Great News – The “Propionic Acidemia Nutrition Guidelines” Are Now Published!
The Nutrition Guideline Committee is happy to announce that the Organic Acidemia Workgroup has published the “Propionic Acidemia (PROP) Nutrition Guidelines” in the February, 2019 issue of Molecular Genetics and Metabolism. The article is available and can be downloaded at no cost at https://doi.org/10.1016/j.ymgme.2019.02.007.
Publication of the PROP/PA Nutrition Guidelines in Molecular Genetics and Metabolism brings the latest evidence- and consensus-based nutrition management recommendations to the attention of clinicians, researchers, policy makers, insurers, and patients.
The new Nutrition Management Guidelines for PROP/PA provide:
Two consumer-oriented pieces, Frequently Asked Questions and a Consumer Summary, provide patients and families with information to use when interacting with their providers. The summary highlights key recommendations and suggests questions that patients and families may want to discuss with the metabolic team.
These nutritional guidelines are part of a multi-year project designed to optimize the nutritional management of individuals with inborn errors of metabolism. The guidelines establish nutritional recommendations based on the best available evidence, contribute to reduced uncertainty and variability of practice and identify needs for future research. Nutritional management guidelines have already been published for other metabolic disorders including phenylketonuria (Singh R et al., Updated, web-based nutrition management guideline for PKU: an evidence and consensus based approach. (2016) Mol Genet Metab, 118(2); 72-83) and maple syrup urine disease (Frazier D et al., Nutrition management guideline for maple syrup urine disease: an evidence- and consensus-based approach. (2014) Mol Genet Metab, 112(3); 210-217), and in the very near future for fatty acid oxidation disorders. This project received funding from the Southeast Newborn Screening and Genetics Collaborative (by a Maternal and Child Health Bureau HRSA grant #2-U22 MC010979), and additional support from Genetic Metabolic Dietitians International (GMDI) to achieve the goal of developing nutritional guidelines for metabolic disorders where there is little published scientific evidence.
Principle Investigators: Rani Singh, PhD, RD and Fran Rohr, MS, RD
Organic Acidemia Workgroup Co-Chairs: Elaina Jurecki, MS, RD and Keiko Ueda, MPH, RD
Organic Acidemia Workgroup members: Dianne Frazier PhD, MPH, RD, Christie Hussa, RD, MBA, Julie McClure, MPH, RD, Laura Nagy, MSc, RD, Lisa Obernolte, MS, RD, Matthew Rasberry, RD, LD, CNSC, Bridget Reineking, MS, RD, CD, Ann Marie Roberts, RD, CSP, CNSD, Amie Thompson, RD, LD, Steven Yannicelli, PhD, RD
Publication of the PROP/PA Nutrition Guidelines in Molecular Genetics and Metabolism brings the latest evidence- and consensus-based nutrition management recommendations to the attention of clinicians, researchers, policy makers, insurers, and patients.
The new Nutrition Management Guidelines for PROP/PA provide:
- New directions including:
- A greater emphasis on nutritional needs such as nutrient intake, nutritional interventions, supplementation, etc.
- Less emphasis on medical management which has been covered in previous publications;
- Additional topics such as monitoring to ensure nutritional adequacy, nutritional issues with pregnancy and lactation, nutritional management for secondary complications such as pancreatitis, and finally a section addressing liver transplantation and the nutritional management before, during, and after the procedure.
Two consumer-oriented pieces, Frequently Asked Questions and a Consumer Summary, provide patients and families with information to use when interacting with their providers. The summary highlights key recommendations and suggests questions that patients and families may want to discuss with the metabolic team.
- When patients and health care providers (HCPs) have the same information, they can work together as a team to identify the treatment that is best for the patient’s situation.
- You can access these pieces at the Genetic Metabolic Dietitians International (GMDI) or Southeast Genetics Network websites located at http://www.Southeastgeneticsnetwork.org/ngp and http://www.GMDI.org
- The new guidelines should lead to greater consistency of care across centers.
