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Acute Porphyria

Medically reviewed by Last updated on Oct 3, 2022.

What is acute porphyria?

Acute porphyria is a disorder that affects how your body makes red blood cells (RBC). Your body needs a chemical called porphyrin to make heme, a part of RBC that carries oxygen. Porphyria prevents your body from creating enough enzymes to control the process, and porphyrin builds up. High levels of porphyrin can cause problems throughout your body, depending on where it builds up. Low levels of heme can also cause organ damage, because your blood cannot bring the organs enough oxygen.

What can trigger an acute porphyria attack?

An acute porphyria attack may happen for no reason. The following can trigger an acute porphyria attack:

  • Certain medicines
  • Sun exposure
  • Cigarettes and alcohol
  • Hormone changes, especially during pregnancy or a monthly period
  • Physical or mental stress
  • Dehydration, fasting, or crash dieting

What are the signs and symptoms of an acute porphyria attack?

Abdominal pain is the most common symptom of an acute porphyria attack. The pain is usually located in the lower abdomen, and may last for hours to days. You may also have any of the following:

  • Pain, especially in your arms, legs, back, chest, neck, or head
  • Constipation or diarrhea
  • Dark or reddish urine or problems passing urine
  • Muscle weakness or twitching
  • Problems thinking clearly, such as confusion, memory loss, or hallucinations
  • Seizures or vomiting
  • Trouble breathing, fast heartbeat, or high blood pressure

How is acute porphyria diagnosed?

Your healthcare provider will ask about your symptoms. Your provider will ask about your medical history and if any family members have porphyria or other blood disorders. Samples of your blood, urine, or bowel movement may be collected. Blood tests check for the enzymes needed to control RBC production, and your porphyrin level.

How is acute porphyria treated?

Certain medicines can relieve your symptoms. These may include certain medicines to treat high blood pressure, seizures, pain, nausea, or vomiting. You may also need any of the following:

  • Hemin: Hemin, also called hematin, is an enzyme made from RBC. It works by telling the body to slow down the production of the building blocks that make heme.
  • Glucose: Glucose may be given if your attacks are triggered by a low-calorie or low-carbohydrate diet.

Treatment options

The following list of medications are in some way related to or used in the treatment of this condition.

How can I prevent an acute porphyria attack?

  • Do not smoke cigarettes or drink alcohol: Cigarettes and alcohol can make your acute porphyria worse. They may also damage the liver and further worsen your problems. Talk to your healthcare provider if you have trouble quitting smoking or drinking.
  • Eat enough food: You can trigger an attack if you do not eat enough, especially if you cut out carbohydrates. Ask your healthcare provider how much you should eat each day. You may need to work with a dietitian to create a food plan.
  • Reduce stress: Try to decrease or avoid stress. Ask healthcare providers how you can learn to relax.
  • Be careful with medicines: Certain medicines can trigger an acute porphyria attack, so ask your healthcare provider about the medicines you take.

What are the risks of acute porphyria?

  • Treatment for acute porphyria may cause side effects. Some medicines may even make your signs and symptoms worse. You may have a headache, rash, itchiness, or slow heartbeat. Hematin can make your veins swell and cause kidney or bleeding problems.
  • Left untreated, acute porphyria can be life-threatening or damage your brain, liver, muscles, or kidneys. The earlier acute porphyria is found and treated, the better the chances of preventing future problems. Your health, quality of life, and ability to function may decrease without treatment.

When should I contact my healthcare provider?

Contact your healthcare provider if:

  • You vomit everything you eat or drink.
  • You have a fever.
  • You get a skin blister or lesion when your skin is exposed to light.
  • You have questions or concerns about your condition or care.

When should I seek immediate help?

Seek help immediately or call 911 if:

  • You have pain in your abdomen, chest, or back.
  • You have signs of dehydration, such as urinating less or not at all.
  • You cannot eat or drink.
  • You are confused, groggy, or weak.
  • You have a seizure.
  • You have trouble breathing or walking.
  • You cannot move one side of your body.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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