by Virginia Schuett, Director and Editor, National PKU News
The Winter issue of National PKU News touched you are no other issue has. The theme, PKU: Lives in Danger," prompted an avalanche of responses from subscribers. And by spreading the message, you have created a genuine "ripple effect": parents have told other parents, parents have told their children, siblings have told their siblings, grandparents have told their grandchildren, clinics have told their young adults, and on. A number of people also have contacted me after finding the newsletterís main content on the National PKU News Web site. Can the diet help me (a young adult) or my brother-in-law or my grandson? I heard over and over that the off-diet descriptions fit many young adults "to a T." Some families requested copies of the issue to give out to other families and to their own doctors. Clinics ordered over 1000 extra copies to send out to young adults and othersóclinics from Tennessee, Arizona, Utah, Illinois, California, Washington, Wisconsin, Montana, New Hampshire, North Dakota, Ohio, Kentucky, Pennsylvania, Arkansas, Texas, New York, South Dakota, Iowa, Nebraska, Maine, Alabama, Canada, New Zealand . . . and hopefully more to come.
I think it is important for us to maintain such excitement and motivation; we need to confront the dangers of high phenylalanine levels with an earnestness that we have not had before. To that end, I have decided to devote the entire front section of this issue to print a sampling of your responses to the Winter 1997 issue:
Responses from Young Adults
I remember when my sisterís behavior changed in high school and I thought it just had to be her diet. She would go to barbecues and eat hamburgers and brag about it. She was tired and had crying spells. I remember lying in bed listening to my parents fight about my sisterís changed personality and behavior. She has gone through phases since then, that canít be understood based on her early years. She became inconsiderate, sleepy and depressed, lies a lot and has started and stopped so many things. She was so bright . . . once.
Over the years she also gained weight, lived with an abusive boyfriend, became withdrawn, hardly called, and caused family upset at holidays. She also lost her personality, became frightened to go out, neglected her looks, had aggressive behavior problems at work, and got straight Dís when she finally graduated from college. My mom has admitted a few times throughout the years that my sister is not mentally right and asked that I talk to her about taking better care of herself. She has had physical problem as well, including headaches, and "arthritis" in her legs. Lately, she has been a little better, but "hyper." She lost a lot of weight and exercises constantly since giving up meat and milk. My mom says she is more pleasant (she is living at home now). But she is losing her friends, some of whom have been around for years. They just donít call her anymore. It is very sad. I worry about her, especially about her future years. She thinks she is fine. Maybe the Winter issue will hit home." Name withheld
"I always look forward to National PKU News. The heading on the front page of the Winter issue really caught my eye. It is my wish also to get all PKU adults back on the diet again because for people with PKU "diet is life." I am an adult with PKU in my 30ís (featured in the Winter 1996 issue). I have been back on the diet for four years now. I am doing really well, but I work in a supermarket and I find some foods tempting to eat. After reading the newsletter, I am going to be even tougher on myself. I know I can get my levels a lot lower ! The lead article in the Winter issue really opened my eyes to what can go wrong if you are not on the diet. I found the article so helpful, and Jackieís story of change, really good. I am trying to become a more confident person by sticking with the diet. I am in the PKU Support Group here and I am helping some adults with PKU in Australia to go back on the diet. Thank-you for all of the inspiration!" Debbie Colyer, Sydney, Australia
"I am now 30 years old and have been totally removed from the whole PKU scene for 12 years. I was started on the diet at the age of six weeks. When I turned 18, I was off to college. I was very worried about dealing with PKU on my own and having to explain it to so many new people. So I begged my doctors to let me go off the diet. They said it was a bad idea (so did my mom) but they also knew they couldnít stop me. When I got to college, I went crazy trying every food I had watched my friends and family eating for so long.
I noticed the first side-effects of my new lifestyle after I got
my first job as an oil broker in New York City. My co-workers and managers
noticed that I had a serious lack of short-term memory. Some-times I would
become silly and "punchy" for no reason. I had no idea why.
My mother suggested these things may be related to PKU. So we tested it out. I modified my diet back to the low protein style and sure enough . . . my head seemed clearer and my memory was better. But after 6-7 years of eating freely, I just couldnít bring myself to go back on the formal, strict low protein diet. So I modified my diet just to reduce protein intake. I went back to school to get my computer programming degree two years ago. I graduated from school very successfully and am currently employed as a computer programmer working as a contracted employee for IBM in New York.
