Treatment

Treatment depends on the type of porphyria you have and the severity of symptoms. Treatment includes identifying and avoiding symptom triggers and then relieving symptoms when they occur.

Avoiding triggers

Avoiding triggers may include:

• Not using medications known to trigger acute attacks. Ask your doctor for a list of safe and unsafe drugs.
• Not using alcohol or recreational drugs.
• Avoiding fasting and dieting that involves severe calorie restriction.
• Not smoking.
• Taking certain hormones to prevent premenstrual attacks.
• Minimizing sun exposure. When you're outdoors, wear protective clothing, and use an opaque blocking sunscreen, such as one with zinc oxide. When indoors, use window filters.
• Treating infections and other illnesses promptly.
• Taking steps to reduce emotional stress.

Acute porphyrias

Treatment of acute porphyria attacks focuses on providing rapid treatment of symptoms and preventing complications. Treatment may include:

• Injections of hemin, a medication that is a form of heme, to limit the body's production of porphyrins
• Intravenous sugar (glucose), or sugar taken by mouth, if able, to maintain an adequate intake of carbohydrates
• Hospitalization for treatment of symptoms, such as severe pain, vomiting, dehydration or problems breathing

In 2019, the FDA approved givosiran (Givlaari) as a monthly injection for adults with acute hepatic porphyria to reduce the number of porphyria attacks. But it’s important to discuss safety information and potential serious side effects with your doctor. These include but are not limited to nausea, liver and kidney toxicity, and a small risk of anaphylaxis.

Cutaneous porphyrias

Treatment of cutaneous porphyrias focuses on reducing exposure to triggers such as sunlight and reducing the amount of porphyrins in your body to help eliminate your symptoms. This may include:

• Periodically drawing blood (phlebotomy) to reduce the iron in your body, which decreases porphyrins.
• Taking a drug used to treat malaria — hydroxychloroquine (Plaquenil) or, less often, chloroquine (Aralen) — to absorb excess porphyrins and help your body get rid of them more quickly than usual. These medications are generally used only in people who can't tolerate a phlebotomy.
• A dietary supplement to replace vitamin D deficiency caused by avoidance of sunlight.