|
The Big DanceThe Road to Self-Management of the PKU DietReview of Annual MeetingHeld November 7, 1998 - Chicago, Illinois |
This page is rather long You may read it either by scrolling down to the various sections or by using the bookmarks below to skip directly to a section.
Link back to PKU Press
Winter 1999
Link back to Index of
PKU Press Articles Available on the Internet
Welcome and Keynote Speaker Introduction. The 1998 Annual meeting kicked off with a Jammin' video that highlighted the social events of the year including the cookie exchange, Picnics, Halloween party, discovery day and PKU camp. The film director worked very hard all year to record all those special moments, and to put together the video.
A dietitian for the UIC clinic followed with a "History of PKU for Dummies", from 1934 to the present. The first diet to show positive results was instituted in 1953, and as early as 1963, doctors suspected that discontinuation of the diet could be linked to a loss in IQ points in their patients. The National PKU Collaborative Study showed definitive evidence in the 1980s that going off diet can be detrimental to patients and their offspring, thus, the "Diet for Life" strategy was born.
Keynote Speaker Cris Trahms, Dietitian at the University of Washington PKU Clinic, spoke in everyday terms about the changing role of both parents and PKU clients in managing the diet. She and her staff have developed a specific curriculum for working with families to help them learn to meet the challenges of diet management by integrating knowledge of child development, psychology, nutrition and a healthy dose of common sense. The actual program encompasses stages from infancy through transition into the adult clinic. It all sounds pretty daunting, doesn't it? Well, here is the good news. If you follow your child's readiness cues, it's not hard at all!!! The basics of the program are that parents have 2 basic tasks that they are responsible for with any child:
Now, HOW do you do THAT?
Easy. Do whatever works at the moment. If your child wants to eat the same food for a month straight, don't let it make you crazy. If she will only eat cold or crunchy foods, that's OK too. In the long run, if he is growing and developing normally, it all evens out. With PKU in particular, don't forget that much of their nutritional needs are met through the medical foods anyway. However, do promote health and fitness. Therefore, it is not all right for your child to live on skittles, even if they try to convince you the different colors are different food groups. Cris Trahms did have some other valuable pointers as well.
Cris covered developmental stages in children as a way of reminding us how we help shape our children's attitudes toward food, and also help them move toward self management. For instance, feeding a hungry baby seems like a no brainer. However, overfeeding a baby because we want her to finish the bottle or jar teaches her that she does not have much control, and cannot trust her own body to tell her when to stop eating. As children grow, Cris reminded us, it is important to let them be involved with food however they can for their age. Very small children learn through playing, so try to let them play with food a little bit, even if it gets messy. Slightly older kids like to pour and measure. As a bonus, kids who are involved in managing their diets, through measuring, cooking, and counting phe's are exposed to math, English, science and social skills very early. Hey, maybe this diet really can make our kids smarter! Cooking helps teach sequencing ( what order, prediction) when kids add ingredients, read a recipe, or make the pizza dough before they chop the mushrooms. They learn to plan their diets to balance how hungry they might be later against how badly they want that bag of french fries, and they learn to share and be gracious, as they may have to decline well-meant offers of food often. They also learn the basic properties of foods, like that they change texture and flavor when they are cooked. Wow! That is a lot to get out of a little time in the kitchen.
Older children move toward independence at sometimes lightening speed. That includes diet management. Think about it. At around age 6, you send your child out to all-day school, and they have to feed themselves without you there to run interference. Some kids are better at it than others, but developmentally, here are some things that may help, starting in the grade school years.
Adolescents and young adults should eventually transition into fully managing their diets. However, parents are often enlisted to help them carry out that plan, and to meet new challenges. Learning to go out, especially on a budget, is important to practicing self management. So is grocery shopping, but how many teens do that by themselves? Last, there may be unique situations that require a willing parent. What if your child wants to go to South America for a year in the foreign exchange program, or travel the U.S. by bicycle? One family agreed that the young person would keep a rough itinerary, and the parents would ship the products ahead to meet her. Other teens often spend at least as much time at a friend's house as at home. How about stashing product at those homes as well?
