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By Sarah Foster
The following story is from Sarah Foster, a young adult with PKU who candidly shares reflections on growing up and her decision to return to the diet as an adult. It is a long story, but well worth the reading.
On January 1, 1997, I sat in my parents' living-room in Oakland, Maine, and I did something I hadn’t done in over ten years--I talked about PKU with my parents. I said, "This is very difficult for me. We all know that I haven’t been on the PKU diet for a long time. I would like to try to go back on the diet, but I am not even sure I know how to anymore."
My name is Sarah Foster. I was born in Silver Spring, Maryland on August 18, 1970. I was seen at Johns Hopkins Hospital in Baltimore. I was told that I was very good with my diet when I was little. I owe a lot of my success to my parents and to the health professionals who cared for me through the years. There isn’t a lot I remember about having PKU when I was little that is positive. I guess that is one of my problems with PKU--I have a selective memory and, it seems that I only remember the bad things. For example,
My family moved to Maine a few weeks before my seventh birthday. The officials in Maine told my parents, at the time, kids in Maine were taken off the diet at the age of six. My parents disagreed and kept me on the diet. In hindsight, I am very thankful they kept me on the diet.
My Mom and I attended a PKU picnic during one of my yearly trips to Baltimore for a check-up at Hopkins. The only person with PKU I remember meeting had been diagnosed late and was much younger than me. The late diagnosis had an effect on her, and she was not someone I felt I could relate to because she was not like me. I am sure there must have been other kids with PKU at the picnic, but I don’t remember them.
I can’t really say when I started to stray off the diet. My definition of going off the diet differs from everyone else’s. To me, I went off the diet when I went to college. I say this because that is when I ate anything I wanted, whenever I wanted. I stopped drinking the formula and started eating things I shouldn’t much more frequently when I was twelve or thirteen -- so, technically, that is when I went off the diet. When you're little and your Grandfather tells you that when you are able to go off the diet he is going to buy you the biggest steak he can find, you get the impression you are missing something.
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 a lot of this was my distorted perception rather than reality; however, for me, my distorted perception was my reality.
I was very angry about having PKU. When I was Sophomore in high school, I had to write a report for biology class about an experiment I had conducted. I chose to write about the "genetic experiment" that I called my life. (That is one thing PKU is good for -- if you need a topic for a report in school, having PKU is choice material.) My conclusion for the report was that I was "alive and well in Waterville, Maine." I wrote the report at the height of my hatred of having PKU. Looking back, I didn’t make a very good scientist. The facts that I did report were very skewed, and I failed to mention very important variables in the experiment -- like the help and care that I received from my parents, family, and health professionals.
I graduated from the University of Maine with distinction in Political Science in 1992. Following college, I attended the University of Maryland Baltimore County, where I received my Master’s in Policy Sciences in 1996. During that period of my life, PKU was not a factor. I didn’t let it. No one knew I had PKU; and as far as I was concerned, I didn’t have it.
I didn’t tell my best friend from college about having PKU until August 1997. I felt as if I had been lying to her for over eight years. She is pregnant, so I explained to her that when her child is born they are going to prick its heel and screen the baby for a disease called PKU. When they did that test on me when I was a baby, it came out positive. I explained the diet as much as I could. When I finished, she said, "I wished you would have told me when we were in school; I would have helped you with your diet." I underestimated many people for a long time, including me.
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. The change in the answer from no to yes was a long and difficult process. It was a process I didn’t even know I was going through until it was complete. Many people have asked me why I have returned to the diet, but I can’t pinpoint any one thing. I don’t have a magic answer. No one does. The reasons why people go on or off the diet vary from person to person. There are several factors that influenced my decision to return to the diet, and there are several things that were not factors in my decision.
The factors that did not influence my decision to return to the diet are much easier to enumerate than those that did. I did not return to the diet because I was having health problems. I did not return to the diet because I wanted to get pregnant. I did not return to the diet because of the article in National PKU News entitled "Young Adults Off Diet - Lives in Danger." I had made my decision before reading the issue, but it certainly was nice to have some reinforcement that the decision I made to return to the diet was the correct one.
