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.
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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:
"It’s been really eye-opening to see how important patient involvement is in rare disease. Patients can drive the field, move the field. Rare diseases like porphyria are very life-altering. Patients have an important role in how the regulatory agencies and how physicians are looking at the disease and what the outcomes are."
Question: I have AIP. I haven’t had a porphyria attack for several years and I don’t have a porphyria specialist. Is there anything I should ask my doctor to check for during my annual check-up?
Question: My daughter (now age 26) has AIP. How will acute porphyria attacks impact her liver as she ages? She was diagnosed a few years ago and her last attack was several months ago.