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

Acute Side Effects of Ketogenic Diets and Issues with Transition

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 1 – used in Faculty presentation only.

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 2

Mitigating Potential Bias

• None

CFPC CoI Templates: Slide 3

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 3rd 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 Cholesterol (3.20‐4.40 mmol/L) LDL cholesterol (0.93‐3.62 mmol/L) HDL cholesterol (0.31‐1.66 mmol/L) Triglycerides (<1.70 mmol/L) Baseline 3.58 1.76 1.35 1.04 6 month follow up 6.50 4.23 1.45 2.35

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: 10th Percentile – Height: Remains <3rd 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 Cholesterol (3.20‐4.40 mmol/L) LDL cholesterol (0.93‐3.62 mmol/L) HDL cholesterol (0.31‐1.66 mmol/L) Triglycerides (<1.70 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 conspaon → voming “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 Calcium (1.22 ‐ 1.37 mmol/L) Magnesium (0.65 – 1.05 mmol/L) 25‐hydroxy Vitamin D (70 ‐ 249 nmol/L) Phosphate (1.10 ‐ 2.0 mmol/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
• f 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
• ngoing 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?