I returned to the diet for a variety of reasons, not all of which are known even to me. I returned to the diet in part for the following reasons:
When I decided to go back on the diet, I didn’t know exactly what approach to take or whom to contact. I wrote a letter to the Maryland Department of Health and Mental Hygiene inquiring about the programs and services available for adults with PKU who are interested in going back on the diet. The letter was forwarded to Betsy Emmerick, a State dietitian. She wrote to me, and I called her to set up an appointment. I was scared and nervous about confronting such a difficult issue for me. At the same time, I was very confident and certain going back on the diet is what I wanted to do; so that made the whole process easier.
During my visit with Betsy, we talked about several things. I made it clear to her I wanted to take this process slowly so I could become used to each aspect without being overwhelmed. I had a lot of questions, concerns, and apprehensions. One of my biggest apprehensions was regarding the formula. When I was a child, I drank Lofenalac. To put it mildly, I don’t have very fond memories of it. When I was a child, I didn’t understand its importance and the role it played in maintaining the diet. Betsy explained that without the formula my body would not be able to maintain the proper levels of phe. She gave me a couple of samples of different formulas on the market to find out which one I liked the best (or the one that made me gag the least). She had some Phenyl-free mixed, and she gave me a sample of it to taste there. Anything that I had forgotten about having PKU rushed back to me when I took that sip.
Betsy told me she thought I should probably be eating around 300 mg of phe a day. She told me I could count milligrams or exchanges. The whole concept of exchanges was just being developed when I was deciding that I didn’t like having PKU so the concept was pretty foreign to me.
While I was talking with Betsy, someone else in the office happened to be making a phone call to parents whose newborn’s initial Guthrie Test had come out positive. She asked the parents to contact their doctor to bring the baby in for a second test. It was obvious that the mother on the other end was very concerned and didn’t understand what she had just been told. I am sure that is not an easy telephone call as a new parent to receive. I asked what the likelihood was the baby would have PKU. I was told that out of all the births in Maryland in a year, about five hundred of the initial tests come out positive -- of those, maybe five would be diagnosed with PKU.
When I got home from my visit with Betsy, I ordered a gram scale. I explained to Trish at the New England Alliance for PKU I was going back on the diet and I was very apprehensive about the formula. She suggested I add French Vanilla Coffee Creamer to the formula. Thanks to that suggestion and XP Maxamum, I knew I had found something that I could deal with for a lifetime.
It took awhile, but I fought the fight with my health insurance company for coverage of formula that many parents have gone through or are going through now. The company I work for is based out of Cincinnati; consequently, that is where my insurance comes from. So, despite living in Maryland, where there is legislation requiring coverage for formula, I still had to fight the fight. I won. Persistency has its rewards.
By late April, I had all of the materials necessary to be truly on the diet, though I had been eating in keeping with the PKU diet since mid January. I knew that I was "going to make it" when the people at work decided to go out for ice cream sundaes. In the past, when they asked me what I wanted I would have agreed with the sundae, but I asked them to see if the place had any sorbet. I got the strangest look from everyone. That is when I explained to them that I had PKU and what it meant. For some reason, that is when I knew I could do this.
I have been sending my blood levels to Betsy. Shortly after I started drinking the formula, my blood levels dropped drastically. Betsy was concerned that I wasn’t eating enough Phe. I took that as a license to eat more potatoes, etc., and my level rose to unacceptable levels. My levels are now back down where they should be. My last few blood levels have been 3 mg/dl (180 micromol/L). I just sent one in for which I have not yet received a level. I anticipate that it will be higher. (It was higher. It was 3.4 mg/dl)
The hardest thing for me so far is measuring and keeping track of what I eat. It is very difficult. My problem is that I want to be able to do the diet without having to work at it. It is not that easy. I don’t know the values of all the foods yet, and I am not great at estimating amounts of food. Following the diet takes a great deal of planning, which is very difficult when you work as much as I do. I have discovered it is really important that I never let myself get too hungry. When I am, I tend to grab whatever I can; and I am more likely to go off the diet. I try not to let a day when I have probably eaten more than I should have get me down. I just try to eat low the next day. I am a big fan of college hockey. The coach of the University of Maine hockey team, after losing to a fierce rival in overtime, said, "It’s not how hard you fall, it’s how fast you get up." The team went on to win the National Championship that year. They finished the season 42-1-2; that game was their only loss.
