RARE Daily

Food for Thought

August 24, 2022

There’s been a lot of discussion about rising prices at the grocery store, but imagine paying $13 for a box of spaghetti, $15 for a loaf of bread, or $18 for a two-pound bag of flour. That’s the reality for people with the rare metabolic condition phenylketonuria, who must rely on medical formula and medical foods to avoid protein they are unable to properly break down.

Kala McWain’s son Braxton was only a few days old when he was diagnosed with phenylketonuria (PKU) through newborn screening. People with the condition PKU have a deficiency of phenylalanine hydroxylase (PAH), an enzyme that is needed to metabolize Phe, an essential amino acid that is present in most protein-containing foods. In the absence of adequate PAH, Phe accumulates in the blood and becomes toxic to the brain. It can cause a variety of complications including severe intellectual disability, seizures, tremors, behavioral problems, and psychiatric symptoms. Though there are existing therapies on the market, Braxton is unable to use them because of his age and his particular type of PKU.

In addition to caring for Braxton, now 2, McWain spends her time fighting with insurance companies and lobbying lawmakers. Like other people with PKU, Braxton relies on medicinal formula and medical foods to get nourishment his body needs without being exposed to Phe. Formula can cost $1,000 a month and medical foods are four to five times the cost of their everyday counterparts. The problem is that it is insurance companies, not doctors, who make the determination about the medical necessity of these items. It’s a fact that has turned McWain into a patient advocate.

As the current legislative session moves into its final months, McWain has been meeting with staff of her two senators and her congressional representative in Wyoming with the hope of winning their support for the Medical Nutrition Equity Act, legislation that would require insurance companies to extend the same type of coverage they have for drugs to medical foods. The need extends beyond people with PKU, who must adhere to a lifelong regimen of medical formulas and foods, but also to people with other inborn errors of metabolism, as well as more common digestive diseases. Current laws vary state-by-state with 38 states having some type of legislation in place, but it is inconsistent.

“There’re people who get divorced because coverage would be better. There’re people that have moved states because there’s better coverage in a different state. There’re people who leave their dream jobs or are stuck in a job they’re miserable in because of the coverage they have,” said McWain, who lives in Casper, Wyoming today, where her husband works in the oil and gas industry. “I’m originally from Wisconsin. If we moved back to Wisconsin, we would have free formula from the state. We would have a portion of free medical foods from the state, and all of our labs would be covered by the state. But at this point we pay for everything subject to deductible or don’t have coverage at all.”

Insurance companies generally don’t cover medically necessary nutrition. When they do, they sometimes require that the formulas be administered through a nasogastric tube, or through a surgically placed tube directly into the stomach, according to Patients & Provider for Medical Nutrition Equity, a group of about 40 patient and physician organizations supporting passage of the Medical Nutrition Equity Act.

The act would require public and private insurance cover medically necessary foods as a treatment option. It would only cover patients for whom medically necessary nutrition is used as a treatment. Proponents note that there is precedent for such action. In 2016, Congress passed legislation that provided medical nutrition coverage for military families covered under TRICARE, the healthcare program for service members and their families.

Sarah Chamberlin, executive director of National PKU News said while efforts to pass the Medical Nutrition Equity Act or similar legislation have persisted for about ten years, people haven’t appreciated the need given the obscurity of conditions like PKU. The recent infant formula shortage, though, has helped create some newfound appreciation.

“For better or for worse, the formula crisis has helped us because I took my can of formula off my desk and took it to my congresswoman’s office yesterday and held it up because now Americans and legislators understand the value of that white powder in a can,” she said. “They understand that it is essential for life and that’s where we’ve been able to make inroads and say, “Okay, now you got it for all the newborns. You need to understand that our community uses this for life. We don’t just use it for nutrition, but we use it for medical treatment.

Representative James McGovern, D-Massachusetts, is the lead democratic sponsor in the house. He is also involved in the White House Conference on Hunger, Nutrition, and Health that’s scheduled for the end of September. Advocates have been working with his staff and people at the U.S. Department of Health and Human Services to get medical nutrition on the agenda and Chamberlin is hoping proponents of the bill will be able to capitalize on that and use momentum from the event to get the legislation passed.

In the meantime, McWain, Chamberlin, and other advocates have been paying visits to the representatives during the August recess. In the past, she’s brought a one-pager and a can of formula, but now she’s brining her daughter who has PKU to these meetings, to show them what this is about.

“You were able to see in the baby formula shortage all those cute babies, all those newborns, and all those parents driving around, searching the shelves. There was that human face on it,” she said. “If we can put our human faces on it and put our stories together, I think there’s a chance that we can move it along this term.”

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