Justin Young's Story

This story is taken from the Fall 1999 issue of National PKU News

My tenuous entry into this world took place early in the morning of June 15, 1973 in a hospital located in Parma, Ohio. According to later boisterous accusations and threats of retaliation from my mother, I was a difficult delivery! The situation was not improved when she was notified that I had PKU. Apparently, my mother was not told immediately of my condition as the Guthrie card used for my individual screening sat inactive, in a pile of others, until the actual testing took place a couple of days later. My mother was rightfully concerned with my postpartum condition, which she described as "agitated and lethargic." With the assistance of a most adept and competent physician at the Cleveland Clinic in Cleveland, Ohio, my mother was able to negotiate the chaotic first few weeks of my existence, but was ever concerned that I was a fragile and compromised newborn. With time, her confidence in managing my diet grew. When my brother, Kevin, was born August 18, 1976, also into a life and diet aimed at managing his PKU, she was a seasoned advocate of the diet and took great steps to promote awareness among other parents. Balancing a demanding work schedule with the challenge of managing the diets of two children with PKU (and one without) was undoubtedly a daunting task. For her efforts I am ever grateful.

My sister, Kimberly, was the other and eldest child, born without PKU. Kimberly attended Stanford University in California, endured a brief stint at the Medical School of the University of California San Diego, and is currently in the second of a three-year residency in Family Medicine in Santa Rosa, California. Kevin, the youngest and my only brother, recently graduated from Miami University in Oxford, Ohio and moved to Chicago to begin his career as an aspiring producer. His college major was Mass Communications with a specialization in video production. Kevin plans to enroll in a graduate program which will ultimately yield his A.C.E. My mother, Joan Shropshire, lives in Lexington, Kentucky with my stepfather, Walter. She owns a graphic design company. My father, Kent Young, is a clinical psychologist with a private practice in Mentor, Ohio.

Very soon I will be a fourth year dental student at the Ohio State University College of Dentistry. Even before high school, I recall that my love for basic sciences was very intense, and I was in advanced classes for math and science in both private and public elementary schools. During high school I enrolled in several honors classes, but always found biological sciences the most stimulating. When I began Miami University my major was Microbiology, and I never wavered from that course. After graduating from Miami University I moved to San Diego, where I lived until I entered dental school. I was fortunate to find excellent employment as a manufacturing Chemist, and then Scientist with Isis Pharmaceutical in Carlsbad. Dental school has been a wonderful experience for me. I have strived to immerse myself in every academic opportunity possible. I have been on the Dean’s List since my first year, and am currently in the top 15 percent in my class of near 100. I am an officer of the American Student Dental Association, a member of the AADR (American Association of Dental Research) and the IADR (the international equivalent), and have won three research scholarship appointments in the last three years. During my sophomore year in dental school, I was invited to apply for a National Institutes of Health grant to promote dental research as part of a combined dental Ph.D. program. I also make a point of lecturing to third and fourth graders on the hazards of smoking as part of my involvement with the American Cancer Society. Additionally, I volunteer at local nursing homes as part of a study to determine the effects of medication on the oral condition of patients in assisted care settings. My research interest has been specifically studying the effect of stress on the immune system, and the group that I work with at Ohio State is a national leader in this type of research.

I understand the necessity of the PKU diet now more than at any time in my life. As I gain more insight into the systemic interactions of the human body, I realize what a miracle it is that I am able to thrive and function, where several years ago this may have been impossible. Being on the PKU diet is difficult, and it takes discipline for an individual to maintain it. I don’t mean that adherence to the diet is difficult, rather that management of blood phe levels from day to day is difficult. I have learned to recognize the effects of even small fluctuations in my blood phe levels on my mood and ability to think clearly and quickly.

Now my everyday regimen consists of a light breakfast, for example some cereal and a banana or other fruit supplemented by my formula (Phenyl-free). For lunch I usually have a salad in the hospital cafeteria, along with steamed green beans, carrots, or broccoli. Sometimes I have steamed rice or French fries depending on how heavy in phe my breakfast was. When I expect to have a longer day, such as those when I have a patient in the evening, I tote my formula and a small cup and fork with me in my book bag. I mix it up on an as needed basis and this usually holds me until I have dinner. For dinners, I usually have various types of stir-fries, with fresh produce and a sauce that I adapt from recipes in cooking magazines. I have become quite adept at creating delectable vegetarian recipes utilizing fresh produce and blending an array of cooking styles. This, however, is the regimen that works for me, and I realize that my nutritional needs with PKU are different from others with PKU, especially adolescents. I relish taking entire afternoons to prepare something that is healthy for me and my diet, and which will maximize my chances of operating at a high level of intellectual performance. This makes me feel very strong and proud of myself.

