Woman suffering from rare ‘maple syrup urine disease’ says she could die from formula deficiency

One woman who suffers from the rare “maple syrup urine disease” says the lack of formula that afflicts much of the country could kill her.

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

He goes through about three cases of formula Ketonex-2 each month, but due to a nationwide shortage of supplies, he has not been able to purchase a new case for three months.

He’s running low on supplies, and he tells Insider he fears the famine will lead to death. A recent COVID-19 infection also made matters worse.

Although she is an adult, many parents across the country tell similar stories of how these recent famines have placed them in a place where they take care to feed their babies every day.

Dolins (pictured) warned she could die if bottle feeding ends due to a nationwide famine

‘I am 28 years old. I’m not ready to die. But without my special metabolic formula, ketonex-2, I could die. I need my formula to live,” he wrote in an essay.

Note that the rare condition only affects about 2,000 people in the United States each year.

It is called “maple syrup” because patients often have sweet-smelling urine.

This prevents your body from breaking down amino acids, minerals, and vitamins found in many common foods.

Dolins says that if he doesn’t receive the formula within 24-48 hours, his brain will begin to deteriorate and he will likely fall into a coma and die.

I want the country to know that if I run out of ketonex-2, hospitalization is the only way to keep me alive, but it’s a short-term solution that probably won’t save my life. At the hospital I take D10 drops with dextrose to keep my calorie intake high but not protein from it,” she explained.

“I need my formula for this, but hospitals are running out.”

He says he goes through about three samples of the formula each month. There are six cans in each and he consumes more than half a can every day.

Recent shortages have left him unable to purchase multiple formulas for three months, and he has since had to start using the inventory he has left.

Many shelves across the country are empty due to formula shortages. It started when the FDA closed an Abbott facility in Michigan due to poor sanitation.

“I have Covid-19. I need my formula to fight it. My stocks are getting low and I’m scared,” she says.

“The formula shortage can kill me, and I want everyone in this country to understand that,” he said.

The baby food shortage is the result of a relatively small list of companies that manufacture the product in the United States and high tariffs that prevent it from being imported.

Abbott Nutrition is one of the four largest fine producers in America. Earlier this year, the Food and Drug Administration (FDA) forced the company to close a factory in Sturgis, Michigan, due to conditions that executive Robert Califf described as “extremely unsanitary.”

But this factory is critical to the US formula supply chain, as Claire Kelloway of the Open Markets Institute, National Public Radio, explains.

“They’re intensifying production in a few very large factories, but that comes with a lot of risk,” he said.

“Most of the crisis we’re seeing right now is due to a factory closing.”

In response to the shortages, the FDA has allowed Abbott to reopen the plant soon and will allow imports of formulas from abroad.

Due to the factory closure, there are very few boxes of baby food in the United States.

To alleviate the problem, the FDA has partnered with Abbott to get the facility back up and running and is expected to be operational on June 6.

Restrictions on imports of foreign formulas were also relaxed, as the government hoped to buy from abroad to alleviate the problem.

Dolins says the FDA hasn’t been transparent about this process, leaving people like him in the dark.

“I understand why the FDA should stop making Abbott’s formulas to make sure they’re safe,” he said.

“But what I don’t understand is why the FDA isn’t providing people like me with more information about what’s going on in our single food source.”