Leah’s Story: Epilepsy & the Ketogenic Diet

My precious daughter, Leah, is 13 years old. She loves to listen to music and swim. She also enjoys riding her golf cart with her brothers, Noah and Elijah, and her little sister Eden. She enjoys listening to the birds while relaxing on her swing outside.

Concerning Symptoms

Leah was developing on track and meeting all milestones until she was around 5 ½ months old. On Labor Day of 2005, I noticed Leah’s eyes were rolling up and to the right. Since Leah is my third child, I knew that something was off and that it wasn’t normal. I actually worked in Leah’s pediatrician’s office and mentioned it to the pediatrician at work the following afternoon. My husband, Brandon, and I brought Leah into the clinic the following morning. The pediatrician discovered that Leah wasn’t visually tracking with her right eye and referred us right away to a pediatric ophthalmologist at a children’s hospital 2 hours away. The ophthalmologist thought Leah might have delayed visual maturation. He wanted to monitor her and keep an eye on it over time.

At Leah’s 6-month check-up, Leah’s pediatrician discovered that she wasn’t visually tracking with either of her eyes anymore. The next couple of months were pretty busy. After more appointments and exams, they discovered that Leah developed cortical visual impairment. As time went on, we also noticed that Leah wasn’t developing properly and was showing some regression. We saw a neurodevelopmental pediatrician. He could see something wasn’t right, but wasn’t sure what was going on. He did some testing, labs, and recommended a referral to a pediatric neurologist.

Then, in November, before we could meet with the pediatric neurologist, I was holding Leah when I noticed that she was throwing her head, arms, and legs outward in a rhythmic pattern. I started counting them. It was happening in clusters every 8 seconds. I thought she was having a seizure. The Internet wasn’t widely used in our rural area back in 2005, so I asked my Aunt Jamie to help look some things up for me. We found some information on Infantile Spasms, also known as West Syndrome, and thought it sounded a lot like the symptoms we were seeing with Leah.

Infantile Spasms

The following morning, I told Leah’s pediatrician that I thought Leah had Infantile Spasms. I told her about the episodes the previous night and she agreed that it sounded exactly like Infantile Spasms. She said it would need to be diagnosed with an electroencephalogram (EEG), but that it would explain Leah’s recent symptoms and developmental regression. The pediatrician called the neurologist that Leah was scheduled to see in a couple of weeks, who agreed that it sounded like Infantile Spasms. She said sometimes it takes a little while to show up on an EEG and to keep our appointment we had scheduled. By that time, if it was Infantile Spasms, it would more than likely show on the EEG.

Fast forward to appointment day. Leah was scheduled for an EEG and another test called a visual evoked potential (VEP) in the morning, and then to see the neurologist in the afternoon. After the first two tests, the EEG tech told us that she had to call the neurologist. I knew then that she saw something abnormal. She came back in and said that the neurologist wanted her to do one more test. After that was done, we had lunch, then went to the neurology clinic for Leah’s appointment. The neurologist told us that our suspicion was right and officially diagnosed Leah with Infantile Spasms.

Leah’s neurologist recommended a medication called ACTH for Leah and told us that it wasn’t a medication that they kept on hand at the hospital so it would need to be ordered in. She explained that Leah would need to be admitted to the hospital to start the medication and that we would be trained to give the injections at home. She told us to go home, make arrangements for our boys, pack a bag, and come back the following afternoon. We were scared, but mostly relieved that we finally had an answer. It took a little while, but the ACTH stopped the Infantile Spasms. Leah had some feeding difficulty, reflux, and was very developmentally delayed, but was doing pretty well otherwise. She had a lot of therapies through Early Intervention each week. She started preschool and was learning new skills.

Seizures Return

Five years later, shortly after Leah turned 5 years old, I had a flashback to November of 2005. I was holding her on my lap one evening and I felt rhythmic moving. Her right shoulder was moving back and her head kept turning to the right. Like years before, I started counting and got a sick feeling in the pit of my stomach. I knew she was having a seizure. The following day, I contacted her neurologist and she scheduled an EEG. The episodes started happening more often. When she had the EEG done, it confirmed that the episodes were indeed seizures. We tried one medication and it didn’t work. Over the next 2 years we tried a second and then a third. They weren’t working.