- There are several important resources included in the guidelines including recommended nutrient intakes, monitoring schedules, and nutritional interventions tables.
- A web site that provides all the resources and references used to develop the guidelines is available so that health care clinicians and others can readily obtain the background information related to the guidelines at the websites listed above.
- The guidelines development method utilized evidence from published research, practice-based medical literature and expert consensus processes.
- The new recommendations were developed following a rigorous, and systematic process based on published standards for guideline development and modified to incorporate consensus methodology.
- This process took just over 2 years to complete and included:
- 13 experienced metabolic dietitians who conducted an extensive review of 250 scientific and grey (unpublished) publications.
- Two Delphi surveys of practice to obtain input from an additional 54 experienced HCPs,
- A nominal group meeting, which included 7 HCPs and 2 parent group representatives, to clarify discrepancies and determine consensus regarding the proposed recommendations.
- External review and field testing.
- Inclusion of all reference materials and each step of the guideline development process stored on a secured web-based platform which are accessible on the websites listed above.
- Next steps:
- Nutrition Guideline Committee will continue to periodically update these guidelines as new evidence to support best clinical practices are discovered.
- In addition, a PROP tool kit is being developed to facilitate the implementation of the PROP Nutrition Guidelines, including educational resources.
These nutritional guidelines are part of a multi-year project designed to optimize the nutritional management of individuals with inborn errors of metabolism. The guidelines establish nutritional recommendations based on the best available evidence, contribute to reduced uncertainty and variability of practice and identify needs for future research. Nutritional management guidelines have already been published for other metabolic disorders including phenylketonuria (Singh R et al., Updated, web-based nutrition management guideline for PKU: an evidence and consensus based approach. (2016) Mol Genet Metab, 118(2); 72-83) and maple syrup urine disease (Frazier D et al., Nutrition management guideline for maple syrup urine disease: an evidence- and consensus-based approach. (2014) Mol Genet Metab, 112(3); 210-217), and in the very near future for fatty acid oxidation disorders. This project received funding from the Southeast Newborn Screening and Genetics Collaborative (by a Maternal and Child Health Bureau HRSA grant #2-U22 MC010979), and additional support from Genetic Metabolic Dietitians International (GMDI) to achieve the goal of developing nutritional guidelines for metabolic disorders where there is little published scientific evidence.
Principle Investigators: Rani Singh, PhD, RD and Fran Rohr, MS, RD
Organic Acidemia Workgroup Co-Chairs: Elaina Jurecki, MS, RD and Keiko Ueda, MPH, RD
Organic Acidemia Workgroup members: Dianne Frazier PhD, MPH, RD, Christie Hussa, RD, MBA, Julie McClure, MPH, RD, Laura Nagy, MSc, RD, Lisa Obernolte, MS, RD, Matthew Rasberry, RD, LD, CNSC, Bridget Reineking, MS, RD, CD, Ann Marie Roberts, RD, CSP, CNSD, Amie Thompson, RD, LD, Steven Yannicelli, PhD, RD
Metabolic Balancer APP replaces Dietwell
A new Metabolic Balancer App to replace the DietWell App to help people living with Metabolic disorders to feel balanced, feel better with key functionality to create recipes, track phe and protein intake, share information with friends and learn about Cambrooke products and much more. In addition to all the features and user interface enhancements, with this new release we have rearranged recipes, meals ingredients, food items and their classification to make the new app more relevant, efficient and organized.
Metabolic Balancer – is designed to assist in the dietary management of Phenylketonuria (PKU) and other inborn errors of protein metabolism where tracking exact protein is essential in the management of a specific metabolic disorder. This iPhone app guides you through your meals, snacks, and formula intake by offering phenylalanine (phe), dietary protein, formula protein equivalent (P.E.), and calorie information for over 7500 food items, specially manufactured low protein food products, metabolic formula products, Cambrooke recipes and meals, along with your Kuvan® intake, blood levels and wellness - all at the touch of your fingertips.
