CHEYENNE — Little did Kala McWain know she would be lobbying federal lawmakers after the birth of her youngest son or that she would not get their support.
She was at Casper in Wyoming Medical Center two years ago when medical staff came to her hospital room to take her son for a newborn screening. It was the same step taken there after the birth of their first son and she had no qualms when she and her husband Jake were told they would have the results in a few days.
The tide turned when McWain received a call from a nurse the next day.
She was informed that her son had tested positive for a genetic disorder called phenylketonuria, often referred to as PKU. She was asked to return to the hospital for further testing, was given little information other than that it was a rare condition and was told to wait the weekend at home for more information.
McWain turned to Google for answers. She was afraid of breastfeeding and had little hope.
This prompted her to travel to urge more people with the disorder to be covered by medical food and infant formula insurance. As one of the supporters of a pending bill in Congress to that effect told the Wyoming Tribune Eagle, obtaining the necessary food, which is expensive, could be a matter of life and death.
Your son is either missing or has a defective enzyme needed to break down part of a protein called phenylalanine. According to the National PKU Alliance, he is one of approximately 16,500 people in the United States who cannot eat or process regular foods.
If the condition is not recognized or newborn screening is not done, a child may not reach the first month mark or many other important growth milestones, according to McWain.
Once diagnosed, it doesn’t get any easier.
“It’s a pretty traumatizing introduction to parenthood. My first child had PKU, so I learned how to be a mother and how to take care of those really special needs,” said Heidi Maxfield, director of the Intermountain PKU and Allied Disorders Association. “And then there are other parents who have had a bunch of kids and never knew they had the PKU gene, and they’re thrown into this world where they’re raising a kid with a metabolic disease.”
There is no treatment other than through proper nutrition. Left untreated, people with PKU develop high levels of phenylalanine in their blood, and their brain development and function is impaired. McWain, Casper’s mother, described the possibility of seizures, institutionalization, an inability to walk or develop fine motor skills, and in some cases death.
As patients age, they should maintain their diet. Teens and adults will struggle to get jobs and good grades while without them they will suffer from lightheadedness, irritability, headaches, and general nausea.
The price of avoiding these consequences is high. McWain’s son will have to live on infant formula and medically approved groceries for the rest of his life, which can cost well over $1,000 a month. Although her family has insurance that covers the formula, it doesn’t cover medicinal foods like bread or pasta, and he won’t be insured after a certain age, once he’s an adult.
“A loaf of bread costs $16,” McWain said. “But if he ate those groceries from the grocery store like you and I do, he would suffer brain damage.”
McWain considers himself relatively lucky.
Self-insured health insurance plans in Wyoming are exempt from coverage due to an exemption from the federal Employee Retirement Income Security Act, and public insurance is limited to certain diagnoses. There are some states where it is even more difficult to get coverage.
McWain scheduled meetings with Wyoming Republican Legislators Rep. Liz Cheney and Sens. John Barrasso and Cynthia Lummis and asked them to co-sponsor the Medical Nutrition Equity Act. She encourages other residents to ask their federal lawmakers to support the bill reintroduced by Rep. Jim McGovern, D-Mass, last year.
“This food is medicine for people and without it their health is deteriorating,” McGovern told WTE. “Or in some cases they could die.”
The bill would require private insurance companies and state health programs such as Medicare, Medicaid and the Children’s Health Insurance Program to provide coverage for medically necessary nutrition if doing so will prevent severe disability and death. There are more conditions than just PKU that require patients to survive on medicated food and formula and have been shown not to be covered by health insurance plans.
She wants support not just for her own family, but for thousands across the country who are struggling with these hurdles.
Both versions of the bill have sat in committee since last June, despite signatures from more than 90 lawmakers and 22 senators.
McGovern said he was trying to enforce the law and that political pressure from voters on their elected officials was an important component.
“The more co-sponsors we have and the more support we can demonstrate, the more effectively we can sidestep the jurisdiction to report them and get them in for a vote,” he said.
McWain said she was disappointed that her own representation in Congress did not endorse or openly support the bill. She spoke to a spokesman from each of the Lummis and Cheney offices, and they withdrew the information last year with no action.
She also met with Barrasso, who she thought would be more comfortable given his background as an orthopedic surgeon for two decades. She said it was an emotional experience and she felt particularly down.
Neither of them agreed to co-sponsor the bill, but she said she plans to meet with them again this month to revisit the issue. McWain said she wants to know why they didn’t endorse it at the time, especially since they all took pro-life positions.
“You say every life matters,” she said. “Then why not support the needs of these communities?”
Barrasso and Lummis both responded to inquiries from WTE about their position, and Cheney did not comment.
The speakers said US senators will closely monitor the bill’s progress through the legislative process and understand the importance of the issue. Lummis confirmed that she will not be co-sponsoring the Medical Nutrition Equality Act at this time.
“Sen. Barrasso appreciates the McWain family for meeting him and raising awareness of this important issue and the legislation,” a spokesman said. “As co-chair of the Senate Rare Disease Caucus, he understands the importance of having access to medically necessary foods and dietary supplements for Wyoming families.”
McWain is not alone in her efforts. Healthcare organizations across the United States and those affected by rare digestive and metabolic diseases have campaigned for similar legislation to be passed for more than a decade.
In May, Dr. Moira Szilagyi, president of the American Academy of Pediatrics, sent a letter to congressional leaders calling for action. She said Congress should require both public and private insurance to cover medically necessary foods as a treatment option, not just pharmaceuticals or biologics, because many of the families looking for specialized formulas are the ones denied coverage.
“Congress has previously recognized the importance of medically necessary nutrition coverage and has mandated TRICARE coverage for such therapies in the National Defense Authorization Act 2016,” she wrote. “We urge Congress to expand this requirement to other insured populations with rare digestive and metabolic disorders.”
Maxfield of the Intermountain PKU and Allied Disorders Association said this bill was necessary for individuals to lead normal lives. It’s already a difficult condition to live with because it’s so rare, and expensive because there’s no market for drug companies to mass produce. In rural areas like Wyoming, this becomes more difficult as there are few families who can afford thousands of dollars in medical food and travel far to other states for their treatments.
She doesn’t want individuals to grow up without resources, suffer neurological damage and possibly risk becoming a burden to the state and taxpayers by ending up in institutions.
“It would be great if we could get the Wyoming delegation on board,” she told WTE. “It’s really nice when representatives who are elected by the constituents are willing to speak to their constituents and hear their stories because they have a great job representing our states and we need their support for this Medical Nutrition.” Equity Act.”