My responsibility for my own diet came early, as my mother had to work as a teacher during most evenings when I was younger. I believe that as early as sixth or seventh grade I began cooking vegetable concoctions for my brother and myself because we did not like the canned fruit and frozen vegetables that our baby-sitter (my own sister) made for us. She realized our needs, I’m sure, but did not have a vested interest in teasing our palates. So this responsibility soon became mine, and I was good at it.

My diet has never been "liberalized" so to speak; meaning that I have never taken a sabbatical or hiatus from the diet and purposefully taken in more phe than I was allowed to or should have. There was an episode when I was younger when my diet was accidentally mismanaged, though, so let this caution parents who send their children to camp. Hearing this story now, as told by my mother, is humorous, but as with other things of this nature, it was a very serious incident at the time. In preparation for an elementary school sponsored week-long camp, my mother and father met with Lena the Amish cook. Lena was the person who was to have sole accountability for my diet throughout the week. All details were arranged, or so it seemed, and I was to report to the kitchen of the dining hall for my formula, and to help measure my food. This never happened and the only times that I reported to the dining hall was for regular meals where I did my best, with my knowledge of the diet at that time, to measure out and manage my intake of phenylalanine. This came before I really had comprehensive knowledge of the complexities of the diet, so you can realize that my management was fairly poor. The tragedy ends with me coming home from the week at camp, uttering nonsense to my mother when she asked me how things went, and falling asleep until the effects of my high phe level wore off. Despite this incident, the camp was still a positive experience. But it left me with a resounding impression of what a liberalized diet may have been like—not for me.

During my education I have encountered many people who are curious about why I eat the way I do. During junior high school when everyone (myself included) is concerned about image, I used to tell people that I was "just a vegetarian." This was before it became vogue to actually be a vegetarian, and my explanation necessarily had to become more involved. It was not always as easy as it is now for me to openly explain my diet, but I have become quite confident and secure with sharing the facts of my diet with those people who are interested. In dental school, most of my classmates have learned something about PKU. When I tell them I have it, they usually want to know more about the diet, enzyme deficiency, and formula. I tell them openly.

Most students are interested, and the conversation usually ends with "That sucks," or "That must be really hard" and, "You will probably live forever." There never have been any problems with people viewing me as "weird" or "different" when I tell them about the diet. I used to worry about this when I was younger. My explanation usually begins with "one in every ten thousand babies is born deficient in the enzyme that converts phenylalanine to tyrosine. I am one of those kids, so I can’t eat things that have a lot of phenylalanine in them. That includes basically anything that you can think of that has a lot of protein in it such as meat, fish, eggs, milk, and that stuff in the middle of your sandwich there. . .that would not kill me, but it would be bad if I ate that."

I have written a grant application for the Federal Department of Public Health and the American Association of Nursing Schools in an effort to increase awareness of funding problems for the state formula and metabolic foods programs. My goals for personal involvement with PKU include lobbying for insurance mandated coverage of low protein foods. Recently a group that I am involved with met with a state legislator in an effort to draft a bill that would require insurance mandated coverage of the formula in the state of Ohio. Progress looks imminent and I am very excited about the long-term benefit of these efforts. I also will serve as a counselor at a PKU camp in Cleveland where my PKU is followed by Judy McConnell, RD, and Dr. Douglas Kerr, MD, PhD.

Children with PKU may sometimes feel alone in their struggle with the diet, and in their decisions about how to deal with the rigors of maintaining their basic level of biological functioning. Being able to talk about my diet to others has allowed me to deal with my condition in a candid way. I am not afraid of the notion of having to eat a certain way for the rest of my life, but I am also not giving up faith in science to negotiate a remedy for the diet. The winners of the Guthrie Scholarship and Merit Awards speak volumes about what people with PKU are capable of. There is no need for special treatment or prejudice when dealing with our diets, and more importantly, with our personalities. I do not see why we as a group should feel, subconscious or otherwise, any level of inferiority or ineptitude! We all possess the natural capability to achieve, and indeed may have a greater compassion for others as a result of dealing with PKU.