Version May 2024
Acute intermittent porphyria (AIP); attack: Note: Prior to administration, ensure accurate diagnosis including quantitative urine porphobilinogen (PBG) and delta aminolevulinic acid (ALA).
Adolescent females ≥16 years: IV: Usual dose: 3 to 4 mg/kg/day of hematin once daily for 4 days; for severe cases, more frequent dosing may be necessary and should be separated by at least 12 hours; reported dose range: 1 to 4 mg/kg/day for 3 to 14 days; maximum daily dose: 6 mg/kg/24 hours (Ref).
Dosage adjustment for concomitant therapy: Significant drug interactions exist, requiring dose/frequency adjustment or avoidance. Consult drug interactions database for more information.
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
(For additional information see "Hemin: Drug information")
Porphyria:
IV: 3 to 4 mg/kg once daily for 4 days (Ref).
Manufacturer's labeling: Dosing in the prescribing information may not reflect current clinical practice. IV: 1 to 4 mg/kg/day for 3 to 14 days; maximum: 6 mg/kg per 24 hours.
Dosage adjustment for concomitant therapy: Significant drug interactions exist, requiring dose/frequency adjustment or avoidance. Consult drug interactions database for more information.
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Frequency not defined.
Central nervous system: Pyrexia
Hematologic: Leukocytosis
Local: Phlebitis
Postmarketing and/or case reports: Coagulopathy, fibrin split products increased, hematocrit decreased, hypofibrogenemia, prothrombin time increased, partial thromboplastin time increased, thrombocytopenia
Hypersensitivity to hemin or any component of the formulation
Concerns related to adverse effects:
• Anticoagulation: Transient, mild anticoagulation effects have been observed, although the extent and duration of hypocoagulation have not been determined. Avoid concurrent anticoagulation therapy.
• Iron overload: Repeat administration cycles may result in iron overload; monitor iron and serum ferritin; consider iron chelation therapy if elevated.
• Renal effects: Asymptomatic oliguria, increased nitrogen retention and reversible renal shutdown has been observed (case report) at a single dose above the recommended dose. Recommended dosage guidelines should be followed; worsening of renal function has not been observed with the recommended dose.
Disease-related concerns:
• Porphyria: Clinical benefit depends on prompt administration. Porphyria attacks may progress to result in irreversible neuronal damage. Hemin is intended to prevent attacks from becoming critical; hemin is not effective in repairing neuronal damage resulting from attacks. After discontinuation, although remission may be prolonged, acute porphyria symptoms generally return.
Dosage form specific issues:
• Human plasma: Product of human plasma; may potentially contain infectious agents that could transmit disease, including a theoretical risk of Creutzfeldt-Jakob disease. Screening of donors, as well as testing and/or inactivation or removal of certain viruses, reduces the risk. Infections thought to be transmitted by this product should be reported to Recordati Rare Diseases at 1-888-575-8344.
• Latex: Packaging may contain natural latex rubber.
Other warnings/precautions:
• Appropriate use: Although the manufacturer's labeling recommends initial therapy with glucose (ie, 400 g/day for 1 to 2 days), glucose may be appropriate for mild cases only and hemin should be initiated early for most acute attacks, although glucose may be utilized while awaiting hemin therapy (Anderson 2005). For moderate to severe attacks, immediate hemin treatment is recommended. Follow recommended dosage; excessive single doses have resulted in reversible renal shutdown, with asymptomatic oliguria and increased nitrogen retention. Administer through a large vein (or central line) to prevent phlebitis.
• Preparation in albumin: Reconstitution in albumin 25% (instead of sterile water for injection) is recommended to prevent formation of degradation byproducts and reduce phlebitis, as well as prevent transient coagulopathy/bleeding (Anderson 2006).
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous [preservative free]:
Panhematin: 350 mg (1 ea)
No
Solution (reconstituted) (Panhematin Intravenous)
350 mg (per each): $12,496.03
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous:
Panhematin: 268 mg (1 ea)
IV: Infuse over at least 30 minutes through a 0.45 micron or smaller filter since undissolved particulate will not be visible. Administer through a large vein or central line to prevent phlebitis. After infusion, flush with 100 mL of NS.
IV: For IV infusion only. Administer through a large vein or central line to prevent phlebitis. Preparing the solution in albumin has been reported to decrease phlebitis (Ref).
Products reconstituted with sterile water for injection: Infuse over at least 30 minutes through a 0.45 micron or smaller filter. After infusion, flush with 100 mL NS.
Products prepared with albumin 25% (Anderson 2006): Infuse over at least 60 minutes at a rate not to exceed 1 mL/minute; infuse piggybacked through a line containing NS. Information related to use of an in-line filter is not available; however, a larger filter size may be necessary to avoid slowing the infusion. Following completion of hemin, infuse NS for ~10 minutes to clear the IV line.
Store intact vials at 20°C to 25°C (68°F to 77°F). Solutions prepared in sterile water for injection (SWFI) should be used immediately after preparation. Solutions prepared in albumin 25% should be protected from light and should be infused within 1 hour of preparation (Anderson 2006).
Management of recurrent attacks of acute intermittent porphyria (AIP) temporally related to the menstrual cycle after initial carbohydrate therapy is known or suspected to be inadequate (FDA approved in females ≥16 years and adults)
None known.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the Lexicomp drug interactions program by clicking on the “Launch drug interactions program” link above.
Anticoagulants: Hemin may enhance the anticoagulant effect of Anticoagulants. Risk X: Avoid combination
Barbiturates: May diminish the therapeutic effect of Hemin. Risk X: Avoid combination
Estrogen Derivatives: May diminish the therapeutic effect of Hemin. Risk X: Avoid combination
Use of hemin for acute attacks of porphyria during pregnancy has been described (Anderson 2005; Marsden 2010; Stein 2013). Avoid use in severe pre-eclampsia (theoretical risk of potentiation of the coagulation disorder).
Urinary levels of ALA, uroporphyrin (UPG), PBG, and coproporphyrin (baseline and during therapy); iron and serum ferritin (if multiple doses); serum creatinine and electrolytes (including magnesium) (daily during therapy)
Limits hepatic and/or marrow porphyrin synthesis by inhibiting synthesis of aminolevulinic acid synthase, the enzyme that regulates the porphyrin/heme pathway
Onset: Clinical improvement begins within 1 to 2 days if hemin is initiated early (Anderson 2005).
Excretion: Feces; urine (as metabolites)
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