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S T E F A N I E W E I N E R
M A R C H 6 , 2 0 1 5
Treating Intractable Pediatric Epilepsy With the Ketogenic Diet
Introduction
Why the
KD?
May 2102
GBM
Jury is out
CHOP
Powerful
Dedication
The Plan
Wrap Up
Presentation of Patient
Medical Nutrition Therapy
Medical Treatment
Epilepsy Anatomy & Physiology
The Brain
Anatomy & Physiology
Neuron Synapse
Anatomy & Physiology
Seizure
Anatomy & Physiology
physical findings or changes in behavior that occur after an episode of abnormal electrical activity in the brain
Source: MedLine Plus http://www.nlm.nih.gov/medlineplus/ency/article/003200.htm
Types of Seizures
Focal seizures
Simple Complex
Generalized seizures
Tonic-clonic Absence Clonic Myoclonic Tonic Atonic
Definition
History
Etiology
Diagnosis
Medical Treatment
Non-Surgical
Surgical
Epilepsy
Definition
2+ unprovoked seizures occurring 24+ hours apart
Diagnosis of an epilepsy syndrome
Resolved if: Past applicable age of
age-dependent syndrome
Seizure-free for 10 years, with no seizure medicines for the last 5 years.
Fisher RS. A practical definition of epilepsy. Epilepsia. 2014; 55:475-482.
Epilepsy
Migraine
Stroke
Alzheimers
2.2 million
What did
Alexander the Great, Julius Caesar, Joan of Arc, Hndel,
Flaubert, van Gogh, and Gershwin
have in common?
POP QUIZ
History
History
1516-1520, Italy The Transfiguration by Raphael
History
Stigma
Misconception
Unpredictable
Helplessness
Etiology
Genetic
Structural/Metabolic
Unknown
Diagnosis
Clinic Visit
Medical History
Seizure History
Neurological Exam
Laboratory Results
Imaging (EEG, MRI, CT)
Diagnosis
Medical Intervention
Dietary Treatment
Surgical Resection Disconnection Stimulation
Seizure Medications
First developed 150 years ago
NON-SURGICAL Seizure Medications
Source: Brodie MJ. Antiepileptic drug therapy the story so far. Seizure. 2010; 19(10):650-655.
NON-SURGICAL Seizure Medications
Carbamazepine
Clobazam
Clonazepam
Diazepam
Divalproex Sodium
Felbamate
Gabapentin
Lamotrigine
Levitracetam
Lorazepam
Phenobarbital
Phenytoin
Pregbalin
Rufinamide
Topiramate
Valproic Acid
Vigabatrin
Zonisamide
First line of treatment
Mechanism: neurotransmitters
Side effects: weight, appetite, dizziness, depression, sedation
Careful choice by MD
% Seizure Control
1st
2nd
3rd+
Intractable
SURGICAL TREATMENT
Not helped by medications Clear focal point or lesion Success rates vs. risks
SURGICAL
Resection
Removal of seizure focus
SURGICAL
Disconnection
Disrupts seizure pathway
Corpus callostomy
SURGICAL
Hemispherectomy
Last resort
< 13 years old
Intense rehab
SURGICAL
Stimulation
Source: The Mayo Clinic. http://www.mayoclinic.org/tests-procedures/vagus-nerve-stimulation/multimedia/vagus-nerve-stimulation/img-20006852
Vagus Nerve Stimulator
Fasting
Metabolic Changes During Starvation
NON-SURGICAL Dietary Treatment
Source: Murray RK, Granner DK, Mayes PA, Rodwell VW, eds. Harpers Biochemistry. Norwalk, CT: Appleton & Lange, 1996.
Fasting
Ketogenesis Pathway
NON-SURGICAL Dietary Treatment
Fasting
Metabolic Changes During Starvation
NON-SURGICAL Dietary Treatment
Fasting
Metabolic Changes During Starvation
NON-SURGICAL Dietary Treatment
Source: Murray RK, Granner DK, Mayes PA, Rodwell VW, eds. Harpers Biochemistry. Norwalk, CT: Appleton & Lange, 1996.
