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By Andrew McLean
I was born on January 19, 1970, in Montreal, Quebec. My parents had already had two girls before me, both normal and healthy births. Because I was born 5 weeks premature, the Guthrie test was administered within 24 hours and I tested positive for PKU. The premature nature of my birth meant that I was kept in the hospital for a prolonged period, ensuring that my PHE levels were closely monitored for the critical first weeks of my life.
My memories of childhood as it related to having PKU are generally good. My parents were always very strict with me on the diet; for me it was simply a fact of life that I wasn’t allowed to eat certain foods. If friends’ parents were not aware of my dietary restrictions, I could always be trusted to decline whenever I was offered restricted food. One of the funniest things I remember from elementary school is that every couple of months we would have a "hot dog and donut" day, when all the students would buy a donut and a couple of hot dogs for lunch. On these days my father would leave work on his lunch hour, go to McDonald’s, and bring me french fries for lunch. All the other kids in my class weren’t interested in their hot dogs; all they wanted were some of my fries! Similarly, at birthday parties I would show up with my own lo-protein birthday cake my mother had made, and more often than not the other kids would want some of my "special" cake. Formula, or my "milk", was never a problem. We always had a Tupperware container of Phenyl-Free in the fridge ready to drink, and I never had any trouble drinking it. I don’t recall ever having to be pressured to drink my formula.
One thing I remember vividly is that in grade 1 or 2, when I would have been 6 or 7 years old, I ate a couple of cookies I was offered at school. For weeks after I was deathly afraid that I would get sick, and I didn’t approach my parents for fear of getting in trouble. I realize now that my fears would have been dispelled if I knew that small amounts of protein don’t affect those with PKU, but I now believe that it was my lack of understanding that helped me to control the diet as strictly as I did.
It would seem that my parents had the hardest time of it. My mother had no PKU cook book or ListServ to get recipes; she basically created everything she made for me from scratch. My father relates stories of getting dirty looks from restaurant staff when I informed them that "I’m not allowed to have a hamburger" while my two sisters order burgers right beside me. When dining out I usually ordered french fries and/or ate from the salad bar.
When I went off the diet at age 14, I was told that if I had been female I would have had to stay on diet for life. It was definitely a memorable time in my life. I went to Disneyland with a friend right after I was taken off diet, and to this day I remember the first time I tasted REAL toast, in the restaurant in the Disneyland Hotel. For about 5 years I ate pretty much what would be considered a vegetarian diet, having no real desire to try meat. That all changed when I tried pizza for the first time. My first experience with beef was a McDonald’s Big Mac, and I have to say that I wasn’t that impressed at all. Over time I acquired a taste for homemade hamburgers, and eventually went on to eating chicken as well.
In 1994, after 10 years off diet, I was approached by the Vancouver Children’s Hospital and went in to speak to a counselor. After the standard battery of tests, both physical and mental, it was stated that I "seemed to be a functioning member of society". This statement did wonders for my self-esteem, as you can well imagine; especially since it was said in a tone that indicated that this was not to be expected. I was then told that I should consider returning to diet because being off diet had been shown to affect mental functioning in people with PKU. I had experienced no such effects to my knowledge, but agreed to try the diet for a while. I was given a case of Phenyl-Free and basically left to my own devices. Needless to say, this attempt lasted all of a week or so.
Ironically, the aforementioned consultation with the Children’s Hospital clinic happened mere days after I met the person who would be my partner through the rest of this adventure. My wife and I met in November of 1994 and dated for 3 ½ years before marrying in May of 1998. I had mentioned in passing to Terri that I had been born with a condition requiring strict dietary controls during my childhood, but that the effects of PKU in adulthood were negligible unless I were a woman attempting pregnancy. I was always a little apprehensive of talking about PKU, as my understanding of it was limited to recollections of my childhood.