The Ketogenic Diet

When Leah’s seizures started, she began to develop problems with eating and drinking. We noticed that the periods of time when she wasn’t eating and drinking much at all, her seizures lessened. I mentioned that to Leah’s pediatrician and she said she wondered if the ketogenic diet would work for her. I didn’t know a lot about it. I had some friends I met in the Infantile Spasms Community Facebook group that had children that were on or tried keto. Some had success and others didn’t. At our next neurology appointment, I mentioned it to the neurologist and she agreed that the ketogenic diet was our next option.

After we decided to try the ketogenic diet, our neurologist had the nutritionist come meet with us to talk more about it. We discovered that Leah was malnourished and dehydrated because of not wanting to eat and drink. Our team decided that until we could get her healthy, we would not be able to start the ketogenic diet. They decided the best way to get her healthy was to have a feeding tube inserted. Leah had her feeding tube placed in August of 2011. She started gaining weight and looked so much better, but by that time she was having hundreds of seizures a day.

On November 1, 2011, Leah was admitted to Geisinger’s Janet Weis Children’s Hospital to start the ketogenic diet. We started out slowly by using her formula mixed with KetoCal® 4:1 powder. Then we kept adding more KetoCal® while decreasing the other formula until she was getting all KetoCal. We had some bumps along the road during the admission. Leah was having some low blood sugars and was vomiting. The neurologist and nutritionist worked together to find that KetoCal® 3:1 powder, which has a lower ketogenic ratio, was a better match for Leah. The lower ketogenic ratio kept both her blood sugars in range and her stomach tolerated it well. We were discharged and returned home where I monitored and charted seizures every day. I started noticing a decrease in seizures. They decreased more and more every day. On day 8, Leah didn’t have any seizures. It was the same on day 9 and 10 and 11. Eventually, we were able to successfully wean Leah off of the last seizure medication that she was on. Leah was doing very well. She was happy. She was healthy. She was interacting with family and friends. She was enjoying life. Leah remained seizure-free on the ketogenic diet for nearly 3 years.

Leah Today

Just a few days shy of Leah being on the ketogenic diet for 3 years, things changed. I got a call from Leah’s school nurse, who said that Leah was having some involuntary rhythmic movement. Unfortunately, 4 years later we’re still seeing it despite continuing the ketogenic diet and adding in many more medications.

Leah is now 13 years old. We have finally found a cause for her epilepsy. She has a spontaneous mutation of her ALG13 gene. It is a very rare mutation, but is now a known epilepsy gene. She is having many seizures a day. We recently had a discussion with her neurologist about what we do next. Part of our new plan is to gradually wean Leah off of the ketogenic diet. It kind of makes me feel bad, but I know we gave it our all. Keto has been a big part of our life for almost 7 years now and I will forever be thankful for those 3 wonderful years that the ketogenic diet and KetoCal allowed Leah to be Leah. To be happy, healthy, and learning new things.

Advice to Parents Considering the Ketogenic Diet

My advice to parents considering the ketogenic diet is to discuss it with your neurologist, and if recommended, try it. We were cautious when starting. We didn’t want to get our hopes up just to be let down. I said the day we started that we’ll hope for the best and pray it’s our miracle, and it was. After the first month, when you establish a routine and find your groove, it gets easier. We measured our KetoCal out ahead of time when we got our shipment. Then all we had to do was add water and mix it daily. The ketogenic diet is definitely worth a try. Just remember to take it one day at a time and keep your eye on the prize…seizure control!

~Krista

I was paid by Nutricia for my time to write this blog, however, my opinions are my own.

KetoCal is a medical food intended for individuals age 1 year and up, and is intended for use under medical supervision.

The ketogenic diet for epilepsy should be used under medical supervision.

Please note that this is one family’s report of their experience with the ketogenic diet and may not be the experience of others. Individual results will vary. Please discuss the potential benefits of the ketogenic diet with your healthcare provider.

The Benefits of Using KetoCal® 4:1 Once Per Day

KetoCal 4-1 productsThe ketogenic diet can help manage seizures in patients with refractory epilepsy. In fact, research shows that just over half of children on the ketogenic diet will have 50% or more improvement in seizures, about 1/3 of children will have over 90% improvement in seizures and about 10-15% will become seizure-free. While it can be helpful with seizure management, being on the ketogenic diet can be challenging at times. In today’s blog post, I will tell you a little about how KetoCal can be a helpful addition to the ketogenic diet.

