NYU’s Dravet Center Director Shares Insights from Conference
Dr. Judith Bluvstein, Director of the Dravet Center at NYU Comprehensive Epilepsy Center, recently returned from Dravet.org’s 2012 biennial conference Advancing Research and Enhancing Comprehensive Care: Progress for Patients with Dravet Spectrum Disorders, held last month in Minneapolis. The forum provided Dr. Bluvstein opportunity to interact face to face again with Dr. Charlotte Dravet, and other recognized colleagues about clinical advancements for treating Dravet syndrome (severe myoclonic epilepsy in infancy), and related epilepsies. Read on to learn about some of the recent developments discussed among attendees.
Epilepsy Diets: Foods for Thought
Since joining the NYU Comprehensive Epilepsy Center as its full-time Registered Dietician Coordinator, Courtney Schnabel, MS, RD, CDN—working in close coordination with the Center’s other experienced doctors—has helped many of its patients receive medical nutrition therapy as part of their epilepsy treatment. The team is currently managing over 90 patients on one variety of the diet where many patients, such as those Dravet Syndrome, have tended to respond particularly well. Read more on Epilepsy Diets.
Life is a Balancing Act
Our daughter, Sylvie, has lived with daily seizures and physical and cognitive challenges for 15 years. Nearly that long ago, someone in an emergency room once tried to comfort me by saying, ”After you have seen 10 seizures or so, you kind of get used to it.” I have never felt that way. For me, each seizure is traumatic both for the one who experiences it and for those who love and care for her.
In caring for Sylvie, who has been diagnosed with Dravet Syndrome, our family lives in a constant state of alertness. We have to anticipate signs of a low seizure threshold, accidents, or other threats to her health and we have to be ready to prevent or remedy them. This may require making (or changing or canceling) a plan, grabbing the paper towels, giving medicine (or taking some away), providing a rest, oxygen, a bandage (or more), closing the blinds, singing a song, telling a story, hugging and kissing and often, saying a prayer. And all the while, Sylvie and her three siblings have beds to make, school to attend, friends to play with, homework to do, practice to get to, dinner to eat, books to read, games to play and a dog to walk. My friend calls it a “balancing act.”
As with any balancing act, there are challenging and discouraging times, as well as hopeful and successful times. Sylvie keeps us all going with her happy, funny, loving demeanor and her true zest for life. There isn’t anything she doesn’t like or want to try. She can befriend anyone, and it doesn’t take very long. She is a model daughter and sister if ever there was one. We are completely inspired to never fail her. Sylvie gives us opportunities to reach and grow all the time. We have reason to examine our lives and how we live them often. In doing so, we strive to be patient and flexible and to see humor in our predicaments and to “never give up” on anything. Sylvie never does. Being Sylvie’s mom has taught me to look inside myself and to think and try and live like I never knew I could.
We are grateful every day to Dr. Devinsky and his colleagues at FACES for the work they are doing to bring attention and much needed advances in treatment to those who live with Dravet. It will take some time for these advances to come. While we wait, if we families can share our experiences with each other we might be able to make a significant therapeutic impact on the lives of Dravet patients and provide meaningful support to their caregivers. For these reasons, we are encouraged by the founding of the NYU Dravet Center, and are thankful to all who have had the vision and determination to create and support this important place.
- Kathy Lammert
Vincent was diagnosed with an SCN1A mutation in May 2011. He was six months old at that time and had been having myoclonic and atonic seizures since he was two months old. I had read enough online to understand that Dravet Syndrome was going to become a big part of our lives. In spite of this devastating diagnosis, I have come to realize we were blessed to find out so early why he was having seizures. Receiving confirmation that our son had this rare disorder also prompted us to make an appointment with Dr. Orrin Devinsky. Who better to see than the man who wrote the book on Epilepsy? [Epilepsy, Third Edition, Patient and Family Guide.]
With four pages of notes, and a video of Vincent's first prolonged seizure, I was armed. After four months of "firsts" that no parent should have to experience – first seizure, first 911 call, first ambulance ride, first spinal tap, first hospitalization, first EEG, first anti-epileptic drug – we were overwhelmed. Yet, I will never forget the kindness and understanding in Dr Devinsky's voice during that appointment. He quickly got us scheduled for a video EEG, and it was during that admission that we met Dr. Judith Bluvstein. Dr. Bluvstein has since been able to get Vincent's seizures under control.