In 1998, the Adult Metabolic Diseases Clinic was started at Vancouver General Hospital. Their list of contacts originated from the Children’s Hospital. I was contacted several times by the clinic during 1998 and 1999, but ignored their attempts to get me to attend one of their information sessions. Late in 1999 I went online for the first time, and decided to look up PKU on the Internet. I found the PKU News site in short order, and started to learn about my condition. The section, "Adults Off Diet: Lives in Danger!" caught my eye, and I read the information, albeit at first with trepidation. It’s still difficult to explain, but I dreaded the possibility of finding out that my actions during my teens and twenties may have irreparably damaged me in some unforeseen way. As I read on, however, I was relieved to find that returning to diet had been proven to reverse many of the damaging effects of prolonged exposure to high levels of phenylalanine.
Around the same time that I was learning more about my situation, the AMDC invited me to another of their information sessions at VGH. Their selected date for the session was January 19, 2000, which also happened to be my 30th birthday. If there was any more symbolic way to enter adulthood and deal with my metabolic disorder at the same time, I couldn’t imagine it! As it turned out, the information session was postponed due to lack of interest, but my decision had been made and I went to the clinic in March of 2000.
The nurse at the clinic noted several things that she attributed to my being off diet for 16 years. These included a slight tremor in my hands, a twitch in my eyelids, and eczema on my legs. I was given samples of several types of new formula to take home and try. I chose the vanilla flavour of PhenylAde as the one I was most likely to stick to. My blood samples taken before I returned to the diet were unfortunately not tested for PHE levels, but I was found to be low in vitamin B12 and was prescribed B12 supplements. My first blood PHE levels were taken after I had started drinking the PhenylAde, but before I started restricting my protein intake. The levels were 15.7 mg/dL. A month later I had managed to get my levels down to 5.6. I was extremely happy with these results, as were the AMDC staff.
Even though I had noticed no adverse effects to being off diet which I could directly attribute to my PKU, within 2 months of returning to diet I noticed a pronounced improvement in my overall health. My eczema, which I had been dealing with for years, cleared up completely within a matter of weeks. But the most dramatic improvement was in my mental functioning. My concentration and attention span increased immediately. Where I used to find it hard to finish reading one book in a month, I read through 5 novels during a 2-week vacation which my wife and I took in May of 2000.
My wife has provided tremendous support since my decision to return to diet. It was not easy for her, having no preparation for such a drastic change in lifestyle for the both of us. As with most couples, dinners both at home and in restaurants are an important part of our interaction. Dinners at friends’ can be difficult, but we find that our friends always go to great lengths to accommodate my diet and are very interested in learning more about PKU. Terri and I have also made new friends through the AMDC and have had opportunity to experiment with PKU-friendly dinners with other couples in the same situation as us.
My experiences returning to diet have inspired me to try and help others with PKU who may feel that there is no reason to return to diet. I especially want to reach men with PKU, because much of the emphasis seems to be on getting women back on diet, specifically those approaching child-bearing years. Maternal PKU is an important issue, and an excellent reason to return to diet, but I don’t want men to be overlooked by clinics attempting to contact adults with PKU who have been off diet for years and have lost contact with PKU-trained professionals.
To this end I have created a website (www.pkuadults.com) specifically for adults who have been or are still off diet. I also had the privilege of attending the NIH Consensus Development Conference on PKU Screening and Treatment in Washington, D.C. in October of 2000. This was an amazing personal accomplishment, and I amassed a huge amount of information on PKU. Most importantly, though, I met many people who have dedicated their lives to helping people with PKU and their families. And I feel very fortunate to count myself, along with Sarah Foster, Stephanie Stremer, and her sister Valerie, as one of the representatives at the conference with PKU.
I have recently joined the Canadian Society for Metabolic Disease, which is a non-profit organization formed in 1990 to support families with children born with metabolic diseases. I am contributing my time and efforts to create and administer a website for the CSMD, and hope to be much more involved with the organization in the future.
This pretty much brings my story up to date, but in a lot of ways I believe that my story is just beginning.