Archive for Saturday, March 4, 2006

Special diet for genetic disorder could be cut from state funds

March 4, 2006


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.

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.


Godot 12 years ago

Well, Bettie, maybe you haven't had contact with the legions of lawyers, CPA's and financial planners who earn a very nice living by helping wealthy people hide their assets in order to receive state aid. One of the reasons rich people are rich is because they don't spend money when they don't have to.

I'm all in favor of preserving state aid money for the people who truly need it.

leeker 12 years ago

This bill is about increasing newborn screening and make some changes to help Kansas be able to do that. They are not trying to take things away from people, they are trying to help more kids. I have been on both sides, paying out lots of money for medical expenses for a special needs child and also planning a funeral for that child because it was not screened for at birth. Don't judge the bill until you read the whole thing!

candy 12 years ago

It would be cheaper for the state to help pay for their food then have to pay for around the clock care for the profoundly retarded. That is how it was explained to us by the state of MN.

As for insurance, good luck. Gerber newborn plan wouldn't even acept my granddaughter. There is a lot of misunderstandings about PKU.

bugmenot 12 years ago

I think Jane had a pretty good point. As far as the people attacking her comment, kettle meet pot. Don't call her an ass while acting like an ass yourselves.

james bush 12 years ago

This sounds like a tough problem these families are experiencing but i'm thinking the legislature is not unmerciful and uncaring. There's more to this legislation than LJW is telling us. Could it be that the LJW is being less than objective with this "news" article?

bettie 12 years ago

Yes, Godot. You're surely right. I bet applying for state assistance is a favorite pasttime of the wealthy, right after going stopping by the soup kitchen to pick up some snacks to take on that cruise you mentioned. Rich people just love to hobnob with the less-fortunate and famous.

Seriously. Think about it. Think about the kind of people you're talking about - the Lexus, the cruises, the investments - sounds like the kind of people who would rather eat paint than be caught dead in an SRS office.

plainzwomyn 12 years ago

Jane... you ignorant _ _ ! You don't sound harsh, you sound superficial, uncaring, and insensitive. Perhaps you should try to develop some compassion.

tolawdjk 12 years ago

Let's see Jane, they eat what appears to be a higher carb/ higher fat diet to get their caloric intake because they can't handle the proteins.

You think they might be a little on the higherweight side?

You aren't harsh, you're clueless to the realities.

Jamesaust 12 years ago

"...because it would require they exhaust all other means of assistance before tapping the state for reimbursement."

Shouldn't every assistance program require this? I doubt this family qualifies but if they're as rich as Canada then they need to use that money to provide for themselves before relying upon the taxpayer. There is also private charity.

As far as budgets, every budget is potentially not fundable in the longrun. There's nothing special here. Indeed, if the State were "to guarantee" funding, then those with private insurance would soon find their condition dropped from coverage. After all, what insurance company covers items that the government covers for 'free'?

frogtoad 12 years ago

Sometimes it's a bit cheaper to live on "normal" foods if you don't have a disease or condition. Please try to educate yourself. Even if you don't have a disease or condition, buying healthy foods are more expensive.

Here's a good general link to PKU from medlineplus.

Godot 12 years ago

I don't see that the legislature is trying to cut off funding, I see that they are trying to make sure the money is reserved for the needy.

If insurance or some other state aid program can contribute to the cost of the food, then that should be tapped first. And if it just so happens that some people who have a child with PKU have high household income, what is wrong with having them carve money from their budget for their childrens' food, leaving the state aid for people who are less fortunate? Granted, a sliding scale for aid might cause some well-heeled people to take one less cruise per year, or maybe buy a Chrysler instead of a Lexus, or make fewer investments. That's not harsh, it makes sense. We should expect those who can take care of their own to do so.

DoubleQ 10 years, 4 months ago

LJW said Sammy said "I hate it, I just hate it." and he told me he said "It sucks!"

Commenting has been disabled for this item.