|
|
Presented at the SHS North America Metabolic Conference on PKU (April 7, 2000, in Washington, DC).
Thank you very much for inviting me to talk to you about my experiences with PKU. If any of you have any questions about PKU adults leading normal lives, I’m fitting this speech in on the off day between the semi-finals and the national championship for college hockey that is being held in Providence, Rhode Island. (Just in case any of you are wondering, North Dakota and Boston College will be playing for the national championship on ESPN tomorrow at 7:30.) But, if you look at this graph, you will know that I was asked to come here today to talk with you about returning to diet and not college hockey.
My feelings and views about PKU have been shaped by my experiences. I have been everything from the star patient to a hell-child. I have gone from loving my formula to dumping it down the drain. I have gone from asking, "why me?" to asking, "why me?"
I was born not too far from here in Silver Spring, Maryland at Holy Cross Hospital on August 18, 1970. When I was three days old my Guthrie test came back with a positive of reading of 6mg/dl. At fourteen days, my blood phenylalanine level was 52.4mg/dl.
I am one-forth Irish and three-fourths French Canadian. My parents, Peter and Judy Foster, live in the Waterville, Maine area where we moved just before I turned seven. My sister, Erin, will turn 35 in September. My Brother, David, just turned 33 in March. Neither of them have PKU.
After I was diagnosed with PKU, I was enlisted to be one of the participants in the National Collaborative Study of Children Treated for PKU. My parents were told to keep my blood levels between 1.5 and 5.5 mg/dl.
At the age of five or so, my parents were told I was in the pool of participants in the National Collaborative Study that was slated to go off diet. My parents saw what positive affects the diet had on me and they refused to have me go off diet. When we moved to Maine the officials there told my parents that all the kids in Maine were off diet and they again refused to have me taken off diet.
I don’t recall many details about my childhood concerning my diet except for the fact that my parents were very strict with me. At that time, Lofenalac was the only formula that was available. I am told that for a long time I actually liked it, although I have a hard time imagining that now.
Compared to today, there were very few low-protein products available. I remember two shapes of pasta, cracker toasts, and wheat starch from which my mom made bread, crackers, and pancakes. Or at least she tried. For the most part, what I remember is my diet primary consisted of fruits, vegetables, Rice Krispies cereal, and jellybeans (not necessarily in that order).
For the most part, I was a happy-go-lucky little kid growing up. For a long time I didn’t know or think that I was different from anyone else. But, gradually, I figured out the games the psychologist played with me weren't games. They were IQ tests. It was all being done so that they could assign a number to me that they said told them how smart I was.
All anyone needs to know is that I was smart enough to know that I didn't like being subjected to the physical or psychological tests year after year. I was smart enough to know I didn't want to be put on display. I was smart enough to know I didn't want to answer question after question from doctors who had never seen someone quite like me. I was smart enough to know I didn't want to have electrodes stuck to my head for an EEG or any other reason.
I wanted to know why all those doctors weren't smart enough to look past the numbers and the mountain of medical records to see me. I wanted to know why all those doctors weren't smart enough to see that I was scared and lonely. I wanted to know why all those doctors weren't smart enough to understand. I wanted to know why all those doctors weren't smart enough to know the answer to the question "why me?"
PKU and the diet became my weapon in a war that had no victors. It was my way of making my parents hurt as much as I did. When I got mad at them, I'd eat things I shouldn't. At the age of nine or ten, I gradually started to eat more and more things that I knew I shouldn't. I refused to drink my formula. It got to the point where I was essentially off diet even though we pretended that I was. I created a communication barrier that made PKU an issue that was not open for discussion.
When I was a kid, I wasn't smart enough to know that being off diet wasn't the answer to any of the questions I had. I wasn't smart enough to know being off diet didn't make being scared or lonely go away. I wasn't smart enough to know that being off diet didn't make PKU go away. I wasn't smart enough to know that being off diet wasn't the answer to the question "why me?"
If I had to choose, the biggest factor in my going off-diet was my lack of self-confidence and self-esteem. Growing up is hard enough, without kids making fun of you. I know some of this was my distorted perception rather than reality; however, for me, my distorted perception was my reality.
I majored in Political Science at the University of Maine. I definitely had to study much harder than my friends. When they were out drinking or sleeping I usually was in the alcove studying. I don’t know if my having to work harder than my peers was more a product of the fact that I had learning disability throughout my academic life or that my blood levels were probably really high. It probably was some of both but more the later than the former. I graduated from The University of Maine in 1992 with academic distinction.