This past August, I attended the Maryland Alliance for PKU camp for a few days. I was apprehensive about attending the camp, but I can never be thankful enough that I did. Short of returning to the diet, it was the best thing I have ever done for myself. For the first time in my life, I felt as if I wasn’t alone anymore. I have a picture of some of the kids from camp on my refrigerator. I look at that picture every day.
I met kids with PKU of all ages and their parents. I had equally rewarding conversations with the kids and the parents. I think I offered a unique perspective in that I could relate with both the kids and the parents.
I got so much from talking with the kids. I was very inspired by them. It was so nice to talk with someone who really understood--like how annoying it is when your parents go through this huge explanation to a waiter in a restaurant about how you have PKU. To this day, my mother still doesn’t understand. I was very honest with the kids, and I think they were with me. Some of the parents asked me what we talked about, but I wouldn’t tell them because I do understand.
Some of the kids were very well adjusted with having PKU while others were not. I didn’t have to be told who they were; I could tell by talking with them. I know what it looks like--I have looked in the mirror often enough. I saw my younger self in some of them.
I learned a lot from the kids and I hope they learned a lot from me. I learned how to count exchanges. I learned that it is okay to have PKU. One of the girl’s philosophies was that she can’t hate having PKU because she is PKU. So, if she said she didn’t like having PKU, she was essentially saying that she didn’t like herself. I think she is a very wise nine-year old. I learned I have a lot to learn.
I talked with some of the parents at great length. I often wondered what it is like for parents to have a child who has PKU. One of the things I used to say to my parents when I was mad at them was that they didn’t know what it was like to have PKU. No matter how hard anyone tries to understand what it is like to have PKU, the only way to know is to have it. I have had people say to me they think they could do the diet if they had to. However, that is precisely the point--they don’t have to--that makes all the difference.
In contrast, I don’t know what it is like to have a child with PKU. I know I certainly didn’t make it easy on my parents. No matter how much I try to put myself in my parents’ shoes, I can never know what it is like to have a child with PKU. (Unless I have one of my own in the future.) It is a two-way street and I am only coming from one direction. I know the PKU side of the street very well, and I hope I was able to give some of the parents a road map to the PKU side of the street.
Toward the end of one of our conversations, one of the parents said, "I guess your right. Kids with PKU have a little more to deal with than the average kid." I corrected him. Kids with PKU have a lot more to deal with than the average kid. It is hard enough being a kid, whether or not you have PKU. The issues, whatever they may be, may appear to be minor or inconsequential to an adult, but to the kid they mean everything.
One of the parents told me about how she caught her daughter in the mall eating Chicken McNuggets with her friends. She said she went out to the car and cried. She said she wondered why they are going through all of this trouble and expense. (Because she is your daughter. You love her, and you want only the best for her. That is why.) Another told me how she caught her daughter eating pizza at the pool and proceeded to lie about it when caught and confronted. I know it hurts. I did very similar things. I was just like them when I was their age. I saw the hurt in my parents’ eyes and heard it in their voices.
I don’t have the answers. I wish I did. I can only implore parents to keep up with the enforcement of the diet. It is not easy. Kids don’t make it easy. Despite the fact you will always be the parent, there comes a time when the child has to make his or her own decision about the diet and take responsibility for his or her own life. You can only hope that when that time comes, the child will use what you have reinforced their entire life and make the decision to stay with the diet. Inevitably, the child will think they have reached that point much sooner than the parents do. As odd as it may sound, I think the "game" my family played (that I was still on the diet when I really wasn’t) helped me return to the diet in the long run. I knew that my parents’ expectations with respect to the PKU hadn’t and wouldn’t change. It was my expectations of me that changed, not my parents’. I don’t, and would never, advocate that kids go off the diet--ever. I understand why they do. I did. I wish I hadn’t, but I can’t change the fact that I did.
