Pediatric Research Update Rosamund Vallings MNZM, MB BS ME/CFS - - PowerPoint PPT Presentation

Pediatric Research Update

Rosamund Vallings

MNZM, MB BS

ME/CFS Diagnosis a nd Management in Y

• ung P

eople: A Primer

Published in Frontiers in Pediatrics, June 2017

• Vol

5, article 121 Produced by panel

• f

10 experts with 20 years experience in ME/CFS (199 refs)

Epidemiology

• Prevalence: 0.1 -0.5%

(studies vary)

• Peak age of onset: 11-19 (I.Bakken)
• Female:Male =

3-4:1 (S.Nijhof)

• Family history – 20%

(55% monozygotic twins)

• Wide

spectrum of severity

• Commonest cause of longterm school absence
• Unpredictable course,

but more favourable than adults

R Vallings’ patients 2015-17

• 126 cases aged

4-18 (=10% total CFS database)

• 7 misdiagnosed

– did not fit criteria

• Of 119 cases

– 18 had a family history (15%)

• Sex

ratio – male:female 1:3

• Onset trigger:
• Infectious

mono – 36 (30%)

• Other

infection – 47 (40%)

• Accident

– 9 (7.5%)

• Overexercise

– 9 (7.5%)

• Severe

allergy – 4 (3.4%)

Melbourne Study

• f

700 (K.Rowe)

• 60-88% reported “recovery”

(over 1-15yrs)

• May have learnt to manage life better,

but some symptoms persist.

• Most important determinant of

functioning was maintaining education.

• 95%

are studying

• r

working fulltime

Aetiology/Pathophysiology

• Infection

common trigger (partic EBV)

• Overexertion,

stress, sleep disorder, trauma etc.

• High co-morbidity with orthostatic intolerance and

joint hypermobility (60% - 95%) (P.Rowe)

• Immune dysfunction

– oxidative stress, ↑WBC apoptosis (F.Khan)

• Beneficial response

to IV immunoglobulin (K.Rowe)

• Brain

imaging: deficits in emotional conflict functioning (L.Wortinger)

• Pittsburg sleep

quality index = dysfunction (E.K.Josev)

Diagnosis

• No specific

test

• Case definitions tend

to be for adults

• 2006

pediatric definition (D.Bell)too complex

• Adapted to 2017 – simpler,

more specific

• Included

PEM (Ped Primer)

• Ongoing

work into case definitions (L.Jason)

• Long

delay in diagnosis (I.Bakken)

• Importance of recognising

co-morbidities

Clinical diagnosis

• Diagnostic worksheet (Pediatric Primer)
• Exam

to include neurological and psych aspects

• Look for co-morbidities and

treat

• PEM after

physical and mental exercise raises probability of CFS

Co-morbid conditions

• Orthostatic intolerance 60-95%

(P.Rowe)

• Ehlers Danlos Syndrome - 60%

(P.Rowe)

• Gynae symptoms premenstrually
• Non-IgE mediated

food allergies – 33% - commonest is milk protein (P.Rowe)

• Mast-cell activation syndrome (assoc with EDS

and OI) – similar symptoms to CFS

Psychological reactions

• = response to chronic illness
• Study 400 patients (K.Rowe)

– 25% clinically depressed (cf 20% healthy controls)

• O.I. and

EDS leads to greater anxiety

• Suicidal thoughts only if CFS associated

with major depression

• Adversity ++ due to misdiagnosis as MDD (J

Newton)

• NB Factitious disorder by proxy (ME Ass UK)
• School phobia (W.Fremont)

Management/treatment

• Paucity of literature
• Primer

recommendations from very experienced clinicians

• Aim to improve function,

relieve symptoms,

• ngoing support,

educate school personnel

• Most important issues: Validation of illness,

supportive doctors, removal of fears, management plan (K.Rowe)

Support/coping sk ills

• Need

sense of achievement (K.Rowe)

• Management plan

important

• Include social & emotional needs (E.Crawley)
• Include gentle paced

exercise – not GET(F.Twisk)

• Avoid

“boom/bust” (L. Jason)

• CBT of limited

value (no change in immune abnormalities)

• Lightning Process – (E.Crawley)

– outlandish claims – does not “cure”

Pharmacological Rx

• Extreme

sensitivity to medication

• Very small doses
• Treat: Sleep,

Pain, Nausea, O.I.

• No trials in children for

treating fatigue with drugs

Diet/supplements

• Balanced nutrition
• Extra

salt for O.I. (P.Rowe)

• Vit D
• Magnesium
• B12 (B.Regland)
• ?probiotics

Alternative/complementary

• Mainly anecdotal
• Watch for interactions
• Massage

and good diet – only approaches deemed helpful (K.Rowe – 700 patients)

• Sense of relief when

parents stopped the search for cure (K.Rowe) – may have tried up to 30 approaches!

Severely affected children (N.Speight)

• Severe – 5-10%
• Very severe – 2-5%
• Physicians unprepared and inexperienced
• Parents best caregivers
• NB Vit D,
• steoporosis,

sensitivity to meds

• Effects on family → sibling anxiety
• Prognosis – 24pts
• ver

7-10 years duration: 2 recovered, 15 improved, 7 still severely ill

Education

• Commonest cause of long term

absence

• Poor cognitive function
• Engagement

in education – key issue leading to improved ability to function (K.Rowe)

• →Fulfilment
• f aspirations &

peer support

• Intellectual

reasoning OK – can keep up if paced (F.Newton)

• Disbelief is a major stress (F.Newton)

Educational support

• Educational accommodations required
• Maybe

homebound

• Effects of travel,

noise etc (P Tucker)

• Cognitive testing may not be accurate
• Educational fact sheet required for teachers
• Letter to doctors to support education

plan

• Robot in classroom (Norway,UK)

(J BØrsting)

The Fu ture

• S

Knight leading pediatric research programme in Australia looking at brain imaging associated with cognition.

• L

Jason further studies into epidemiology

• David

Bell overview and conclusion (Written for Open Medicine Foundation)