Nutrition in Glycogen Storage Disease Recorded Lecture Presentations

Published On: 15th April 2014

Vitaflo is pleased to bring you the following lecture presentations which were recorded at the 1st Nutrition in Glycogen Storage Disease (GSD) Education Day held in Raleigh-Durham, North Carolina on September 19th, 2013. This one day educational program for dietitians and other healthcare professionals included discussions on the nutritional management of GSD types I and III, current guidelines and practices for management using uncooked corn starch and Glycosade® (Vitaflo®), as well as a review of the needs for protein in GSD type III.  A copy of the full program agenda and speakers can be downloaded here.

Access to the videos below requires a password which healthcare professionals can obtain from their local Vitaflo representative or by emailing Vitaflo@VitafloUSA.com

NOTE: The views and opinions expressed in the video presentations are based on the presenters’ experiences and do not reflect any recommendations or opinions of Vitaflo USA, LLC.

Nutritional Management of GSD

Segment 1:

Nutritional management of GSD I (minute mark 00:00 to 26:54)

Kathy Ross RD, LDN, Pediatric Metabolic Dietitian, Glycogen Storage Disease Program, University of Florida

Kathy Ross, Registered Dietitian, gives a comprehensive overview of the nutritional management of GSD type I based on her own practices and experience in working with a large GSD population at The University of Florida Glycogen Storage Disease Program. The nutritional management of infants, young children, adolescents and adults is discussed. Practical tips on dietary restrictions (fructose, simple sugars and galactose), feeding aversions, sick day management and dosing of UCCS are included. Case examples are used to illustrate specific aspects of nutritional management.

Segment 2:

Nutritional Management of GSD III (minute mark 26:55 to 58:12)

Anne Boney, MEd, RD, LD, Pediatric Metabolic Dietitian, Clinical Science Liaison, QOL Medical

Anne Boney, Registered Dietitian, discusses and reviews the evidence to support nutritional management of GSD type III including guidance on dosing of UCCS and protein in context of the overall macronutrient composition of the diet. Guidelines for short and long term monitoring and follow up are also reviewed. Practicalities of nutritional management are illustrated through a case example.

PLEASE NOTE: This video is intended for health care professionals only and requires a password for access. If you are a heath care professional, and would like to request the video password, please email us here.

 

Use of Glycosade in Management of GSD

Segment 1:

Evidence and practices in use of uncooked corn starch (UCCS)/Glycosade in GSD (minute mark 00:00 to 38:39)

Dr. David Weinstein, Director, Glycogen Storage Disease Program, University of Florida College of Medicine

Dr. David Weinstein describes the use of UCCS and Glycosade in the management of GSD type I in context of overall clinical goals and outcomes. He provides a review of the evidence to support dosing of UCCS and provision of carbohydrate to meet glucose requirements at various ages. The advantages and limitations of UCCS compared to continuous overnight feeds are reviewed. Dr. Weinstein also discusses the history and development of Glycosade, and provides a description of the experiences in using Glycosade as an overnight source of glucose in over 100 patients at the University of Florida Glycogen Storage Disease Program. Data is presented on 69 patients with GSD type I which includes dosing, monitoring and long term experiences. Use of Glycosade in other types of GSD (III, VI, IX, O) is also presented.

Segment 2:

Setting up an inpatient protocol for initiation of Glycosade (minute mark 38:40 to 54:52)

Dr. Laurie Tsilianidis, Center for Pediatric Endocrinology, Glycogen Storage Disease Program, Cleveland Clinic Foundation

Dr. Laurie Tsilianidis describes her experiences in setting up a new Glycogen Storage Disease program at the Center for Pediatric Endocrinology, Cleveland Clinic Foundation. Her presentation describes steps taken to set up an inpatient protocol for Glycosade including patent selection, initiation, monitoring and follow up. She presents data on her experiences in using Glycosade in 17 patients and illustrates aspects of management through case discussion.

PLEASE NOTE: This video is intended for health care professionals only and requires a password for access. If you are a heath care professional, and would like to request the video password, please email us here.

