Emerge Australia Seminar 24th July 2014 Over the past 40 years, Dr Kathy Rowe has achieved outstanding national, and international recognition for her work in adolescent health. She is a consultant paediatrician at the Royal Children’s Hospital and an expert in the area of ME/cfs and the relationship between health and education. She has learned much from her extensive cohort and feedback from the young people with this illness.

1. Understanding ME/CFS in
young people
Dr Kathy Rowe
Royal Children’s Hospital, Melbourne
July 24th 2014, The Alfred Hospital

3. ME/CFS
What is it?
Precipitating factors
How can it be managed?
What is the outcome?
Role of pacing and exercise
Importance of maintaining education
What do young people think is helpful?

4. Chronic Fatigue Syndrome
Neuraesthenia 1869
DaCosta’s Syndrome 1871
Royal Free Disease 1955
Icelandic Disease 1958
Chronic EBV
Post viral fatigue syndrome
Myalgic Encephalomyelitis

5. Different diagnostic criteria – mostly
for research purposes
Holmes
Fukuda
Canadian
Oxford
Pediatric

6. Diagnostic criteria CFS (Fukuda et al 1994)
Fatigue
– clinically evaluated
– unexplained
– persistent or relapsing
– 6 months or more
new onset
not result of ongoing
exertion
not substantially
alleviated by rest
substantial reduction
in previous levels of
occupation,
education, social or
personal activities

7. Diagnostic criteria CFS (Fukuda et al 1994)
4 or more of the following - concurrent
persistent, did not predate fatigue
– impaired short term memory or conc.
– sore throat
– tender cervical or axillary lymph nodes
– muscle pains
– multi-joint pains without arthritis
– headaches - new type, pattern, severity
– unrefreshing sleep
– post-exertional malaise lasting more than 24 hrs

8. CFS and adolescents
Concerns have been expressed that:
– symptoms may be different
– may reflect somatization disorder
– diagnosis of CFS is counterproductive in
young people
– Several studies describing outcomes -
many subjects had illness of only 3
months duration when entered study (Carter,
1995: Bell, 1997; Krilov 1998)

9. Sample characteristics
189 young people
mean age 15 (10.6-18.6) years
M:F ratio 1:3
All socioeconomic groups represented,
although ‘unskilled’ under-represented
compared with census data
All Caucasian except for 3 with at least one
S.E. Asian parent (Anglo-celtic ~85%)

10. Sample characteristics
Duration: minimum 6 months
defined onset over hours or days
fatigue exacerbated by exercise and not
relieved by rest
neurocognitive symptoms
at least 3 of following: myalgia, arthralgia,
headaches, sleep disturbance, abdominal pain,
dizziness, nausea, pharyngitis lymphadenopathy

11. Method
Completed 38 item symptom
questionnaire
– Designed for adults, and to identify other
illnesses (Lloyd Hickie)
– Symptom frequency and severity
Analyses
– frequency of responses
– confirmatory factor analysis

12. Method
Comparison with control group
– 68 young people from youth group
– age mean 14.5 (range 12-18) years
– M:F ratio 1:3
– Similar age, gender and socioeconomic group
characteristics

13. Data analyses
Analyses
– frequency of responses
– fitted first- and second-order confirmatory factor
analytic models (CFA) to the data
– fitted a structural equation regression model to
estimate the magnitude and direction of the
interdependent effects among the identified
factors

15. CFS symptoms: Clinical group
24 of 38 symptoms considered relevant
remainder - had:
- non-significant relationship with the
underlying scale
- low occurrence and severity response
frequencies
- marginal relevance to CFS symptomatology
in the clinical experience of first author

16. Symptoms experienced by
87% of clinic group
prolonged fatigue following minor activity
headache
the need for excessive sleep
loss of ability to concentrate
disturbed sleep
myalgia following minor activity
severe or moderately severe in 70%

