ketogenic diet presentation
DESCRIPTION
Clinical Case Study PresentationTRANSCRIPT
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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
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Introduction
Why the
KD?
May 2102
GBM
Jury is out
CHOP
Powerful
Dedication
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The Plan
Wrap Up
Presentation of Patient
Medical Nutrition Therapy
Medical Treatment
Epilepsy Anatomy & Physiology
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The Brain
Anatomy & Physiology
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Neuron Synapse
Anatomy & Physiology
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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
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Types of Seizures
Focal seizures
Simple Complex
Generalized seizures
Tonic-clonic Absence Clonic Myoclonic Tonic Atonic
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Definition
History
Etiology
Diagnosis
Medical Treatment
Non-Surgical
Surgical
Epilepsy
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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.
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Epilepsy
Migraine
Stroke
Alzheimers
2.2 million
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What did
Alexander the Great, Julius Caesar, Joan of Arc, Hndel,
Flaubert, van Gogh, and Gershwin
have in common?
POP QUIZ
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History
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History
1516-1520, Italy The Transfiguration by Raphael
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History
Stigma
Misconception
Unpredictable
Helplessness
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Etiology
Genetic
Structural/Metabolic
Unknown
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Diagnosis
Clinic Visit
Medical History
Seizure History
Neurological Exam
Laboratory Results
Imaging (EEG, MRI, CT)
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Diagnosis
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Medical Intervention
Dietary Treatment
Surgical Resection Disconnection Stimulation
Seizure Medications
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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.
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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
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SURGICAL TREATMENT
Not helped by medications Clear focal point or lesion Success rates vs. risks
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SURGICAL
Resection
Removal of seizure focus
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SURGICAL
Disconnection
Disrupts seizure pathway
Corpus callostomy
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SURGICAL
Hemispherectomy
Last resort
< 13 years old
Intense rehab
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SURGICAL
Stimulation
Source: The Mayo Clinic. http://www.mayoclinic.org/tests-procedures/vagus-nerve-stimulation/multimedia/vagus-nerve-stimulation/img-20006852
Vagus Nerve Stimulator
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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.
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Fasting
Ketogenesis Pathway
NON-SURGICAL Dietary Treatment
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Fasting
Metabolic Changes During Starvation
NON-SURGICAL Dietary Treatment
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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.
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Medical Nutrition Therapy
Background
Nutrition Care Process
Assessment
Diagnosis
Intervention
Monitoring & Evaluation
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MNT Background
Dr. Russell Wilder
1921 Effects of fasting without starvation Ketogenic 50-70% experience >50% seizure reduction Popular in 1920s and 1930s
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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
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MNT Background
Charlie Abrahams Dateline 1994 1971: 2-8 articles per year Now: 40 articles per year The Charlie Foundation
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MNT Nutrition Care Process
Assessment Medical History
Diet History
Ketogenic Diet Selection
Contraindications
Growth Parameters
Energy, Protein, Fluid Needs
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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
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MNT Nutrition Care Process
Diagnosis Inadequate oral intake
Inadequate fat intake
Excessive carbohydrate intake
Food- and nutrition-related knowledge deficit
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MNT Nutrition Care Process
Intervention Inpatient Admission
Advancement of ratio
Education
All-liquid
Parenteral administration
Supplements
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MNT Nutrition Care Process
Intervention
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MNT Nutrition Care Process
Intervention
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MNT Nutrition Care Process
Intervention
GOALS
Seizure freedom/reduction Age-appropriate weight gain Linear growth
Increased cognition
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MNT Nutrition Care Process
Monitoring & Evaluation
Ketosis/Euglycemia
Other Labs
Food-Drug Interactions
Close Outpatient Follow-Up
Common Challenges
Discontinuation
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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
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MNT Nutrition Care Process
FAQs
Do kids get fat?
High cholesterol?
How strict?
Life long?
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Meet
SP
Presentation of Patient
Admitted to CHOP for Ketogenic Diet initiation on December 8, 2014
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SP Assessment
Social History
14 3/12 year old male
Sociable
Lives at home with mother, father, younger
brother
Special life skills class
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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
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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
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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
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SP Assessment
Weight
67.8 kg
75-90th %ile
120% IBW
Borderline obese
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SP Assessment
Height
170.5 cm
75th %ile
102% standard height for age
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SP Assessment
BMI
23.32 kg/m2
85-90th %ile
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SP Assessment
Head Circumference
56 cm
50-98th %ile
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SP Diagnosis
PES Statement
Food- and nutrition-
related knowledge
deficit
related to ketogenic
diet initiation
as evidenced
by need for diet
instruction.
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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
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SP Monitoring & Evaluation
Hospital Course
Tolerated advance
No N/V
Complaints of hunger resolved
Music therapy &
video games
Progression of ketosis
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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)
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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
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M E R Y L
Summary
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Looking Ahead
Other applications
Asia
Hindmilk?
Keto pill?
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Comments
Dr. Christina Bergqvist
Separation from child
Mobile app
KD room
service
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Cagla Fenton, RD, LDN
Sue Groveman, MS, RD, LDN
Donna DiVito, RD, LDN, CNSC
Avi Weiner, DMD
Mollie & Ron Makar
Acknowledgements
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Acknowledgements
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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
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Questions?