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    Agents for Acute Treatment of Hereditary Angioedema (HAE)

    DEA CLASS

    Rx

    DESCRIPTION

    A selective bradykinin B2 receptor antagonist
    Used subcutaneously to treat acute attacks of hereditary angioedema
    May be administered by the patient

    COMMON BRAND NAMES

    FIRAZYR, SAJAZIR

    HOW SUPPLIED

    FIRAZYR/Icatibant/Icatibant acetate Subcutaneous Inj Sol: 1mL, 10mg

    DOSAGE & INDICATIONS

    For the treatment of acute attacks of hereditary angioedema (HAE).
    NOTE: Given the potential for airway obstruction during acute laryngeal HAE attacks, advise patients to seek immediate medical attention in an appropriate health care facility in addition to icatibant treatment.[45450]
    Subcutaneous dosage
    Adults

    30 mg subcutaneously once. If response is inadequate or symptoms recur, may repeat dose at intervals of at least 6 hours. Max: 90 mg/24 hours.[45450]

    For the treatment of ACE-inhibitor induced angioedema†.
    Subcutaneous dosage
    Adults

    30 mg subcutaneously once.

    †Indicates off-label use

    MAXIMUM DOSAGE

    Adults

    30 mg/dose subcutaneously. May repeat at intervals of 6 hours; no more than 90 mg/24 hours subcutaneously.

    Geriatric

    30 mg/dose subcutaneously. May repeat at intervals of 6 hours; no more than 90 mg/24 hours subcutaneously.

    Adolescents

    Safety and efficacy have not been established.

    Children

    Safety and efficacy have not been established.

    Infants

    Safety and efficacy have not been established.

    Neonates

    Safety and efficacy have not been established.

    DOSING CONSIDERATIONS

    Hepatic Impairment

    No dosage adjustments are needed.

    Renal Impairment

    No dosage adjustments are needed.

    ADMINISTRATION

    Hazardous Drugs Classification
    NIOSH 2016 List: Group 3
    NIOSH (Draft) 2020 List: Table 2
    Observe and exercise appropriate precautions for handling, preparation, administration, and disposal of hazardous drugs.
    Use double chemotherapy gloves and a protective gown. Prepare in a biological safety cabinet or compounding aseptic containment isolator with a closed system drug transfer device. Eye/face and respiratory protection may be needed during preparation and administration.

    Injectable Administration

    NOTE: Patients may self-administer icatibant upon recognition of acute hereditary angioedema (HAE) attack, after appropriate training under the guidance of a healthcare professional.
    For subcutaneous injection only.
    Visually inspect parenteral products for particulate matter and discoloration prior to administration whenever solution and container permit. Icatibant should be clear and colorless.

    Subcutaneous Administration

    Supplied as a prefilled 30 mg syringe (10 mg/mL).
    Remove the prefilled syringe and 25-gauge needle from the carton. Attach the needle to the syringe hub; do not use a different needle. Do not remove needle cap until immediately before administration.
    Choose a site on the abdomen roughly 2 to 4 inches (5 to 10 cm) below the umbilicus on either side. The injection site should be at least 2 inches (5 cm) from any scars. Do not choose an area that is bruised, swollen, or painful.
    Disinfect injection site (abdominal area) and allow to dry.
    Uncap the needle. Hold prefilled syringe between fingers and thumb.
    Gently pinch the fold of disinfected skin. Hold the syringe at a 45 to 90 angle to the skin.
    Bring syringe toward skin and quickly insert the needle into the skin fold. Push the plunger, administer over at least 30 seconds, and until no medication remains in the syringe.
    Release skin and gently pull needle out. Dispose of the used syringe and needle in sharps disposal container immediately after use.
    May repeat at intervals of at least 6 hours if response is inadequate or symptoms recur. Do not administer more than 90 mg (3 doses) in 24 hours.[45450]

    STORAGE

    FIRAZYR:
    - Discard product if it contains particulate matter, is cloudy, or discolored
    - Discard unused portion. Do not store for later use.
    - Do not freeze
    - Store between 36 to 77 degrees F
    - Store in original package until time of use
    SAJAZIR:
    - Discard product if it contains particulate matter, is cloudy, or discolored
    - Discard unused portion. Do not store for later use.
    - Do not freeze
    - Store between 36 to 77 degrees F
    - Store in original package until time of use

    CONTRAINDICATIONS / PRECAUTIONS

    General Information

    Due to the potential for airway obstruction during an acute laryngeal HAE attack, patients should seek immediate medical attention in an appropriate health care facility in addition to icatibant treatment.

