Conventional Medical Treatment for Porphyria

What is porphyria?

Porphyria is a group of rare disorders that affect the skin (cutaneous porphyria) and nervous system (acute porphyria). It involves a buildup of natural chemicals called porphyrins in the body. Porphyrins are necessary for the production of heme, which is a part of hemoglobin. Hemoglobin carries oxygen to organs and tissues.

In order to convert porphyrins into heme, eight enzymes are needed. Without these enzymes, porphyrins build up in the body. This results in major issues, especially regarding the nervous system and skin.

Conventional medical treatment for porphyria

Treatment is dependent on the type of porphyria and the severity of symptoms. It often includes medications and identifying and avoiding triggers.

Acute porphyria

Treatment for acute porphyria attacks usually involves symptom management and prevention of future complications. This can include, but is not limited to, the following:

• Hospital care for the treatment of symptoms, such as dehydration, vomiting, breathing problems, and severe pain
• IV or oral glucose to ensure carbohydrate intake
• Hemin injection to limit the body’s production of porphyrins
• Givosiran monthly injections for acute hepatic porphyria to reduce the frequency of attacks
• Invasive treatments, including blood transfusion, surgery to remove the spleen, or liver or bone marrow transplant

Cutaneous porphyria

Treatment for cutaneous porphyria is generally focused on the reduction of triggers, which is typically sunlight. It may also involve decreasing the amount of porphyrins in the body. This may be accomplished with the regular removal of blood from the body in order to lower excess iron. The removal of one pint, or approximately 450mL, of blood every two weeks can reduce iron levels. This treatment can result in remission.

In Canada, this procedure can be done at a hospital or possibly at Canadian Blood Services (Canada’s blood bank). In the United States, it can be performed at a physician’s office, hospital, labs, and blood banks.

For those who cannot tolerate the procedures to have blood removed from the body, the medication hydroxychloroquine, a drug typically used for the treatment of malaria, is used. It helps absorb and dispose of extra amounts of porphyrins to prevent buildup in the body.

Additional source: Verywell Health