Acute Side Effects of Ketogenic Diets and Issues with Transition CFPC - PowerPoint PPT Presentation

Acute Side Effects of Ketogenic Diets and Issues with Transition

CFPC CoI Templates: Slide 1 – used in Faculty presentation only. Faculty/Presenter Disclosure • Presenters: Alex Printis, RD Helen Lowe, MSc, RD Maria Zak, MN, NP ‐ Paediatrics • Relationships with financial sponsors: – Grants/Research Support: Nothing to disclose – Speakers Bureau/Honoraria: Nothing to disclose – Consulting Fees: Nothing to disclose – Patents : Nothing to disclose – Other: Membership of Matthew’s Friends Canada Medical Advisory Board

CFPC CoI Templates: Slide 2 Disclosure of Financial Support This program has not received financial support • This program has not received in ‐ kind support • • Potential for conflict(s) of interest: – None to disclose

CFPC CoI Templates: Slide 3 Mitigating Potential Bias • None

Objectives • Recognize acute side effects of the ketogenic diet (KD) • Distinguish the management of acute side effects in patients following a KD • Identify how to transition a patient following a KD to adult care

Patient Referred for Ketogenic Diet • 5 y.o. boy with Dravet syndrome; non verbal • Recurrent generalized seizures, occasional myoclonic seizures • Current seizures: 1 – 2 per week • Current meds: valproic acid, clobazam, topiramate, stiripentol • MRI: Normal • EEG: mild slowing of background activity; generalized epileptiform discharges

Diet Assessment • Orally fed but small eater; puree texture • No dysphagia, no aspiration pneumonia • Drinks adequate fluids; safe with thin • Ht and Wt below 3 rd percentile for age PLAN: Initiate a 2.5:1 ratio Classic KD

Food Refusal • Day 1: Pt takes all shakes; drinks well • Day 2: Pt starts to refuse food at full diet – Urine ketones: 8 – 16 mmol/L – Blood glucose (BG): 3.2 – 3.9 mmol/L • Day 3: Pt vomiting – Urine ketones >16 mmol/L – BG: 2.2 mmol/L

Food Refusal: Contributing Factors • Everyone goes into ketosis differently – some faster than others • High ketones suppress appetite; cause nausea

Food Refusal: Management • Treat with juice • If necessary: – Lower diet ratio by 0.5 – Continue to treat with juice as needed – Offer shakes rather than food • Adjust food choices for Pt’s preferences

Food Refusal: Our Patient • Stabilizes on 2:1 ratio – BG: 3.5 – 4.2 mmol/L – Ketones: 8 – 16 mmol/L – Consuming 85% of food SUCCESS!

Hyperlipidemia • Ratio increased to 4:1 over the next 6 months • Eating 95 ‐ 100% • Fasting blood panel: Date Total LDL cholesterol HDL Triglycerides Cholesterol (0.93 ‐ 3.62 cholesterol (<1.70 (3.20 ‐ 4.40 mmol/L) (0.31 ‐ 1.66 mmol/L) mmol/L) mmol/L) Baseline 3.58 1.76 1.35 1.04 6 month 6.50 4.23 1.45 2.35 follow up

Hyperlipidemia: Contributing Factors • Typically normalize within 1 – 2 years • Diet intake: 90% of calories from fat – Pt primarily eating butter, heavy whipping cream, coconut oil, bacon – Low fibre intake • Anthropometrics: – Weight: 10 th Percentile – Height: Remains <3 rd percentile

Hyperlipidemia: Management • Questions to ask: • Blood work done fasting? • Compliant with diet? • Check free carnitine; supplement if low • Adjust diet: • Reduce saturated fats • Replace with liquid oils, avocado, consider MCT oil • Omega ‐ 3 supplementation • Decrease calories

Hyperlipidemia: Management Date Total LDL cholesterol HDL Triglycerides Cholesterol (0.93 ‐ 3.62 cholesterol (<1.70 (3.20 ‐ 4.40 mmol/L) (0.31 ‐ 1.66 mmol/L) mmol/L) mmol/L) Baseline 3.58 1.76 1.35 1.04 6 months 6.50 4.23 1.45 2.25 12 months 4.50 2.43 1.30 2.01 Consider pancreatic enzyme supplementation • Consider decreasing diet ratio •

Vomiting • Pt stable on 4:1 ratio and now 10 y.o. • Get a call: – Pt is vomiting; having breakthrough seizures. – No fever; no one sick at home. – Parents report “diarrhea – just liquid”. • Ketones: 8 – 16mmol/L; BG: 3.5 ‐ 4.3mmol/L. • Pt starts to refuse food.