Medical Nutrition Therapy
Background
Nutrition Care Process
Assessment
Diagnosis
Intervention
Monitoring & Evaluation
MNT Background
Dr. Russell Wilder
1921 Effects of fasting without starvation Ketogenic 50-70% experience >50% seizure reduction Popular in 1920s and 1930s
MNT Background
The Ketogenic Diet
Ratio by weight Total grams of fat : Total grams of protein + CHO
4:1 ratio: 90% kcal as fat Total CHO 10 grams per day
0%
20%
40%
60%
80%
100%
Typical Ketogenic
30
90 15
7
55
3
CHO
Protein
Fat
MNT Background
Charlie Abrahams Dateline 1994 1971: 2-8 articles per year Now: 40 articles per year The Charlie Foundation
MNT Nutrition Care Process
Assessment Medical History
Diet History
Ketogenic Diet Selection
Contraindications
Growth Parameters
Energy, Protein, Fluid Needs
MNT Nutrition Care Process
Assessment
Estimations
WHO Equation, REE x Activity Factor
Age Males Females
0-3 60.9W 54 61.0W 51
3-10 22.7W + 495 22.5W + 499
10-18 17.5W + 651 12.2W + 746
18-30 15.3W + 679 14.7W + 496
Activity Factor
Indication
1.3 Well-nourished child at bedrest with mild/moderate stress
1.5 Normally active with mild/moderate stress
Inactive with severe stress Minimal activity requiring catch-up
growth
1.7 Active requiring catch-up growth Active with severe stress
RDA
Age Kcal/kg gm protein/kg
0-6 months 108 1.52 (AI)
7-12 months 98 1.2
1-3 years 102 1.05
4-6 years 90 0.95
7-10 years 70 0.95
11-14 years 55 0.85 (14-18)
15-18 years 45 0.85
11-14 years 47 0.85 (14-18)
15-18 years 40 0.85
Fluid Requirements
1-10 kg 100 mL/kg
10-20 kg 1000 mL + 50 mL each kg over 10 kg
>20 kg 1500 mL + 20 mL each kg over 20 kg
MNT Nutrition Care Process
Diagnosis Inadequate oral intake
Inadequate fat intake
Excessive carbohydrate intake
Food- and nutrition-related knowledge deficit
MNT Nutrition Care Process
Intervention Inpatient Admission
Advancement of ratio
Education
All-liquid
Parenteral administration
Supplements
MNT Nutrition Care Process
Intervention
MNT Nutrition Care Process
Intervention
MNT Nutrition Care Process
Intervention
GOALS
Seizure freedom/reduction Age-appropriate weight gain Linear growth
Increased cognition
MNT Nutrition Care Process
Monitoring & Evaluation
Ketosis/Euglycemia
Other Labs
Food-Drug Interactions
Close Outpatient Follow-Up
Common Challenges
Discontinuation
MNT Nutrition Care Process
Monitoring & Evaluation
Side Effects
Short Term Long Term
Hypoglycemia Hyperlipidemia
Acidosis Heart disease
Dehydration Kidney stones
Nausea/Emesis Reflux
Diarrhea/Constipation Linear growth failure
Lethargy Osteoporosis
Anorexia Vitamin deficiency
Weight loss Pancreatitis
MNT Nutrition Care Process
FAQs
Do kids get fat?
High cholesterol?
How strict?
Life long?
