What's UP Doc?

What's UP Doc? Is a monthly column where we feature a patient question along with a response from a member of the UPA Scientific Advisory Board.

Do you have a question you would like to ask a Porphyria Expert?  Send us an email at info@porphyria.org. We’d love to hear from you!

In the September What's UP Doc, it said if you have acute hepatic porphyria your liver function should be checked annually. Why is it important to have your liver function checked? Is there anything you can do to prevent issues with your liver?

Liver function is checked with a blood test that measures liver enzymes in the blood. Elevated liver enzymes can be temporary or indicate a liver disease. Establishing a baseline of liver function identifies what is normal for you and can help determine if your liver function is changing over time. During an attack of acute porphyria, liver enzymes may also increase, but this increase is usually minor and temporary.

You should also have your liver function checked if you are considering treatment with givosiran or prophylactic (preventative) hemin. Increased liver enzymes have been reported with givosiran, so it is important that it is monitored during treatment. Hemin can increase the iron stores in your liver over time, which can also impact liver function.

I also want to mention that with acute hepatic porphyria, there is a significant risk of liver cancer compared to the general population. This isn’t something to lose sleep over. Liver cancer is very treatable if it is caught early, which is why it is recommended you have a screening ultrasound every 6 months after you turn 50.

In terms of protecting your liver, here are some steps you can take:

• Get vaccinated for hepatitis A and B
• Avoid unnecessary medications.
• Do not use herbal and dietary supplements without a discussion with your doctor.
• Ideally, you should not take any alcohol.  If you must, limit alcohol intake to less than 5 standard drinks per week in women and less than 10 standard drinks per week in men. This is important both for preventing porphyria attacks and protecting your liver.
• Live a healthy lifestyle: exercise and eat well.

Thank you to Dr. Thapar for this What's UP Doc? answer! Do you have a question for a porphyria expert? Send it to info@porphyria.org.