Porphyria

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Prevention and Treatment

Once a person is known to have a particular porphyria, there are precautions that can be taken to prevent signs and symptoms from occurring.

For the acute neurological porphyrias such as AIP (acute intermittent porphyria), VP (variegate porphyria) and HCP (hereditary coproporphyria), the most important precaution is to avoid using drugs that are known to precipitate attacks. Lists of drugs that are considered safe and unsafe for people with a neurologic porphryria are available (See the drug lists on the web sites in the Related Pages section of this article.). If attacks are related to the menstrual cycle, hormone manipulation to change the menstrual cycle may prevent attacks. Avoidance of strict dieting may be important in some people.

In people having an attack, treatment involves identifying and removing any precipitating factors, giving an intravenous infusion of a heme derivative such as hematin or heme arginate, and supplying enough nutrition by intravenous infusion if necessary. Intensive medical care may be required in severe cases.

People with PCT (porphyria cutanea tarda) need to avoid alcohol consumption. Sun protection with clothing and complete sunblock lotions is also very important. The treatment for PCT is phlebotomy (removal of blood), which rids the body of excess iron. People who are eligible (i.e., do not have hepatitis or other transmissible disease) may become regular blood donors. People who cannot tolerate phlebotomy may be treated with chloroquine to help remove excess porphyrins.

Children and adults with EPP (erythropoietic protoporphyria) benefit from sun protection using thick clothing. Conventional sunscreens have limited effectiveness. Treatment with beta-carotene reduces sun sensitivity and skin symptoms.

People with VP and HCP need to take the same precautions as people with AIP to avoid neurological symptoms. They also need sun protection with clothing and complete sunblock lotions if they suffer from sun sensitivity.

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