I was born on February 19, 1973 at the Miami Valley hospital in Dayton, Ohio. I was tested for PKU at birth, as testing was mandatory in the state of Ohio at the time. The Children's Hospital of Cincinnati put me on diet at three weeks of age to bring my blood Phe level down from ~39. I am classified as having "classical" PKU.
My immediate family consists of myself, my father James, my mother Vicki, my older sister Lysah (age 28, non PKU) and my younger brothers Brett (age 21, classical PKU) and Scott (age 19, classical PKU). My parents and siblings live in Southern California but I currently make my home in Stony Brook, New York.
My father is a business consultant for Cyber-Tech Computer College. My mother is an accountant at a company that makes GPSs'. Lysah is married and works as an accountant at a business. Brett and Scott both played varsity baseball and basketball in high school and graduated with honors. Brett graduated in the Spring of 2000 with a B.A. in Communications from California State University at Fullerton; he hopes to break into the movie industry. Scott is studying Communications/Broadcasting at CSUF and works weeknights and weekends Sportscasting for Orange County (southern California) high school sporting events. He hopes to make sportscasting his carrer & my predicition is that he will be the next Vin Scully!
When I was in high school I had an aptitude for science and math courses. My favorite classes were Biology, Chemistry and Math. After graduating from Valencia high school, in Placentia, CA, in 1991 I started college at Fullerton Community College. Two years at FCC with a combined science/math G.P.A. of 3.55 (out of 4.0) enabled me to transfer to the University of California at Los Angeles. After two years of grueling physics and astronomy courses, I graduated from UCLA in the spring of 1995 with my Bachelors Degree in Astrophysics. While at UCLA I did a senior research project and the results were included in an article published in the Astrophysical Journal, a journal of modern astronomical research.
In the Fall of 1995 I moved from California, my home of nearly 22 years, to Long Island, New York to continue my education at the State University of New York at Stony Brook. During my first two years at Stony Brook I taught the astronomy laboratory courses offered by the university. In the Spring of 1996 I was awarded the departmental award for excellence in teaching. In the Spring of 2001 I completed my Doctoral dissertation and earned my Ph.D. in Astronomy. To the best of my knowledge, I am the first person in the world with PKU to earn a Ph.D. There is also a woman in Germany (Dr. Miriam Hein) who has PKU & has an MD and practices pediatrics. I'm sure there are going to be many, many more PKU adults obtaining advanced degrees in the future!
My dissertation research utilizes state of the art technology in application to high resolution imaging techniques (such as speckle mode shift-and-add analysis and adaptive optics) to add to the current knowledge of proto-planetary disks around very young stars and newly forming clusters of young stars (more than 1000 times younger than our sun). My research will add insight into the environment in which stars form and how material in a proto-planetary disk evolves to form solar systems such as the one we live in today.
I can't really remember exactly when I decided I wanted to be an astronomer. It was a gradual decision that extended over most of my younger years. However, when I was a senior in high school I had made up my mind that Astronomy was the field of study that I wanted to pursue. I am presently working as a science research fellow at Gemini North Observatory in Hilo, Hawaii. My position requires that I typically spend a week or so every month observing at the summit of Mauna Kea, a dormant volcano. What a unusual & exciting job it is! :)
Like many astronomers, I have often dreamt of becoming an astronaut and flying in the space shuttle. However, I have run into problems when dealing with my diet while merely traveling across the state of the country, I can't begin to imagine what those problems would be like in orbit around the earth! Unfortunately, present diet limitations are not very compatible with space travel.
When I get free time I like to watch movies, go to concerts and sports events, and hang out with friends. I also enjoy rollerblading and playing squash. I recently got a membership to the local health club and now swim 3-5 kilometers per week. I also love animals. I always snuggle and pet my cat, Benisch, and I keep and care for two freshwater aquariums. I inherited artistic talent from my mother so I enjoy watercolor painting, macrame and making jewelry. In addition, I have a definite green thumb. I love keeping and caring for a wide variety of houseplants, herbs and sometimes fruits and veggies...
My parents always kept a large vegetable garden and numerous fruit trees when i was growing up and every summer we would always have plenty of fresh produce. That is probably one of my favorite memories when I think about growing up with PKU. My brothers and I would always have our choice of fresh green beans, cucumbers, green peppers, peaches, pears, apples...etc...
Another one of my favorite memories was whenever a birthday rolled around our mom used to make each of us a special birthday dinner. We used to get to choose meals that took inordinate amounts of time to fix, such as gyoza (a special little Japanese dumpling), tempura (batter fried vegetables), vegetable fondue, stuffed peppers, fried rice... etc...