After reading the Winter issue, and getting inspiration from another young adult with PKU in New York, I finally took the bull by the horns. I made an appointment and went to the PKU clinic in New York City and got myself back on the diet! I have only been back on the diet a short time, but already I have a better feeling about myself. The diet is going extremely well. I was skiing in Vermont this weekend, as I do often, and I had a noticeable amount of extra energy. I drank my formula at breakfast and lunch, perfect with a nice tossed salad. It was a great energy provider for a strenuous day of skiing. Thanks again for all of the inspiration from the Winter newsletter." Craig B, New York City, NY
"I just read the Winter issue. There was some very scary stuff about the long term effects of being off the diet, some of which I did not know. I went off the diet at age 10. Some of the symptoms described I had experienced before returning to the diet. I am 32 years old now and have now been back on the diet for five years. I can remember the tremors in my hands (starting when I had been off the diet for 10 or 12 years) and feeling very nervous and out of control. There was a time while I was off the diet that I remember my legs feeling extremely shaky; I could not walk down a flight of stairs without hanging onto the railing. I did not really connect this with the diet. But after reading the Winter issue it made me think a little deeper about what kind of symptoms I actually had while I was off the diet.
As I said in my story (Fall 1996 Hang in There section), I also had headaches that I no longer get. And now I feel much more relaxed and calm, donít have tremors anymore, and have better concentration and memory. Many symptoms that I experienced off the diet were very hard to recognize. It was really my mom who brought it to my attention and who suggested it may be due to being off the diet. Until that time, I didnít want to accept that I needed to go back on the dietóeven though I knew I needed to do it in the future in order to have normal children." Debbie Tobey, New Richmond, WI
"After reading the Winter newsletter, I just thought I would write and give you a brief history of my ups and downs with PKU. I was diagnosed with PKU in 1969, at five days of age. As with many other PKU patients of this era, I was taken off the diet at age seven. Then, when I was 13 years old, I was selected to do an on-diet study for the local PKU clinic in my area. Out of the ten participants I was the only one who completed the study; everyone else went off the diet. There seemed to be a lack of will power to stay on such a rigid diet when doctors were telling everyone that they didnít need the diet after age seven. While I was on this study, my grades improved.
After I finished the study, I went off my diet completely. My concentration from grades 8 to 12 was very low and my grades faltered. I left high school with a high school "leaving" certificate. I started having panic attacks, convulsions and blackouts. I decided that I needed to go back on my diet. My pre-diet blood test came back at 43 mg/dl, extremely high even for someone with PKU. I then cut my blood level down to 23 mg/dl and struggled for years to get it where it is today.
I am now 27 years old, and happily married. I keep my blood levels at 2-6 mg/dl. I feel better than ever. I not only have better concentration but I also have a sense of well being and greater self- esteem than ever. I realize that this is the way I want to feel all the time. There is no food in the world that is worth losing that feeling over." Lisa A. Whyte, Saskatoon, Saskatchewan, Canada
"In response to the Winter 1997 issue and "Lives in Danger," I agree with you . . . itís very scary! My daughter, Kelly, age 10, has been very well controlled since birth, generally in the 2-3 mg/dl range. As she gets older and gains more independence, I hope I have established a good foundation for heróone that will help her through the peer pressure, the curiosity of food and the desire to "cheat." Reading the articles only broadens my fears because I want Kelly to have as normal a life as possible. The thought of some of these tragic things possibly happening to her in life is devastating. Please, in the next issue, once again address the status of research for a cure for PKU! Let parents know what we can do to keep it moving in the right direction. Thank-you for all your time and effort for PKU." Eileen M. Rickard, Hamburg, NY
"Our daughter, Tammy, is 37 and has had a very hard time with the diet. She was born before newborn screening and was not diagnosed until she was eight years old. With great difficulty, we were able to get her on the diet for a number of years and she has done remarkably well while living at home. But she has not been fully on the diet for quite a long time. We were so happy to receive the Winter issue from our clinic. Tammy is now trying to get back on the diet. Keeping her informed and reading of othersí experiences has really helped. She says she it was like a "pep talk" to get back on the diet fully again. Thank-you!" Patricia M. Allen, Everett, WA
"Iím writing to see if you still have copies of the latest newsletter. I have two young, early-treated children with PKU of my own, and I also work in an institution for the retarded (for the past 10 years). I have been trying for several years to get two never-treated PKU brothers on the diet. We finally have a doctor here who will listen to me, so I am going to bring in the Winter newsletter for him and others to read. Maybe someone will see the light. One brother has horrible eczema, to the point where he looks scalded and seems that he would like to crawl out of his skin. They have tried countless creams; none work. The other brother has many bad behavior problems. Thanks so much for this issue. It has rejuvenated me and I donít feel quite so alone." Cheryl Evans, Boyd, WI
