coagulation factor IX (Recombinant), Fc fusion protein

General

Pronunciation:
ko-a-gyoo-lay-shun fac-tor nine eff-cee fyoo-zhun proh-teen

Trade Name(s)

• Alprolix

Ther. Class.
hemostatic agents

Pharm. Class.
clotting factor replacements

Indications

Factor IX replacement (DNA-derived) to control and prevent bleeding episodes perioperatively or prophylactically in patients with Hemophilia B.

Action

Replaces deficient Factor IX in patients with Hemophilia B.

Therapeutic Effect(s):

Decreased incidence and severity of bleeding .

Pharmacokinetics

Absorption: IV administration results in complete bioavailability.

Distribution: Unknown.

Metabolism and Excretion: Used up in clotting process.

Half-life: Adults– 86.5 hr; children 12–17 yr– 84 hr; children 6–11 yr– 72.2 hr; children 2–5 yr– 66.4 hr.

TIME/ACTION PROFILE (decreased bleeding/risk of bleeding)

Contraindication/Precautions

Contraindicated in:

• Known hypersensitivity.

Use Cautiously in:

• OB: Use in pregnancy only if clearly needed;
• Lactation:Use caution if breastfeeding;
• Pedi: Children <12 yr have ↑ clearance, shorter duration of action (↑ dose/kg or more frequent dosing may be required).

Adverse Reactions/Side Effects

CNS: headache

CV: THROMBEMBOLISM (↑RISK WITH CONTINUOUS INFUSION VIA CENTRAL CATHETER)

GI: perioral paresthesia

Misc: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS, production of neutralizing antibodies (inhibitors)

* CAPITALS indicate life-threatening.
Underline indicate most frequent.

Interactions

Drug-Drug

Bleeding risk may be ↑ by anticoagulants , antiplatelet agents , aspirin , fibrinolytics , NSAIDs , SSRIs and SNRIs .

Route/Dosage

One unit/kg increases circulating Factor IX (FIX) level by 1%.

IV: (Adults and Children) Minor and moderate bleeding– amount needed to produce a circulating FIX level of 30–40 IU/dL, repeated every 48 hr as needed; Major bleeding– amount needed to produce a circulating FIX level of 80–100 IU/dL, repeat after 6–10 hr, then every 24 hr for three days, then every 48 hr until healing occurs; Perioperative management (minor procedures)– amount needed to produce a circulating FIX level of 50–80 IU/dL as a single infusion, may be repeated after 24–48 hr until bleeding ceases and healing has occurs; Perioperative management (major procedures)– amount needed to produce a circulating FIX level of 60–100 IU/dL as a single infusion, may be repeated after 6–10 hr and then every 24 for 3 days until bleeding ceases and healing occurs (additional doses may be given every 48 hr if needed);Routine prophylaxis– 50 IU/kg once weekly or 100 IU/kg every 10 days. Doses may be modified according to need/response.

Availability

Lyophilized powder for IV injection (requires reconstitution): 500 IU vial, 1000 IU vial, 2000 IU vial, 3000 IU vial

Assessment

• Assess for signs and symptoms of allergic reactions (angioedema, chest tightness, hypotension, rash, nausea, vomiting, paresthesia, restlessness, wheezing, dyspnea). If symptoms occur, discontinue therapy and treat symptomatically.

Lab Test Considerations:

If expected Factor IX activity levels are not attained or if bleeding is not controlled with recommended dose, perform a Bethesda assay to determine if inhibitors are present.

Potential Diagnoses

• Ineffective tissue perfusion (Indications)
• Deficient knowledge, related to medication regimen (Patient/Family/Teaching)

Implementation

Patient/Family Teaching

• May be self-administered with proper training. Instruct patient to read Patient Information and Instructions for Use prior to starting therapy and with each refill in case of changes.
• Advise patient to notify health care professional immediately if signs and symptom of allergic reactions.
• Advise female patient to notify health care professional if pregnancy is planned or suspected or if breastfeeding.

Evaluation/Desired Outcomes

Control and prevention of bleeding in adults and children with Hemophilia B.