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.

To learn more about the medical ketogenic diet for the management of intractable epilepsy, visit www.ketogenicdietforepilepsy.com.

Keto-friendly Thanksgiving Recipes!

Chef Rachel

Chef Rachel

Thanksgiving is right around the corner! For keto parents, it can be difficult to come up with keto-friendly variations of traditional Thanksgiving recipes. Chef Rachel Finn, from Kansas City, KS, has saved you some time! Rachel specializes in creating recipes for individuals on special diets and often works with families of children on the ketogenic diet. Her keto Thanksgiving recipe creations are sure to please your little one’s taste buds! Below are instructions for making these recipes at a 4:1 or 3:1 ratio. We hope that your family has a wonderful Thanksgiving!

Note, the recipes below are intended for individuals on a 4:1 or 3:1 ketogenic ratio. As always, consult with your health care provider to ensure that a recipe is appropriate for your child’s diet. Work with your provider to adjust recipes to meet your specific ratio and calorie requirements. 


Recipes for a 4:1 Ratio

Thanksgiving Tart (4:1)

tart

Ingredients:
16 g Butter
20 g KetoCal® 3:1 powder, Nutricia NA
18 g Oil, Olive
10 g Cream Cheese, Philadelphia Brand
8 g Flour, Coconut – Bob’s Red Mill Organic Hi Fiber
11 g Egg (raw, mixed well)
2 g Bouillon, Wylers Instant-Beef/Chick granules
7 g Turkey, dark meat (no skin) – cooked
5 ml Water

Directions:
1. Preheat oven to 325 °F
2. To make the tart crust, place coconut flour and KetoCal into bowl and rub in butter with fingertips. Add a pinch of poultry seasoning, dried rosemary or rubbed sage, if desired.
3. Dissolve bouillon granules in the 5 mL water. Sprinkle bouillon over top tart crust dough and mix until it becomes a stiff, crumbly pastry dough. Save 5 grams of the pastry dough for the tart topping.
4. Line tartlet tin with your crust dough (minus the 5 grams saved for the top), using your fingers to press the pastry into the edges.
5. In a bowl, mix together cream cheese, egg, olive oil, and turkey. Pour the mixture into the crust.
6. Season with salt and pepper, and top with remaining 5 g of crust dough. Add another pinch of dried rosemary on top.
7. Place on oven tray and cook for 10-15 minutes. Allow to cool before removing from tart pan.

Nutrition Information (for entire recipe):
Carbohydrate: 3.87
Calories: 507
Fat: 50.87
Protein: 8.8
Ratio: 4.01:1

Mashed Fauxtatoes (4:1)faux

Ingredients:
76 g Cauliflower, cooked
8 g KetoCal® 4:1 LQ Unflavored – Nutricia
14 g Butter

Directions:
1. In a small saucepan, heat KetoCal 4:1 LQ with butter and cooked cauliflower until warm.
2. Puree mixture in a blender or food processor until smooth.
3. Season with salt and pepper.

Nutrition Information (for entire recipe):
Carbohydrate: 1.44
Calories: 129
Fat: 12.88
Protein: 1.77
Ratio: 4.01:1

Thanksgiving Green Bean Casserole (4:1)gbc

Ingredients:
10 g KetoCal® powder 3:1, Nutricia NA
36 g Cream, 36%
50 g Green Beans – cooked
28 g Butter
46 g Sour Cream – cultured (not low-fat)
10 g Durkee French’s fried onions

Directions:
1. Melt butter; Mix with KetoCal 3:1 powder, sour cream, and cream.
2. Stir in green beans.
3. Pour mixture in a ramekin or a small baking dish. Season with salt & pepper.
4. Top with fried onion (if desired, save some of the onions to place on top after baking for a crunchy texture).
5. Bake in 350 °F for 15 minutes.

Nutrition Information (for entire recipe):
Carbohydrate: 9.77
Calories: 566
Fat: 56.66
Protein: 4.38
Ratio: 4:1


 

Recipes for a 3:1 Ratio

Thanksgiving Tart (3:1)tart

Ingredients:
17 g Butter
20 g KetoCal 3:1 Powder
7 g Oil, Olive
10 g Cream Cheese, Philadelphia Brand
10 g Flour, Coconut – Bob’s Red Mill Organic Hi Fiber
14 g Egg (raw, mixed well)
2 g Bouillon, Wylers Instant-Beef/Chick granules
6 g Turkey, dark meat (no skin) – cooked

Directions:
1. Preheat oven to 325 °F
2. To make the tart crust, place coconut flour and KetoCal into bowl and rub in butter with fingertips. Add a pinch of poultry seasoning, dried rosemary or rubbed sage, if desired.
3. Dissolve bouillon granules in the 5 mL water. Sprinkle bouillon over top tart crust dough and mix until it becomes a stiff, crumbly pastry dough. Save 5 grams of the pastry dough for the tart topping.
4. Line tartlet tin with your crust dough (minus the 5 grams saved for the top), using your fingers to press the pastry into the edges.
5. In a bowl, mix together cream cheese, egg, olive oil, and turkey. Pour the mixture into the crust.
6. Season with salt and pepper, and top with remaining 5 g of crust dough. Add another pinch of dried rosemary on top.
7. Place on oven tray and cook for 10-15 minutes. Allow to cool before removing from tart pan.

