Givlaari for acute porphyria

What's UP Doc?
Written By Dr. Bruce Wang

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

I am thinking about starting Givlaari injections. What tests should I have done before and during my treatment?

This week our response was provided by Dr. Bruce Wang of UCSF.

Prior to starting Givlaari: confirm AHP diagnosis both biochemically (urine ALA and PBG) and genetically. There should be multiple ALA and PBG results since patients should only consider Givlaari if they have experienced multiple acute attacks. This helps establish the patient’s own baseline as well as levels during attacks. Additional baseline tests before starting Givlaari include blood tests for liver function, creatinine and homocysteine.

Before each Givlaari injection (within a few days): urine test for ALA, PBG, and creatinine. The goal is to ensure that ALA and PBG are well controlled at the current Givlaari interval.

Other tests to consider while on Givlaari: Patients should be tested for liver function tests to look for liver toxicity, serum creatinine for renal toxicity, and homocysteine levels. The optimum frequency and intervals remain to be determined.

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

 

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