Meet
SP
Presentation of Patient
Admitted to CHOP for Ketogenic Diet initiation on December 8, 2014
SP Assessment
Social History
14 3/12 year old male
Sociable
Lives at home with mother, father, younger
brother
Special life skills class
SP Assessment
Medical History
Followed by CHOP Neurology since 2005
Myoclonic jerks in AM, generalized seizure
every 2 weeks
MRI + EEG + cognitive assessment
Generalized intractable epilepsy and mental
retardation
SP Assessment
Diet History
3-day diet record
Creamy, fatty foods
No rice, pasta
Chocolate milk
Needs
REE = 1838 kcal
AF: 1.2-1.4
2205-2573 kcals per
day
Protein: 0.85
g/kg/day
Fluid: 1842-2456 mL/day
SP Assessment
Labs Medications
Klonopin
Depakote
Topamax
Diastat
Tranxene
Glucose mg/dL 90
Cholesterol mg/dL 131
Triglyceride mg/dL 114
HDL Cholesterol
mg/dL
32 (L)
LDL-Cholesterol
mg/dL
76
Betahydroxybutyrate
mmol/L
SP Assessment
Weight
67.8 kg
75-90th %ile
120% IBW
Borderline obese
SP Assessment
Height
170.5 cm
75th %ile
102% standard height for age
SP Assessment
BMI
23.32 kg/m2
85-90th %ile
SP Assessment
Head Circumference
56 cm
50-98th %ile
SP Diagnosis
PES Statement
Food- and nutrition-
related knowledge
deficit
related to ketogenic
diet initiation
as evidenced
by need for diet
instruction.
SP Intervention
Meal plan
2456 kcal
Protein: 44.7 gm
CHO: 16.3 gm
Classes
Weighing Food
Exchanges
Keto Kitchen
Cooking Demos
U Arts Interns
SP Monitoring & Evaluation
Hospital Course
Tolerated advance
No N/V
Complaints of hunger resolved
Music therapy &
video games
Progression of ketosis
Ref.
Range
Dec 9
21:40
Dec 10
01:30
Dec 10
09:00
Dec 10
12:08
Dec 10
17:50
Dec 10
22:15
Dec 11
02:11
Dec 11
06:10
Dec 11
12:40
Dec 11
17:42
Dec 12
06:42
Glucose 70-106
mg/d
92 105 80 82 86 91 68 (L) 82 94 87
BHB 0.0-0.3
mmol/L
0.38 (H)
Follow Up Jan 22, 2015
No seizures since 1/6/15
- 1.3 kg (2%) in 6 weeks
No linear growth
Struggle to eat at school
alert and interactive
M E R Y L
Summary
Looking Ahead
Other applications
Asia
Hindmilk?
Keto pill?
Comments
Dr. Christina Bergqvist
Separation from child
Mobile app
KD room
service
Cagla Fenton, RD, LDN
Sue Groveman, MS, RD, LDN
Donna DiVito, RD, LDN, CNSC
Avi Weiner, DMD
Mollie & Ron Makar
Acknowledgements
Acknowledgements
References
Herculano-Houzel S. The human brain in numbers: a linearly scaled-up primate brain. Frontiers in Human Neuroscience. 2009;3:1-11.
National Research Council. Epilepsy Across the Spectrum: Promoting Health and Understanding. Washington, DC: The National Academies Press, 2012.
Fisher RS, Boas WvE, Blume W, et al. Epileptic Seizures and Epilepsy: Definitions Proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE). Epilepsia. 2005; 46:470-472.
Kwan P, Brodie M. Early identification of refractory epilepsy. The New England Journal Of Medicine [serial online]. February 3, 2000;342(5):314-319. Available from: MEDLINE with Full Text, Ipswich, MA. Accessed January 19, 2015.
Brodie MJ. Antiepileptic drug therapy the story so far. Seizure. 2010; 19(10):650-655 Murray RK, Granner DK, Mayes PA, Rodwell VW, eds. Harpers Biochemistry. Norwalk, CT:
Appleton & Lange, 1996. Morris AA. Cerebral ketone body metabolism. J Inherit Metab Dis. 2005;28:109-121. Hirtz D, Thurman DJ, Gwinn-Hardy K, et al. How common at the common neurologic
disorders? Neurology. 2007;68(5):326-337. Wheless JW. History of the ketogenic diet. Epilepsia. 2008; (Suppl. 8):3-5. doi: 10.1111/j.1528-
1167.2008.01821.x
Plogsted S. The Ketogenic Diet. ICAN. December 2010;2:370-376. Zupec-Kania B, Neal E, Schultz R. An Update on Diet in Clinical Practice. Journal of Child Neurology.
Aug 2013;28:1015-1026. doi: 10.1177/0883073813487597
Questions?