Cris was informative and down to earth. She has helped develop a curriculum that takes the families in her clinic from infancy to adulthood and beyond in managing PKU. The focus is to empower the parents first, and then the child, to see diet management as just one tiny piece in a life full of experiences and opportunities, and to treat it as such. Whenever possible, the child is handed, first, involvement in, and then, responsibility for their relationship with food. And that is as it should be.
Our Own Curriculum in Illinois. Linda, known to many as the social worker from Children's Memorial, and the co-creator of Discovery Day announced that she has been drafted to develop a developmentally based curriculum for the PKU clinic at the University of Wisconsin. Eventually, that should translate to a more standardized approach to teaching our kids about PKU and managing the diet, with age appropriate activities. Due to Linda's ties to the clinics here in Illinois and the large PKU population of those clinics, Children's Memorial and UIC will be the field test sites for the components as they are developed. Specifically, you may be asked to try out a game or activity designed to teach about PKU and the diet, and evaluate it. An educational game is only good if it is fun and the student learns the desired lesson, so she will be asking for feedback over the next year. The program is expected to reach teens and adults as well, possibly resulting in the much-requested adult clinics.
PKU Panel - STUDENT BECOMES THE TEACHER/WHO IS THE EXPERT NOW?
This year, the attendees were treated to something we haven't seen in a while. A number of PKU "kids" assembled to let us see the world through their eyes. They ranged in age from 11 to 28 and included several adults and a PKU mom, her 2 beautiful kids and her husband, and a PKU dad. Members and parents posed these questions from the floor.
Q.How do you deal with social situations such as going out to eat with friends or parties?
A .Answers varied, but many said their friends were very helpful, and worried about getting them something to eat more than they themselves did. Some panel members said they just don't eat at such times, because it is easier not to explain, especially if the friends are not close.
Q.Is variety in your diet important?
A.The overwhelming response was NO. One young lady admitted to having eaten French fries for just about every lunch for the last 10 years, and others agreed. It is easier to stick with a few foods they know they like than to worry about counting phe's.
Q.How do you handle eating out? Do you measure portions precisely?
A.Most panel members just "eyeball" the portions. Some people bring along Low phe foods to the restaurant, and some "save" a large percentage of their daily phe allowance for going out so they can order from the menu. Vegetarian dishes are fairly common now, and appetizers and side dishes are good choices.
Q.What do you tell restaurant staff?
A.Nothing, especially the older panel members. If asked, some will mention dietary restrictions in a vague way.(This is effective when you bring your own food and want them to heat it.)
Q.Do you have to be really organized to keep track of what you eat?
A.No. For most, they do not monitor the diet so closely that they count every gram of Phe. One young man did admit that sometimes he will choose not to eat because he "just doesn't want to do math".
Q.Tell us about dating and marriage.
A. PKU was not really an issue in dating. After getting to know someone, they will tell them a little about the diet. When it becomes "serious", a longer discussion usually takes place, especially considering the possibility of marriage and children. The husband of the PKU mom, joked that he partly married her because she was a "cheap date." Another indicated that, even though PKU has not really limited him, he was worried that his little girl would have PKU.
Q.What do/did your parents do that makes you crazy?
A. Amid laughter, the panel listed their pet peeves. Nagging, making a big deal of the diet, and threatening (you can't go out if you don't finish...) topped the list. Ordering for them or giving long explanations to people who don't need to know were next. Surprisingly, the older panel members didn't have too many complaints.
Q.Did you go through a rebellious stage?
A. Some did, some did not. In general, one summed it up by saying that she got suspicious of her parents when they tried too hard to get her to try something, and others said they got fed up with being Different.
Q.What brought you back to the diet?
A.Growing up, both in responsibility and attitude helped. Wanting to feel their best for the sake of self or sports contributed. The best answer? "I started to do it when my parents gave up."