"I wanted to write to you to thank you for the last newsletter. Thanks for stepping into the light with your opinions. Many people are with you. Stories in the newsletter of success, and also of personal difficulties and how young people and families deal with them, are great. They are the best way for kids like our daughter, Beth, to know they are not alone. Thanks for bringing us these important stories, and for making a strong statement about the need for "diet for life." Sean & Dorothy Corry, Mill Creek, WA
"I just wanted to let you know that there is further remarkable improvement in my son, Kevin, since I shared his story for the Winter 1997 newsletter ("Kevinís Story"). The staff at the group home where he is now living says that he has become "too high functioning" for this home! They feel he needs to be moved to a home with a higher functioning population. Kevin is interacting with the staff more than the other residents. He has become more verbal, makes better eye contact and is more independent in all personal care skills. He is the only one in his group home to be working (in a sheltered workshop assembling small parts for General Motors). The supervisor tells me he is one of their most productive workers and they are going to move him on to more complicated assembly. A year or two ago, I would have said this was impossible for him. The group home manager has subscribed to the newsletter. He has also obtained the food list and cookbook and one of the staff is baking bread in her machine for Kevin. The staff is really enjoying meeting the challenge of Kevinís diet. Still, we are fighting to get treatment for Kevin at Michigan's only PKU clinic. They seem to feel no responsibility for serving the needs of PKU patients like my son who are returning to the diet." "Kevinís mother"
"We just received your Winter issue and it is one of the best ones ever. Thank-you so much for addressing the concerns of older PKU teens and adults. My daughter, Erika, is 16. She is really struggling with her diet. She wants to do well, but finds staying on diet very frustrating (and has for years). The theme of this issueóthe importance of staying on dietówas presented in a realistic way as teens and young adults honestly expressed how hard it has been for them. I hope and pray that Erika will take the message to heart. Thank-you so much!" Jeanne Clark, Richmond, TX
"You did a tremendous job on the Winter issue. It is the talk throughout the North Dakota PKU group. Doctors also say the information is "eye opening." I must have cried a million tears as I read the stories. I recently did a small newsletter for our stateís families and talked about your article and if they needed a copy to contact me. I explained how vital this information is. Also, for a "strategy meeting" of some of the parents involved in our stateís legislative efforts, I brought several copies of the Winter issue. As they read your article, they were hit pretty hard. I sincerely think that your article made us more "mother tigers defending our young" (as Bev Benda-Moe, our nutritionist, called us). I think thatís why we "rocked the Human Services Committee" the next dayóthe newsletter gave us more factual and emotional fuel to give to the committee. Thank-you for your "mission." Brenda Rakoczy, Reynolds, ND
"As the mother of a young daughter with PKU, I found the recent issue very informative. I had no idea so many young adults were off the diet. Also, these stories should serve to keep clinics aware of the need for counseling for both young adults with PKU and their parents. Much of this diet is a partnership between parents and their children. Our daughter has been doing excellently on the diet, but she is only four years old. I hope our clinic will provide advice and counseling to our family when Brittany begins the tougher stages of her life. Again, all PKU clinics should read this issue and learn from it. Thanks so much for all the information you provide to affected families." Rita Mercante, Garden City, NY
"Please send five copies of the Winter newsletter. My niece paid for a yearís subscription for me last year and I canít tell you how very much I have appreciated the News. I read every word when I received my copy last week. My 51 year-old daughter has PKU and is profoundly retarded. She has been a resident at a state facility since 1961. Her PKU was not diagnosed until she was 11 years old. We tried Lofenalac formula with diet for over a year, until the pediatrician advised us to discontinue it. Again, in the late 1960ís, she tried a trial of diet and formula. It was an impossible task under the circumstances of her environment. Currently, she has episodes of agitated, erratic, almost violent behavior. She has poor mobility and is given a medication daily for arthritis. After reading the PKU News, especially the personal stories, I believe much of her behavior can be attributed to a high phe level. For the past four years she has lived in a more homelike apartment with caring caretakers. I am hoping that possibly some effort can be made to control her phe level. I long to see her a calmer, less agitated person so she can be happier and easier to care for. Thank-you and God bless you for the work you are doing with PKU persons." Frances Polansky, Irving, TX
"Thank-you for this issue of the newsletter. My granddaughter is 21 years old and is very much in denial concerning her PKU. I pray she will read it from cover to cover, and it will help her to understand her condition and that she needs to go back on her diet. This issue really "tells it like it is" and I wish we had known all of this many years ago. Thank-you for your hard work, in trying to help each of us." A concerned grandparent