Vomiting: Contributing Factors • New EA at school so not getting necessary fluids. • Parents stopped PEG 3350 a week ago. • Last formed BM: 1 week ago • Vomiting started 2 days ago; Pt bloated. Suspect cons � pa � on → vomi � ng “Diarrhea” ₌ “overflow”

Vomiting: Management • Bowel routine • Communicate with school regarding importance of following fluid schedule • Could use MCT oil or food (avocado, flax) to soften stools

Bone Health • Pt now 16 y.o. and continues with 4:1 ratio – Ambulatory • Presents to ER with 1 week history of crying, decreased mobility • X ‐ ray shows fracture of right femur, generalized osteopenia

Bone Health: Contributing Factors • Limited physical activity • Multiple AEDs (VPA) • Acidosis ‐ KD, topiramate • Pt not taking vitamin/mineral supplements • Blood work results: Labs Ionized Magnesium 25 ‐ hydroxy Phosphate Calcium (0.65 – 1.05 Vitamin D (1.10 ‐ 2.0 (1.22 ‐ 1.37 mmol/L) (70 ‐ 249 mmol/L) mmol/L) nmol/L) Levels 1.0 0.52 45 1.05

Bone Health: Management • DEXA scan • Reinforce importance of taking supplements • Replace food with formula • Potassium citrate or bicarb supplementation • Refer to Endocrinology • Refer for Physiotherapy • Reconsider KD therapy? Decrease ratio? Transition to modified diet?

Transition to Adult Care • Suddenly John will be turning 18 yrs in six months and will be transitioning to adult care • Ketogenic diet – very efficacious in seizure management ‐ parents wish to maintain on treatment • Adult ketogenic diet clinic available • How do you prepare John and his family for transition?

Transition Management • Transitioning adult neurologist/epileptologist and adult ketogenic diet program – genetic etiology, management of epilepsy syndrome • Medical documentation/imaging/EEGs confirming diagnosis, treatments to date • Provide diet information • Engage the primary care provider

Transition – Early Preparation • Introduced concept of transition with John’s parents when 15 yrs old • Documentation on transition, checklists provided • Risk assessment completed • Contact with social worker • Documentation to new team

Transition – 7 Key Steps Step 1 (ages 12 ‐ 15 yrs): Introduce the concept of transition • Step 2 (ages 12 ‐ 17 yrs): Explore financial, Community, and Legal support • available Step 3 (ages 16 ‐ 17 years): Determine transition readiness patients and • their parents Step 4 B (ages 12 ‐ 19 years): Identify and address risk factors for • unsuccessful transition in adolescents with epilepsy and intellectual disability Step 5 (ages 16 ‐ 19 years): Reevaluate the epilepsy diagnosis • Step 6 (ages 16 ‐ 17 years): Identify obstacles for continuation of treatment • of drug ‐ resistant epilepsies Step 7 (ages 17 ‐ 18 years): Prepare pediatric discharge package • Andrade D, Bassett AS, Bercovici E, et al. Epilepsy: Transition from pediatric to adult care. Recommendations of the Ontario implementation task force. Epilepsia 2017;58(9);1502 ‐ 1517.

Transition – No Adult Keto Program • John turning 18 years in six months! • Transitioning to adult neurologist unfamiliar with genetic diagnosis • No adult ketogenic diet program

Transition – Management • Family physician – management • Communication: dietitians to family physician • Referral to clinical dietitian • Documentation and information exchange

Transition – It’s Possible • With advanced preparation transition is possible • Need to ensure all aspects in place for safe ongoing management

Rapid Fire Question #1 • Unusual skin rash appears soon after starting the ketogenic diet

Rapid Fire Question #2 • Not able to use the gut – What do we do if unable to use the gut for 4 days? – What do we do if unable to use the gut for 2 weeks?