Monday 16 July 2018

Brothers can't eat chips, chocolate or steak because of rare disease

Imagine not ever being able to have a plate of chips, a bar of chocolate or a good steak. Two brothers tell Joy Orpen about their daily challenges living with PKU, a rare genetic disease that seriously affects their metabolism

Brothers Mark and Peter Lavery. Photo: David Conachy
Brothers Mark and Peter Lavery. Photo: David Conachy

Mark (26) and Peter Lavery (19) have never knowingly eaten meat, chicken or fish. They are also barred from having cheese, eggs, beans and tofu. For most people, that would seem like the ultimate deprivation. But these young, southside Dubliners fully understand that having a diet that is almost totally protein-free ensures their survival.

Mark says his problems began soon after his birth when a heel prick test was done. This routine procedure checks newborn babies for six medical conditions including cystic fibrosis. "Five days later, my parents were told I had a disease called phenylketonuria (PKU)."

According to a spokesman for the PKU Association of Ireland (PKUAI), this is a rare genetic disorder that affects a person's metabolism. "People with PKU are unable to break down an amino acid called phenylalanine (phe)," they said. "This is a natural substance found in food. However, in people with PKU, phe builds up in the blood and in the brain and if left untreated can result in permanent brain damage."

Since PKU affects the body's ability to process all protein effectively, a huge range of foods becomes hazardous for people living with this disease. Mark and Peter have access to certain low-protein foods "on prescription". They say, however that while the range of food currently available in Ireland is "abysmal", people living with PKU in other countries have access to a much wider range of low-protein foods.

Peter, who was also diagnosed with PKU, says his first major dietary challenge arose when he went to the Gaeltacht with fellow primary school pupils.

"I had to take bags of my own special food - especially snacks," he explains. "When you're a kid you don't want to seem different. At secondary school, there was nothing in the canteen I could eat."

His brother Mark, an actor, had similar experiences packing bags of food into his suitcase when he went to America for four months following his studies at the Dublin Institute of Technology Conservatory of Music and Drama.

"I attended the William Esper Studio in New York and loved it," Mark says. "I had wonderful, enriching experiences in that city; I also spent every cent I had on going to the theatre. Luckily, I brought my own food from Ireland, which mainly consisted of low-protein pasta." Since returning to Dublin, Mark has appeared in the award-winning movie Handsome Devil and also in the Vikings series

So, while being clear that their diet does present challenges, Peter believes they are ones that can be overcome. "I used to help Mum bake different dishes using our own special ingredients and that's when I developed a real passion for food," he says.

So, it's not surprising that this committed foodie is now running the recently reinvented Tropica Cafe and Restaurant in Dalkey.

"All the food at Tropica is home-made," Peter says, "and that comes from baking cookies, special breads and cakes when I was a child. We home-baked rather than bought ready-made."

So where did this rare disease come from? "PKU is genetic," explains Peter. "Both parents must be carriers. It's caused by a Viking gene. So, you will find higher numbers of people with PKU in countries where the Vikings landed, such as Canada, Scotland, Turkey, Spain and, of course, Ireland. One in 4,500 babies in Ireland is diagnosed with the disease, while in the UK, it's closer to one in 12,000 babies."

Having recently returned from a conference in Norway, Peter says specialists from all over the world agree that diet alone is not sufficient to manage the dangers that PKU presents. "A strict, low-protein diet isn't enough to prevent damage to the brain," Peter says.

"In Norway, they were talking about Kuvan, a drug which is actually manufactured by a company with a significant presence in Ireland," he adds. "This has proven to lower phe levels in some people living with PKU. That means they may be able to increase their protein intake. It has also seen a lessening of brain damage and the very real side effects that some people with PKU experience.

"It's available and reimbursed in most other countries including America, Austria, Bulgaria, Germany, Romania, Russia and France, but ironically, it's not allowed in Ireland, which has one of the highest rates of PKU," Peter says.

A staggering 95pc of normal food is not permitted on the PKU diet. Mark is only allowed 6g of protein a day, while Peter is on 5g. Mark explains that one Weetabix is equivalent to 2g of protein. A small bowl of rice is between 2g and 3g.

"Everything has to be weighed and spread out over the course of the day," Mark explains. "All high-protein foods are absolutely out of the question - so I've never, ever, had meat, chicken, fish or eggs. But you can also find lower levels of protein in foods such as potatoes, rice and avocado. One of our staple foods is special low-protein pasta. I often take pasta to restaurants when I want to eat out with friends."

The brothers are extremely supportive of each other and share the preparation and cooking of their food. Every night, one of them will pop a loaf made from low-protein flour into their bread-making machine. And if one of them makes a vegetable curry, he will share it with the other. They are unable to drink beer, but they are allowed to have cider, spirits and some wine.

Mark says it makes life a bit easier that he has several friends who have chosen to forgo meat in favour of a vegetarian or vegan diet. "We can eat most fruits and vegetables," he says, "but unlike vegetarians, we cannot eat pulses, nuts or grains."

Nonetheless, Mark fervently believes that people in Ireland should have access to treatments that are readily available in other countries; treatments that assist in controlling phe and help prevent long term brain damage.

"If we could increase real protein intake [by having access to the appropriate medication], it would make a dramatic difference to the lives of all people living with PKU," he says. "It would also give us choices we don't have right now.

"It's proven to help the physical body," concurs Peter. "But even more than the choices and the freedom it brings, it helps improve concentration and most crucially, it can help protect the brain from long-term damage."

See pku.ie

Sunday Independent

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