Ketogenic diet therapies. Pilar Gennaro ARNP, CNRN

Transcription

1 Ketogenic diet therapies Pilar Gennaro ARNP, CNRN

2 Definition

3 Definition What is the ketogenic diet? It is a high fat, very low carbohydrate, adequate protein diet,used as a non drug treatment for epilepsy. The high ratio of fats induces and maintains ketosis which results in an antiepileptic effect

4 History HIPPOCRATES fasting 550 BC GEYELIN ketosis 1921 IN New York WILDER High fat diet 1921 Mayo Classic Ketogenic diet HUTTENLOCHER MCT oil diet NBC Dateline 1994 Back to classic ketogenic diet

5 The Charlie Foundation 1994

6 First do no harm

7 Charlie now

8 Classic Ketogenic diet back 1994 MCT oil LGID Low Glycemic Index Diet 2002 Dr. Eric Kossof M.A.D Modified Atkins Diet History

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10 Which diet is most effective Classic Ketogenic diet

11 Metabolic pathways are shifted in the brain: Function - reduction in glycolysis - increase in beta-oxidation of fatty acids - increase in ketone bodies - activation of ATP Potassium channels - Increased GABA synthesis - reduced reactive oxygen species generation - increased energy production in brain tissue

12 How does it work? Ketosis is induced by fasting and maintained by a high fat diet. We are mimicking a fasting state. This results in ketones bodies being used as a PRIMARY energy source fro Brain metabolism.

13 Statistics Half the children started on the KD will have at least 50% reduction in seizures within 6 months Half of these will have >90% improvement; including approximately 15% seizure free

14 We use it in Epilepsy Dravet syndrome Infantile Spams Myoclonic -astatic Tuberous Sclerosis ESES Electrical status epilepticus during slow wave sleep LG Lennox Gastaut Ohtahara

15 Neuro metabolic uses Pyruvate dehydrogenase deficiency Glut 1 Glucose Transport Defect

16 Candidacy Social issues Educational level Any possibility of lipemic aspiration? Referral to keto team

17 Classic ketogenic diet Classic Ketogenic Diet Admission 87 to 90% of calories come from fat Strict measuring, weighing of food Close follow up specially with the youngest children

18 LGID

19 LGID Low Glycemic Index Diet admission not needed best for older children Allow the most carbohydrates 60 grams of low glycemic carbohydrates a day Liberal calories and protein Household measurements -cups LGID

20 LGID Most recent study after 6 months, 34% had a >90% reduction, 20% had 50-90% reduction, 20% had <50 % reduction and 26% had no change or increase in seizures. Although the LGIT is less restrictive than the ketogenic diet, about 1 4 of families who have used this treatment report that it is too difficult to follow long-term.

21 M.A.D

22 M.A.D. Modified Atkins Diet Admission not needed Best for older children Calories not controlled 10 to 15 grams of carbohydrates for children 20 to 30 grams of carbohydrates teens/adults Liberal calories and protein minimum of 2 tbps/4 oz heavy whipping cream/meal Household measurements- cups Carb free Vitamin- mineral supplementation M.A.D

23 MCT oil

24 MCT oil Diet MCT oil Medium Chain Diet 60% of the calories come from MCT oil No need for hospitalization Calories are controlled 40 to 60 grams of carbohydrate Often discontinued due to diarrhea and emesis We used MCT oil as add on any of the 3 other diets to enhance ketosis and treat constipation.

25 Education, Education, Education! Verbal written pamphlets What is the diet Why we hospitalize What do we do in the hospital What to expect What to bring scale, cooler Special diets- Kosher, blenderized, breast milk Education

26 CK Calculations Parameters for Calculation Diet ratio Calories Age Ratio Age Cal/kilo Up to 2yrs 3:1 up to 18 mo 75 cal/kilo 2-12 yrs 4:1 18mo 3 yr 70 cal/kilo 13 yrs 3:1 4 to 6 yr 65 cal/kilo 7 to 8 yrs 60 cal/kilo 9 to 10 yrs 55 cal/kilo > 11 yr 40 cal/kilo

27 Videofluorscopy if at risk of aspiration - Possible GT placement - Microaspiration of fats is very dangerous Initiation of CK

28 Ideal weight for Height Starting the CK is best to be at ideal weight for height If over weight it takes a while to achieve high levels of BHB If underweight we have to start at a lower ratio say 1:1 and event then they can become acidotic and hypoglycemia

29 Initiation of Ketogenic Diet Admission to hospital No longer after a period of fasting Upon admission need to change all meds to tablet/capsule forms Lab studies upon admission, pre diet: CBC BMP Lipids (fasting) Vitamin D Selenium EKG Renal ultrasound