Use Metabolic Balancer to:
1) Improve Dietary Compliance- learn about the phe content of your food in order to make better choices. Metabolic Balancer provides a quick reference to more than 7500 food items. Know what each portion or recipe actually contains. Know when you need to adjust your dietary phe intake. Save your favorite foods to Favorites or add new items, recipes and meals.
2) Prepare Diet Log and export it via email in preparation for Clinic Visit. You can now easily record your dietary protein, supplemental protein, and Kuvan® intake along with your blood levels and wellness using this handy iPhone app. Many U.S. metabolic clinics recommend 3-5 days of dietary records before the clinic visit.
3) Plot data overtime.
Metabolic Balancer app can help you better understand how dietary compliance correlates to your overall feeling “wellness”.
Metabolic Balancer features:
• Over 7500 USDA and manufacturers' product data. Includes data on many specialty low protein food items and formula.
• Log data for multiple users.
• Easy search.
• Save your frequently used food items or recipes to Favorites.
• Add new food items, recipes or meals.
• Share custom items, recipes and meals with friends.
• Track of your Kuvan® intake, blood levels and daily wellness.
• Monitor your daily goals with the graphing feature.
• Review, edit, and export to email (by date range) daily log entries by user.
• Enter quantities in three convenient units: gram, ounce, or serving size.
• All data is contained within the app. No Wi-Fi connection is needed except when downloading updates or exporting logs via email.
Metabolic Balancer – is designed to assist in the dietary management of Phenylketonuria (PKU) and other inborn errors of protein metabolism where tracking exact protein is essential in the management of a specific metabolic disorder. This iPhone app guides you through your meals, snacks, and formula intake by offering phenylalanine (phe), dietary protein, formula protein equivalent (P.E.), and calorie information for over 7500 food items, specially manufactured low protein food products, metabolic formula products, Cambrooke recipes and meals, along with your Kuvan® intake, blood levels and wellness - all at the touch of your fingertips.
Use Metabolic Balancer to:
1) Improve Dietary Compliance- learn about the phe content of your food in order to make better choices. Metabolic Balancer provides a quick reference to more than 7500 food items. Know what each portion or recipe actually contains. Know when you need to adjust your dietary phe intake. Save your favorite foods to Favorites or add new items, recipes and meals.
2) Prepare Diet Log and export it via email in preparation for Clinic Visit. You can now easily record your dietary protein, supplemental protein, and Kuvan® intake along with your blood levels and wellness using this handy iPhone app. Many U.S. metabolic clinics recommend 3-5 days of dietary records before the clinic visit.
3) Plot data overtime.
Metabolic Balancer app can help you better understand how dietary compliance correlates to your overall feeling “wellness”.
Metabolic Balancer features:
• Over 7500 USDA and manufacturers' product data. Includes data on many specialty low protein food items and formula.
• Log data for multiple users.
• Easy search.
• Save your frequently used food items or recipes to Favorites.
• Add new food items, recipes or meals.
• Share custom items, recipes and meals with friends.
• Track of your Kuvan® intake, blood levels and daily wellness.
• Monitor your daily goals with the graphing feature.
• Review, edit, and export to email (by date range) daily log entries by user.
• Enter quantities in three convenient units: gram, ounce, or serving size.
• All data is contained within the app. No Wi-Fi connection is needed except when downloading updates or exporting logs via email.
Nutrition & Recipes
The Metabolic Diet App Suite: Metabolic Diet Tracking on the Go!
