Porphyria

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Treatments and drugs

By Mayo Clinic staff

Acute porphyrias
Treatment of acute porphyrias focuses on eliminating symptoms. This may require hospitalization in severe cases. Treatment may include:

  • Stopping medications that may have triggered symptoms
  • Medication to control pain
  • Prompt treatment of infections or other illness that may have caused symptoms
  • Intravenous sugar (glucose) to maintain an adequate intake of carbohydrates
  • Intravenous fluids to combat dehydration
  • Hemin or hematin (heme arginate) injections, medication that is a form of heme, which can reduce the burden placed on your body to produce heme, thus lowering porphyrin levels

Cutaneous porphyrias
Treatment of cutaneous porphyrias focuses on reducing the amount of porphyrins in your body to help eliminate your symptoms. This may include:

  • Phlebotomy. Phlebotomy involves drawing a certain amount of blood from one of your veins. This reduces the iron in your body, which decreases porphyrins. You may need to undergo phlebotomy several times before cutaneous porphyria goes into remission.
  • Activated charcoal. Taken orally, this medication can absorb excess porphyrins and help your body get rid of them more quickly than usual. Doctors also use the medication cholestyramine for this purpose.
  • Beta carotene. Long-term treatment of cutaneous porphyrias may include daily doses of beta carotene or other carotenoid, such as canthaxanthrin. Your body converts beta carotene to vitamin A, which is necessary for healthy eyes and skin. Beta carotene may increase your skin's tolerance to sunlight.
References
  1. Porphyria. National Digestive Diseases Information Clearinghome (NDDIC). http://digestive.niddk.nih.gov/ddiseases/pubs/porphyria/. Accessed March 24, 2009.
  2. Porphyria. Genetics Home Reference. Accessed March 24, 2009.
  3. Sassa S, et al. Understanding the porphyrias. http://www.uptodate.com/home/index.html. Accessed March 24, 2009.
  4. Learning about porphyria. National Human Genome Research Institute. http://www.genome.gov/pfv.cfm?pageID=19016728. Accessed March 24, 2009.
  5. About porphyria. American Porphyria Foundation. http://www.porphyriafoundation.com/. Accessed March 24, 2009.
  6. Anstey AV, et al. Liver diseases in erythropoietic protoporphyria: Insights and implications for management. Gut. 2007;56:1009.
  7. Sassa S, et al. Porphyria cutanea tarda, hepatoerythropoietic porphyria, and toxic porphyria. http://www.uptodate.com/home/index.html. Accessed April 2, 2009.

DS00955

May 9, 2009

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