX deficient patients requires factor IX levels of 30 to 50 percent. 30-50 IU/kg every 16-24 hours for 7-10 days until healing is achieved. For dental extractions, the factor IX level should be raised to 50 percent immediately prior to procedure; additional factor IX complex may be given if bleeding recurs. |
Rebinyn
| Indication | Dose |
On-demand treatment and control of bleeding episodes
Congenital Hemophilia B | Minor and Moderate
40 IU/kg of actual body weight. A single dose should be sufficient for minor and moderate bleeds. Additional doses of 40 IU/kg can be given.
Major
80 IU/kg of actual body weight. Additional doses of 40 IU/kg can be given. |
Perioperative management of bleeding
Congenital Hemophilia B | Minor
Pre-op: 40 IU/kg of actual body weight (single pre-op dose should be sufficient) Post-op: Additional doses can be given if required
Major
Pre-op: 80 IU/kg of actual body weight
Peri/Post-op: 40 IU/kg of actual body weight. As clinically needed for the perioperative management of bleeding, repeated doses of 40 IU/kg (in 1-3 day intervals) within the first week after major surgery may be administered. Due to the long half-life the frequency of dosing in the post-surgical setting may be extended to once weekly after the first week until bleeding stops and healing is achieved. |
Rixubis
| Indication | Dose |
| Control and prevention of bleeding episodes Hemophilia B | One IU per kilogram body weight increases the circulating activity of Factor IX by 0.7 IU/dL for patients <12 years of age and 0.9 IU/dL for patients ≥ 12 years of age.
Initial dose = body wt (kg) x desired factor IX increase (percent of normal or IU/dL) x
reciprocal of observed recovery (IU/kg per IU/dL)
Minor
Circulating Factor IX level required (% or IU/dL) = 20-30 every 12 - 24 hours for at
least 1 day, until healing is achieved
Moderate
Circulating Factor IX level required (% or IU/dL) = 25-50 every 12 - 24 hours for 2 – 7 days, until bleeding stops and healing is achieved
Major
Circulating Factor IX level required (% or IU/dL) = 50-100 every 12 - 24 hours for 7 –
10 days, until bleeding stops and healing is achieved |
| Routine prophylaxis Hemophilia B | Dosing for previously treated patients (PTPs):
Patients <12 years of age
60 – 80 IU/kg twice weekly
Patients ≥ 12 years of age
40 – 60 IU/kg twice weekly
Adjust the dose based on the individual patient’s age, bleeding pattern, and physical activity. |
Perioperative management
Hemophilia B | Minor
Circulating Factor IX level required (% or IU/dL) = 30-60 every 24 hours for at least 1 day, until healing is achieved
Major
Circulating Factor IX level required (% or IU/dL) = 80-100 every 8 - 24 hours for 7 – 10 days, until bleeding stops and healing is achieved |
IV. For continuing therapy, Alphanine SD, Alprolix, Bebulin, BeneFIX, Idelvion, IXINITY, Mononine, Profilnine, Rebinyn, and Rixubis 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, as well as dosing 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);
o Thromboembolism may occur when using factor IX products and has been associated with the use of factor IX containing products. Because of the potential risk for thromboembolism, monitor for early signs of thromboembolism and consumptive coagulopathy when administering factor IX products to patients with liver disease, fibrinolysis, perioperative status, or risk for thromboembolic events or disseminated intravascular coagulation.
· Development of neutralizing antibodies (inhibitors); AND
o To determine if factor IX inhibitors are present, perform a Bethesda assay. The presence of inhibitors should be suspected if the expected factor IX 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.).
· 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 a 12 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 Bebulin will only be reviewed for retrospective requests, not prospective requests as the drug is discontinued and not currently available.
V. All other uses of Alphanine SD, Alprolix, Bebulin, BeneFIX, Idelvion, IXINITY, Mononine, Profilnine, Rebinyn, and Rixubis are considered investigational.
Medicare Coverage:
There is no National Coverage Determination (NCD) for Hemophilia Products – Factor IX (Hemophilia B). In the absence of an NCD, coverage decisions are left to the discretion of
Local Medicare Carriers. Novitas Solutions, Inc, the Local Medicare Carrier for jurisdiction JL, has determined that this service is covered when LCD L35111 criteria is met.
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 IX (Hemophilia B): Alphanine SD, Alprolix, Bebulin, Benefix, Idelvion, Ixinity, Mononine, Profilnine, Rebinyn, and Rixubis
Alphanine SD
Alprolix
Bebulin
Benefix
Idelvion
Ixinity
Mononine
Profilnine
Rebinyn
Rixubis
Factor IX
Hemophilia B
References:
1. AlphaNine SD [package insert]. Los Angeles, CA; Grifols Biologicals Inc.; June 2018.
2. Alprolix [package insert]. Cambridge, MA; Biogen Idec, Inc.; July 2017.
3. Bebulin [package insert]. Westlake Village, CA; Baxalta US Inc. September 2015.
4. BeneFIX [package insert]. Philadelphia, PA; Wyeth Biopharma; July 2019.
5. Idelvion [package insert]. Bagsvaerd, Denmark; Novozymes Biopharma A/S; October 2019.
6. Ixinity [package insert]. Winnipeg, Manitoba, Canada. Cangene Corporation; December 2018.
7. Mononine [package insert]. Kankakee, IL; CSL Behring LLC; December 2018.
8. Profilnine [package insert]. Los Angeles, CA; Grifols Biologicals Inc.; May 2014.
9. Rebinyn [package insert]. Plainsboro, NJ. Novo Nordisk. May 2017.
10. Rixubis [package insert]. Westlake Village, CA; Baxalta US Inc.; December 2019.
11. 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.
12. 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.
13. 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.
14. Annual Review of Factor Replacement Products. Oklahoma Health Care Authority Review Board. Updated April 2016. Access June 2016.
15. Graham A1, Jaworski K. Pharmacokinetic analysis of anti-hemophilic factor in the obese patient. Haemophilia. 2014 Mar;20(2):226-9.
16. Croteau SE1, Neufeld EJ. Transition considerations for extended half-life factor products. Haemophilia. 2015 May;21(3):285-8.
17. 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).
18. Blanchette VS, Key NS, Ljung LR, et al. Definitions in hemophilia: communication from the SSC of the ISTH. J Thromb Haemost 2014; 12:1935.
19. 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.
20. 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.
21. Shire Discontinuing Manufacture and Distribution of Bebulin. July 2018. National Hemophilia Foundation. Available at: https://www.hemophilia.org/Newsroom/Medical-News/Shire-Discontinuing-Manufacture-and-Distribution-of-BEBULIN
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*
HCPCS
J7193
J7201
J7194
J7195
J7202
J7195
J7193
J7194
J7195
J7200
* 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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