Everyday Convenience

If you have a child on the ketogenic diet, I don’t have to tell you that it can be challenging to plan for keto meals or snacks while running errands, traveling, or going to after-school activities. Using KetoCal once per day can help provide everyday convenience when you are on-the-go. KetoCal® LQ is ready-to-drink and provides a simple and nutritious solution for busy schedules. It also tastes great! (Request a sample to taste it for yourself!)

Nutritional Reassurance

As a busy mom and dietitian, I know a little about the struggle to find meals and snacks that are easy AND nutritious. KetoCal was designed to be both! Using KetoCal® 4:1 once per day is not only convenient, but can also help fill nutrient gaps in the ketogenic diet. Unlike heavy cream (a popular milk substitute with the ketogenic diet)*, one serving of KetoCal 4:1 (356 calories) provides:

  • An excellent source of calcium and vitamin D, two nutrients that are important for bone health
  • Fiber to promote gut health
  • DHA, an Omega-3 fatty acid that plays a role in brain and eye health
  • 4 times LESS saturated fat when compared to heavy cream!

There are multiple ways to incorporate KetoCal 4:1 into your daily routine. You can drink it once per day or use it in everyday recipes. KetoCal 4:1 is available as a powder (great for baking!) or as a ready-to-drink liquid (convenient while on-the-go). Work with your dietitian or visit www.myketoplanner.com to find tasty recipes using KetoCal 4:1.

Ellen Avery, MS, RD
Medical Advisor, Ketogenics
Nutricia North America

KetoCal is a medical food for use under medical supervision.

*Comparing an equal amount of calories from heavy cream.

 

 

Recipe: KetoCal Pumpkin Spice Shake

yogurt with spiced pumpkin puree

In case there was any doubt, the surplus of pumpkin-flavored foods confirms that it is officially fall. Like many, I look forward to pumpkin lattes, pumpkin cheesecakes, and anything else pumpkin all year long. I decided that we needed to come up with a pumpkin KetoCal recipe, so I set out to create one. I must say that this delicious KetoCal Pumpkin Spice Shake exceeded my expectations. I hope that your keto kids enjoy it as much as I did!

-Mallory

As always, consult with your health care provider to ensure that this recipe idea is appropriate for your child’s diet and calorie requirements. The instructions for preparing this recipe at a 4:1 and 3:1 ketogenic ratio are provided, along with the instructions for preparing this recipe for the Modified Atkins Diet. Be sure to use the appropriate version for your child. If your child is on a different ratio, your dietitian may be able to help you adjust it by changing the amount of oil used. Note that the nutrition values were calculated using KetoCalculator, so if you are in another country or using a different system, the recipe may need to be adjusted.

Ingredients:

4:1 Version:

  • 120 g Vanilla KetoCal 4:1 LQ (you could also use Unflavored KetoCal LQ but you may want to add a carbohydrate-free sweetener if you do)
  • 15 g pumpkin, canned, unsweetened
  • 1 g ground cinnamon or unsweetened pumpkin pie spice
  • 5 g canola oil

3:1 Version:

  • 120 g Vanilla KetoCal 4:1 LQ (you could also use Unflavored KetoCal LQ but you may want to add a carbohydrate-free sweetener if you do)
  • 15 g pumpkin, canned, unsweetened
  • 1 g ground cinnamon or unsweetened pumpkin pie spice

MAD Version:

  • 1/2 cup (120 mL) Vanilla KetoCal 4:1 LQ (you could also use Unflavored KetoCal LQ but you may want to add a carbohydrate-free sweetener if you do)
  • 1 tablespoon pumpkin, canned, unsweetened
  • 1/2 teaspoon ground cinnamon or unsweetened pumpkin pie spice

Directions:

  • Add ingredients to a blender and blend until smooth.
  • Once prepared, serve immediately or store in a refrigerator for up to 24 hours.

Serving ideas:

  • Blend with ice for a cold treat!
  • Serve warm for a comforting treat on a cold day!
  • Freeze into pumpkin spice popsicles or ice cream!

Nutrition Information:

4:1 Version:

Ratio: 4:1
Calories: 228
Fat: 22.8 g
Protein: 3.9 g
Carbohydrate: 1.8 g

3:1 and MAD Versions:

Ratio: 3.1:1
Calories: 183
Fat: 17.8 g
Protein: 3.9 g
Carbohydrate: 1.8 g