Dept. of Public Health. We hear the name "Sydney", and once in a while get a letter from her, but she really is a phantom for many of us, or a guardian angel. She Is in charge of the program at the Department of Public Health that gets formula to your doorstep. Beyond that, what do any of us know?
Sydney has been with the Department of Public Health pretty much since the beginning of PKU time. 35 years ago, there were about 5000 people with disabilities in state institutions, and 89 of them had PKU. Her department persuaded the state to make PKU testing mandatory, and when the private, out of state, labs proved to be slow and unreliable, they protested because it caused delays in treating our kids, and eventually took on the mammoth task of centralizing and tracking newborn screening. They developed a PKU registry, and even today continue to improve the program with more sensitive tests, and better tracking and retesting of borderline patients. In 1997, The State tested over 185,000 newborns for a variety of inborn errors of metabolism. Remember that when your Maxamaid order takes an extra few days!
Due to the success of screening and treating PKU, the state now tests for 9 other diseases, and infant auditory screening will soon be implemented. Those children are just as precious as ours. The programs have been kept alive and even expanded in the face of having funding cut twice, through the tenacity of a small core of people, including Sydney, who have shown the legislature time and again the benefits of addressing and treating PKU and other debilitating conditions. However, one caveat: with the advent of Diet for Life, there will be a gradually increasing strain on the resources allotted to the Department of Public Health, as people will no longer go off diet, and the numbers treated will only grow. Let's hope Sydney is there to lend her expertise and wisdom, as well as considerable political and medical knowledge to our cause.
Clinic Update - DOCTOR, DOCTOR, GIVE ME THE NEWS. We were once again pleased to have 2 of our own clinic doctors present updates about what's new in PKU.
Dr. Margaret O'Flynn has recently announced that she will be retiring at the end of 1998. She arrived in 1956 and was told "There is no PKU in Illinois," and has continued on here to befriend and treat countless families over the years. She spoke at the annual meeting of developments in Maternal PKU. In the 1960s, when all mothers with PKU were untreated because of the practice of taking grade-schoolers off diet, colleague DR..Levy's research showed that "Phenylalanine is the strongest poison known to man in damaging the fetus." From there, Dr. O'Flynn and others had to decide what to do about it. While she used to advise women with PKU to adopt because of the grave and certain consequences, research has shown that a very strict PKU diet can result in a healthy baby. Most important factors are that the diet be started before pregnancy, because so much damage can be done even before a woman knows she is pregnant. Second is that blood levels below 6mg/dl are much better than levels elevated only as high as 10mg/dl in assuring a healthy child. These findings are important for parents to note.
Dr. George Hoganson spoke about developments in research. Highlights included the news that there has not been much direct progress in gene therapy. However, as always, most of the research is conducted for other diseases, but may have more widespread applications..... Modified vectors (delivery systems) have resulted in slightly longer expressions of the desired gene. It still has a long way to go.....The newest thinking came from chance...a liver organ transplant into a PKU patient results in PKU being eliminated. While organ transplants are not a viable option, redesigning some liver cells genetically and replacing them into the liver may be able to get the liver to produce enough of the enzyme to do the job. This is still in the theoretical stages. .........Theoretically, synthetic enzymes are possible, but are being investigated for cost-effectiveness.....Putting the right gene into a lactating mammal, such as a rabbit may yield some surprisingly logical results. The enzyme needed to digest the food (in our case, Phe) is excreted in the milk, and the patient drinks the milk. This would essentially result in phe-free milk, with all the other proteins intact, and someday (in a galaxy far, far away) might replace the engineered "milk." Also, depending on the chemistry that takes place in the body, if enough enzyme is in the milk, it might aid digestion of other high phe foods, when eaten in conjunction with them.
Closing. Here's to another year of hoping for the future to hurry up and get here. In the mean time, enjoy the organization and activities you have now, and see you at next year's meeting!
Link back to PKU Press Winter 1999
Link back to Index of PKU Press Articles
Available on the Internet
