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By Sally Milbury-Steen
Blythe is our only child. I am the Executive Director of Pacem in Terris, a small nonprofit "peace with justice" organization in Wilmington, Delaware. My husband, John, teaches English as a Second Language at Temple University in Philadelphia, Pennsylvania.
We were living in England when our daughter was born, and we joined the National PKU Society while we were there. We moved back to the United States when Blythe was almost three. Since then, Blythe has been followed by Dr. David Valle at the Pediatric Genetics Clinic at Johns Hopkins University Hospital in Baltimore, Maryland. He and Betsy Emerick, dietitian, have been most helpful and encouraging. Their pride in Blythe is very evident whenever they see her or call her with a blood test result or recommend a diet change.
Both John and I have been deeply involved in the management of Blythe’s diet. John mixed up her formula faithfully every day from the time she was an infant until she began doing it herself during the summer before she entered eighth grade. He also did her blood tests until Blythe started doing them herself during her senior year in high school. I kept her diet sheets and calculated her food intakes until she began her freshman year at Bryn Mawr College.
The Diagnosis and Beyond
Blythe was born in 1978 in Brighton, England and was given her first Guthrie Test when she was six days old. When the results came back from her first test, our general practitioner came rushing to our home and told us that we had to take her to the hospital right away. We knew very little about PKU, so we were very frightened and nervous when we took her to the Royal Alexandra Children’s Hospital for her first appointment. We both were weepy while we waited for the doctors, not knowing what to expect and anticipating the worst. A definitive diagnosis was made when she was 20 days old, after watching her levels for 2 weeks. When she was started on the diet. I had to stop breast-feeding her abruptly, which only added to our difficulty and distress at the time. The diet got off to a rocky start because of a mistake made by the consultant in charge of Blythe’s treatment. The dilution of her formula was too weak, so she cried from hunger during the first week on the diet, eventually becoming so weak that I had to feed her with an eyedropper. Finally, when the formula was made properly, Blythe turned into a happy, satisfied baby.
The "turning point" for us was when the physician in Brighton arranged for us to take Blythe for a consultation with Dr. Hugo Wolff and Dr. Isabel Smith at the Great Ormond Street Hospital for Sick Children in London. They ran a wonderful PKU clinic there and were most reassuring.
After that visit, we knew that we had found our PKU "home," so we transferred Blythe’s treatment there. Taking Blythe to Great Ormond Street was always a treat. The office was full of toys and both doctors were very supportive, patient, and knowledgeable. They listened to our questions carefully and talked us through all of our anxieties. They made arrangements for us to transfer Blythe’s treatment to Johns Hopkins Hospital in Baltimore when we came back to the States.
Raising a Successful Child
The strongest factor in Blythe’s success is Blythe herself. As parents, we tried to provide the nurturing she needed to follow her curiosity, develop her talents, and grow up with self-discipline and self-confidence. We always tried to give her the love she needed to serve as a buffer in tough times and a springboard in expansive times, helping her leap into new experiences with more excitement than trepidation. We recognized early on that words and music fascinated her, so we talked and read to her from infancy and began violin lessons at her insistence when she was only four years old. She later switched to the piano, which she still enjoys playing even though she is too busy now to continue taking lessons.
When Blythe was little, I would sometimes tell her, "Papa wears glasses, I wear glasses, and you have PKU." PKU was a fact of her life, not a disability or a problem, so there was no need to fixate on it as the measure of who she was. She was always very cooperative and compliant about the diet, which made its management quite easy. John and I tried, with little success, to get her to eat some of the things we made from recipes in the PKU cookbook. We never took her rejection of these offerings personally, and we realized that although she had some favorites, her life did not center on food. By the time she went to pre-school, she knew pretty well what she could eat. The way she tended to exercise choice over her diet was to make it more restrictive than it needed to be. She took packed lunches and snacks with her. I can never forget our English dietitian, Dorothy Francis, who warned us once, "When PKU kids get to be teenagers, they may rebel by sneaking out and eating a hamburger." Considering what other parents have to worry about —sex, drugs, and alcohol—John and I never saw this as such a bad option! Fortunately, Blythe seemed to have little desire to experiment with high protein foods.
In talking to Blythe about PKU, we tried to give her age-appropriate information. Explaining an enzyme to a five year-old was a bit tricky, but I told her it was like the juices in her mouth that helped make crackers soft and dissolve. A couple of months later, I overheard her telling her violin teacher, "I have PKU, which means that I have a juice missing in my liver."
