How should I manage a patient with porphyria who presents with acute abdominal pain and is at risk of an attack?

Management of a patient with porphyria presenting with acute abdominal pain and at risk of an attack involves prompt recognition and supportive care to prevent progression. Initial steps include immediate cessation of any precipitating factors such as certain drugs, alcohol, or fasting, as these can trigger an acute attack Pischik & Kauppinen 2015. Supportive management should focus on pain control using safe analgesics, intravenous glucose administration to suppress hepatic porphyrin synthesis, and correction of electrolyte imbalances, particularly hyponatraemia, which is common during attacks NICE CG143 Pischik & Kauppinen 2015.

Hospital admission is often necessary for close monitoring and treatment. If symptoms worsen or neurological signs develop, intravenous hemin therapy should be considered as it is the specific treatment to reduce the production of porphyrin precursors and shorten the attack duration Pischik & Kauppinen 2015. Avoidance of known porphyrinogenic drugs is critical throughout management NICE CG143.

In summary, management integrates prompt identification, removal of triggers, supportive care with glucose and analgesia, correction of metabolic disturbances, and use of hemin in severe cases to prevent complications and progression of the acute porphyria attack NICE CG143 Pischik & Kauppinen 2015.