Fall Research Roundtable Summary
Hello and Happy Fall!
For this new edition of Science with Sandra, I would like to share a summary of our recent Fall Research Roundtable that took place on Tuesday October 22nd. The topic for this gathering was the ketogenic diet. We had the privilege of having two wonderful guest speakers, dietitian Kelly Faltersack who shared information about the adult experience with the ketogenic diet, and Dr. Juan Pascual, who shared information about his studies with triheptanoin or C7 oil.
The meeting was a wonderful opportunity to gather with dietitians who are key players in the care of our loved ones, and we had a record number of participant at the meeting, 50 attendees!
Executive Director Glenna Steele and I started the meeting started with a presentation from the patient and family experience around the diet, as well as updates about our Keto Care Project.
The data shared was a combination of information gathered around the diet in our Collective Voices Survey, the Research Ready Survey, as well as a recent Ketogenic Diet survey (see slides here). The results gathered sowed that about 91% of participants in these surveys have tried the ketogenic diet as a form of treatment for Glut1 Deficiency and the most common type of diet used, reported by respondents, is the classical ketogenic diet 3:1 to 4:1 ratio.
Regarding the benefits of the diet reported by participants, the majority report the diet is very effective for seizures but less effective for other symptoms such as movement issues, or speech and language. Results of the recent Ketogenic Diet survey indicate that families feel the diet brings a variety of benefits in different aspects with the most common benefit being seizure freedom. Other benefits include increased energy, developmental gains, or being off medication.
According to the Collective Voices Survey, participants report that challenges around the diet is one of the top three things impacting their quality of life on the social aspects. Most of the responders (54%) indicated the diet makes them feel socially different, it impacts their family celebrations and holidays (52%) and prevents them from being spontaneous (43%). About 41% of participants indicated experiencing diet side effects with constipation being the most common (59%). The recent Ketogenic Diet survey indicated that families feel the diet brings a variety of challenges with the most common being the amount of time for preparation for the diet. Other challenges include the lack of variation, limitations for eating out, and eating different foods, to name a few.
However, despite the challenges, the majority of participants (83%) in our Collective Voices Survey indicated that the benefits of the diet outweigh its challenges. In addition, participants rated the quality of the ketogenic diet support received from their care team with an average of 3.8 stars out of 5.
Glenna also shared some results of the Keto Care Project that the G1DF was able to launch thanks to a grant received from the Baszucki Group. The Keto Care Project is a way to further support families who don’t have access to a keto care team or families who needed extra support. The program partners with 3 expert dietitians who have provided virtual consultations for 24 patients and families from 3 different countries and 12 states within the US. Some of the useful tips or strategies the dietitians participating in the program have reported are that there should be more diet resources available for families, as well as more support options. Some of the targeted assistance has been to provide more recipe options and Ketocalculator access for patients and families.
One of the outcomes of the Keto Care Project has been a resource for families who are not yet on the diet and are trying to learn more about the diet and how to implement it. We thank especially dietitians Victoria Whiteley, Kelly Faltersack, Prof. Dr. Joerg Klepper, and Matthew’s Friends and the Charlie Foundation for their assistance and the collaboration with different Glut1 Deficiency patient organizations around the world. You can find this new resource on our website if you follow this link. You can also find the insights from the recent keto survey here in a blog post.
Dietitian, Kelly Faltersack continued the presentations at the meeting as our first guest speaker. Ms. Faltersack is an expert registered dietitian nutritionist at UW Health in Madison, Wisconsin, and a member of our Medical and Scientific Advisory Board. She specializes in ketogenic metabolic therapy for adults with epilepsy and other neurological conditions.
One of the topics Ms. Faltersack talked about was transition from the pediatric to adult care. She emphasized the importance of starting to plan for transition early during adolescence and she shared a publication by her team at the University of Wisconsin which talks about this transition and is a great resource “Journal of Pediatric Epilepsy– Transition of Care for Adolescent and Young Adult Patients on Dietary Therapy for Epilepsy”. In addition, she recommended to share this publication with the adult care team when planning the transition process “ International Recommendations for the Management of Adults Treated With Ketogenic Diet Therapies”.
