Porphyria

Alternative names:

acute intermittent porphyria; congenital erythropoietic porphyria; erythropoietic protoporphyria; hereditary coproporphyria

Treatment:

During an acute attack, these treatments can be expected.

• maintain electrolyte balance
• monitor blood gasses
• increase carbohydrate intake (may be IV glucose solutions)
• pain medication as needed
• sedation for restlessness and agitation
• propranolol for hypertension
• intravenous hematin

Long-term management:

• avoid all alcohol
• avoid drugs which may precipitate an attack
• high carbohydrate diet
• avoid sunlight
• sunscreen (if not contraindicated)
• avoid skin trauma
• beta-carotene
• consider splenectomy

Expectations (prognosis):

The porphyrias are life-long intermittent illnesses, but with good long-term management, the affected person can expect long problem-free periods.

Complications:

• progressive muscle paralysis
• respiratory failure
• gallstones
• disfigurement
• coma

Calling your health care provider:

Call your health care provider if you have any of the above symptoms suggestive of a porphyria. If you have already been diagnosed with a porphyria, call your health care provider at the first signs of an acute attack.