Once when she asked why she had to be on a special diet, I told her that if she weren’t on the diet, she would not be able to ride a bike. I did not mention mental retardation, since I did not want her to become worried, fearful or anxious about losing intelligence if we had a dietary indiscretion. When Blythe went to school, she always took her lunch and a thermos of her formula with her. She preferred it to be cold, so I would keep her plastic thermos bottle in the freezer overnight, pour her lunchtime formula into it in the morning and put it into her lunch box. Sometimes, she would tell me that other kids would ask her about her "special milk" and ask her how she could drink such "smelly stuff." She usually told them that she liked it and was used to it; she had been drinking it since infancy.
I never had difficult explaining PKU to teachers and often gave them a publication with a simple explanation of PKU prepared by the British PKU society. I always stressed that Blythe knew what she could and couldn’t eat, making it clear that the responsibility for the diet rested on her and me—not the teacher.
The elementary school that Blythe attended always took the children on at least one camping trip a year. Blythe would take a cooler with ice packs and little containers of her formula already mixed up. When she had to camp for two nights, I would freeze some of these containers, and put the ones for the second day in a different cooler labeled "Day 2." If that cooler stayed closed with its ice packs until the second day, the formula was thawed out but still cold by the time she had to drink it.
PKU may have complicated logistics at times, but we never saw it as an excuse to deny Blythe experience or travel. During the times when Blythe would rail against PKU and feel sorry for herself, I was always honest in telling her that I wished the genetic dice had shaken out differently, but they hadn’t. We have some good friends who are blind, and after a little while, Blythe would often remark, "I think that being blind is worse than having PKU." Fortunately, she had the resilience to embrace PKU as an unalterable part of her life rather than an impediment or handicap.
At the end of seventh grade, Blythe went to Japan with my sister and her family for two weeks. She took complete charge of her diet and mixed up her formula herself. When she got back home, she was very proud of the fact that once she had mixed it up in the ladies’ rest room in a train station!
When Blythe was very small, John and I decided that we would not change our own eating habits to conform to hers. We felt that it was important for her to learn at home, especially from the people she loved and trusted, that not everyone had to follow a diet like hers. Once when she was six and John and I were having ham, she asked us, "Are you eating dead pig?"
During her teenage years, Blythe seemed to have no problems adhering to the diet, but she did not take much interest in learning more about PKU. Once in her biology class in tenth grade, they had a lesson on PKU, which quite a bit later she told me was full of inaccuracies. However, at the time, she said that she preferred to let them go uncorrected rather than explain why she knew so much about PKU. When she was applying to colleges, a friend of ours who had done alumni interviews for her alma mater, advised Blythe to write about her PKU on her application because it might help her stand out from some of the other applicants. To my pleasant surprise, Blythe took this advice to heart and wrote a very touching essay about her experiences with PKU.
A Chance to Brag
From my subjective perspective as Blythe’s mom, I feel that being on the PKU diet all of these years, has given her self-discipline, conscientiousness, commitment, and maturity beyond that of many young persons her age. She has a strong sense of justice as well as deep empathy and understanding for others. She has the ability to set clear goals for herself and to do the work necessary to achieve them. For example, when she began as a freshman at Bryn Mawr College, she decided that she wanted to spend her junior year abroad at Cambridge University in England. Sure enough, that’s what she is doing this year. Love of words has turned her into an English major with a lively wit and sense of humor. At Cambridge, where she started in October, she even has her own Sunday evening radio show on which she reads American short stories to her English audience!
Blythe is easy to get to know and has made a circle of wonderful friends at school. She has earned the respect of her professors and thrives under the rigors of scholarship. I tease her that she was the only incoming freshman in her class who received a brand new whisk as a going-to-college gift. She uses it to mix her formula, and it traveled with her to the Institute of French Studies in Avignon, France this summer and now has made the trip to Cambridge. After that, I know that many more adventures lie in store for Blythe and her whisk.
Reflections of a Parent
We have lived with PKU for so long as a family that it has just become a part of who we are—not a primary or all-consuming part —but just another facet of our lives. The cost of the formula, since it is not covered by our health insurance, has been considerable at times. For the past ten years, a special fund created by the legislature in Delaware has provided us with much of the Phenyl-Free that Blythe requires, which is a great help.
In retrospect, I do not think that there is really anything that John and I would have done differently. I believe as a parent that raising a child is a matter of love, trust, and luck. My job was to get to know Blythe as well as I could by caring for her, listening to her, and communicating with her as openly and as patiently as I could. The strong bonds that we have developed have given her responsibility and have given me the strength to know when to hold on, when to let go, and how to help her move into ever wider worlds. I have to admit that over the years I made plenty of mistakes, but the bonds of love are sufficiently elastic to bounce back each time.
My primary advice to other PKU parents is to accept PKU for what it is—a fact of life—not the full measure of your child or the focal point of your lives. Try to do your very best with the diet, without becoming compulsive or obsessive or making formula and food intakes a tug of war. Above all, trust in your child, and allow your love and common sense to prevail.