30 Initiation CK Baseline lab work blood and urine as well as renal ultrasound and an EKG Glucostix every 6 hours Blood work daily to determine if the Electrolytes, glucose and CO2 are close to normal Monitor meal intake Monitor fluid intake Check urine ketones Monitor seizure frequency and duration Education

31 Protocol ORAL Started at goal calories and ratio Light carbohydrate meal before admission 3 meals and 1-2 snacks Source of fat Butter, mayo, oil, dressings Heavy whipping cream Source of CHO Only from vegetables/fruits, no starches Source of protein All

32 TUBE FEEDING Ketocal/Ketovie 4:1 Liquid (+ water + modulars) Beneprotein, oil, sugar RCF (+ modulars) Start at half strength half home formula + half Ketogenic formula Advanced as tolerated to ¾ strength and full strength May be daily or q 2-3 feedings GT Protocol

33 TPN TPN Total Parenteral Nutrition Used in PICU Pentobarb coma due to status Decreased gut motility Start trophic enteral feeds via Ketogenic formula when able Composition: Dextrose 0 to 2.5% AA 0.8-1g/kg IL 3 g/kg (soy bean oil, powdered egg yolk) and glycerin ( sugar alcohol) and water Carnitine Monitor Blood glucose: goal of TRIG closely: below 400

34 Monitoring ACIDOSIS/Hypoglycemia Symptoms: CO2<16 + Blood sugar <40-50 Lethargy Vomiting () STAT P8 Correct by giving 3oz juice Causes: Calories too restricted Ratio too high Not eating 100% meals, not drinking 100% fluids Pt is very thin/underweight Too much ketones produced too quickly

35 Monitoring Betahydroxybutaric Acid level (BHB) Ketone Body Used to test the level of ketosis in the blood Best indicator of ketosis (vs. urine ketones) Ideal level = 4-8mmol/L If < ideal: increase ratio (more strict=higher acidosis) If > ideal: too acidotic and must decrease ratio. If not good seizure control, stop diet.

36 Nursing Roles Obtaining current/accurate weight and height Diet calculations based on IBW/HT Changing all medications to CHO/sugar free/ tablets NO med flushes with Pedialyte/Enfalyte NO Dextrose in IVF I STAT/BMP if Glucose below 40 or CO2 16 or less or symptomatic -treat

37 Calendars

38 Discharge from Hospital Education, Education Education!! Discharge with written information Sick day diet How to treat signs and symptoms of hypoglycemia and acidosis What to do if your child is seen in the Emergency room All medications should be in tablet or powder form. NO LIQUID NO CHEWABLE dose forms Please

39 Supplements needed Carnitine ( amino acid derivate transports fatty acids into cells for energy production Calcium and Vitamin D, keto diet low in this Potassium Citrate to alkalinize urine and help prevent kidney stones Multi vitamins and minerals Sometimes salt as the diet is low in sodium Sometimes Baking soda or sodium bicarbonate to aide in preventing low CO2 Table salt as keto diet is low in salt

40 Supplements needed Sometimes selenium ( chronic low seleniumcardiomegaly) If we do not know the carbohydrate content of the supplement do not give it

41 Possible side effects of CK Acidosis Low CO2 vomiting, weakness Hypoglycemia weakness, clamminess, vomiting Constipation/fecaloma DO not let more than 2 days pass without a bowel movement- most common issue Increased liver functions- usually transient Increased in lipids- usually transient if not tx with pancreatic enzymes Kidney stones Growth deceleration

42 Possible side effects of CK Hyponatremia low sodium-salt Hypokalemia Hunger Selenium deficiency can lead to cardiac disease Pancreatitis Electrolyte imbalance Calcium multivitamin Deficiency Certain children at risk for osteoporosis

43 Follow up Monitor weight, Height ideal body weight for height Adjust the diet accordingly Increase ratio or decrease calories Monitor seizure frequency Monitor Bowel movement frequency Monitor urine ketones

44 4 to 6 weeks after discharge When do we follow? First year on ketogenic diet every 3 to 4 months Second year 4 to 6 months In between many s!!!

45 In most of the children we will know if the ketogenic diet is working within 3 months after starting the diet with an adequate level of betahydroxybuterate in the serum

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47 Tools we use Websites: Books Dr. Atkins New Diet Revolution The CalorieKing Calorie, Fat and Carbohydrate Counter

48 Tools we use

49 Products Ketogenic diet formulas Ketocal, ketovie, ketovie cafe Wholesome bread 3.5:1 ratio Cinnamon donuts 3:1 ratio Pizza petites 3:1 ratio

50 Products

51 Products

52 Products

53 Case studies-

54 Prior to keto diet * WT 37.5 kg >> 97% * HT 110 cm 50-75% * Weight for height above 97% * Ideal body weight 18.8 kg (200%) MM On 6/18/12 started at 2.5:1 ratio 700 cal ended up on 1/21/14 On 550cal/day diet with a weight 25.3 kilos 9.46% IBWT for HT 19.8 kilos, total loss 12.2 kilos = lbs since keto was started 75% decrease in seizure frequency