"You printed my letter in the Letters to the Editor section of the Winter issue ("name withheld," mother of the 19 year-old daughter). I have to tell you how much that newsletter has helped us. My daughter did read all of it, including my letter to you, and was at least touched and frightened by what she read. Recently our local health department agreed to change her medical food to Periflex. She is doing much, much better, drinking almost all of what she is supposed to. As for eating habits, though, I am not seeing much change yet. Still, she has moved at least one step forward by drinking her milk (from "precontemplation" to "contemplation") and I think the newsletter is to thank for that. She has kept a job for several months now, so on some fronts there are successes. I hope the newsletter will have even more of an impact on her as she really thinks about the stories of young people like herself. Thank-you again for all of your hard work." Name withheld
"Thank-you for the excellent service you provide all who are interested in PKU. This latest issue about "off-diet young adults" is superlative. As the mother of a 14 year-old son with PKU I worry about his choices. Heís doing pretty well with good blood levels, but flirts with denial especially in social situations. I read Chris Piersonís story to him and we discussed it a bit. I plan to read the other articles together and discuss the whole issue more. National PKU News consistently invites people to move out of denial by supplying solid information and encourages parents to trust their instincts regarding their childrenís welfare. Thank-you!" Mary DeLateur, Spokane, WA
From the Editor
Thank-you, everyone, for your responses to the Winter 1997 issue. My intention was to provoke serious thinking about the risks of high phenylalanine levels. A special goal was to reach young people who have been out of touch with a PKU clinic and are not aware of the potential off-diet problems. From the responses I have received, and others I have heard about, I think we accomplished a lot toward those ends in a very short time. As I said in the Winter issue, I strongly believe that everyone has a right to know the facts. Everyone needs to make up their own mind about the benefits of returning to diet or improving blood phenylalanine control.
From your responses, I think most of you agree with that philosophy. You felt the reports and stories in the Winter issue were both frightening and enlightening. I believe most of you were grateful to have the facts, as troubling as they might be.
I did receive several letters after all but my comments in this section were finalized. They bring up important issues. While I donít have space to reprint them, let me summarize the basic thoughts of the letters and my reaction.
Two siblings with PKU, ages 26 and 31, wrote to tell me they are both doing "just fine" after being off the diet for 20 and 25 years, respectively. Both have college educations, are happily married and have good jobs. They wanted to emphasize that some adults do perfectly O.K. after stopping the diet and that I should have pointed this out in the Winter issue.
My response: I did not intend to imply that everyone with PKU will have the problems described in the Winter issue. My point was that no one can predict who will have problems due to high phe levels, what the severity of the problems will be, and when in life they will occur. At ages 26 and 31, this is still young adult life. What about mid-life and older? And even now, there is no guarantee that brain and central nervous system biochemistry are completely normal for these young adults. I myself would want to see brain MRI results before declaring myself neurologically unaffected. Finally, I have heard from many young adults who thought they had no problemsóuntil they went back on the diet. Then they discovered they felt better or had better memory or better concentration, etc. with low phe levels. I know not everyone will have severe problems off the diet. But I believe it is a mistake for young adults to think because they do not notice any problems now, they are forever immune to the often subtle and insidious effects of high phe levels.
The third letter I received was from a PKU professional, a psychologist. It reminded me of the thinking of the 1970ís. The gist of the letter was that the Winter issue caused undo anxiety among young people with PKU and their families. It went on to say that professionals should not "overstate their case," lest they lose their credibility; later, it said that "we should not resort to testimonials that may be more frightening than helpful." The author wrote that she knew of many off-diet adults "functioning very normally" in the community. She chided me for causing in parents "undeserved guilt for failing to enforce dietary control" and for causing "frustration and anxiety for adolescents who are unable to maintain diet control." The letter went on to describe the many practical difficulties of returning to the diet (obtaining formula, preparing special foods, etc),"which your article made no mention of."
Is our goal as professionals really to "not produce guilt" for those with high phe levels? Just as "practical difficulties" and "guilt-avoidance" should not have been reasons to stop the diet in young children in the 1970ís and beyond, I believe they should never be reasons for withholding the truth about the risks of high phe levels from parents and young people. I fully appreciate the problems of returning to the diet, as I mentioned in my closing on p. 3 of the Winter issue. But I do not believe that is a reason for shielding families from reality. Also, I would add the same response as I had for the two young adults who wrote to me: Does functioning "normally" in society mean that high phe levels have been completely benign? Show me the evidence! Could these young people not be doing even better with low phe levels? Convince me with data! High phe levels simply are not normal for the human body. It is up to professionals and families to search for ways to help young people who want to return to the diet. If we believe in the need, we will find a way ó even if it takes time and is not easy.