Nutrition Information (for entire recipe):

Carbohydrate: 4.32
Calories: 424
Fat: 9.18
Protein: 9.18
Ratio: 3.05:1

Mashed Fauxtatoes (3:1)

Ingredients:faux
107 g Cauliflower, cooked
10 g KetoCal® 4:1 LQ Unflavored 
14 g Butter

Directions:
1. In a small saucepan, heat KetoCal 4:1 LQ with butter and cooked cauliflower until warm.
2. Puree mixture in a blender or food processor until smooth.
3. Season with salt and pepper.

Nutrition Information (for entire recipe):
Carbohydrate: 2.01
Calories: 138
Fat: 2.4
Protein: 2.4
Ratio: 3.02:1

Thanksgiving Green Bean Casserole (3:1)gbc

Ingredients:
13 g KetoCal 3:1 Powder
28 g Cream, 36%
54 g Green Beans – cooked
16 g Butter
34 g Sour Cream – cultured (not low-fat)
12 g Durkee French’s fried onions

Directions:
1. Melt butter; Mix with KetoCal 3:1 powder, sour cream, and cream.
2. Stir in green beans.
3. Pour mixture in a ramekin or a small baking dish. Season with salt & pepper.
4. Top with fried onion (if desired, save some of the onions to place on top after baking for a crunchy texture).
5. Bake in 350 °F for 15 minutes.

Nutrition Information (for entire recipe):
Carbohydrate: 10.44
Calories: 462
Fat: 4.41
Protein: 4.41
Ratio: 3.01:1

Tube-Feeding and the Ketogenic Diet

feeding_tubes_bannerThe ketogenic diet can be much easier to manage when your child is tube-fed since the diet can be given with a special ketogenic formula. In addition, research suggests that an all-liquid ketogenic diet may even be more effective, perhaps because there is less room for errors with measuring foods[1].

Tube-feeding can be given in a variety of ways. Whether given via a bolus, syringe, or feeding pump, the ketogenic diet can easily be given with a feeding tube. Ideally, the feeding schedule should be similar to the way your child was previously fed, just with the new formula.

As usual, the ketogenic diet will be started in the hospital under close medical supervision. You will work with your doctor and dietitian to determine the appropriate ketogenic diet ratio and amount of formula you should give to your child daily. Oftentimes your child will be gradually transitioned from their previous formula to the new ketogenic formula over a period of a few days. While in the hospital for diet initiation, your ketogenic diet health care team will teach you everything you need to know about preparing, administering, and managing your child’s new ketogenic diet when you go home.

KetoCal is a nutritionally-complete ketogenic formula that can be used to provide the ketogenic diet for tube-fed children. It comes in a 3:1 or 4:1 ratio, and as a ready-to-feed liquid or easy-to-prepare powder. KetoCal is milk-based so it may not be appropriate for children who have milk protein allergies. However, your dietitian can put together a modular ketogenic tube-feed with a variety of ingredients that are safe for your child’s food allergies. The KetoCal 4:1 powder and KetoCal 4:1 LQ are both low in lactose, so they are usually okay for children with lactose intolerance.

Tips for Tube-Feeding KetoCal:

  • KetoCal LQ Unflavored is the ideal formula for tube-fed children because it is ready-to-feed. No need to mix! Just shake and administer the formula! In some cases, your child’s dietitian may recommend adding extra water or other ingredients to the formula, so be sure to follow these instructions carefully.
  • If your child is using the KetoCal powder, be sure to mix your formula per the instructions given by your child’s dietitian. For best results, be sure to mix with the correct temperature of water to allow the formula to dissolve fully. It is recommended to use water between 113-122 °F. For more information and tips on mixing KetoCal powder, check out our KetoCal mixing video on YouTube!
  • Once the powdered formula is mixed, store in a closed container in the refrigerator and use within 24 hours. Discard after 24 hours and mix a new batch. Be sure to mix or agitate the formula just prior to feeding since some settling may occur.
  • Once the KetoCal 4:1 LQ has been opened, it can be stored in a closed container in the refrigerator for up to 24 hours.
  • For both the KetoCal Powder and KetoCal LQ, the recommended hang time is 4 hours. The hang time refers to the amount of time the formula can safely remain at room temperature after preparation.
  • Some settling of Ketocal may occur in the feeding bag which is a normal feature of the product. This settling should not block the feeding tube, however, it is recommended that the tube is flushed with water between each feed. It may also help to occasionally agitate the feeding bag.

If you have any questions or concerns, contact your child’s health care team for assistance. For questions about KetoCal, you can also call our Nutrition Services support line at 1-800-365-7354, Option 2.

For assistance with getting insurance reimbursement for KetoCal, contact our Nutricia Product Coverage Navigator program.

Ellen

[1] Kossoff EH, McGrogan JR, Freeman JM. Benefits of an all-liquid ketogenic diet. Epilepsia. 2004 Sep;45(9):1163.