Sammy Dowell’s favorite sandwich is ketchup, mustard and pickles on bread.

It’s not just the tastes of a 10-year-old. It’s a medical necessity.

Sammy has the rare genetic disorder phenylketonuria, or PKU. He follows a strict, low-protein diet, because veering from it could cause severe problems.

“I hate it. I just hate it,” Sammy said of his disease.

Proposed legislation, which will be discussed in a hearing Tuesday, would change the way the state reimburses families for the special diet that those with PKU follow. The bill has some families, including Sammy’s, worried.

“I feel like PKU is getting picked on a little bit because we are a small group,” said Sammy’s mother, Rayanne Dowell. “We’re scattered. We’re not a coalition. We don’t have a voice.”

PKU is one of the genetic disorders for which physicians test babies at birth. Those with PKU are unable to process food normally. They must follow a rigid low-protein diet to prevent buildup of the amino acid phenylalinine. If untreated, the disorder causes mental retardation.

“I could tell you anything my daughter has ever eaten in her whole life,” said Randy Jolitz, an Overland Park father, whose 2-year-old daughter was born with PKU.

Jolitz is used to meticulously planning meals and weighing his child’s foods as is required to “stay on diet.”

The Dowells stack boxes of a special PKU food formula in their closet. Sammy’s sister, Jesse, also has PKU.

Jesse used to be shy about her illness, only revealing it to her closest friends. But she’s grown more comfortable with it.

The vanilla-flavored formula she drinks costs $50 for a six-glass serving. She must drink three glasses a day.

Rayanne Dowell rattles off the list of specially formulated foods the children eat.

Bag of pasta: $6.89.

Rayanne Dowell, center, has two children who have PKU, Jesse 13, left and Sammy 10, right, have to follow a very special diet because of the genetic disease. Much of the food on their diet is expensive and is covered by state funds, but a bill before the Legislature could cut that funding.

Special mix for omelets: $21.

Jar of peanut butter: $6.75.

These foods and the formula keep people with PKU from a potentially devastating result.

“If we didn’t have that, we wouldn’t be as normal,” Jesse Dowell said of the formula.

State help

The Dowells and the families of roughly 60 other Kansans, ranging in age from birth to 53, benefit from a state program that reimburses them for the cost of the expensive special food formula.

But a bill before the Legislature has the families of PKU victims worried because it would require they exhaust all other means of assistance before tapping the state for reimbursement.

House Bill 2971, calls for people with PKU to first apply for insurance coverage for the PKU formula. And the bill also proposes that the Kansas Department of Health and Environment establish a sliding scale for reimbursement pegged to family income.

The sliding scale has not been determined, said Sharon Watson, spokeswoman for KDHE.

Those who depend on the state aid are jittery about moves to modify the program.

“Some parents are concerned that the state will say, ‘We just don’t have the money.’ Then they’ll be stuck,” said Norm Hess, director of program services for the Greater Kansas Chapter of the March of Dimes. “I can see the parents’ point of view – that this doesn’t provide them with any assurances in the long run.”

According to the governor’s proposed budget, the state anticipates a deficit for the program next year.

The state plans to cover the program costs for this year, Watson said.

Rep. Peggy Mast, R-Emporia, vice chairwoman of the Health and Human Services Committee, said she supports the program.

Mast said the state has supported the program for some time.

“I don’t want to back off on that now,” she said. “I think it’s crucial that the state continues to support the program.”

Some are torn about the bill because it also includes a proposal to expand newborn screening.

“We’re obviously supportive of the end of this,” Hess said of the portion that deals with newborn screening. “It’s a little awkward for us.”

Rayanne Dowell said her insurance doesn’t cover the special food or the formula. Dowell estimates the food products would cost her $14,000 a year.

“We’re just an average family,” she said.

By all appearances, you can’t tell someone has PKU by looking at them.

Dowell said she believes their anonymity and other factors make them an easy target for cost-cutting efforts.

But she said she plans to speak up. She’s calling agencies that she hopes can help. She’s written letters. And she plans to take her case to lawmakers Tuesday when a hearing is set for the bill.

“If one of these kids slipped through the cracks, the results could be devastating,” she said.