The parents asked me for some tips of how to improve the camp and to help keep the children on the diet. The camp is a wonderful thing. It is vitally important for kids with PKU to have exposure to other kids with PKU. It is comforting to know that you aren’t alone dealing with these issues although there are times when you might feel as if you are. The nicest thing about the camp is it is the one place where PKU isn’t an issue. That may seem a rather odd statement considering the whole focus of the camp is PKU. However, when everyone has PKU, it is no longer a factor. I suggested that some elements might be added to the camp, such as:
I also have some tips for keeping children on the diet:
I went home to Maine for a vacation at the end of August 1997. My parents where very surprised at all the changes and advances in the treatment of PKU that have been made since I was on the diet years ago. My mom was astonished I could make bread in the bread machine. She told me how she was literally in tears after attempts to make me bread when I was little. They tried some of my pasta and couldn’t tell the difference between it and regular pasta. The advances that have been made in food are astounding. While nothing can ever take away the fact that kids with PKU want to be like other kids in every way, the advances with respect to the development of foods will go a long way in easing some of those issues.
On September 18, 1997, I went to Johns Hopkins for my first check-up since I was sixteen. I wanted to wait until I was totally comfortable with the changes I had made. In addition, the check-up was precipitated by the fact that I was loosing a lot of my hair since going back on the diet full force. Returning to Hopkins was a strange experience, to say the least. I had the option to take the easy way out -- I could have seen different people at Johns Hopkins or gone to a different hospital than when I was a kid. I didn’t. I owed it to them and to myself. After all these years, I was finally able to say thank you.
I felt as if I was in a strange episode of the Twilight Zone. Suddenly, eleven years vanished. I met with Dr. Wessel, the psychologist I had seen my entire life (or at least the only one I ever remember). I did poorly on the math portion and well on the verbal portion of the IQ test. It wasn’t something I didn’t already know. My definition of the word "tirade" was what I used to do to him. He said he didn’t remember; I told him not to lie. He laughed. I don’t know if going off the diet had an effect on my IQ.
After, I met with Dr. Valle, the only doctor I remember ever having seen, though I know I saw another doctor when I was younger. We talked about my loss of hair. He thought the hair loss may be my body reacting to such a major change in diet and it would stop. Because hair is primarily comprised of protein, another possible explanation for the hair loss was that I was not getting enough protein so the amount of formula I drink was increased. (My hair has stopped falling out and I am growing back the hair that was lost.) I had blood drawn. The tests came back showing that I was severely anemic, so for the next couple of months I will be taking iron pills. (There is no evidence that my anemia was related to PKU.) Generally, Dr. Valle found me to be in good health. One thing that was surprising to me -- when he had me hold out both of my arms straight, we noticed a slight shaking in my left hand. I had always thought that going off the diet had had no effect on me. I was wrong.
In March of 1998, I attended the New England Connection for PKU and Allied Disorders conference in Taunton, MA. The New England conference differed from the Maryland camp in its focus, but the intent was the same: a gathering of people with something in common, to share, learn, and grow.
Personally, this conference was much more difficult for me than the camp because other adults on diet attended the conference. At the camp, I was the only adult on diet. There were two other women with PKU at the camp that I didn’t really have a chance to talk to at any length. Because of varying circumstances, they were not on diet. At the conference there were eight adults on diet, and one not on diet, with whom I personally had some form of contact.
Meeting the other adults with PKU on diet was a very positive experience. But, it was difficult because I had to deal with things I never had to before. When you spend your life thinking you are the only one in the world dealing with certain issues you have to rethink a lot of things. It isn’t exactly easy to look at myself in the proverbial mirror of the past because often when it comes to PKU I don’t like what I see.
The adult portion of the conference mainly focused on the realities of dealing with PKU in the box, can, microwave society that we live in today. (Issues that parent of children with PKU have to deal with as well.) The over riding issue was the lack of time. The majority of us live by ourselves so the problems of not wanting to cook for one and spoilage of food were common concerns.
The morning portion focused on breakfast. We all handle breakfast in our own way: nothing to vegetable stir-fry. Dianne Sullivan demonstrated and gave us samples of easy to make French toast. (Which, despite the comment that it tasted like chicken, did taste like French toast.)
