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)

MUSCLEPAIN & 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

ABDOMINALHEAD & CHEST PAIN

MUSCLE PAIN & FATIGUE

Model Goodness-of-fit Indices:

χ2 (246) = 33.9; p = 0.999RMSEA = 0.035; SRMR = 0.01GFI = 0.996; AGFI = 0.971

Mus

cle p

ain &

fatig

ue

Neuro

cogn

itive

ME

AN

FA

CT

OR

SC

OR

E Clinical Group (n = 189)

Normals (n = 68)

Abdom

inal, h

ead

&

ches

t pain

Neuro

phys

iolog

ical

Imm

unolo

gical

Somat

ic 1

Muscle

sens

ation

s

Somat

ic 2

Symptom Factors: Clinical and control groups

Structural equation regression model:Standardized solution

IMMUNOLOGICALModel Goodness-of-fit Indices:

χ2 (4) = 1.1; p = 0.893RMSEA = 0.00; SRMR = 0.01GFI = 0.998; AGFI = 0.991

NEURO-PHYSIOLOGICAL

ABDOMINAL, 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)

05

1015202530354045

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/neurally

mediated 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’