    Labor, obstetric delivery, pregnancy

    Available data from published literature and the pharmacovigilance database on icatibant use in pregnant women have not identified a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes. Icatibant displayed no teratogenic effects in rats or rabbits at maternal doses up to 2.7 times and 13 times the maximum recommended human doses (MHRD). In embryo-fetal development studies involving rabbits that received subcutaneous icatibant on gestation days 7 to 18 and at doses approximately 0.025 times the MHRD and higher, the rates of premature birth and abortion were increased. Dose-related increases of percent pre-implantation loss, as well as, dose-related decreases of total implantations and in total number of live fetuses were observed when pregnant rabbits received icatibant doses up to 13 times the MRHD. In pre- and post-natal development studies in rats that received subcutaneous icatibant from gestation day 6 to post-partum day 20, delayed parturition and resulting deaths of dams were observed at doses 0.5 times the MRHD and higher and 2 times the MRHD and higher, respectively. At doses 2 times MRHD, fetal death and increased pup deaths were observed through post-partum day 4. Decreased pup hair growth and pup righting reflex impairment were observed with doses of 7 times the MRHD. Use icatibant during pregnancy only if the benefit outweighs the potential risk to the fetus. The effects of icatibant on labor or obstetric delivery have not been investigated; however, animal studies showed that icatibant causes delayed parturition and associated fetal death in rats and premature birth and abortion in rabbits. Delayed parturition occurred in rats at half the maximum recommended human dose.[45450]

    Breast-feeding

    There are no data on the presence of icatibant in human milk, the effects on the breastfed infant, or the effects on milk production. The manufacturer states that systemic absorption in infants following oral exposure of icatibant through breast milk is not expected. Consider the benefits of breast-feeding, the risk of potential infant drug exposure, and the risk of an untreated or inadequately treated condition. If a breast-feeding infant experiences an adverse effect related to a maternally administered drug, healthcare providers are encouraged to report the adverse effect to the FDA.

    Driving or operating machinery

    Tiredness, drowsiness, and dizziness have been reported following the use of icatibant. Patients should be advised against driving or operating machinery if they feel tired or dizzy.

    ADVERSE REACTIONS

    Moderate

    elevated hepatic enzymes / Delayed / 4.0-4.0
    erythema / Early / Incidence not known
    hematoma / Early / Incidence not known
    edema / Delayed / Incidence not known
    antibody formation / Delayed / Incidence not known

    Mild

    injection site reaction / Rapid / 97.0-97.0
    fever / Early / 4.0-4.0
    dizziness / Early / 3.0-3.0
    rash / Early / 1.0
    urticaria / Rapid / Incidence not known
    ecchymosis / Delayed / Incidence not known
    pruritus / Rapid / Incidence not known
    headache / Early / Incidence not known
    nausea / Early / Incidence not known

    DRUG INTERACTIONS

    Amlodipine; Benazepril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Angiotensin-converting enzyme inhibitors: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Benazepril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Benazepril; Hydrochlorothiazide, HCTZ: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Captopril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Captopril; Hydrochlorothiazide, HCTZ: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Enalapril, Enalaprilat: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Enalapril; Hydrochlorothiazide, HCTZ: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Fosinopril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Fosinopril; Hydrochlorothiazide, HCTZ: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Hydrochlorothiazide, HCTZ; Moexipril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Lisinopril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Lisinopril; Hydrochlorothiazide, HCTZ: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Moexipril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Perindopril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Perindopril; Amlodipine: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Quinapril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Quinapril; Hydrochlorothiazide, HCTZ: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Ramipril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Trandolapril: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.
    Trandolapril; Verapamil: (Minor) Although clinical data are lacking, icatibant is a bradykinin B2 receptor antagonist and may theoretically potentiate the antihypertensive effect of ACE inhibitors.

    PREGNANCY AND LACTATION

    Pregnancy

    There are no data on the presence of icatibant in human milk, the effects on the breastfed infant, or the effects on milk production. The manufacturer states that systemic absorption in infants following oral exposure of icatibant through breast milk is not expected. Consider the benefits of breast-feeding, the risk of potential infant drug exposure, and the risk of an untreated or inadequately treated condition. If a breast-feeding infant experiences an adverse effect related to a maternally administered drug, healthcare providers are encouraged to report the adverse effect to the FDA.

    MECHANISM OF ACTION

    Icatibant is a competitive inhibitor of the bradykinin B2 receptor. Hereditary angioedema (HAE) is caused by a deficiency of C1 esterase inhibitor. A deficiency of C1 esterase inhibitor may result in an increase in plasma bradykinin concentrations. The characteristic symptoms of HAE, localized swelling, inflammation, and pain, are thought to be caused by an excessive production of bradykinin. Icatibant inhibits bradykinin from binding to the B2 receptor, thus treating the symptoms of an acute HAE attack.

    PHARMACOKINETICS

    Icatibant is administered subcutaneously. Vd at steady state is 29 +/- 8.7 L. Icatibant is extensively metabolized by proteolytic enzymes to inactive metabolites that are primarily excreted in the urine. Less than 10% of the dose is eliminated as unchanged drug. Plasma clearance is 245 +/- 58 mL/minute, and mean half-life is 1.4 +/- 0.4 hours.
     
    Intravenous icatibant (0.4 and 0.8 mg/kg over 4 hours) caused dose and time-dependent inhibition in the development of bradykinin-induced vasodilation, hypotension, and reflex tachycardia in healthy young subjects for 6 to 8 hours. Based on exposure-response analysis, a subcutaneous dose of 30 mg is predicted to be effective against bradykinin challenge for at least 6 hours.
     
    Affected cytochrome P450 isoenzymes and drug transporters: none

    Subcutaneous Route

    After a 30 mg subcutaneous dose, icatibant has an absolute bioavailability of 97%. The mean Cmax of 974 +/- 280 ng/mL is seen after 0.75 hours. The mean AUC is 2,165 +/- 568 ng x hour/mL, and there is no evidence of accumulation following three 30-mg doses administered 6 hours apart.