Another important topic Ms. Faltersack talked about was the challenge of managing symptoms related to hormonal changes throughout the menstrual cycle. She talked about three types of patterns called catamenial patterns that can occur at certain times in the menstrual cycle. About one in three patients of child-bearing potential may experience seizures, movement disorders, or struggle with low ketones. Catamenial patterns can be classified as: C1 (perimenstrual), in the three days before menstruation through the first three days of menses. During this time, there is a rapid decrease of progesterone. C2 (periovulatory), prior to ovulation, there is a rapid surge of estrogen and progesterone does not rise until ovulation. And C3 (luteal). During this period, there is a high estrogen to progesterone ratio. In anovulatory cycles (a cycle when the release of an egg from the ovaries does not occur), estrogen surges (just like the C2 pattern), but progesterone stays low.
Some of the tips she gave to try to improve symptoms for people with a catamenial pattern were:
- target nutritional strategies to the type of catamenial pattern
- track menses, symptoms and ketones in a calendar and watch for trends (this can be possible now with apps on smart phones)
- time the intervention to encompass the days of the menstrual cycle that match the type of catamenial pattern (for example, someone with a C1 pattern would increase the amount of fat or MCT oil, start 3 days before menses and continue through the first 3 days of menses).
- individualize the type of nutrition intervention
Additionally, Ms. Faltersack shared some nutritional strategies to incorporate during the targeted window:
- Decrease carbohydrate intake – for example, if a patient is on the Modified Atkins Diet, they could lower the carb intake from 20 g net carb to 15 g net carb.
- Increase fat intake – for example, increase the ketogenic diet ratio, add or increase ketogenic formula, add extra fat bombs, add extra oil, avocado or choose high fat snack
- Supplement MCT oil – for example, use MCT oil daily or increase MCT oil during menstruation. This can vary depending on GI tolerance.
I think it is important to talk to the keto diet care team and share this information if your child is experiencing an increase or change in symptoms around their menstruation, so that the team can guide the incorporation of some of the tips mentioned by Ms. Faltersack.
Our last speaker of the day was Dr. Juan Pascual. Dr. Pascual is the Chief of the Division of Child Neurology in the Department of Pediatrics at New York Presbyterian Komansky Children’s Hospital and Weill Cornell Medicine. He specializes in genetic and metabolic diseases of the nervous and neuromuscular systems in infants, children and adults, with emphasis on complex disorders, and he is also a member of our Medical and Scientific Advisory Board.
Dr. Pascual talked about his clinical studies with C7 oil and shared two publications that came out as a results of the studies. The publications are “Maximum dose, safety, tolerability and ketonemia after triheptanoin in glucose transporter type 1 deficiency (G1D)” and “Combination of triheptanoin with the ketogenic diet in Glucose transporter type 1 deficiency (G1D)”. From these publications, Dr. Pascual shared that according to the results found in the studies, he and his team determined that the correct dosage of C7 oil or triheptanoin, after evaluating the safety and tolerability in all participants, is 35% of the total daily calories the patient consumes.
Other important take aways from his talk are:
- C7 does not produce much beta-hydroxybutyrate as a product of its metabolism, instead, it produces other ketones such as beta-ketopentanoate and beta-hydroxypentanoate.
- None of the participants experienced serious or unexpected adverse effects, except for diarrhea in some of the participants.
- C7 is a powerful anaplerotic nutrient; which means that it helps replenish carbons lost that are necessary for the tricarboxylic acid cycle (TCA) cycle.
The study of the combination of the diet with C7 oil indicated that there was a high variability in the production of beta-hydroxybutyrate (BHB) in the study participants. Because of this, Dr. Pascual and his team decided to separate the data from participants into two groups – compatible with C7 oil and non-compatible. Participants in the compatible group were patients in which the addition of C7 oil to the diet decreased their BHB levels to more than 50%, while participants on the compatible group were patients in which the addition of C7 oil decreased their BHB level to less than 50%. Patients on the compatible group expressed their overall satisfaction related to improvement of symptoms and the fact that their ketogenic diet was compatible with the incorporation of C7 to their diet.