17. Experienced by 70%,
moderate-severe 50%
myalgia after activity
sore throat without coryzal symptoms
tender cervical lymph nodes
feeling of disturbed balance
nausea
abdominal pain
myalgia at rest
experiences of being ‘lost for the word’

18. Muscle Pain Fatigue:
First-order items standardized factor loadings
Muscle pain (not joint pain) after activity
Muscle pain (not joint pain)
even when doing nothing
Excessive muscle fatigue with minor activity
Joint pain
Prolonged feeling of fatigue after physical
activity lasting for hours (or days)
MUSCLE
PAIN FATIGUE

19. Neurocognitive:
First-order items standardized factor loadings
Loss of concentrating ability
Difficulty with speech - ‘lost for the word’
Memory loss
Vivid dreams or nightmares
NEUROCOGNITIVE

20. Abdominal, Head Chest Pain:
First-order items standardized factor loadings
Stomach pain
Nausea
Headache
Recurrent chest pain
ABDOMINAL,
HEAD CHEST PAIN

21. Neurophysiological:
First-order items standardized factor loadings
Recurrent chest pain
Feeling of disturbed balance
Difficulty in focussing vision
Disturbed sleep or disrupted sleep pattern
Persistent dryness in the eyes or mouth
Shortness of breath with minor activityl
Papitations (feeling the heart racing)
Needing to sleep for long periods
NEURO-PHYSIOLOGICAL

22. Immunological:
First-order items standardized factor loadings
Tender glands in the neck
Tender glands elsewhere
Sore throat without common cold symptoms
Repeated fevers and sweats
IMMUNOLOGICAL

23. Chronic Fatigue Syndrome:
Second-order CFA standardized solution
.701
.636
.702
.914
.710
MUSCLE PAIN
FATIGUE
ABDOMINAL
HEAD CHEST PAIN
NEURO-PHYSIOLOGICAL
Model Goodness-of-fit Indices:
c2 (246) = 33.9; p = 0.999
RMSEA = 0.035; SRMR = 0.01
GFI = 0.996; AGFI = 0.971

24. Symptom Factors: Clinical and control groups
Neurocognitive
MEAN FACTOR SCORE
Muscle pain fatigue
Clinical Group (n = 189)
Normals (n = 68)
Abdominal, head
chest pain
Neurophysiological
Somatic 1
Muscle sensations
Immunological
Somatic 2

25. Structural equation regression model:
Standardized solution
Model Goodness-of-fit Indices: IMMUNOLOGICAL
c2 (4) = 1.1; p = 0.893
RMSEA = 0.00; SRMR = 0.01
GFI = 0.998; AGFI = 0.991
NEURO-PHYSIOLOGICAL
ABDOMINAL, HEAD
CHEST PAIN
MUSCLE PAIN
FATIGUE
NEUROCOGNITIVE
.73 .27
.52
.60
.67
.486
.17
.488

26. Identified ‘triggers’
In Australia:
– Typically after infective
process
• EBV - Glandular fever -
15% adolescents (70%
+ve serology)
• CMV, influenza, polio,
Chicken pox,
gastroenteritis, Ross
River, Q fever,
Mycoplasma, Malaria,
Dengue Fever, HHV6,
giardia
– Overlaps with
‘overtraining syndrome’
– trivial illness in the
context of heavy training
schedule – same
symptoms.
In Japan:
– Chronic sleep
deprivation and stress –
very rare to be post –
infective – typical onset -
end primary school

27. Month of onset of illness
16
14
12
10
8
6
4
2
0
Jan Feb Mar Apr May June July Aug Sept Oct Nov Dec
Months

28. Age of onset of CFS (n = 187)
45
40
35
30
25
20
15
10
0 5
6 7 8 9 10 11 12 13 14 15 16 17
Age in years

29. Presence of anxiety/depression
Baseline level depression in adolescents in
Victoria 20%
ME/CFS 27% - severity illness, not being
believed, ‘school’ not working out
Anxiety – worse if absent from school,
cognitive issues severe, loss confidence and
associated social anxiety, occasional panic
attacks if ‘overloaded’, recurrence of
symptoms with intercurrent infection – like
‘post-traumatic stress’

