Ongoing formula shortage has life or death impact for PKU patients

CHARLOTTESVILLE, Va. (WVIR) - The formula shortage is impacting not just babies, but an older demographic that relies on it in day-to-day life.

A dietician from UVA Health says her patients need a medical formula because they cannot process the proteins found in most foods.

This means that the backed up formula supply chain can be a life threatening matter for Phenylketonuria (PKU) patients.

“It’s a genetic condition that affects the body’s ability to break down a specific amino acid called phenylalanine that is found in dietary protein,” UVA Health Dietician Rachel Murray said. “Dietary management is the primary therapy for PKU, and it’s a lifelong therapy. Individuals must follow a restricted protein diet.”

This means that PKU patients must drink a special formula because they are unable to process the protein in most food. The shortage however, is making that formula nearly impossible to find.

Jackson Miller was diagnosed with PKU at birth, and he will need formula for his entire life.

His mother, Susan Miller, helps her son manage PKU, and she is hoping that supply starts meeting demand soon, as it is critical for 16-year-old Jackson’s life.

“It’s been really hard. I haven’t been able to get an order since I think the end of March. Luckily, UVA has sent me a few of what they call ‘bridge orders.’ My dietician has tried to find other clients maybe who had the same formula but didn’t need it,” Miller said.

Without this formula, PKU patients’ blood concentration in their brain spikes, leading to symptoms such as tremors, intellectual disabilities, brain fog, and getting lost going to routine places.

“Jackson has been little worried coming to me and saying: ‘Mom is something gonna happen to my brain?’” Miller said. “...how I think, or how I’m growing, and it’s been hard for him too.

Murray says that there are only a few companies out there that produce these specialty formulas, with one being Abbott Laboratories, which has been at the center of the nationwide shortage.

“Insurance coverage is generally very poor for these, and so when changing formulas, we have to go back and forth with the insurance company, and there’s limited to no coverage for these in Virginia. There’s a really high out of pocket cost for some of these alternatives, so our patients can’t afford to switch,” Murray said. “We need to help them find that formula or they will die.”

Copyright 2022 WVIR. All rights reserved.

Do you have a story idea? Send us your news tip here.