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PROTEIN AS PAIN
Amanda Cosburn had scrambled eggs recently.
At age 27, it was the first time she had ever had them — but, given their expense, it won’t be happening often.
Cosburn has phenylketonuria (PKU), a long name for the disease that is screened for in newborns with a poke into their heel and a blood test. Most tests come back negative — hers didn’t.
“It takes two weeks to get the results and, when they got mine, they called my mother and told her I was mentally retarded and to take me to B.C. Children’s Hospital right away.
“My mom was only 20 years old and she was met at the hospital by this whole team of doctors.”
There, Cosburn’s parents learned the truth she has lived with. PKU is a genetic disorder that can be managed but not cured.
Boiled down to the simplest explanation of what is a complex biological process in the body, people with PKU must limit their intake of protein. That means no meat, dairy, seafood, legumes soy or tofu. Fruits and vegetables are okay, depending on how they are processed.
The bodies of people with PKU cannot handle phenylalaline, an amino acid found in foods that can be toxic to the brain and lead to a variety of conditions.
Treatment involves a special liquid formula, which Cosburn drinks throughout the day, and a restricted diet. Prepared foods for people with PKU are available but are expensive — a box of nine small pizza balls costs $24 while a bag with nine tiny bagels costs $19, for example.
Specially prepared pasta in a bag not bigger than what most people might buy at their grocery store, costs $9 and a baking mix Cosburn relies on to prepare food each week is $55.
A third prong in the treatment is a drug called Kuvan, one that was approved by Health Canada in 2013, by the U.S. Food and Drug Administration even earlier and available in Ontario, Saskatchewan and Quebec — but not B.C.
“This is really confusing for me,” Cosburn said, “We’ve been told research has not been proven but B.C. gets the same information as other provinces that have approved it.”
Cosburn has spoken with her MLA, Health Minister Terry Lake, and she said he’s been sympathetic to her issue.
When contacted by KTW, Lake’s ministry office in Victoria emailed his response.
It noted the province relies on the Canadian Agency for Drugs and Technologies in Health and the B.C. Drug Benefit Council when making decisions on drugs to approve.
The ministry, after receiving input from the two bodies “recommended against provinces covering this drug because of unclear benefits and high drug costs. The annual cost per patient for the medication can be as much as $180,000 per year for an adult, depending on dosing and the patient’s weight, and the available clinical research does not show clear benefits,” the email noted.
Access to the drug would make life easier for her and reduce her food costs, Cosburn said. And, it might go a long way to helping her with her dream of having a child of her own.
To do so, living with PKU, would require monitoring the presence of the amino in her body, keeping it to a level best described as a one or two for six months before conception, during a pregnancy and during nursing.
Given the unpredictability of conception, in essence it means keeping the level that low all the time, something that isn’t easy to do. Often, particularly when she hasn’t enough money to pay for the prepared foods, that level rises to anywhere from eight to 11 in Cosburn’s body, she said.
And, she’s aware she runs the risk of giving birth to a child with PKU.
Cosburn’s childhood was difficult. She was removed from physical-education classes permanently in Grade 6 because of fear muscle development would elevate the level of protein in her body and raise the amino level.
Lunch was four crackers with some jam, some applesauce and a thermos of the formula she lives on.
To go to a restaurant with her partner, Cole Rickett, requires calling ahead of time to ensure the few foods she can eat are prepared properly and, even then, she tends to simply order mashed potatoes and grilled vegetables.
Even then, however, she has to order child-sized portions because the more food, the greater the presence of proteins.
One large potato, for example, can have eight grams of protein and Cosburn needs to restrict her entire daily protein intake to no more than five grams. Anything more than one-quarter cup of corn also exceeds her protein -consumption limit for the day.
She’s speaking out for many reasons. She wants the government to approve the drug and she wants people to see that it is possible, although expensive, to live a healthy life with PKU.
She’s speaking out because she spent many of her teen years in hospital because she didn’t stick to the diet and she wants young people with PKU to understand how important it is to stick to the rules.
But, in the end, she’s speaking out because of a moment during her nursing studies in Vancouver when she discovered the only exposure she and her classmates would receive on the disease was one paragraph in a textbook.
“I was mad when I saw that. That wasn’t good enough for me. So I did a report on it for the class and decided I had to keep speaking out.”