Understanding ME/CFS in young

people

Dr Kathy Rowe

Royal Children’s Hospital, MelbourneCanberra: Capital Health Network Nov 12th 2015

ME/CFS What is it? Precipitating factors How can it be managed? What is the outcome? Importance of maintaining education Role of pacing and exercise 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

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

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:2 (246) = 33.9; p = 0.999RMSEA = 0.035; SRMR = 0.01GFI = 0.996; AGFI = 0.971

Muscle

pain

& fatig

ue

Neuroc

ognit

ive

MEA

N F

AC

TOR

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OR

E Clinical Group (n = 189)Normals (n = 68)

Abdom

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ead &

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Immun

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Muscle

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Symptom Factors: Clinical and control groups

Structural equation regression model:Standardized solution

IMMUNOLOGICALModel Goodness-of-fit Indices:2 (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

Investigations

FBE & ESR U&E LFT TFT Coeliac Screen ANA (EBV/CMV serology/

RRV/ Q Fever)

Differential diagnoses

School refusal Depression Autoimmune disorders Coeliac disease Endocrine – thyroid disease Narcolepsy Inflammatory bowel disease

Co-morbid conditions

Fibromyalgia Orthostatic intolerance Dysmenorrhoea Food intolerances eg FODMAP Depression/anxiety

Month of onset of illness

0

24

68

10

1214

16

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

Months

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

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

Management ofChronic Fatigue Syndrome Physical Symptoms

– headache– sleep disturbance– nausea and dietary disturbance– abdominal pain– fibromyalgia– pain management

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 completeto 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 in Victoria 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) (n=788)– level of functioning– self-reported perception of recovery– duration of illness– usefulness of management strategies.

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

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 1-16)

At 5 years FU ~50% have recovered

For those followed more than 12 years 68% reported recovery (n=93)

Over 70% of all followed for more than 12 years scored 8-10/10 cf 43% at 5 years

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’.

Feed back

The management plan was appreciated for allowing them ‘control over their lives again’

Essential contributors to their quality of life and ability to cope:

‘Believing them’ ongoing support assistance in navigating the education

system

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

Helpful people have included:

Family doctor Pediatrician Visiting teacher Particular teachers School counsellor Support group/ chat rooms Allied health Psychologist

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

Unhelpful

Not being believed – schools, parents, relatives, doctors, friends

Schools and teachers being inflexible Delay in diagnosis “Telling me that I just have to exercise and I will get

better – I love sport and it is killing me not to be able to do it”

“Quacks telling me that they can ‘cure’ me” Implying that it is ‘all in my mind’

Alternative health practitioners

Widely used but: considered of little value (especially supplements and

restrictive diets) ‘Waste of money’ ‘Waste of time’ ‘Sick of promises to cure’‘Helpful’: massage for muscle pain ‘good’ dietary advice

Key predictor of outcomes Continuing engagement in education was identified

as the best predictor of functional outcome Met needs:

– social– educational – developmental – life aspirations– assisted physical activity– ensured that could potentially support themselves

Psychological assistance

most often sought when they were recovering and evaluating how they had managed adolescent developmental tasks

Helped ‘fill in the gaps’ for developmental tasks they were not confident with

Provided confidence that strategies they had learned would serve them well

Helped them deal more constructively with anxiety

What about graded exercise and CBT (cognitive behaviour therapy)?

What does CBT mean?– Goal orientated– Identifying obstacles to achieving that goal– Collaborative agreementHad 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 if intense, may have no

energy left to go to school or see friends

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

Our challenge

To assist: The young person to ‘find their way’ Adequate support for teaching and learning• Families to negotiate with the school Teachers to be comfortable to be flexible Schools to understand illness and assist with peers Educational authorities to allow progress through school

despite not completing a full load Access to higher educational opportunities

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’

ConclusionYoung people valued: regaining the control over their lives that was

lost through illness support to maintain social contacts assistance to achieve educational or life goals feeling confident that they had not missed social

learning tasks of adolescence