It is important to understand what are the potential triggers of acute porphyria attacks. That way, you may be better able to avoid them if you have porphyria.
In addition to genetic risks, environmental factors may trigger the development of porphyria and these painful attacks.
What is porphyria?
An acute porphyria attack comes on suddenly and is marked by severe pain, especially in the stomach, but also in the chest, back, or limbs. It also can lead to breathing problems, seizures, nausea and vomiting, dehydration, anxiety or other mental changes, and high blood pressure. In acute porphyria, the buildup of porphyrins can damage the nervous system.
In cutaneous porphyria, porphyrins build in the skin. Symptoms include pain-causing sun sensitivity, sudden painful skin redness and swelling, blisters on exposed skin, and itching. Red or red-brown urine and excessive hair growth in affected areas also are symptoms of cutaneous porphyria.
What are disease triggers?
A trigger, or precipitating event, is generally something that sets off a disease in people who are genetically predisposed to it. It also could be something that brings on a particular symptom in a person with an established disease.
What triggers porphyria?
Many people with a disease-associated mutation in the HMBS gene do not necessarily develop acute porphyria symptoms. Additional factors — triggers — are also necessary. When exposed to the trigger, the body’s demand for heme production increases. This overwhelms the deficient enzyme, setting in motion a process that leads to porphyrin accumulation.
Of note, attacks can develop over hours or days, and last for days or weeks.
Not all triggers of acute porphyria attacks are the same for each person, and susceptibility to specific triggers may vary during a patient’s lifetime. Such factors can include certain medications, alcohol, and a low-calorie diet.
Patients with acute intermittent porphyria — the most common type of acute porphyria — should be careful in their use of medications. Always check with your physician to see whether a medicine or remedy, including over-the-counter treatments or supplements, is safe for you.
Common drug triggers can include barbiturates, tranquilizers, sedatives, and birth control pills.
Research has shown that alcoholic drinks can cause acute attacks, and patients experiencing any symptoms would do well to stay away from alcohol.
Low-calorie diets and prolonged periods with little food (fasting) may provoke an acute attack. It’s important to maintain a normal diet and healthy body weight. Eating smaller meals more often can help.
People with severe porphyria, especially those who have recurrent attacks, may need special dietary advice from their physician and a dietician. If you are overweight and wish to lose weight, speak with your physician about how you can do so gradually and safely.
Other acute porphyria attack triggers can include smoking, physical stress due to infections or other illnesses, and emotional stress.
Last updated: Feb. 2, 2021
Porphyria News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.
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