Following college, I was accepted into the University of Maryland Baltimore County policy science Master’s degree program. I took two full years of classes and completed an internship in the interim summer. Following completion of my class work, I wrote a seventy-page thesis as well as pass a comprehensive examination. In the summer of 1996 I successfully completed all of the requirements for my Master’s degree.
In college and graduate school, PKU was not a factor. I didn’t let it become one. No one knew I had PKU; and as far as I was concerned, I didn’t have it. For a little while at least, I had found the "cure" for PKU. The cure was called denial.
For a long time when anyone asked me if I would ever go back on the diet, I wouldn’t have even given it a second thought. The answer was easy--the answer was "no." Upon completion of my Master's degree, my focus turned from my books to myself. The books were easier. I realized that over time my reasons for being off diet faded so much they faded away. Returning to the diet was returning to what was normal. For me, having PKU and the diet is what is normal.
Returning diet was relatively easy because it was what I WANTED to do not what I HAD to do. It makes all the difference. There were definite obstacles and there was a definite learning curve but, all in all, it was relatively easy because of the struggle my parents went through in order to keep me on the diet as long as I was.
I was told to eat 300mg of phe and to take 100g of XP Maxamum, the formula I had chosen based on a compromise between taste and fat and calories. It took several months to fight the insurance battle and to get my levels down to where they were consistently between 2 and 6 mg/dl. After I did that I went to Johns Hopkins for my first check-up since I was sixteen. I had the option to take the easy way out -- I could have seen different people at Hopkins or gone to a different hospital altogether. I didn’t. I owed it to them and to myself. After all these years, I was finally able to say "thank you."
Going back to Hopkins brought back a lot of memories, none of them pleasant. I met with Dr. Kenneth Wessel, the psychologist I had seen most of my life. He conducted another IQ test on me. My definition of the word "tirade" was what I used to do to him. He said he didn’t remember and I told him not to lie.
After seeing Dr. Wessel, I met with Dr. David Valle, the medical doctor I had seen most of my adolescent life. One of the things that started happening since I had gone back on diet was that I lost a tremendous amount of hair. We talked about my loss of hair. Because hair is primarily composed of protein, a possible explanation for my hair loss was that I was not getting enough protein, so the amount of formula I drink was increased to 110 grams.
I had blood drawn. The tests came back showing that I was severely anemic so he had me take three times the RDA of iron for three months. Dr. Valle asked me to hold out both of my arms straight and there was a slight shaking in my left hand. The shakiness in my hand was something that I had never noticed.
I noticed another thing that had happened to me since I had returned to diet when I looked at a photograph of my niece and I. I noticed for the first time my hair was changing color. I had heard something about the relation of tyrosine and pigmentation but I never thought my hair had changed color. It was really surprising to see the pictorial evidence.
In October of 1998 I moved from Maryland to Boston. Prior to accepting my new job I asked what health insurance my potential new employer carried and I called the health insurance company directly. I wouldn’t be able to afford the approximately $9,000 a year it costs just for food and formula (not to mention doctor’s visits) without it.
Ultimately, I chose to receive my care from Dr. Mary Ampola at the New England Medical Center. Dr. Ampola operates a little differently than most places in that I go to her office every month to get my blood drawn rather than sending it on filter paper. Dr. Ampola increased the amount of XP Maxamum I drink to 130 grams a day.
I work at First Market Research as the Focus Group Coordinator. The office I work in is very small and everyone knows that I have PKU. Most of my co-workers really don’t know much or care about it. The Vice President of the company, Margi, is probably the nicest person you could ever meet. In the afternoon I drink my formula in her office while I chat with her. She has even tried the Phlexy-10 bar and some of the new Loprofin cereal. That is beyond nice.
The President of the company, Jack, is as opposite as you can get. When I was in Margi’s office downing 40 of the Phlexy-10 capsules she asked Jack, who was also there, if he thought he could take 120 pills a day. He said that he already takes 15 vitamins a day and it would be no problem. He told me that PKU is just an allergy and if I followed his diet and exercise program for six months, PKU would go away, I’d lose weight and I’d be beautiful.
Even without the Jack’s of the world, being an adult with PKU can be very frustrating. I am constantly being asked how old my child with PKU is. I get tired going to children’s hospitals or pediatric departments for my care. I get upset that so many adults with PKU don’t want to get involved. I get upset with the insurance industry and the fact that so many adults have problems getting coverage.
