Brandl Family Story

There are six members of our family—my husband and I, and four sons: Daniel (18), Donald (16), Douglas (12), and David (10). Daniel, who has PKU, is a freshman at the University of North Carolina at Chapel Hill where he is carrying a full load of classes. He is majoring in physics. He is a trumpet-playing member of the Tarheels Marching Band and Pep Band and takes karate three times a week.

All of our children play musical instruments and enjoy martial arts as well as a great variety of other activities. All of them have been (or are) in Academically Gifted classes and are all "A" students. We feel very fortunate. My husband, Dennis, has advanced degrees in physics and computer science, and is a software system engineer currently doing marketing for a high tech company, Sequentia Corp. I have a degree in economics, and currently work part-time at North Carolina State University. I worked for ten years as a volunteer in my children’s schools and for the last two years as a teacher assistant in second grade. Also, we are the parent representatives for the North Carolina Newborn Screening Advisory Committee, which advises on policies for newborn screening in the state of North Carolina.

I have been the parent who mainly controlled Daniel’s diet throughout the years, but we were both actively involved. Dennis was always diligent in being aware of the status of Daniel’s daily intake. Dennis also is the low protein pizza maker. Daniel never knows if he is going to get onions and garlic in his blue, green, or pink crust; or if it will have chives, parsley and some other surprise in it. We have tried to learn everything we can about PKU. We have subscribed to newsletters, purchased cookbooks, tried new recipes, made up our own recipes, tasted (and sometimes wasted) our concoctions.

We had very supportive people at the clinic in Houston, Texas from May, 1980 to May 1984 (especially Dr. Williams, whose calming ways helped us through the early years of PKU). When we moved to Johnson City, Tennessee in 1984 we were so fortunate to have the wonderful Dr. Kirkman and Dianne Frasier to care for Daniel. We could not have done it without them. Dr. Frasier has calmed me, informed me, and taught me all I need to know about Daniel’s diet and, most important, that each day is a new day. For the last three years, Dr. Meunzer at the clinic has helped Daniel and shown special interest in his independence.

Like for all families, our initial reaction to the PKU diagnosis was one of shock, fear, and doubt. We knew nothing about PKU, and the explanation that the pediatrician read to us from his medical reference book did not answer any of our questions. My main worry was "will his life expectancy be affected by this disease?" Tearfully, we called Dennis’ mother who was a nurse in public health in Pittsburgh, Pennsylvania. She knew what PKU was and calmed us with information she got from the PKU clinic there. Since we lived in Houston, Texas at the time, we were referred to the genetic specialists at Hermann Hospital in Houston. We spent a week in the hospital for lots of tests and training for feeding an infant with PKU. These wonderful specialists taught us enough about PKU and the special diet to realize that it was not the end of the world or the loss of our son. We discovered that Daniel could grow up to be a healthy and normal person; we just had to diligently manage his diet. It took us a couple of months to realize that Daniel’s diet was something we could deal with and that it would not become the main focus of his or our lives.

The turning point came when we realized that we had some control of Daniel’s future health. We had to manage his diet until he became old enough to manage it himself. Managing Daniel’s diet simply became a fact of life, something we had to do. It had the same importance as teaching him right from wrong, teaching him how to read, teaching him to respect others, and all of the other things that parents must do. Although management of the diet was difficult at times, we never considered Daniel’s PKU as a burden or problem, and we attempted to teach him that attitude.

During the early months, Dennis’ mother gave us constant support. Her positive attitude helped us to see that, in the whole scheme of things, this very manageable disease was a minor part of who our child was.

Realizing that strict diet management was the key to the success of our child, Dennis and I decided that we simply would not rely on others to manage Daniel’s diet. Instead of continuing my career in banking, my career would be that of an "at home" mom. It was quite an adjustment in our lifestyle, but it has been worth it.

We did not "buy into" the negative side effects we learned could come from having PKU. One particular time we were told that Daniel probably would not be good at math. Dennis and I looked at each other and smiled an "over my dead body" smile. Math is a great strength in our family and we knew that Daniel would have it, too.

When he was young, we thought that PKU must be the reason for some of Daniel’s idiosyncrasies, until we realized that we could see the same things in our other children and other relatives. Many of his "quirks" belong to other first born children everywhere. We now say, "that’s just his personality" and not "maybe this has something to do with PKU."

I began to explain "yes" and "no" foods when Daniel was three years old. When he asked me why he could not have a certain food, I told him that it was not good for his body. And "if it is not good for your body, why would you want to eat it?" I suppose this made sense to him and he accepted this explanation. I would read You and PKU to him, but once it sank in, he didn’t want to hear it anymore. We did not have difficulty with Daniel taking forbidden foods, though; he just wanted more of a certain food he could already have.

We had a problem weaning Daniel from a bottle. It had been the problem-free way he drank all of his Lofenalac. Getting him to drink the volume of formula each day was difficult in a cup and it was a battle every time formula time rolled around. We tried flavoring it with strawberry flavor Quik and even Tang. He would sometimes eat it in powdered form, but he liked to eat it as a paste the best. Finally, we decreased the amount of water and he had less to drink. Even now with Phenyl-Free, he makes it very thick and has only 14-16 ounces a day.

