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PORPHYRIAS TREATMENTS |
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Treatment for porphyria revolves around
avoiding acute attacks, limiting potential effects, and treating
symptoms. However, treatment options vary depending on the type of
porphyria that has been diagnosed. Given the rarity of ALA
dehydratase porphyria (six reported cases), definitive treatment
guidelines have not been developed. Acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria Treatment for acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria follows the same basic regime. A person who has been diagnosed with one of these porphyrias can prevent most attacks by avoiding precipitating factors, such as certain drugs that have been identified as triggers for acute porphyria attacks. Individuals must maintain adequate nutrition, particularly in respect to carbohydrates. In some cases, an attack can be stopped by increasing carbohydrate consumption or by receiving carbohydrates intravenously. If an attack occurs, medical attention is needed. Pain is usually severe, and narcotic analgesics are the best option for relief. Phenothiazines can be used to counter nausea, vomiting, and anxiety, and chloral hydrate or diazepam is useful for sedation or to induce sleep. An intravenously administered drug called hematin may be used to curtail an attack. It seems to work by signaling the heme biosynthesis pathway to slow production of precursors. Women, who tend to develop symptoms more frequently than men owing to hormonal fluctuations, may find hormone therapy that inhibits ovulation to be helpful. Congenital erythropoietic porphyria The key points of congenital erythropoietic porphyria treatment are avoiding exposure to sunlight and preventing trauma to and infections of the skin. Liberal use of sunscreens and taking beta-carotene supplements can provide some protection from sun-induced damage. Medical treatments such as removing the spleen or administering red blood cell transfusions can have short-term benefits, but they do not offer a cure. Oral doses of activated charcoal may offer the potential of remission. Porphyria cutanea tarda As with other porphyrias, the first line of defense is the avoidance of precipitating factors, especially alcohol. Regular blood withdrawal is a proven therapy for pushing symptoms into remission. If an individual is anemic or cannot have blood drawn for other reasons, chloroquine therapy may be used. Erythropoietic protoporphyria Avoiding sunlight, using sunscreens, and taking beta-carotene supplements are typical treatment options for erythropoietic protoporphyria. The drug, cholestyramine, may reduce the skin's sensitivity to sunlight as well as the accumulated heme precursors in the liver. Liver transplantation has been used in cases of liver failure, but it has not effected a long-term cure of the porphyria. Alternative treatment Acute porphyria attacks can be life-threatening events, so it is ill-advised to try self-treatments in these situations. Alternative treatments can be useful adjuncts to conventional therapy. For example, some people may find relief for the pain associated with acute intermittent porphyria, hereditary coproporphyria, or variegate porphyria through acupuncture or hypnosis. Relaxation techniques, such as yoga or meditation, may also prove helpful in pain management. |
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