The theme for the afternoon program was titled "One Thousand and One Pasta Dishes With Only Two Ingredients In Under Five Minutes." A few of the dishes used a few more than two ingredients, but they were quick, easy, and tasty. I learned a lot more than different cooking tips at the conference, however.
Friday night Trish asked me if I would take part in the adult PKU panel and I agreed. During the adult panel, someone in the audience (I believe it was a social worker) asked what effects each of us experienced while we were off diet. Out of the four of us, two had trouble concentrating and behavioral problems and the other two hadn’t experienced any effects from being off diet. The person who asked the question said that often times the person who has PKU doesn’t notice any effects of being off diet because the person learns to live with and adapt to the effects, while those around the person do notice effects of being off diet.
I have thought a lot about what she said. It is difficult for me to objectively evaluate that statement with respect to me. I believe there is some validity to what she said. It is easy to rationalize away many of the reported effects. Did I ever get tired while I was off the diet? Yes. Did I ever have a headache while I was off the diet? Yes. Did I ever emotionally overreact to something while I was off the diet? Yes. Did I ever have trouble concentrating while I was off the diet? Yes. Does anyone without PKU ever get tired? Yes. Does anyone without PKU ever get headaches? Yes. Does anyone without PKU ever emotionally overreact to something? Yes. Does anyone without PKU ever have trouble concentrating? Yes.
Personally, I can’t separate PKU from my behavior. I can’t find where PKU ends and where I begin because there isn’t such a place. I am who I am today because of my life experiences; PKU plays a significant role in those experiences. Can I notice any difference since returning to the diet? Yes. I notice an increased level of energy. Do I still get tired? Yes. Do I still get headaches? Yes. Do I still emotionally overreact to things? Yes. Do I still have trouble concentrating sometimes? Yes. Don’t you? What we need to focus on is the level of intensity and the frequency of these things. While I was off diet I experienced what I perceived to be normal levels of frequency and intensity, but I’m sure my family has a different opinion. I guess that just validates what the social worker said.
We were also asked how the other kids treated us when we were on the diet when we were little. The majority of the adults (all, I believe) except for me were taken off the diet at the age of five. Upon learning that, it made me even more thankful my parents were so insistent I stay on the diet. While I know I was extremely lucky to be on the diet for the length of time that I was, because of that, I have some different issues from the adults who were taken off the diet at the age of five. (And, I’m sure they have issues that I don’t. It must have been even more difficult for them to return to the diet than it was for me.) Like kids with PKU today, I was on the diet while I was an adolescent.
I think self-confidence and self-esteem levels differ greatly between those who were taken off the diet early and those who were on the diet during adolescence. More so than any other time in life, adolescence is a time when any kid wants to be like everyone else and not to stand out from the crowd. I was very self-conscious about having PKU. I did everything I could to not let people know that I had PKU. I was tutored for my learning disability before school started in the morning so I wasn’t taken out of class. I didn’t bring formula to school. The list is endless. In hindsight, I don’t think the kids treated me much differently from anyone else. It was more my distorted perception then anything else. However, just because my perception was distorted didn’t make it any less real to me.
With the new foods today I would think it is getting easier to "blend in" than it was, but I’m sure it is still difficult. I think wanting to be like everyone else is probably the single biggest underlying reason why kids take themselves off the diet.
During the conference, I was able to meet a few of the parents of the adults with PKU. The saddest thing I have to report to kids with PKU is that it doesn’t stop when you get older. The "it" I am referring to is the nagging. It doesn’t matter if you’re eight or twenty-eight, they still ask you if you have drunk all of your formula and if you should really be eating that. They still think that you starve yourself before you have to draw your blood so that you will get a low level. Once a parent, always a parent I guess - especially when it comes to PKU. Just because you’re older doesn’t mean they stop caring.
Following the conference, five of us with PKU (four on diet and one not), along with three other people, had dinner in the hotel dinning room. The one person not on diet apologized to those of us on diet for ordering a chicken sandwich. Talk about peer pressure PKU-style. She said this was the first time she had ever gone out to dinner with anyone who had PKU. I corrected her. I told her she has dinner with someone who has PKU every night. Herself. Just because you aren’t on the diet, doesn’t mean you don’t have PKU.