When I was growing up PKU was just a way of life. When we were young my parents were generally very good about keeping my brothers and me on diet and making sure we knew what we could and couldn't eat. When I was very young my blood level was taken at least once a month and was routinely within the suggested range. However, when I was 18 months old my blood levels suddenly came back abnormally high. It took a while, but my mother finally caught me nibbling cat food out of the food dish that was kept on the kitchen floor!
I definitely remember having difficult times with the diet. When I was nine years old I went through a stage of "curiosity cheating". I used to sneak foods, like slices of cheese and granola bars, and try to hide the evidence. It was very difficult to face the temptation of having the high protein foods around and not be able to eat them. My blood levels were not being checked as often then, but my mother caught up with my cheating because I was sleeping a lot more that i used to. She sat me down and told me that the diet was for my own good and when I ate foods I wasn't supposed to I was damaging my health. It took several weeks, but I quit sneaking food and started to cook and experiment more with the foods I was allowed to have.
For the most part I have been very good with the diet, but when I was a teenager and undergraduate student my control was erratic at times. I was never really off diet, per se, because I was always drinking my formula, but at times I was very liberal with what I would eat. When I was a teenager I would sometimes eat a slice of cheese pizza or too much rice or pasta and my blood levels would shoot up. While I was at UCLA I used to reward myself with a slice of pizza or something else I knew I wasn't supposed to eat after taking a hard test or turning in a tough paper. I don't do this anymore because I have found that the few minutes of pleasure that result from eating the forbidden food do not outweigh the guilty feeling that gnaws at me afterward. More importantly, I can always tell when I have cheated on the diet because a tired/listless feeling creeps over me when my blood levels shoot sky high.
When I was living with my parents in Southern California, my brothers and I went to clinic at Loma Linda Medical Center. I went to clinic at UCLA Medical Center during the two years that I attended classes at UCLA. While I was living in New York I was seen every 6 months at the PKU clinic at SUNY-Stony Brook Medical Center. My blood was tested every time I go to clinic and my recent levels have been in the 8-10 range. These levels have been fairly consistent for the past two years. In the past few years the clinic has held several PKU picnics and parties. I always try to attend these events because I think interaction with the local PKU community is very important. I especially want to set a good example for the new PKU parents who are uncertain about the future of their young one. I am presently attending the metabolic clinic on the island of Oahu in Hawaii - even though I live on the island of Hawaii, itself! After attending metabolic clinics at the University I was studying at for so long, I never thought I would be *flying* to my clinic appointments!
I currently take my formula using the Phlexy-10 formula made by SHS. I used to take 300 gms of Phenyl-Free, but at over 1000 calories a day, I decided that was too large of a calorie expenditure for my diet. Phlexy-10 is a much, much better formula for adults with PKU, in my opinion. It comes in the classical powder formula drink mix, as well as a citrus flavored "candy bar" or even in capsule form. I presently take 120 Phlexy-10 capsules per day. I know that sounds like a lot, but I just sit down with a big drink & take 40 capsules 3 times a day. They are gone before I realize it! This is by far and away the best formula system for me, as I am often too busy (lazy?) to continually mix up a formula drink...
I currently try to take ~400mg of phenylalanine, or about 27 exchanges per day. I probably eat similar foods to other people with PKU. I eat a lot of fruits and vegetables; peaches, pineapple, pickles and carrots are among my favorites. For meals I usually eat limited amounts of pasta, rice, corn or potatoes with lots of various vegetables and sauces added to make a full meal. Lately, my favorite breakfast food is rice krispie squares. I really like to cook a vegetable medley consisting of sliced mushrooms and olives and chopped green pepper, onions and tomatoes. I saute the vegetables together in some butter or oil and keep them in a tupperware container in the refrigerator. This particular combination of veggies is very versatile and can be eaten with Italian or Mexican sauce and a choice of pasta, rice or even on top of bread (sort of as a little personal pizza).
In college I became an expert in unconventional cooking methods. Early on I learned that a small $10 electric hot-pot is an absolute necessity for dorm room living. I always used to cook low protein pasta in the hot-pot in my dorm room. I still use a hot-pot to cook lunch in my office at school when I am very busy and dont get the chance to cook at home. If I didn't like or couldn't have the vegetarian dish being served in the cafeteria in the dorms I used to improvise with what was on hand. I took sliced vegetables from the salad bar and cooked them in some butter in the microwave, then ate them plain, with some spaghetti/soy/mexican sauce or over some rice, pasta or potatoes.
I have never really had any difficulty with other people when dealing with my diet. I always knew what I could and couldn't eat and I never had anyone try to force me to eat anything or tease me because I was different from other people. Most of the people that have known about my PKU have been really supportive and caring. My friends have been fairly indifferent to my PKU. The fact that I can't eat the same things as some people has not kept them from being my friends, and when we all go out to dinner together they always make an effort to choose a restaurant that will have selections that I can eat.
