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When your child can’t eat

The Lemky family (from left): Sienna, 4, Ruth, Steve, and Alayna, seven months. Alayna was born with Phenylketonuria, or PKU, which prevents her body from properly digesting protein. - EVAN SEAL / THE LEADER
The Lemky family (from left): Sienna, 4, Ruth, Steve, and Alayna, seven months. Alayna was born with Phenylketonuria, or PKU, which prevents her body from properly digesting protein.
— image credit: EVAN SEAL / THE LEADER

Bouncing on her father’s knee, Alayna Lemky can’t contain her excitement. Her hazel eyes dart about the room as she screeches and laughs like any other healthy seven-month-old child.

When Alayna was born, like every child born in B.C., she received a Guthrie test, or what is more commonly referred to as a neonatal heel prick.

The procedure tests for a variety of genetic abnormalities in a baby’s blood.

Four day later parents Steve and Ruth received a call at home from the hospital saying Alayna was suffering from a metabolic genetic disorder known as Phenylketonuria or PKU. The disorder occurs when the body is unable to process one amino acid in protein known as phenylalanine or PHE, which is found in almost all foods.

“It was pretty traumatic,” said Ruth Lemky. “Over the phone they tell you if you don’t do anything your child can be severely developmentally damaged.”

PKU patients need to be kept on a very strict diet supplemented by a medical formula containing all amino acids except PHE, along with other nutrients that are essential to a proper diet. This “milkshake” serves as their primary meal three times a day and should be maintained for life.

Early detection of PKU, along with the specialized diet, can result in a child living a normal life with normal mental development.

Each day, Alayna eats 700 ml of a special prescription formula that acts as her protein substitute. Recently apple sauce has been introduced as well, but PHE is found in many fruits and vegetables so constant calculations are essential.

The PHE-free baby formula is covered by MSP, however other low-protein rice and bread medical supplements are not covered and are often expensive.

“If you want them to eat more than just apples or formula shakes you’re going to spend $300 to $500 per month on these special low-protein foods,” said Ruth. “Even a small package of mac and cheese is $10.”

While the medical formula is fully funded across Canada through the provincial health care systems,  in all other provinces expect B.C., the medical low-protein food is also funded – fully or in part – by the province.

In B.C., the protein-free foods are not covered and Ruth says the costs to families are often overwhelming.

There is some hope on the horizon. In 2010, Health Canada approved a new drug called Kuvan, which reduces the levels of PHE in the blood.

Kuvan has shown varying degrees of success for between 30 and 40 per cent of patients suffering from PKU. The drug has been available in the U.S since 2007.

“(Kuvan) is by no means a magic bullet,” says Steve. “She’s not going to be able to go out and eat a steak, but she could potentially have a vegan diet.”

Currently the cost of that drug runs upwards of $50,000 a year and will cost nearly four times that much when Alayna reaches adulthood. Since the drug is not covered by the Lemkys’ medical insurance, the costs are out of reach for them – and most PKU sufferers.

In a statement from the B.C. health ministry, a spokesperson said “because of unclear benefits and high drug costs,” they have declined to cover the costs of the drug.

“In regards to food costs for PKU patients, the ministry currently does not fund a low-protein food subsidy for PKU patients. This is consistent with other medical conditions that require patients to follow a special diet,” the spokesperson wrote in an email response to The Leader.

Steve and Ruth Lemky have written multiple emails and letters to their provincial representatives looking for help, but to no avail.

“We’re at our wits’ end,” said Steve. “We would just like the government to fund Kuvan like they would fund any other drug.”

For more information about PKU, visit www.canpku.org

 

 

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