Understanding ME/CFS in young people

Dr Kathy Rowe

Royal Children’s Hospital, Melbourne

July 24th 2014, The Alfred Hospital

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?

Chronic Fatigue Syndrome

Neuraesthenia 1869

DaCosta’s Syndrome 1871

Royal Free Disease 1955

Icelandic Disease 1958

Chronic EBV

Post viral fatigue syndrome

Myalgic Encephalomyelitis

Different diagnostic criteria – mostly

for research purposes

Holmes

Fukuda

Canadian

Oxford

Pediatric

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

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

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)

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%)

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

Sample characteristics

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

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

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

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

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%

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’

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

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

Abdominal, Head & Chest Pain:First-order items & standardized factor loadings

Stomach pain

Nausea

Headache

Recurrent chest pain

ABDOMINAL,

HEAD & CHEST PAIN

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

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

Chronic Fatigue Syndrome:Second-order CFA standardized solution

.710

.914

.702

.636

.701

NEURO-

PHYSIOLOGICAL

ABDOMINAL

HEAD & CHEST PAIN

MUSCLE PAIN

& FATIGUE

Model Goodness-of-fit Indices:

2 (246) = 33.9; p = 0.999

RMSEA = 0.035; SRMR = 0.01

GFI = 0.996; AGFI = 0.971

ME

AN

FA

CT

OR

SC

OR

E Clinical Group (n = 189)

Normals (n = 68)

Symptom Factors: Clinical and control groups

Structural equation regression model:Standardized solution

IMMUNOLOGICALModel Goodness-of-fit Indices:

2 (4) = 1.1; p = 0.893

RMSEA = 0.00; SRMR = 0.01

GFI = 0.998; AGFI = 0.991NEURO-

PHYSIOLOGICALABDOMINAL, HEAD

& CHEST PAIN

MUSCLE PAIN

& FATIGUE

NEUROCOGNITIVE

.73 .27

.52

.60

.67

.486

.17

.488

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

Month of onset of illness

0

2

4

6

8

10

12

14

16

Jan Feb Mar Apr May June July Aug Sept Oct Nov Dec

Months

Age of onset of CFS (n = 187)

0

5

10

15

20

25

30

35

40

45

6 7 8 9 10 11 12 13 14 15 16 17

Age in years

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’

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

Management issues

Aim to reduce consequences of chronic

illness

– loss of social confidence

– educational disadvantage

– physical de-conditioning

– prevaricating about participating in activities

Management program

Balance the amount of energy spent over

the week:

– social activity

– physical activity

– educational input

– commitment to attend certain activities

Management ofChronic Fatigue Syndrome

Physical Symptoms

– headache

– sleep disturbance

– nausea and dietary disturbance

– abdominal pain

– fibromyalgia

– pain management

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

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

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

Emotional issues

For younger children

– dependence

– anxiety

• general

• social

– depression

– helplessness and powerlessness

– transition to adolescence

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’

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

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

Importance of educational

engagement in functional outcome

Importance school attendance:

– Academic goals

– Social learning

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?

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

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

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.

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

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

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

Management principles

Symptom management– Sleep

– Headache

– Dizziness (POTS/NMH –postural orthostatic tachycardia/neurallymediated hypotension)

– Depression

– Pain

‘Lifestyle’ management - balance– Social

– Academic

– Physical activity

– Commitment to attend ‘something’ regularly

– Review each month

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

Any difference between the 2 groups?

t-test results

ANOVA for duration illness, length of follow up and functional rating score p=.23 (ns)

Mean 1 Mean 2 t df p F-ratio

variances

p

variance

Durn

illness

4.78 5.64 -2.03 237 0.041.72

0.008

Durn

FU

8.3 7.3 2.77 523 0.0051.35

0.018

Score 7.79 7.36 2.94 567 0.003 1.04 0.7

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

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

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)

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.

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’.

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

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

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

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.

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’