http://www.metabolicdietapp.org/
by Keiko Ueda MPH RD, Gloria Ho, Roderick Houben, Jeffrey Joa, Alette Giezen RD, Barbara Cheng RD, Clara van Karnebeek, MD, PhD, FCCMG
Lifelong medical nutrition therapy (MNT) is a key part of medical therapy for many inherited amino- and organic acidopathies. But there is a lack of metabolic disorder specific diet intake tracking and meal planning resources. Many individuals living with metabolic disorders experience difficulties following their daily metabolic diet goals. The Metabolic Diet App Suite is a free, web-based metabolic diet tracking tool developed to help people living with metabolic disorders track and plan their daily diets. It was created with input from biochemical geneticists, metabolic dietitians, metabolic patient caregivers, and application developers for use on both mobile devices and desktop computers (http://www.metabolicdietapp.org). General disorder information is provided for fifteen individual metabolic disorders for example ‘GA1’ (glutaric acidemia), ‘IVA’ (isovaleric acidemia), ‘MMA’ (methylmalonic acidemia), ‘PROP’ (propionic acidemia), as well as a general protein tracker ‘PROT’. Food nutrient content is based on the MetabolicPro™ program food database, compiled by the Genetic Metabolic Dietitians International (GMDI) Technology committee. Each Diet App offers functions such as: secured personal user login/password, user instructions, goal setting, daily intake tracking, food content checks, adding foods and homemade recipes, exportable daily food diary log (update your doctor and dietitian), and developer feedback. Individuals can start by going to the website and creating an account and diet profile, no downloading required. The website provides a user guide: ‘How it works?’ with answers to frequently asked questions (FAQs) such as how to recover forgotten password and usernames and contacting the developers. Initial pilot tester feedback was positive and suggestions used to further improve the App suite. This project was funded by the British Columbia Children’s Hospital Foundation (Treatable Intellectual Disability Endeavor in British Columbia, www.tidebc.org, 1st Collaborative Area of Innovation) and the Rare Diseases Foundation (Vancouver, Canada). The Metabolic Diet App Suite is intended as a patient and caregiver support tool. They do not replace the GMDI Metabolic Pro™ diet analysis program or health care professional advice. We hope that this tool helps ease the daily challenge of metabolic diet tracking and meal planning for individuals living with protein or amino- or organic acid restricted metabolic nutrition therapies. Ho G, Ueda, K, Houben RFA, Joa J, Giezen A, Cheng, B, van Karnebeek CDM. Metabolic Diet App Suite for inborn errors of amino acid metabolism. Molecular Genetics and Metabolism. 2016 Mar;117(3):322-7.
Spotlight on Dietary Fiber & OAs
by: Keiko Ueda, MPH, RD, LDN, Metabolic Dietitian, Floating Hospital for Children at Tufts Medical Center & Emily Evans, MS, Frances Stern Nutrition Center Dietetic Intern, Tufts University Friedman Graduate School of Nutrition, Boston, MA
by: Keiko Ueda, MPH, RD, LDN, Metabolic Dietitian, Floating Hospital for Children at Tufts Medical Center & Emily Evans, MS, Frances Stern Nutrition Center Dietetic Intern, Tufts University Friedman Graduate School of Nutrition, Boston, MA
State Laws on Formula Coverage
What's the Law for Formula Coverage in Your State?
What's the Law for Formula Coverage in Your State?
Low in Protein, BIG in Taste!
Nutricia North America Introduces Milupa Line of Low Protein Foods
http://www.myspecialdiet.com/
Nutricia North America is excited to announce the availability of new Milupa low protein (lp) foods that are low in protein and BIG in taste! These great tasting lp-foods are suitable for children and adults, provide on-the-go convenience and add variety to low protein diets. New offerings include:
Product information, recipes and online ordering are conveniently available at MySpecialDiet.com. Free shipping is available on orders over $25.
Nutricia North America Introduces Milupa Line of Low Protein Foods
http://www.myspecialdiet.com/
Nutricia North America is excited to announce the availability of new Milupa low protein (lp) foods that are low in protein and BIG in taste! These great tasting lp-foods are suitable for children and adults, provide on-the-go convenience and add variety to low protein diets. New offerings include:
- Drinks – unflavored and chocolate
- Snacks – paprika chips and italian mix
- Cereals – chocolate ringlets and flakes
- Cereal Mixes – pear and apple-banana
- Energy Bars – apple-cinnamon, apricot and fruits of the forest
Product information, recipes and online ordering are conveniently available at MySpecialDiet.com. Free shipping is available on orders over $25.
Additional Information
Be sure to check the Nutritional Links Page for More Useful Websites