GENETIC ALLIANCE BY ADRIANNA EVANS
The arrival of the New Year brings with it resolutions of all kinds; people everywhere promise to make the coming year better than the last. Among these resolutions are often promises to be kind, to spend more time with family, to go to the gym – and of course, to stick to a healthy diet. These days, the promise to stick to a diet often involves using websites, social media, apps, and the myriad food blogs of the Internet. The online dieting community is accessible by phone, computer, and tablet, helping dieters to stay on track every moment of the day. Even so, some dieters will lose their way and return to their old habits even before it becomes time to make the next year’s resolutions.
For some dieters, this isn’t an option. For people with certain types of genetic conditions, sticking to a specific diet is crucial, as even the slightest cheat has consequences much greater than simply working off the extra calories at the gym. Such conditions are caused by an inborn error in metabolism, or the body’s ability to perform the chemical reactions necessary to sustain life. Typically, with these conditions, a person will inherit one or two faulty copies of a gene, which causes one of the enzymes that carries out metabolic reactions to malfunction, or even to be completely absent. Without a functional enzyme, the initial molecular components of the reaction go unused and subsequently build up and wreak havoc in the body.
Phenylketonuria (PKU) is a genetic condition that renders a person unable to break down the amino acid phenylalanine (PHE). Amino acids are a component of protein, so people with PKU or similar amino acid disorders can’t digest protein. They must drink a special formula, carefully weigh out every bit of food they eat, and calculate how much protein and PHE they take in every day. Without the diet, PKU and amino acid disorders will cause protein to build up in a person’s body, often causing problems with eating or sleeping, which can escalate into severe symptoms such as seizures, coma, or even death. Even small deviations or cheats can have neurological consequences. Galactosemia is a similar genetic condition. People with galactosemia can’t breakdown galactose, a sugar found in all milk containing products. Consequently, these people must be on a diet free of milk and all galactose-containing foods. Untreated, galactose sugars build up in the body, causing potentially fatal symptoms such as seizures and liver damage.
Fortunately, online dieting has not neglected the community of dieters with genetic conditions. The rising popularity of food blogs and new diet plans has increased the availability of resources for these very specific genetic diets. The following list of resources can be used as a guide to helpful tools online.
Cook for Love is a non-profit serving the PKU community. It was founded by mothers of children with PKU who went through the intense struggle of creating palatable food that wouldn’t skyrocket their children’s phenylalanine to perilous levels. Most of their recipes were created by trained chef and founder, Brenda Winiarski. Like any recipe, Cook for Love recipes provide an ingredient list, instructions for preparation, and cook time. But unlike most food websites, they also provide exact measurements and nutrition information, including calories and PHE content, which is absolutely critical for the PKU chef. There are recipes for breads, desserts, salads, soups, entrees, even resources for planning a low protein holiday feast. In addition to their creative and flavorful recipes, Cook for Love also has some informational content about PKU for families just starting out on their PKU diet journey as well as listing many other useful informational website, cookbooks, and tools specifically designed to serve the PKU community. Cook for Love requires a login to access most of the recipes, but it’s free and simple to sign up.CookforLove.org
How Much Phe is a tool provided by National PKU News designed to make managing the low protein diet easier. Much like Cook for Love, How Much Phe was developed by individuals in the low protein diet community as a guide for others on their low protein journey. The tool has several features that low protein dieters will find useful. Dieters can search for information about the PHE content, protein content, calories, and exchanges for foods they find using information provided by the Low Protein Food List for PKU. Dieters can track their PHE and protein goals, exchange intake, and blood records by inputting their food intake. The program will track information for the day as well as over time, producing charts that allow the dieter to see trends in the form of charts and view information from previous days. The tool also allows the dieter to export this information as reports for personal or clinic use. There is also a calculator that allows dieters to determine how much of a certain food can be eaten based on weight, PHE content, exchanges, or protein content. How Much Phe is available both as a website and an app for iPhone, downloadable from their website (a video tutorial of how to do this is available for free on their website). This website requires a paid subscription to access the information but this money does go back into the funding for improving and maintaining the program. HowMuchPhe.org