In the past year we have had three generalized tonic clonic seizures, all associated with illness and fever. Dravet Syndrome IS a daily presence in our lives. There is constant worry about being ready for the next seizure, hitting developmental milestones, medication side effects, and the future in general. Then I see Vincent's smile.
- Amy P.
Modified Atkins Recipe: Deviled Eggs with Candied Bacon
By Courtney Schnabel, MS, RD, CDN, and Dara Tannariello
This month we are focusing on Dravet Syndrome. Since the therapeutic diets used to treat epilepsy have been notably successful in some patients with Dravet, we felt it appropriate to share this high fat, low carbohydrate modified-atkins recipe for deviled eggs.
Eggs have gotten a bad rap over the past few decades, mainly because they were said to have high amounts of cholesterol in the yolk. The more research that is done on eggs, the more we realize that they may not be so bad after all. Recently we have been able to focus on their positive benefits such as the nutrient-rich egg yolk! The egg yolk contains two important substances: biotin and lutein. Biotin assists in various metabolic reactions and may be helpful in maintaining steady blood sugar levels. Biotin has also been implicated in the strengthening and health of hair and fingernails. Lutein is a phytochemical found mostly in plants, but is found in high concentrations in egg yolks. It works as an antioxidant in your body to reduce the damage done by free radicals. Antioxidants have many healthy benefits and are said to have roles in disease prevention.
Recipe: Deviled Eggs with Candied Bacon, Makes 6-8 servings
Nutrition Facts (per 1 whole egg/2 halves): 195 calories, 17 grams of fat, 1 gram of carbohydrate, 8g protein
*This recipe is very low carbohydrate and the sweet bacon gives it the illusion of a sweet and savory treat. With 17g of fat this recipe is almost a 2:1 ratio*
1/2 teaspoon stevia (powdered)
Pinch of cayenne pepper
Pinch of ground cinnamon
1/8 pound thick-cut bacon (about 3 strips)
1 tablespoon bacon fat
8 large eggs, straight from the refrigerator
1/4 cup mayonnaise, or as much as desired
2 teaspoons whole grain mustard
1 tablespoon finely chopped fresh dill, plus more for garnish
1/2 tablespoon cider vinegar
3/4 cup heavy whipping cream, whipped to stiff peaks
1 teaspoon Worcestershire sauce
2 scallions, minced
1/2 teaspoon kosher salt
Paprika, for garnish
Preheat the oven to 350 °F
In a small bowl, mix together the stevia, a pinch of cayenne and the cinnamon. Place the bacon on a wire rack set over a rimmed baking sheet. Sprinkle each slice of bacon with some of the spiced sugar and bake, about 10 minutes. Flip the bacon, sprinkle with the remaining spiced sugar and continue to cook until crispy, about 10 more minutes. Remove the bacon from the oven and allow to cool. Once the bacon is cool, mince it and set aside, reserving a quarter of it for garnishing the eggs.
Put the cold eggs in the bottom of a medium saucepan and cover with cold water. Bring water to a boil and remove the pan from the heat. Cover the pan with a lid and let stand for 12 minutes. Drain the eggs and rinse with cold water. Let the eggs cool a bit and then peel when they are still warm (eggs are much easier to peel when they are warm). After they are peeled, you can then store them, covered, in the fridge.
Mix together the mayonnaise, mustard, dill, cider vinegar, Worcestershire sauce, ¼ teaspoon cayenne, the scallions, bacon fat and the minced bacon in a medium mixing bowl. Fold in the heavy cream. Season with the salt.
Slice the eggs in half lengthwise. Gently remove the yolks by pressing your thumb against the back of the yolk to pop it out of the white. Add the yolks to the bowl with the mayonnaise mixture. Mash together, using a fork, until smooth.
Put the filling in a re-sealable bag. Cut one end off and pipe the filling into the egg whites. Sprinkle with the paprika, extra bacon bits and dillbefore serving.
Cook's Note: A wire rack is a great way to cook bacon because the air can circulate around the bacon making it crispy and delicious.
Dr. Blanca Vazquez tells you how to Take Charge of your Epilepsy! Friday, September 14th.
REGISTER TODAY:Call 1-866-865-7305
DATE/TIME: Friday, September 14th 6:00pm
LOCATION: 92nd Street Y
1395 Lexington Avenue, NYC 10128
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