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She Suffers From Something Called Maple Syrup Urine Disease, And She Just Got Even More Bad News…

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.

Now, a woman suffering from the rare “maple syrup urine disease” bravely came forward, saying that the formula shortage affecting much of the nation could kill her.

28-year-old Hannah Dolins needs a special metabolic formula at every meal to survive because her condition prevents her body from properly breaking down proteins in other foods.

“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. I’m 28 years old. I am not ready to die. But without my specialized metabolic formula, Ketonex-2, I may die. I need my formula to live,” Hannah said in an interview with Insider.

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.

Dolins says that if she does not receive the formula for 24 to 48 hours then her brain will begin to deteriorate and she will likely fall into a coma and die.

“I want the country to know that when I run out of Ketonex-2, hospitalization will be the only way to keep me alive, but it’s a short-term solution that probably won’t save my life. In the hospital, I’ll be given an IV of D10 dextrose to keep my calorie intake up, but that won’t give me any protein,” she explained. “For that, I would need my formula — but the hospitals, too, are running out of it.”

She says that she goes through around three cases of the formula every month. Each case contains six cans, of which she consumes more than half a can every day.

Recent shortages have left her unable to purchase more formula for three months now, and she has since had to dip into the remaining supply she has left.

“I have COVID-19. I need my formula to fight it. My supply is dwindling every day, and I am scared,” she said. “The formula shortage may kill me, and I want everyone in this country to understand that.”

Sources: AWM, Insider



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