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Prevention and treatment

Once a person is known to have a particular porphyria, or a relative who carries the same gene but has not yet been affected is identified, there are precautions that can be taken to prevent recurrence or a first attack. For the acute neurological porphyrias such as AIP (acute intermittent porphyria), VP (variegate porphyria) and HCP (hereditary coproporphyria) the most important precaution is avoiding using drugs that are known to precipitate attacks. Lists of known and suspected drugs are available (see the drug lists in the related web pages of this article). If attacks occur regularly related to the menstrual cycle then hormone manipulation to change the menstrual cycle may prevent attacks. Avoidance of strict dieting may be important in some people.

Treatment of acute neurological porphyrias involves identifying and removing any precipitating factors, giving an intravenous infusion of a haem derivative such as haematin or haem 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. Treatment is most commonly by venesection which is removal of blood. Patients who are eligible may become regular blood donors. Others e.g. those with hepatitis or HIV will have similar amounts of blood removed regularly. Removal of blood removes iron from the body and excess iron exacerbates PCT. People who cannot tolerate venesection 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.

Last Review Date: December 21, 2018