When I was younger my family never did much traveling. When we did, we drove to our destination and took several coolers full of a wide variety of fruit and vegetables and our formula. While on the road, a significant number of our meals were McDonald's french fries!
Being an astronomer requires a lot of travel. In the last several years I have traveled to the Space Telescope Science Institute in Baltimore, MD and gone to conferences in New York City, Boston, Santa Barbara, New Orleans and even Berlin, Germany. I have a small 20 quart ice chest that is perfect for storing a jug of formula, some ice and some low protein snacks (like pickles, olives and apples). For past trips I would pack several cans of my formula and take the cooler on the plane/train with me, make my formula every night and drink it the next day. Now that I am on the Phlexy-10 system things are so much easier. I just have to pack a couple of containters of the capsules & make sure that I take them 3 times a day. It's so easy and convenient. When I am on an observing run at Mauna Kea the diet is easier to deal with because I have access to a refrigerator. The cooks at the cafeteria at base camp (which is kind of like a ski lodge populated by astronomers) know I am a vegetarian and they always cook me a special batch of grilled vegetables or potatoes if I can't eat what is being served.
When it comes to growing up and dealing with PKU it was great having my brothers around. I know that it must have been really difficult, not to mention expensive, for my parents to raise three of us on the diet, but I am really thankful that I can share experiences with my brothers. Every evening when my family ate dinner half of us were eating low protein foods! This fact made the diet easier for me to deal with. I used to cook meals, bake cookies and experiment with made-up recipes and it was always a comfort to know that I had my brothers to help me eat the finished products.
My general outlook on life is that I can do absolutely anything if I set my mind to it. That thinking applies to managing my PKU as well as my academic life. I remember sitting up late at night trying very hard to do my high school geometry homework. At the time, I thought geometry was the hardest thing in the world and I told myself if I got anything better than a 'C' I could do anything! I persevered and actually finished the class with an 'A'.
Whether it be dealing with my PKU, or the stresses of academic life, since my experience in high school geometry class I think I can accomplish anything if I believe in myself. I think this is a great philosophy for all aspects of life, for everyone. A person should never think that they can't do something simply because they can't eat the same things other people can, because they have been told that they aren't as smart as other people, because they are female or any number of other reasons. A person should only conclude that they can't accomplish something after they have made a valiant effort to do it.
Staying on the diet is my only option. However, living with PKU is something that i am accustomed to. The most challenging aspect of the diet for me has been resisting the temptation to eat high phenylalanine foods that I have tried before. I do sometimes wish I could eat pizza or other high phe foods, but I value my health and well-being above these short-lived cravings.
Dealing with PKU does have positive and negative aspects. The main negative aspects are that the low protein foods (and if you don't have some sort of coverage, the formula) are extremely expensive. The diet also requires planning everything to eat ahead of time, which, of course, makes traveling and eating at restaurants very difficult. On the positive side, if a variety of fruits and vegetables are incorporated, the PKU diet is inherently low-fat and quite healthy. Most of my diet consists of fruits, vegetables and pasta, and my main source of cholesterol is from the butter I eat on pasta. I would imagine that there are probably very few people with PKU that suffer high cholesterol levels. Another positive aspect of PKU is the level of self control one needs to develop with the diet carries over into other aspects of life. I sincerely feel that PKU has caused me to be more self-disciplined.
My main advice for parents of children with PKU is to try not to make PKU a big deal or a central issue in the childs life. What you eat is not even remotely close to being the most important aspect of life. I think that parents should get their children involved in sports and other extracurricular activities at a very young age to help them realize that what they eat does not determine who they are. At the same time, it is also necessary that the child understands the importance of the diet to their health and development. Let the child know what foods they can and cannot have and allow them to actively participate in their diet management at a young age.
My advice to teenagers with PKU is to stay on diet, at all costs! The teenage years are the most difficult for a person with PKU. You have more freedom in decision making than you have ever had before, and you will be more tempted to eat a forbidden food to "fit in" with your friends. Being a teenager is tough enough as it is, it is not worth it to further complicate your life by going off diet and having high blood levels cloud your judgment and damage your health.
Growing up with PKU is not easy. I would be lying if I said that it was. However, when it comes right down to it, PKU is nothing more than a dietary inconvenience. I know PKU parents who have had their doctors say that their child's PKU will never allow them to lead a normal life. In a lecture my UCLA psychology professor told my class that "PKU is *defined as* mental retardation", she never mentioned that it is treatable and not all people with PKU are severely mentally retarded. Productive adults like my brothers myself and the many successful adults that have shared their stories with the world are out to prove that people with PKU can lead healthy, happy, sucessful lives and that "PKU" is *NOT* synonymous with "mental retardation". The fact that we can't eat all the same things "normal" people do has not kept us from living our lives and making our dreams come true!
Stories from other Adults with PKU