Inborn errors of metabolism:
Indications for: PANHEMATIN
Amelioration of recurrent attacks of acute intermittent porphyria temporally related to the menstrual cycle in susceptible women, after initial carbohydrate therapy is known or suspected to be inadequate.
Limitations of Use:
Intended to prevent an attack from reaching the critical stage of neuronal degeneration. Not effective in repairing neuronal damage.
Consider carbohydrate loading (eg, 400g glucose/day for 1–2 days) before initiation. If improvement unsatisfactory, give 1–4mg/kg/day of hematin via IV infusion over at least 30mins for 3–14 days. In more severe cases, this dose may be repeated no earlier than every 12hrs. Max 6mg/kg of hematin per 24hrs.
<16yrs: not established.
Administer into large arm vein or a central venous catheter to avoid phlebitis. Contains human plasma; monitor for possible infection transmission (eg, viruses, Creutzfeldt-Jacob disease agent). Monitor iron and serum ferritin in multiple administrations. Latex allergy. Pregnancy (avoid in severe pre-eclampsia). Nursing mothers.
Delta-aminolevulinic acid synthetase inhibitor.
Avoid CYP-inducing drugs that increase the activity of δ-aminolevulinic acid synthetase (eg, estrogens, barbituric acid derivatives, steroid metabolites). Avoid concomitant anticoagulants.
Headache, pyrexia, infusion site reactions, phlebitis.