55 J M 19 month old male with static encephalopathy manifested by: * Epilepsy- spasms that are resistant to 2 antiepileptic drugs. He did well on steroids and was seizure free for 2.5months. Patient now on Topamax. * Global developmental delay- Patient is not sitting, has marked hypotonia requiring total assistance with ADL * Cortical blindness * Weight for Height 90% * Passed videofluoroscopy, no aspiration, but had penetration as above ETIOLOGY Unknown at present although he has an SCN1A Variant of unknown etiology

56 Seizure History * onset 2 weeks before his first birthday * semiology head drops and upper body jerking movements * frequency Having clusters 2-3 times a day and at times every other day. * duration episode occurs in seconds, and clusters of these episodes last 1 minute * triggers episodes occur while awake, none during sleep. * hx of status None * rescue meds at home :None JM Medications Wt 4/ kilos * Topamax 25 mg tablets gets 75 mg oral BID = 9.4 mg/kilo/day dec on 4/11 from 84 mg (14 ml) po BID (6mg/ml) started in October mg/kilo/day No change in seizure frequency Acidotic * Melatonin 1 mg at HS started a few months ago. Helps him sleep. Past AED * 2 1/2 months seizure free while on ORAL steroids. *was on Potassium Citrate for treatment for low CO2 for 1 month

57 On 4/11/16 started on ketogenic diet at 3:1 ratio 70 cal/kilo = 880 cal/day and fluids at 41 oz per day On 4/15/16 Discharged on 1.5:1 ratio ( due to acidosis) 880 cal/day 41 oz fluids per day ON 5/12/16 dec calories by 60 On 7/7/16 seizures dec by 75% 1.5:1 810 cal/day and 50 ounces of fluids a day Seizure free since September 1 st 2016!

58 J M Laboratory * 4/12/16 Glucostix 72, 87; BMP CO2 16, glucose 62 WBC 13 H/H 12.9/37.1 Plat 491; LFT's normal * 4/11/16 Glucostix 91; BMP normal CO2 20 Glucose 89 Urinalysis ph 7.5 S Gravity 1,004 Ketones negative; urine calcium 3; creatinine 18.9; ratio 0.15 * 2/17/16 BMP CO2 16, glucose 101 * 2/6/16 BMP Co2 15, LFT's normal lipids normal except for triglycerides 236, VLDL high 47 Vitamin D 25 OH 31.5 ; Selenium normal 156 * 2/5/16 urine calcium 4; creatinine 15.8 ratio normal at 0.2

59 Medications/Treatment wt 13.6 kilos AED * Topamax OFF TOPAMAX * Ketogenic diet 1.5:1 820 cal ( 56 cal/kilo present wt) Diet started 4/11/16 * Levocarnitine 330mg 1 tab crushed BID = 48.5 mg/kilo/day * Nano VM 2 scoops BID ( 1-3 yrs) * Na Bicarbonate 1/8th teaspoon BID 1mEq/kilo/day * K citrate Powder crystals ( 30 meq per day) 15 ml TID * Miralax grams po c 4 ounces of water po daily

60 Last visit 1/19/18 Weight: * 1/19/ kilos 54.71% * 4/11/ kilos 99% * 3/17/ kg 99.87% Height * 1/19/ cm 59.25% measured x 2 * 4/11/16 89 cm 95% * 3/17/16 90 cm 98.96% BMI * 1/17/ kg/m2 48.2% Ideal body weight for Height * 1/19/ kilos

61 She is a BELIEVER! J M

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65 Other indications?

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68 Weight loss - Migraines ADHD ASD Autism Alzheimer s disease Parkinson s disease Other Indications

69 Polycystic Ovary Syndrome Diabetes Mellitus type 2 ALS Amyotrophic lateral sclerosis Brain tumors Other indications

70 Brain tumor clinical trials 1 RecruitingKetogenic Diet in Children With Malignant or Recurrent/Refractory Brain TumorBrain Tumors Other: Ketogenic Diet Nicklaus Children's Hospital Miami, Florida, United States

71 Brain Tumors clinical trials Pilot Study of a Metabolic Nutritional Therapy for the Management of Primary Brain TumorsGlioblastoma Other: Energy restricted Ketogenic Diet (ERKD) (Metabolic Nutritional Therapy) Michigan State University/Sparrow Hospital East Lansing, Michigan, United States

72 Brain tumors clinical trials Feasibility Study of Modified Atkins Ketogenic Diet in the Treatment of Newly Diagnosed Malignant GliomaGlioblastoma Other: Modified Atkins Ketogenic Diet UC Health Cincinnati, Ohio, United States

73 Other indications