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Social Editor and Staff Writer
A shortage of infant formula in the United States has put enormous strain on families desperate to keep their babies fed. While the US Food and Drug Administration (FDA) has issued a warning for parents not to resort to making their own, there’s another group for whom the shortage is potentially deadly: people with maple syrup urine disease.
People with maple syrup urine disease can't break down proteins into amino acids, so they need to supplement their diet with formulas containing them. Without these formulas, they can develop life-threatening brain swelling called cerebral edema, making the formula crisis a catastrophic situation for people who rely on products that have been recalled and remain in short supply.
Maple syrup urine disease is a rare genetic disorder occurring in around 1 in every 185,000 births worldwide. People with the disorder lack an enzyme complex needed to break down proteins, leading to an abnormal accumulation of them in the blood.
It's known as maple syrup urine disease simply because it makes people's urine smell like maple syrup.
It’s usually detected within the first two days of life as babies develop increasingly severe neurological symptoms, but can be managed with the right diet. This involves a protein-restricted diet and rigorous control of the ingestion of three branched-chain amino acids (BCAAs) called leucine, isoleucine, and valine.
Special formulas can be used to complement a maple syrup urine disease diet – as is the case for Hannah Dolins, who spoke to Insider about her difficulty accessing the life-sustaining formula.
“The last three months have been some of the most stressful of my life — because from the moment I heard about the Abbott formula recall, I was terrified,” she said, “without my specialized metabolic formula, Ketonex-2, I may die. I need my formula to live.”
Dolins requires three cases of Ketonex-2 formula to get the daily dosage of processed amino acids she needs to stay healthy. but has been unable to get hold of even one for three months. Without it, Dolins says she could experience brain swelling within 24 to 48 hours that could prove life-threatening without intervention – but even hospitals are running out of metabolic-issue-specific formula.
The product has disappeared from shelves following a recall led by the FDA, compounded by an ongoing global supply chain crisis. Recalls of three products from an Abbott Nutrition factory in Michigan began in February 2022 over a string of Cronobacter infections suspected to be linked to the products.
While recalls like this are a normal part of FDA regulation, the effects of this one have been worsened by factors including the COVID-19 pandemic which saw a surge in stockpiling from understandably concerned parents. There was then a brief pause in production while demand fell – but as it sparked back up again in 2022, the factories were unable to fulfill orders.
For now, it remains unclear when the formula crisis will be resolved, but for the families of both infants and people with metabolic disorders, it presents a life-threatening situation.
Social Editor and Staff Writer
Rachael is a science writer and social editor for IFLScience with a Zoology degree from the University of Southampton, UK, and a nose for novelty animal stories.
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