Primary immune deficiency:
Indications for: HIZENTRA
As replacement therapy for primary humoral immunodeficiency (eg, congenital or X-linked agammaglobulinemia, common variable immunodeficiency, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies). As maintenance therapy to prevent relapse of neuromuscular disability and impairment in adults with chronic inflammatory demyelinating polyneuropathy (CIDP).
Adults and Children:
See full labeling. Individualize. Give by SC infusion using an infusion pump into abdomen, thigh, upper arm, or lateral hip areas. May use up to 8 infusion sites (at least 2 inches apart) simultaneously. Rotate sites. Primary immunodeficiency: <2yrs: not established. Ensure patients have received IGIV at regular intervals for at least 3 months before initiation; obtain serum IgG trough level to guide subsequent dose adjustments. Start 1 week after last IGIV or IGSC infusion (for weekly or frequent dosing), or 1–2 weeks after last IGIV infusion or 1 week after last weekly IGSC infusion (for biweekly dosing). ≥2yrs: initial weekly dose: (1.37 x previous IGIV dose [in grams])/number of weeks between IGIV doses. Infuse as tolerated up to max 15mL per infusion site at max 15mL/hr/site for first dose; may increase to 25mL/site at max 25mL/hr/site for subsequent doses. Biweekly dose: multiply the calculated weekly dose by 2. Frequent dosing (2–7 times/week): divide calculated weekly dose by the desired number of times per week. Switching from IGSC: maintain previous IGSC weekly dose. Risk of measles exposure: give a minimum total weekly dose of 0.2g/kg for 2 consecutive weeks; if biweekly dosing: give one infusion at minimum of 400mg/kg. Dose adjustments: based on serum IgG trough levels after 2–3 months of initiation and desired clinical response (see full labeling). CIDP: <18yrs: not established. Start 1 week after last IGIV infusion. ≥18yrs: 0.2g/kg (1mL/kg) per week in 1–2 sessions over 1–2 consecutive days (0.4g/kg/week was also shown effective in clinical study). Infuse as tolerated up to max 20mL per infusion site at max 20mL/hr/site for first dose; may increase to 50mL/site at max 50mL/hr/site for subsequent doses. If worsening CIDP symptoms on 0.2g/kg/week: consider increasing to 0.4g/kg/week. If CIDP symptoms worsen on 0.4g/kg/week, consider re-initiating IGIV while discontinuing Hizentra (see full labeling). Thrombosis risk: give at minimum dose and/or infusion rate practicable.
IgA-deficiency with antibodies against IgA and history of hypersensitivity. Hyperprolinemia (type I or II). Previous severe reaction to human immune globulin.
Advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling central vascular catheters, hyperviscosity, cardiovascular disorders: increased risk of thrombosis. Ensure adequate hydration. Monitor for signs/symptoms of thrombosis and assess blood viscosity in patients at risk for hyperviscosity. Pre-existing renal insufficiency, diabetes mellitus, >65yrs, hypovolemia, sepsis, paraproteinemia: increase risk of renal dysfunction; monitor and consider lower, more frequent dosing. Correct volume depletion; assess renal function before and during therapy; discontinue if renal function deteriorates. Monitor for aseptic meningitis, hemolysis, delayed hemolytic anemia, transfusion-related acute lung injury (eg, respiratory distress, pulmonary edema, hypoxemia). Risk of transmission of blood-borne diseases. Pregnancy. Nursing mothers.
Concomitant nephrotoxic drugs: increased risk of renal toxicity. May affect response to live virus vaccinations. May interfere with serological test interpretation.
Local reactions (eg, swelling, redness, heat, pain, itching), headache, diarrhea, fatigue, back pain, nausea, pain in extremity, cough, upper RTI, rash, pruritus, vomiting, upper abdominal pain, migraine, arthralgia, pain, fall, nasopharyngitis; hypersensitivity reactions (discontinue if occurs).
Single-dose prefilled syringe (5mL, 10mL, 20mL)—1; Single-dose vial (5mL, 10mL, 20mL, 50mL)—1