This was her second year at the conference. She was attending the conference to educate herself on the research that is being done on PKU should she ever want to have children. But, because she didn’t feel like she was experiencing any effects of being off the diet, she was not ready to return to the diet at this time. Many people at the conference were strongly urging her to go back on the diet. While I wasn’t by any means telling her to stay off the diet, I also wasn’t someone who was strongly pushing her to go back on the diet either. I know what it is like to have people always nagging you about returning to the diet. I didn’t like it, and I can’t imagine she enjoys it either.
The results of nagging are often the exact opposite from the desired intent. Short of returning to the diet, I think she is probably doing the best possible thing she can do for herself. She is educating herself and not ignoring the fact that she has PKU. She may return to the diet in the future because she wants to do something positive for herself or for her future children; she will return to the diet only when she is ready and not before then.
Following dinner, we had planned to go out together. I bailed out. There were many things for me to think about for one day. I went up to my room and cried. I did so because I am a thinking and feeling person, not because I had high blood levels.
In addition to all the issues about PKU I was confronting, the thing I found most upsetting did not involve PKU at all. It was to learn about the other metabolic disorders that were also represented at the conference. I never felt so lucky to have PKU as I did then. I had overheard something very briefly about MSUD at the Maryland camp however, this conference was the first time I had really ever learned anything about any other metabolic disorders. The fact that some of these disorders can be tested for during newborn screening and aren’t because they are so rare is utterly appalling. I’m sure at one point in time the exact same thing was said about PKU.
If someone didn’t come away from those lectures emotionally upset, I would question whether that person has any emotions at all. It makes me give thanks to the many people who have worked so long and so hard to make the progress that has been made with PKU. If they hadn’t fought so hard, I couldn’t write this. I wouldn’t be able. More than anything, the one thing in my life I regret the most about having PKU is that I never said thank you to all of the people who have helped me have a normal life.
I was able to combine my trip to the conference with visiting some close friends and family in the Boston area and attending the College Hockey National Championships at the Fleet Center before returning to Baltimore. It was the first time since returning to the diet that I had traveled anywhere other then back home to Maine. It was an interesting learning experience. I pre-weighed and packed all of my formula. I ate PKU pasta I brought with me while I was visiting my friends from college. They were wonderful about asking me what else I could have to eat. They asked me a few questions about PKU out of curiosity, but PKU was never an issue. I underestimated many people for a long time.
The only time PKU did become an issue was when I was at my cousin’s apartment and drank a few glasses of ice tea from a pitcher which I later found out contained NutraSweet (aspartame). When I did realize it, I was so mad at myself for not checking first. I will never make that mistake again. I think I was low enough during my trip that my drinking the tea didn’t have a huge effect on my blood levels. The blood sample I took immediately after I came back from my trip was 4.0 mg/dl (240 micromol/L).
It has been just slightly more than one year since I totally went back on the diet, including drinking the formula. I still am, and always will be, very guarded and protective about whom I tell I have PKU. It is not in any way that I am ashamed to have PKU; it is just that there are only so many people I feel I want to invest my time in to tell them about what PKU is. I am most questioned by strangers about what I am drinking. I accurately and honestly answer their question--it is a nutritional supplement.
I still haven’t become accustomed to the practice of counting exchanges or weighing my food. I just know what I can and can’t eat. And, I know just how much I can have of the food that I can eat. I guess it is working because my last level was 2.8 mg/dl (168 micromol/L). My next challenge is to lose weight without catabolizing so much my blood levels are drastically effected. It will just have to be a very slow and steady process. I am happy with who I am and content with having PKU.
PKU is still a difficult issue for my family and me, however. A lifetime of conflict and old habits don’t get erased overnight; but, it is getting easier. As odd as it may sound, it is easier for me to talk about PKU with a stranger than it is with my family although they have always been extremely supportive of me. With strangers there is no history to deal with.
I am not perfect in my new (old) lifestyle; and I don’t claim to be. I am learning more everyday, and I learn from my mistakes. The genetic experiment I call my life continues, and I remain alive and well.