UH Standard of Practice for Ketogenic Diet

Initiation of the Diet

Before consideration or admission for the ketogenic diet any infant or child with possible feeding difficulties should be evaluated by the OT/Speech Feeding team. This would include an infant/child that has coughing, choking, or gagging with liquids or solids, an inability to advance feeds and textures, food refusal, frequent respiratory illness, and poor growth. If a Modified Barium Swallow is recommended by the team it must be done before proceeding with the diet.

Neurology or Epilepsy service asks the dietitian to call parents interested in the ketogenic diet for their child. The family is contacted to provide overview of the diet, discuss hospitalization and answer questions.

Required Information

If parents wish to pursue the diet the RDN will collect the following information by phone:

1. Diet history
2. List of food preferences detailed likes and dislikes are needed for meal planning
3. Food allergies
   Note: Multiple food allergies may prevent use of the diet
4. Chewing or swallowing difficulties, food texture
5. Medical history
   Note: Patients who have had a cholecystectomy can not do the diet. Also, patients with delayed gastric emptying will likely have difficulty tolerating the diet, further discussion with the PMD or GI specialist may be necessary before attempting the diet.
6. Current height and weight
7. List of medications, how administered, note patient’s ability to swallow pills/capsules.
   Note: All medication must be sugar-free, cannot use chewable tablets or elixirs. If a medication is not available in a sugar-free form the carbohydrate content must be taken into account when calculating the diet.

Laboratory Evaluation

(If not done recently as outpatient recommend on admission)

• Complete blood count with differential
• Hepatic function panel
• Renal function panel
• Magnesium
• Fasting lipid profile
• Urinalysis
• Calcium, urine spot
• Vitamin D 25 Hydroxy

Family Instruction Prior to Admission

(This is by phone by the RDN)

1. Review the Charlie Foundation website, charliefoundation.org
2. Purchase the book The Ketogenic Diet and Modified Atkins Diet: Treatment for Epilepsy and Other Disorders by Kossoff, Turner, Doerrer, Cervenka, and Henry, 6th edition, 2016
   Optional but encouraged.
3. Have the child try heavy whipping cream.
4. Try administering medications with butter to determine child’s acceptance.
5. If possible have prescriptions changed to sugar-free (this is especially true for any refills and new prescriptions).
6. Purchase a digital gram scale. These are available at Target; Bed, Bath and Beyond; The Charlie Foundation or on the internet. A scale that measures 1000 grams with 0.1 gram precision is recommended.
7. For a new patient considering the diet, the dietitian can make arrangements for samples of Ketocal 4:1 or 3:1 from Nutricia or Ketovie from Cambrooke Foods. This is for patients who would be taking the supplement by mouth.
8. The diet should be trialed for at least three months before determining efficacy.
9. Toothpaste: Tom’s Natural or Arm & Hammer are recommended. Children’s toothpaste with sorbitol cannot be used.

Current Practice for Initiation of the Diet

1. Except for special circumstance patients are to be admitted on Monday. Typical length of stay is four to five days. Stay is usually extended if there are problems with tolerance of the diet or hypoglycemia.
2. Parents are encouraged to omit sugars and sugary beverages the weekend prior to admission. Whenever possible the patient should not be fed after 5 p.m. the night before admission. Stress that the patient continue to drink water or diet beverages to prevent dehydration. Medications should be given at usual times. Patients on tube feeds are instructed to maintain usual schedule until admitted.
3. Fluids are not restricted. Base fluids on maintenance needs.
4. If an IV becomes necessary it should not contain any dextrose, use normal saline with added potassium.
5. Inform the resident of the need to change any medications to a sugar-free form. Any new medication must also be sugar-free.
6. Ketones are checked at each void. Use cotton balls in the diaper to collect the urine. I/O should be recorded daily.
7. D-stix checked every four hours if under 2 years old. Should be checked every six hours if over 2 years old. Treat a blood glucose of under 40 mg/dL or if child is symptomatic, treat with 15ml juice or formula. If NPO treat with 25ml D10%, recheck in 30 minutes. Retreat as necessary until blood glucose is over 50 mg/dl and child without symptoms. Notify HO of hypoglycemia.
8. Infants and children under 5 years of age are not generally fasted. They start the diet as soon as meals are delivered to the room. There are typically three meals per day.
9. Older children will start their meals late afternoon on day of admission. If last meal was 5 p.m. the day prior to admission this will be a 24-hour fast.
10. The meals are started at full calorie and ratio goal. Three to four meals are delivered at a time. The formula room prepares and delivers any special formula.
11. Any infant or child fed by bottle or tube feeding will be gradually transitioned to the ketogenic formula. Start with ¼ ketogenic formula plus ¾ of their usual formula for 6 to 10 hours, then ½ and ½ etc., until on full feeds of the ketogenic formula.
12. Goal is ketones are greater than 3+.

Food Service

1. As soon as the admission date is known, the dietitian will contact the Patient Services Manager to make sure heavy cream, canola oil or any other special product is ordered.
2. Special formulas are prepared in the formula room. A 24-hour supply of formula is provided daily.
3. The RDN provides a copy of the meals to the Patient Services Manager, meals are then measured and then delivered to patient’s room.
   Four meals are prepared at a time and stored in the patient’s refrigerator. Each food is labeled with the item, gram weight, and date. Each meal is placed in a separate bag. Plastic ware, salt, pepper, bottled water and diet beverages are sent in bulk.
   Note: Syrofoam containers should not be used. Use only clear plastic containers. The formula powders and oils cling to Styrofoam.

