Hereditary Coproporphyria Treatment & Management

Updated: Jan 21, 2015
  • Author: Thomas G DeLoughery, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Treatment

Approach Considerations

The goals in managing an acute attack of porphyria are to decrease heme synthesis and to reduce the production of porphyrin precursors. [8, 9] For mild attacks (ie, mild pain and no vomiting, paralysis, or hyponatremia), guidelines from the advise that a high-carbohydrate diet (eg, with glucose-containing drinks and high-energy foods) and supportive measures may be used for up to 48 hours. [7]

High oral doses of glucose (400 g/d) can inhibit heme synthesis and are useful for the treatment of mild attacks. Intravenous glucose solutions (eg, 5% or 10% dextrose in water) can be used in patients who cannot eat, but may aggravate hyponatremia.

Treat severe attacks, especially those involving severe neurologic symptoms, with hematin at a dose of 4 mg/kg/d for 4 days. Patients with severe attacks should be hospitalized for symptom control and monitoring of fluid and electrolyte balance, as well as cardiovascular, respiratory, and neurologic function. [7]

Pain control is best achieved with narcotics; high doses are typically required. Administer laxatives and stool softeners with the narcotics to avert exacerbating the patient's constipation.

For seizure control, administer gabapentin. Most of the classic antiseizure medications are contraindicated in acute attacks of porphyria. However, the British and Irish Porphyria Network, while acknowledging that the safety of intravenous diazepam is controversial in porphyria attacks, concludes that benefit outweighs risk in this acute situation. [7]

Treatment options for other manifestations are as follows [7] :

  • Nausea and vomiting - Prochloperazine, promazine, or ondansetron.
  • Severe agitation and anxiety - Chlorpromazine
  • Hypertension and tachycardia - Atenolol, propranolol or labetalol (but use with caution and monitor for hypotension and bradycardia)

Unlike porphyria cutanea tarda, the skin disease in coproporphyria does not respond to phlebotomy or antimalarial drugs.

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Diet

Patients should receive a high-carbohydrate diet during the attack. Administer intravenous glucose if patients cannot eat. Between attacks, patients should eat a constant balanced diet rather than one that is extremely rich in glucose.

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