30. Management issues in adolescents
Symptom management
Lifestyle (energy) management
– social contact
– academic input
– physical activity
– commitment to attend something on a regular
basis
Family and emotional support

31. Management issues
Aim to reduce consequences of chronic
illness
– loss of social confidence
– educational disadvantage
– physical de-conditioning
– prevaricating about participating in activities

32. Management program
Balance the amount of energy spent over
the week:
– social activity
– physical activity
– educational input
– commitment to attend certain activities

33. Management of
Chronic Fatigue Syndrome
Physical Symptoms
– headache
– sleep disturbance
– nausea and dietary disturbance
– abdominal pain
– fibromyalgia
– pain management

34. Effects of chronic ill health
Emotional and developmental
– on the child
– on the family
Educational
Strategies for school absence
– Housebound students
– Getting back into school after prolonged illness

35. What about graded exercise and CBT
(cognitive behaviour therapy)?
What does CBT mean?
– Goal orientated
– Identifying obstacles to achieving that goal
– Collaborative agreement
Had a lot of bad press- some evidence for
usefulness in adults and less so in young people

36. Important to understand the illness including
prolonged recovery
Important to understand priorities in life
Graded exercise may progress but may have
no energy left to go to school or see friends

37. Emotional issues
For younger children
– dependence
– anxiety
• general
• social
– depression
– helplessness and powerlessness
– transition to adolescence

38. Emotional issues
Parental concerns
– cajole and encourage or trust their judgement
– defend and excuse or set limits to behaviour
– protect or allow to make mistakes
– put their own life on hold or ‘get on with life’

39. Emotional issues
For adolescents
– social anxiety/social skills
– autonomy/dependence
– confidence in ability/uncertainty about what
is required
– ‘risk-taking’/extreme caution
– poor self image/self esteem
– depression/hope
– puberty and sexual identity

40. Educational effects
· Delayed or severely interrupted academic
progress
· increased dependence on others – a sense
of ‘loss of control’.
· lack of confidence and reduced self image
· decreased organisational skills

41. Importance of educational
engagement in functional outcome
Importance school attendance:
– Academic goals
– Social learning

42. Flexibility important:
– What were career aspirations before became
unwell?
– What is the minimum that they need to complete
to enable them to get there?
– What teachers do they like?
– What subjects do they like?
– What subjects do they need?
– What fits with the amount of time they can
manage at school and fits with family demands?

43. Educational impact
Headache, fatigue and stamina issues
Recovery after activity (physical or mental)
‘Brain fog’ and cognitive issues
– Difficulty retrieving information quickly
– Difficulty finding the right word
– Difficulty in ordering information
– Effort required to learn
– Stimulus overload
Competing psychosocial/educational needs/
expectations

44. Challenges:
Transition times – year 7 / new school
Upper secondary – issues around VCE
special provision.
Different educational demands in different
states. In US – problems, as not allowed to
progress unless fully completes each year-very
discouraging and educational drop outs
common – depression common – welfare
dependency common

45. How long does it last?
To follow up consecutive patients referred to
the CFS clinic at the Royal Children’s
Hospital over 18 years (1991 – 2009)
– level of functioning
– self-reported perception of recovery
– duration of illness
– usefulness of management strategies.

