Wednesday, January 4, 2017

Other Important Treatment Considerations for the Acute Porphyrias

Other Important Treatment Considerations for the Acute Porphyrias

  • Hospitalization is often necessary for acute attacks. Medications for pain, nausea, and vomiting, as well as close observation are generally required.
  • A high intake of glucose or other carbohydrates can help to suppress disease activity and can be given by vein or by mouth.
  • Attacks are often precipitated by low intake of carbohydrates and calories in an attempt to lose weight. Thus dietary counseling is very important.
  • Premenstrual attacks often resolve quickly with the onset of menses. Hormone manipulations may prevent such attacks.
  • Acute porphyria is particularly dangerous if the diagnosis has not been made and if harmful drugs are administered. The prognosis is usually good if the disease is recognized and if treatment and preventive measures are begun before severe nerve damage has occurred. Although symptoms usually resolve after an attack, some patients develop chronic pain. Nerve damage and associated muscle weakness can improve over a period of months or longer after a severe attack. Mental symptoms may occur during attacks but are usually not chronic.
  • Wearing a MedicAlert® bracelet is advisable for patients who have been diagnosed with porphyria.

Bibliography:
Anderson KE. The porphyrias. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. Philadelphia, Pa: WB Saunders Co; 2004: chap. 223.
Bonkovsky HL, Healey BS, Lourie AN, Gerron GG. Intravenous heme-albumin in acute intermittent porphyria: evidence for repletion of hepatic hemoproteins and regulatory heme pools. Am J Gastroenterol. 1991;86:1050-1056.
Data on file, Recordati Rare Diseases, Inc.

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3 comments:

  1. For just over 40 years I've been using zinc sulphate to deactivate the PBG my AIP produces that poisons my nervous system, about 12-15 mg orally when I have symptoms. It's cheap, effective, available.

    ReplyDelete
  2. This comment has been removed by the author.

    ReplyDelete
  3. What a great suggestion. I want to try this and see if it will help. What brand are you using that helps? Also, do you have any idea or theory as to exactly why it helps? Thank you so much.

    (I tried to edit this earlier to add an additional question... instead it deleted it. Sorry.)

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