About Our Speakers

Dr. David Weinstein

Director, Glycogen Storage Disease Program, University of Florida College of Medicine

GSD webinar Dr. WeinsteinFollowing his graduation from Trinity and Harvard Medical School, Dr. Weinstein completed a residency, chief residency, and fellowship in pediatric endocrinology at Children’s Hospital Boston. He subsequently obtained a Masters in clinical investigation from Harvard and MIT, and became Director of the Glycogen Storage Disease Program at Boston Children’s Hospital. In 2005, Dr. Weinstein moved to the University of Florida where he directs the Glycogen Storage Disease Program. Dr. Weinstein follows the largest cohort of hepatic GSD patients in theworld, and he has published over 50 articles and 25 textbook chapters on this topic. Dr. Weinstein is a former Barry Goldwater Scholar, and he received the Jan Albrecht Award from the American Association for the Study of Liver Diseases.  He also received the George Sacher Award from the Gerontological Society of America. Dr. Weinstein was inducted in the Rare Disease Research Hall of Fame in 2013, and he was recently awarded a United Nations recognized international humanitarian award for his efforts to help children with glycogen storage disease from around the world.


Dr. Laurie Tsilianidis

Center for Pediatric Endocrinology, Glycogen Storage Disease Program, Cleveland Clinic Foundation

Laurie TsilianidisLaurie Tsilianidis, M.D. was raised in Cleveland, Ohio and graduated from the University Of Toledo School Of Medicine in Toledo, Ohio in 2006. She completed residency training in Pediatric at the University of South Carolina/Palmetto Health Richland in Columbia, South Carolina. She began her fellowship training in pediatric endocrinology at the Medical University of South Carolina in Charleston, South Carolina, but transferred to the University of Florida in Gainesville, Florida to complete her last two years of training. At the University of Florida, she trained under Dr. David Weinstein and developed a passion for studying and treating hepatic forms of glycogen storage disease. Upon graduation, she returned home to Cleveland to develop a multidisciplinary Glycogen Storage Disease Program at the Cleveland Clinic. In its first year, the program saw close to 50 patients from around the country. Dr. Tsilianidis has published several articles and book chapters on the subject and spoken nationally and internationally to raise awareness about these disorders.


Kathy Ross

RD, LDN, Pediatric Metabolic Dietitian, Glycogen Storage Disease Program, University of Florida

Kathy Ross GSD speakerKathy Ross received her BS in Dietetics and Nutrition from Florida International University in Miami, Florida and is currently a Registered Dietitian at the University of Florida in Gainesville where she has served as the Clinical Coordinator of the Glycogen Storage Disease Program since 2010. Kathy is responsible for providing nutritional care and counseling to infants, children and adults who are followed in the UF Division of Pediatric Endocrinology, working specifically with patients with GSD Types 0, I, III, VI and IX. Kathy has spoken and presented at many national and international meetings on the topics of Diabetes and Glycogen Storage Disease management, and has been nominated for the Emerging Leader Award for the Florida Dietetic Association.


Anne Boney

MEd, RD, LD, Pediatric Metabolic Dietitian, Clinical Science Liaison, QOL Medical

Anne Boney received her BS in Public Health Nutrition from the University of North Carolina and then a MEd in Foods and Nutrition from the University of North Carolina at Greensboro.  After completing a clinical rotation at Duke University Medical Center (DUMC) she began her career at Duke as a clinical dietitian.  Later Anne became the Nutrition Manager for the General Clinical Research Center (GCRC) at DUMC where she first learned about inborn errors of metabolism and Glycogen Storage Disease.  When the DUMC metabolic clinic opened in 1999, she joined the Department of Pediatrics, Genetics and Metabolism and worked there until 2013.  Anne is now a clinical science liaison for QOL Medical in Raleigh, NC where she works with Sucraid (sacrosidase), an oral enzyme replacement therapy used to treat genetic sucrase Isomaltase deficiency (GSID).

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