46. 788 young people with CFS
age 6-18 years (mean 15 years)
M:F 1:3
30% rural (reflects population distribution), 2% interstate
Ethnic origins
– 85% with either parent with Scottish / Irish surname
– 10% northern European (Dutch, Scandinavian, German)
– No Middle Eastern, African, 1 Sri Lankan (with Scottish
Grandfather), 1 Taiwanese, 3 Chinese descent and only
1 with both parents Chinese
CFS patient ethnic origins not representative of Victorian
population or those that visit RCH

47. 2 groups followed up
398 with standardized history, baseline
symptom and psychological data (anxiety
scales, depression, parental bonding)
– Prospective questionnaire follow up each 2 years
390 with standardized history and symptoms
– Phone contact during 2010 and 2011
– Questionnaire sent if consent obtained

48. Follow up questionnaire
Demographics
Functional outcomes
– Academic level
– Nature of work
– Use of social security support
Duration of illness if reported recovered
Additional illnesses
Use of alternative health practitioners
Reported usefulness of management strategies

49. Management principles
Symptom management
– Sleep
– Headache
– Dizziness (POTS/NMH –postural orthostatic tachycardia/neurally
mediated hypotension)
– Depression
– Pain
‘Lifestyle’ management - balance
– Social
– Academic
– Physical activity
– Commitment to attend ‘something’ regularly
– Review each month

50. Follow up
1st group
– At least one return from 342 of 398 (86%)
– 6 occasions between 1998 and 2008 provided 804 returns
50% reported recovery
2nd group
– 78% contacted and provided information
33% reported recovery
Both groups follow up 1.7-21 years

51. Any difference between the 2 groups?
t-test results
Mean 1 Mean 2 t df p F-ratio
ANOVA for duration illness, length of follow up and
functional rating score p=.23 (ns)
variances
p
variance
Durn
illness
4.78 5.64 -2.03 237 0.04
1.72 0.008
Durn
FU
8.3 7.3 2.77 523 0.005
1.35 0.018
Score 7.79 7.36 2.94 567 0.003 1.04 0.7

52. Functional ratings (mean 7.6, sd 1.7) at follow up
mean 7.8 years sd 4.3 (range 1-21) n=570

53. Distribution of duration of illness
mean 5 years (sd 2.8) n=240

54. Of those who report recovery
– 60% are well by 5 years
Average duration is 5 years in young
people (range 1-13)
For those followed more than 12 years
68% reported recovery (n=67)

55. Functional status
Of those who reported recovery ~1/3
indicated that they were conscious of
monitoring their workload.
Less than 5% were not either studying or
working part or full time, often due to other
factors than CFS.
Many had married (n=38) and those with
children (n=15) reported being well.
90% completed or intended to complete
post-secondary school training.

56. Follow up of clinical group
– 10% Distance Education Services
– 33% used the Visiting Teacher Service
– 15% received a Disability Support Pension
– 30% considered that they ‘no longer suffered
from CFS’.

57. Anything helpful during illness?
82% said “yes”
management strategies (30%)
positive outlook
supportive family
‘pushing themselves’ to try new things
balancing rest periods with gentle
exercise
assistance with education

59. Could anything have been handled better?
50% said “yes”
– 30% found their encounter with the medical
profession frustrating
• not believed and many doctors were unaware
of the illness
• Similar issue with schools
– 20% earlier diagnosis
– general ignorance of the illness and lack of
understanding of the illness

60. Is there a role for an intensive program?
Not been answered yet but the impression is
that it is very useful if the young person and
family are ready for intensive input.
Especially if:
– If ‘educational issues’ cannot be resolved
– If they are ‘stuck’ in progress
– May be conflict at home/school about
management that has not been resolved

61. Adolescent CFS ?
Young people remarkably consistent in responses
Not likely to respond in a ‘halo’ fashion to ‘any
symptom’
85% had onset following ‘viral’ or febrile illness
Headache/abdominal pain, muscle pain and fatigue,
and neurocognitive symptoms were mediated by
‘neurophysiological’ symptoms and ‘immunological’
symptoms were central to responses.

62. Conclusion
Illness has significant impact on all aspects
of development
Frustratingly long period (av 5 years 1-13
years). Most recover.
Functional outcomes significantly related to
being engaged in education
Depression related to severity, not being
understood, difficulties with schooling
They are impressively resilient, persistent
and determined and deserve a ‘fair go’