Factor IX (Human)

• If you have an allergy to factor IX (human) or any part of this medicine.
• If you are allergic to any drugs like this one, any other drugs, foods, or other substances. Tell your doctor about the allergy and what signs you had, like rash; hives; itching; shortness of breath; wheezing; cough; swelling of face, lips, tongue, or throat; or any other signs.
• If you are allergic to mouse proteins, talk with the doctor.

This medicine may interact with other drugs or health problems.

Tell your doctor and pharmacist about all of your drugs (prescription or OTC, natural products, vitamins) and health problems. You must check to make sure that it is safe for you to take factor IX with all of your drugs and health problems. Do not start, stop, or change the dose of any drug without checking with your doctor.

NOTE: Contains nondetectable levels of factors II, VII, and X. Therefore, NOT INDICATED for replacement therapy of any other clotting factor besides factor IX or for reversal of anticoagulation due to either vitamin K antagonists or other anticoagulants (eg, dabigatran), for hemophilia A patients with factor VIII inhibitors, or for patients in a hemorrhagic state caused by reduced production of liver-dependent coagulation factors (eg, hepatitis, cirrhosis).

Control or prevention of bleeding in patients with factor IX deficiency (hemophilia B or Christmas disease): Infants, Children, and Adolescents: IV: AlphaNine SD, Mononine: Dosage is expressed in units of factor IX activity; dosing must be individualized based on severity of factor IX deficiency, extent and location of bleeding, and clinical status of patient. Refer to product information for specific manufacturer recommended dosing. Alternatively, the World Federation of Hemophilia (WFH) has recommended general dosing for factor IX products.

Formula to determine units required to obtain desired factor IX level: Note: If patient has severe hemophilia (ie, baseline factor IX level is or presumed to be <1%), then may just use “desired factor IX level” instead of “desired factor IX level increase”.

Number of factor IX units required = patient weight (in kg) x desired factor IX level increase (as % or units/dL) x 1 unit/kg

For example, to attain an 80% level in a 70 kg patient who has a baseline level of 20%: Number of factor IX units needed = 70 kg x 60% x 1 unit/kg = 4200 units

Alternative recommendations (off-label): Infants, Children, and Adolescents:

Prophylaxis, primary: Refer to adult dosing.

Treatment: Refer to adult dosing.

Solution should be infused at room temperature.

IV administration only: Should be infused slowly over several minutes: Rate of administration should be determined by the response and comfort of the patient.

AlphaNine SD: Administer IV at a rate not exceeding 10 mL/minute

Mononine: Administer IV at a rate of ~2 mL/minute. Administration rates of up to 225 units/minute have been regularly tolerated without incident (when reconstituted as directed to ~100 units/mL).

World Federation of Hemophilia recommendations: Infuse by slow IV injection at a rate not to exceed 3 mL/minute (adults) or 100 units/minute (young children); may also administer as a continuous infusion in select patients. With patients who have anti-factor IX inhibitors who have had allergic reactions during factor IX infusion, administration of hydrocortisone prior to infusion may be necessary (WFH [Srivastava 2013]).

Factor IX levels (measure 15 minutes after infusion to verify calculated doses) (WFH [Srivastava], 2013), aPTT, BP, HR, signs of hypersensitivity reactions; screen for factor IX inhibitors if the patient experiences hypersensitivity reaction or when patient is to undergo surgery, if suboptimal response to treatment occurs, if patient is being intensively treated for >5 days within 4 weeks of the last infusion, or at the following intervals (WFH [Srivastava], 2013):

Children: Screen for inhibitors every 5 exposure days until 20 exposure days, every 10 exposure days between 21 to 50 exposure days, and at a minimum of twice a year until 150 exposure days is reached.

Adults (with >150 exposure days apart from a 6 to 12 monthly review): Screen for inhibitors when suboptimal response occurs.

Animal reproduction studies have not been conducted. Factor IX concentrations do not change significantly in pregnant women with coagulation disorders and women with factor IX deficiency may be at increased risk of postpartum hemorrhage. Pregnant women should have clotting factors monitored, particularly at 28 and 34 weeks gestation and prior to invasive procedures. Prophylaxis may be needed if factor IX concentrations are <50 units/mL at term and treatment should continue for 3 to 5 days postpartum depending on route of delivery. Because parvovirus infection may cause hydrops fetalis or fetal death, a recombinant product is preferred if prophylaxis or treatment is needed. The neonate may also be at an increased risk of bleeding following delivery and should be tested for the coagulation disorder (Chi, 2012; Kadir, 2009; Lee, 2006).