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Name /bks_53161_deglins_md_disk/sodiumphenylacetatebenzo 02/17/2014 10:18AM sodium phenylacetate/sodium benzoate proic acid may contribute to hyperammonemia and negate beneficial effects. (soe-dee-um fen-il-as-e-tate/soe-dee-um ben-zo-ate) Route/Dosage Ammonul Classification Therapeutic: antidotes Pharmacologic: none assigned Indications Adjunctive therapy of acute hyperammonemia associated with urea cycle disorders; when lack of specific enzymes results in an inability to breakdown and eliminate waste nitrogens. Action Provides an alternative pathway for nitrogen elimination in patients without a fully functioning urea cycle. Therapeutic Effects: Decreased sequelae of hyperammonemia including encephalopathy and death. Pharmacokinetics Absorption: IV administration results in complete bioavailability. Distribution: Unknown. Metabolism and Excretion: Metabolized in the liver as part of the alternative pathway in the urea cycle; also metabolized in the kidney. Half-life: Unknown. TIME/ACTION PROFILE (blood levels) ONSET rapid PEAK 1–3 hr pg 1 # 1 Interactions Drug-Drug: Penicillin and probenecid may compete for renal secretion. Val- 1 ROUTE IV Plate # 0-Composite DURATION 14–26 hr Concurrent IV arginine is required. IV (Children 0– 20 kg): Loading dose over 90– 120 min— 2.5 mL/kg (provides 250 mg/kg of sodium phenylacetate and 250 mg/kg sodium benzoate) followed by maintenance infusion— 2.5 mL/kg (provides 250 mg/kg of sodium phenylacetate and 250 mg/kg sodium benzoate) over 24 hr, continued until oral therapy is initiated. IV (Children ⬎20 kg): Loading dose over 90– 120 min— 2.5 mL/kg (provides 250 mg/kg of sodium phenylacetate and 250 mg/kg sodium benzoate) followed by maintenance infusion— 55 mL/m2(provides 5.5 g/m2 of sodium phenylacetate and 5.5 g/m2 sodium benzoate) over 24 hr, continued until oral therapy is initiated. NURSING IMPLICATIONS Assessment ● Assess neurologic status frequently during therapy. ● Assess infusion site frequently during therapy. Extravasation into peripheral tis- sues may lead to skin necrosis. If extravasation is suspected, discontinue infusion and resume at a different site. Treatment of extravasation may include aspiration of residual drug from catheter, limb elevation, and intermittent cooling using cold packs. ● Lab Test Considerations: Monitor plasma ammonia levels frequently during therapy. ● Monitor CBC and serum electrolytes frequently during therapy; maintain normal levels. May cause hyperglycemia, hypocalcemia, hypokalemia, and anemia. ● Monitor blood chemistry, pH, and pCO2 frequently during therapy. May cause metabolic acidosis and hyperammonemia. Contraindications/Precautions Contraindicated in: Hypersensitivity. Use Cautiously in: Hepatic/renal impairment; OB: Use only if clearly needed; Potential Nursing Diagnoses Lactation: Safety not established. ● Must be diluted and administered through a central line; administration through Adverse Reactions/Side Effects CNS: SEIZURES, mental impairment. GI: vomiting. Endo: hyperglycemia. F and E: hypokalemia. ⫽ Canadian drug name. ⫽ Genetic Implication. Risk for injury (Indications) Implementation peripheral lines may cause burns. ● May cause nausea and vomiting; administer an antiemetic prior to infusion. ● Do not repeat loading dose; phenylacetate plasma levels are prolonged. CAPITALS indicate life-threatening, underlines indicate most frequent. Strikethrough ⫽ Discontinued. PDF Page #1 Name /bks_53161_deglins_md_disk/sodiumphenylacetatebenzo 02/17/2014 10:18AM Plate # 0-Composite pg 2 # 2 2 ● Begin infusion as soon as the diagnosis of hyperammonemia is made. ● Caloric supplementation and restriction of dietary protein are required during PDF Page #2 therapy. Caloric intake of ⬎80 cal/kg/day should be attempted. Non-protein calories should be supplied as glucose (8– 10 mg/kg/min) with Intralipid added. ● Once elevated ammonia levels have been reduced to normal range, oral therapy, such as sodium phenylbutyrate, dietary management and protein restrictions should be started or reinitiated. IV Administration ● Intermittent Infusion: Diluent: Dilute with D10W at ⱖ25 mL/kg before ad- ministration. Use a Millex Durapore GV 33 mm Sterile Syringe Filter (0.22 m) during the admixture process when injecting Ammonul into the 10% Dextrose IV bag, regardless of whether particulate matter is seen in the vial; particulate matter may not be seen on visual inspection. Solution is stable for 24 hr at room temperature. Do not administer solutions that are discolored or contain particulate matter. Rate: Administer loading dose over 90– 120 min. ● Continuous Infusion: Diluent: Maintenance infusions use same dilution as loading dose and may be continued until elevated plasma ammonia levels have been normalized or patient can tolerate oral nutrition and medications. Rate: Administer maintenance infusion over 24 hr. ● Additive Compatibility: arginine 10%. ● Additive Incompatibility: Do not mix or infusion other solutions or medications with sodium phenylacetate and sodium benzoate. Patient/Family Teaching ● Explain purpose of medication to parents/caregivers. Evaluation/Desired Outcomes ● Decreased sequelae of hyperammonemia including encephalopathy and death. Why was this drug prescribed for your patient? 䉷 2015 F.A. Davis Company