Calculating the Diet

1. Use ratio of 4:1 children older than 1 year old. Use ratio of 3:1 for infants.
2. The dietitian will use the ketodietcalculator program to calculate protein and calories. Calories on based on patient’s current intake or tube feeding.
3. Provide maintenance fluid needs.
4. In general, most tube feedings are made 1cal/ml. Formula can be concentrated to higher levels or diluted if needed.
5. Use the vitamin/mineral function on the ketodietcalculator to determine supplementation for home going.
   For patients eating regular foods, a multivitamin with minerals and calcium with Vitamin D are recommended
   For patients on special formula products, additional vitamin or mineral supplements may not be necessary since these products are nutritionally complete.
6. Sodium and potassium can be supplemented with salt and salt substitute as needed after discharge.
   Note: For inpatients salt and salt substitute cannot be added to any formula, additional sodium and potassium must be ordered through pharmacy.
7. When planning meals with oil, try to use canola or olive oil.
8. Optional extra supplementation, based on RDN and MD recommendations:
   • Oral citrates (Polycitra K)
   • Laxatives: Miralax, Milk of Magnesia™, Glycerin suppository, Senna tablets.
   • Vitamin D
   • Carnitor
   • MCT oil

Note: Any medication or supplements must be provided as sugar-free. If a medication or supplement is not available sugar-free then the carbohydrate content must be calculated into the diet.

Instructions for Going Home

1. Provide the caregiver with copy of diet calculations, menus, diet handouts, list of vitamin/mineral supplements needed.
2. The following instruction sheets are also given on discharge from the hospital:
   • How do you test your urine for ketones?
   • Ketogenic Diet Guidelines: Scale, Sweeteners, Monitoring, & Follow-up
   • Reading Labels for Sugars
   • Resources
   • Sick Day Guidelines
   • Troubleshooting Problems on the Ketogenic Diet
3. The family is instructed to check for ketones twice a day for the first two weeks, thereafter they will check ketones twice a week. Check once in am before the first meal or tube feeding and once in the late afternoon or evening. Record ketones along with the occurrence of seizures on your seizure calendar. If there is a change in diet or seizure activity ketones may need to be checked more frequently.
4. Patient needs to be weighed weekly, record all weights.
5. In most cases, D-stix are not continued at home. However, some younger children may have problem with hypoglycemia and may require monitoring the first three to five days after discharge. Arrangements can be made for a glucometer, strips and education if blood glucose needs to be monitored at home.
6. The family will contact the inpatient dietitian the first three months on the diet for issues and concerns. At three months the family has usually met with the neurologist to decide if they will continue with the diet. At that point follow-up of the diet is transferred to the outpatient dietitian.
7. Typical length of time on the diet is two years.
8. Based on their comfort and interest parents can be given access to the ketodietcalculator program to create meals and snacks with the dietitian’s supervision.

Treating Hypoglycemia

1. Before altering the diet be sure patient is on full volume of food or tube feeding for two to three days. Younger children may need a longer time to adjust to the diet.
2. Before changing the calories or ratio divide food or formula into greater number of feeds. Run tube feeding overnight.
3. Add bedtime snack.
4. Change ratio.
5. Increase calories.

Follow Up Care

First Three Months

• Follow-up by phone and/or email by the inpatient dietitian.
• During the first two to four weeks after initiation, changes to the diet should be minimal unless there is a significant adjustment problem. The child needs this time frame to adjust to the changes of diet to their metabolism.
• If adjustments are made, only one change should be made at a time. If diet is changed, medications stay the same (and vice versa).
• Schedule three month appointment with Outpatient Dietitian.

Follow-up with Outpatient Dietitian

• At three months, six months, one year and then annually.
• Each visit will include:
  • Assessment of growth.
  • Discussion: growth, any hunger, food refusal, mealtime issues and any necessary diet changes.
  • If calorie increase is determined necessary: a gradual increase in calories for infant and small children should be made. Gradually increase of 25 calories every one week to goal calories.
  • Older children typically do well with increases of 100 calories every one to two weeks to goal calories.
  • Assessment of labs when available.

Laboratory

• Labs should be ordered at each follow-up interval: three months, six months, one year and then annually.
  • Complete blood count with differential
  • Hepatic function panel
  • Renal function panel
  • Magnesium
  • Fasting lipid profile
  • (frequency based on initial results)
  • Urinalysis
  • Calcium, urine spot
  • Vitamin D 25 Hydroxy (frequency based on initial results)
  • Any drug levels as deemed necessary by physician
• Additional labs to consider are:
  • Carnitine
  • Selenium
  • Zinc

Weaning of medications is typically done after six months of being on the diet.

Discontinuation of the Diet

Parents are encouraged to trial the diet initially for at least three months. If the diet is ineffective it can be stopped without any weaning process. If a family chooses to continue the diet for a longer period of time despite no or minimal seizure control, their wishes should be supported.

The diet may be stopped abruptly in an emergency.

Typically, the diet is slowly tapered over one to three months by gradually lowering the ratio from 4:1 to 3:1 to 2:1; then the calories are liberalized. Once ketosis is lost a regular diet can be resumed. The pace of the taper can be altered to the child’s individual need. Vitamin and minerals supplements should continue until a regular diet is taken.

If seizures return the ketogenic diet can be increased back to the previously effective diet ratio.

The Long-term Considerations: Over Two Years on the Diet

Adverse effects of the ketogenic diet need to be considered always in comparison to the benefits to each individual patient.

Patients continuing on the diet beyond two years must continue with the minimum of an annual follow-up with the dietitian, including labs. More frequent visits can be scheduled if needed.

Particular attention must be made to growth, risk for development of kidney stones, and bone health. Obtaining a Dexa scan needs to be considered.