Go Dairy Free is a website created to service everyone interested in a dairy-free diet for any reason, genetic condition, milk allergy, personal interest, or otherwise. This website is no simple food blog (although you can stay updated with founder Alisa Fleming’s blog on the site). It is a comprehensive, community centered resource for dairy-free eaters of any level. They provide start-up guides, definitions, and plenty of other information for beginners. There is a cornucopia of recipes in every category from appetizers to entrees to milk-free substitutes, including kid-friendly recipes. They have a grocery guide, which provides recommendations on useful ingredients, milk-free
brands and products, as well as useful kitchen items. Additionally, Go Dairy Free has complied a restaurant guide, which makes choosing a restaurant much easier for any dairy-free dieter. They have gathered their information from a combination of reader experiences and information from the restaurants themselves. Depending on the restaurant, there is information on acceptable menu choices, milk-containing products to avoid, and whether or not the food processing is done in a plant that also processes dairy (for those with sever dairy allergies). Go Dairy Free has a store, which sells its related books, but all the other information is completely free and accessible without a login or subscription. GoDairyFree.org
Pinterest has become the place to go for hair and make-up tutorials, wedding ideas, crafting projects, and recipes of all kinds. From Mason jar salads to meal prep plans, recipes for any occasion and taste can be found on Pinterest, including recipes for genetically restricted diets. Weighinginonpku.com, pkunews.org, and Minnesota PKU Foundation all post tasty low protein recipes. Galactosemia Dietary Tool also posts galactosemia safe recipes. Pinterest.com
DIET FOOD COMPANIES
Much like Jenny Craig or Nutrisystem, there are specialized diet food companies that prepare formulas and premade foods for people
on restricted diets due to genetic conditions. Nutricia Metabolomics and Cambrooke Theraputics are two such companies. They provide several formulas specific to many different genetic disorders, as well as low-protein foods such as pastas, baking products, and snacks. All of these foods are available to order through their websites. There are also some free recipes, advice for different stages of life, blogs, and information about reimbursement for formula. Cambrooke Theraputics also offers an app called DietWell for PKU that is similar to How Much Phe. It is available for purchase on their website. In addition to the more metabolic focused companies, there are companies that produce alternative foods acceptable for some genetic condition diets. So Delicious, for example, is a company that provides dairy free alternatives to milk, ice cream, yogurt, creamers, and baking milk. They provide recipes and coupons through their website, but their products can be found in certain stores as well as through Amazon.
THEN AND NOW
In many ways, navigating the world of genetically-restricted diets is like trying to navigate a minefield; one misjudgment and the consequences can be explosive. In the past, information about the proportions of different metabolic compounds such as protein, phenylalanine, or fatty acids was information only accessible by reading cookbooks or consulting with a doctor, making everyday meal planning frustrating and difficult. Everything had to be made from scratch and every ingredient had to be meticulously measured. Now, the online dieting community has created a wealth of services that have eased the process of meal planning and prep for those who have no choice but to stick to their diets. There is a map to guide dieters through the minefield. In a few short years, the online dieting community has developed so many useful websites and tools for people with genetic conditions. But why stop there? What will the next development be? •
ABOUT THE AUTHOR:
Adrianna Evans is a Program Intern working with Baby’s First Test at Genetic Alliance. She is currently a first year graduate student at George Washington University working on her Master’s in Public Health, specializing in Global Health Program Design, Monitoring and Evaluation. She received her BS in Biology at Penn State in the spring of 2015.
GENETIC ALLIANCE
The world’s leading nonprofit health advocacy organization committed to transforming health through genetics and promoting an environment of openness centered on the health of individuals, families, and communities.