Something important to point out is that Dr. Pascual mentioned that when using C7 oil it is important to make sure it is taken 1 hour before any meal for it to be effective!
Finally, I wanted to share some of the questions asked on chat during the meeting with answers provided by Kelly Faltersack. Please see the Q&A below (answers provided in blue).
- Can you please state again where we can find the information about recommended lab monitoring for Glut1. Thank you! International Recommendations for the Management of Adults Treated With Ketogenic Diet Therapies – PMC
- For those who have weaned off the diet for seizure control, how did you do it? was it successful?
I have never weaned an individual with Glut1DS off KT. If this was done, hopefully, they would be transitioning to another treatment option.
- we are starting to see our 14-15 year old Glut1 patients by themselves alone for 10-15 minutes before the parents to start fostering independence. Young adults need a cooking class.
Here are other suggestions for pediatric to adult transition.
Journal of Pediatric Epilepsy– Transition of Care for Adolescent and Young Adult Patients on Dietary Therapy for Epilepsy
- Does genetics or neuro typically drive the ratio decisions for patients (especially with new diagnosis)?
This may vary by institution. At our hospital, the keto team resides in neurology. The. ketogenic therapy team would work with patients/families to make this shared decision together. The medical team can provide suggestions/recommendations, but the families need to be involved in the decision as well.
- What about fiber? In what way too many fiber (more than 14g on 1000 kcal) affect ketosis?
This is a tricky question. We typically count net carbs (subtract out fiber) since it is not absorbed. However, as Diana mentioned live, many keto teams have seen issues with some “keto” products on the market such as “keto bread” that claim to be zero net carbs. I have often seen these types of products decrease ketone levels, kick peoplemout of ketosis, or cause seizures to return. I suspect the products may be rounding down carb-containing ingredients and rounding up added fiber, so they can claim “zero” net carbs. I am very skeptical about these types of products and generally recommend avoiding them. In the diabetes world, some people advise counting 50% of added fibers from these types of products. I’ve considered this approach, but it seems overly complicated as there’s not a great way to capture this in mobile apps used for carb counting. It’s easier to limit the use of “keto” products and focus on getting fiber from whole foods like avocados, berries, and vegetables which are rich in nutrients.
- We have been told that we need to send all meals/snacks into school. I believe Kelly mentioned that schools are mandated to provide keto food. Could you expand on how common that is and how that looks? Like if keto options provided or would they be able to get trained and weigh out food? Just curious. Thanks!
This is not my area of expertise since I work with adults. The USDA requires the National School Lunch Program to make accommodations when needed due to a disability. It requires a letter from the physician. We have provided a letter of medical necessity from the physician and dietitian stating the needed accommodations and appropriate substitutions. My understanding is that some schools will provide a ketogenic formula as a meal replacement or instead of milk. A pediatric dietitian may be better able to elaborate on school lunch program practices. I suspect many families choose to pack their own meals because they can make sure things are done correctly (which could be an issue if the school has staffing constraints or different people preparing food), they know what types of foods their child enjoys and will actually eat, etc. I would hope schools are able to provide reasonable accommodation for more liberal versions such as the modified Atkins diet since it would be easy to offer protein and veggies and skip the carbs, but I would be skeptical about their ability to offer a reliable ratioed ketogenic diet that requires the use of a gram scale. I have worked with some universities, and I’ve been really impressed with some of the accommodations provided for people on the modified Atkins diet.
We thank our wonderful guest speakers for taking the time to present at our meeting and to all attendees for participating, we really appreciate the support and care dietitians and clinicians provide to all the people in our community!
Please do not hesitate to contact me if you have any questions at [email protected] and thank you for visiting our blog!