When Daniel was young, the standard treatment was to go off the diet at age 6 years. However, as Daniel got older, the cut-off age also raised to 12. By the time he was 10, the standard treatment was to stay on the diet for life. I believe that Daniel was disappointed, but there does not seem to be any long-term resentment of a "broken promise." He knew that it was best for him in order to reach his full potential.

I learned not to give the school too much information about PKU. We never discussed any of the known side effects of PKU with Daniel’s teachers. We did not want to influence them into seeing what they expected to see. We learned to write "Daniel is on a very restricted diet and can eat only food sent from home," or "on a very restricted diet: cannot eat protein." I believe his math and science teachers would be astonished to hear that PKU kids were "expected" to have problems in math and science since these are two of the areas where he excels.

I have always offered to donate food and time for class parties. Room parents were most understanding in helping Daniel to be included in class parties. For the surprise in-class food occasions, I always supplied the teacher with Daniel’s favorite hard or sugar candies (for the whole class) and he knew that he could go to this candy can. Many times the other children in the class would want what he had instead of what was offered.

Daniel has always taken his lunch to school. There are so many foods that a child with PKU can eat that the only difference in the lunches many times is that his appeared to be healthier, with more fruits or vegetables. In elementary and middle schools the majority of the cafeteria had bag lunches. In high school, a large number of students brought lunch to school because to stand in the lunch line would take up too much of the lunch period. He loved the lunch he packed.

Daniel wanted to take karate when he was 8 years old. We agreed because we felt it would help his self-confidence. We don’t know if Daniel’s knowledge of PKU led to a lack of self-confidence at this time in his life, or if that was his nature. We do know that, even before he got his black belt in seventh grade, he believed in himself again and had confidence in his abilities.

Daniel has had every kind of vegetable from asparagus (a favorite) to zucchini (not a favorite). He hates tomatoes but loves catsup so much that we recently purchased Heinz 57 stock! There are hundreds of foods that he can eat and we have always concentrated on trying those. We have not made any effort to offer him high phe foods.

As our family grew larger, meal planning had to get simpler. My goal has always been to have as many people eat each dish as possible. The whole family will eat the vegetables and fruit that are planned to complete Daniel’s daily requirement. Of course, low protein products are prepared for Daniel, but it usually takes no more than one pan to accommodate Daniel’s special food preparation. Fortunately, Daniel has always eaten his meals without a fuss, unlike his brothers who went through some major fussy-with-food years.

Daniel has three younger brothers who view him as The Boss and The Man. "PKU" is not a description they use when referring to him. Daniel and Donald have been close all their lives. The two younger boys look up to Daniel and are in awe that he is so smart, plays a mean trumpet, and always beats the video games.The "special need" that Daniel had was that of a strict diet. The only times this has really affected his siblings has been on the choice of restaurants where the whole family can eat.

The teen years were harder at school because there were more multi-day field trips and camps. For example, band camp was for one week each summer at a college 60 miles away. I called in advance to ask for the daily menus. I made an appointment with the people in charge and visited them with Daniel’s low protein pasta (with my cooking instructions, date and meal to be served taped to each box) —and a blender. I had learned years ago that to talk or meet with those feeding the students could guarantee a worry-free week of camp for me. Also, conscientious Daniel didn’t have to worry about his diet. They gave him a place to make and store his formula and cooked his low-protein pasta just the way he likes it.

There were a few "bumps in the road" during his teen years that made his mother grayer. He had some high phe levels in his mid teens. Thankfully, our clinic soon determined that his caloric intake was too low for all the physical exercise associated with the marching band.

Daniel had the normal teen rebellion over restrictions about driving, staying out late, and spending too much money on Magic Cards—but none over food. When we had asked him as a little boy why he would want to eat anything that was not good for his body, he decided then that he didn’t want to. We have talked "phes," "too many phes," and "free food" since he was a little boy. He learned what "line" on the bowl he could pour his cereal to without our help. He had "free" foods that he could eat without asking us. We started to make Daniel fully responsible for his own diet in his teen years (along with doing his own laundry). He resisted this and continually asked us to decide for him. We were there to guide him and had to be firm in getting him to make the choices for himself so he could take full responsibility in college and beyond.

We have been very lucky to have such a wonderful son as Daniel. He has grown to be a fine young man with a good sense of humor, a lot of perseverance, and a lot of intelligence. He is a responsible, mature, and well-rounded son who loves his family. Most of all, he’s lots of fun to be around. We believe that these qualities would belong to Daniel, with or without PKU.

Reflections of a Parent

Looking back, there are several things that I would have done differently. I would live fewer days fearing how a low or high phe day might be affecting Daniel negatively. I would be less stressed when he was sick, though his levels were probably higher. I would never again scoop up the spilled food and weigh it all and deduct it from his day’s totals (yes, I really did this quite often when Daniel was a toddler.) I would live every day as a brand new day with a fresh start and not try to make up for missed or too many phes from prior days. We have found that doing one’s best to handle the diet is an attitude and does not need to be an obsession.