And, I’d be lying to you if I said managing the diet was easy all the time. Even though I use just about all of the low protein products that are available, there are times when I get bored with my diet. I try to plan ahead and cook on weekends but I don’t always have time or feel like it. Cooking ahead is also difficult because I live in an apartment and my freezer and storage space is only so big.
The hardest aspects about having PKU for me however aren’t the dietary ones; they are the psychological ones. Now that I am almost 30, I still ask "why me?" The question still remains the same; but twenty years has changed the inflexion and the intonation, thus it has changed the very question itself. I used to think that having PKU was the worst thing in the world that could ever happen to anyone. Now, I can only wish I was able to think that is true.
Shortly after returning to the diet, I met a person who had not been diagnosed with PKU until the effects of high levels of phenylalanine had severely taken their toll. It was a strange feeling knowing how easily the tables could have been turned. I was born 10 miles from D.C. where they didn’t do newborn screening at the time. Why me?
At the first metabolic conference I ever went to, I learned about much more rare and much more devastating diseases that in many ways are very similar to PKU. I met a four year old who was unable to walk or talk because he hadn't been diagnosed with one of those diseases and had suffered a debilitating stroke. Why me?
I wish I could say that all of the thoughts and feelings about PKU that I have are all like this but that would be a lie. There are times when I think I won't be able to choke down another restaurant salad even if my life depended on it. There are times when it still feels like I'm the only one with PKU. There are times when I want to kick and shout and scream and cry. There are times when I do.
At the same time, there are so many positives of having PKU and returning to diet. The fact that I can even stand up here before you today and talk about PKU is evidence of probably one of the biggest positives from returning to diet. I am starting to break out of my shell that I have been in for over twenty years. It is a slow process but it is a very thick shell. I am rediscovering the devilish glint in my eyes that Dr. Neil Holtzman observed when I was a little kid, and I am developing an adult version of it. The positives of returning to diet far outweigh any of the negatives and they are what I focus on.
I focus on the people I have met and the support they have given me. I focus on the Mullaley family and how Trish and Mark have made me a part of their family. Trish Mullaley is an unbelievable person. She has an incredible amount of energy and has been an incredible source of support. She helped give me more self-confidence than any one person has.
I focus on Trish’s daughter, Jennie, who is ten years old and has PKU. Through our friendship, Jennie is teaching me the joys of being ten that I never knew existed. Whether it is wearing face paint to an Austin Prep Girl’s Hockey game or pretending to be a manikin in a store window, she makes me laugh more than I can tell you.
I focus on Tracy Beck, who is twenty-seven and has PKU. I met Tracy a year ago through the PKU Listserv and she has become probably the best friend I have. Tracy is in her fifth year of her Ph.D. in Astronomy at the State University of New York- Stony Brook and has two brothers with PKU. When I was out in Los Angeles in January to have an MRI done for the follow up to the National Collaborative Study for PKU, I was able to meet Tracy’s entire family and get lots of embarrassing "dirt" on her.
I focus on the project Tracy and I have undertaken to write a book about what it is like being an adult and having PKU. We have sent out over 100 questionnaires to individuals with PKU in the U.S. and beyond and we have only just started.
I focus on my parents and family who have been there for me from the start and who will always be there for me. I focus on my friends who couldn’t care less whether I have PKU or not.
I focus on creating a full-time paid position so that I can focus my time and energy to be an advocate of issues and coordinator events involving PKU and other metabolic disorders.
And, I focus on things that don’t involve PKU at all like whether North Dakota or Boston College will win the college hockey national championship tomorrow in Providence.
I wish I could give you a laundry list of tips and tricks that you could take away from here so that you would know what to do every time an adult comes to you looking to go back on diet. But, I can’t. The reason I can’t is that we are all different. And, if I were to give you one piece of advice that would probably be it. We are all individuals. Treat us that way: as individuals.
There are two things I can say, however, that might help. Please don’t give u! You never know when your former hell-patient will come to you wanting to go back on diet. Speaking as a former hell-patient, we can change and you just might be pleasantly surprised.
The last thing I can say is "thank you." I know you don't hear it a lot or often enough. Thank you for your time, dedication and support. Without it, I couldn't make this speech. I wouldn't be able to.
(Just in case you were wondering... North Dakota beat Boston College in the championship game 4-2. It was a great time!!!! )