Milestones in the trajectory of lifelimiting illness in children Children and Young Peoples Research

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Milestones in the trajectory of life-limiting
illness in children Children and Young Peoples
Research Network 6th Nov 2007

• Richard Hain (PI)
• David Fone
• Emma Barnes

Sharon Simpson Fiona Wood
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Palliative care stream

• Heart of PPC is patient-centred
• Scoping study identified three streams
• Effectiveness of interventions
• Access of clinicians to research work
• Access of researchers to needs of clients (ie
  patients, families, carers)

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Defined four categories that include (by
definition) all life-limiting conditions
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ACT category I

• Life-threatening conditions for which curative
  treatment may be feasible but can fail.
  Palliative care may be necessary during periods
  of prognostic uncertainty and when treatment
  fails (eg cancer, cardiac anomalies).

Characteristic possible cure
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ACT category II

• Conditions in which there may be long periods of
  intensive treatment aimed at prolonging life and
  allowing participation in normal childhood
  activities, but premature death is still possible
  (eg cystic fibrosis, muscular dystrophy).

Characteristic normal period
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ACT category III

• Progressive conditions without curative treatment
  options, in which treatment is exclusively
  palliative and may commonly extend over many
  years (Battens disease, mucopolysaccharidosis).

Characteristic relentless
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ACT category IV

• Conditions with severe neurological disability
  which may cause weakness and susceptibility to
  health complications, and may deteriorate
  unpredictably, but are not considered progressive
  (eg severe cerebral palsy).

Characteristic unpredictable
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Using ACT categories

• Countinue to be foundation of development of PPM
  services.
• Based on disease trajectories
• Potentially useful for streamlining funding by
  predicting need.
• BUT completely unvalidated.

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Aim of Millipaed

• Do life-limiting conditions within ACT
  categories have more in common with each other
  than they do with those in other categories ?

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Aim of Millipaed

• Pilot Describe disease trajectories
• Identify important experiences
• Document when they occurred
• Then Develop mathematical model of trajectories
• Ultimately Use to
• Validate ACT structure (comparison)
• Establish predictive value as funding model

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MilliPaed Pilot

• Q1 What sort of thing do professionals think is
  important to families ?
• Q2 What are the milestones identified by
  families, and when do they occur ?
• Q3 What are the main problems facing families
  with LLC ?

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Aim of Pilot

• Phase I
• Focus groups of professionals to provide
  structure
• Phase II
• Interviews of families (/- children themselves)

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Method -Phase 1

• 4 occupationally themed focus groups with
  professionals who work with children with LLC and
  their families.
• Health
• Education
• Social Care (respite carers, social workers, play
  therapists)
• Mixed professional group.
• Vignettes for each ACT/RCPCH group used to
  structure the discussion - beginning/end
• Asked to identify milestones or events between
  the two stages.

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Phase 2 Interviews with families

• Parents of children with a life-limiting
  condition randomly chosen from clinical records
  of the Palliative Care Team.
• Letter sent to family inviting them to be
  interviewed about the milestones (times or events
  that were important or challenging) during their
  childs illness.
• In a one to one interview participants asked to
  identify milestones, their timings and to tell
  their story.
• For both focus group and interviews data analysed
  qualitatively (thematically) to provide data on
  the nature of milestones and quantitatively
  (summary statistics) on timing of events

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Phase 2 Interviews with families

• Total 27 individuals in 24 families
• Aiming for 5 families in each ACT group.
• Actual interviews
• Group I (5)
• Group II (2)
• Group III (6)
• Group IV (11)

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(No Transcript)
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Results 1 - milestones

• Symptoms
• Referrals
• Tests
• Diagnosis
• Seeking information
• Telling others
• Changes in routine
• Leaving work
• Hospitalisation / operations
• Remission (group 1)
• Loss of mobility
• Leaving mainstream school
• Starting special school
• Family adaptations
• Treatment regimes
• Modifications/ equipment
• Behavioural changes
• Problems feeding

• Incontinence
• Leaving special school
• Cognitive Decline
• Loss of Speech
• Hospice Care
• Respite Care
• Sudden decline
• Pain and palliation
• Ventilation
• Infections
• Plan death and funeral
• Death
• Funeral
• Genetic counselling
• Anniversaries

Diagnostic phase
Loss of normality
Palliative phase
Adjusting to new norm
Death phase
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Milestones - all ACT categories combined
Diagnosis
Time from diagnosis (months)
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What does this show ?

• Enormous range
• Major issues may be before diagnosis
• Palliation may be well before death
• Some suggestion of ACT-group specific
  trajectories
• Different milestones
• Different timing of milestones
• Different duration

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Results 2 - Professional focus groups
ACT/ RCPCH I
ACT/RCPCH II
Psychosocial Impact Shock, Optimism for a
cure, Concern over genetic implications
Psychosocial Impact Shock, Fear, Optimism for a
cure, Disclosure to others
Practical Impact Hospital visits
Time out of school Time off work
Care for siblings
Stigma around illness, Identity
change, Isolation from peers
Condition Milestones Mobility problems Seeking
help Tests Diagnosis
Practical Impact Hospital visits Time
out of school Time off work
Care for siblings
Routine of treatment Role
change for parents, may leave work
School absence
Professional involvement
Condition Milestones Symptoms, Seeking help,
Tests, Diagnosis
Illness from treatment Appearance
changes Hospital visits
Try to fit back into pre-illness lives, Identity
change, Fear of condition returning, Return to
peer group
Increasing difference to peers, Move to special
school, Isolated from peers, Grief at each new
decline
Back to pre-illness routine Return to school
Return to work
Diagnosis
Period of normality after treatment Routine
check ups
Loss of normality
Fails to keep up with peers Losses
start Mobility, Feeding, Speech Increasing
ventilation required
Increasing professional involvement Home
modification Increasing personal care needed
Disclosure, Grief, Loss of hope, Guilt
Adjusting to abnormality
Relapse Decision to stop treatment Change to
purely palliative care
Palliation
New professionals enter care Home
modifications Time out of work
Care of siblings
Loss of hope/optimism Guilt, Exhaustion
Plan for future plan for death
More frequent infections Cycle of
illness/recovery Increased physical size
Accept respite, Caring becomes normal daily
routine, Denial
Death
Caring becomes normal daily routine
Denial as a coping
mechanism Changing
identity Grief at each new loss
Respite care Attend special school
Condition relatively stable, Infections and
complications due to physical growth
Routine of care New modifications Parents leave
work
Continuing losses in Mobility, Feeding,
Speech Increasing ventilation required
Planned vs. unpredictable, Relief vs. shock,
Family readjustment, Ongoing contact with
professionals.
Death of peers, Frustration, Isolation, Guilt at
DNR discussion
Loss of hope, Grief, Guilt, Exhaustion
Discussions about DNR New modifications May move
to adult services (lose services)
Increased cardiac involvement Cycles of
illness/recovery
???
Discussions about DNR Increasingly medicalised
home environment May move to adult services (lose
services)
Exhaustion from caring Grief for childs future
Cycle of illness/recovery from infection
Increased professional care
Home modifications Routine of care Parents
become carers
Problems emerge with development Problems with
mobility, speech, feeding, etc.
Family adjustment
Denial as a coping mechanism
Parents leave work Home modifications
Respite care
Appearance changes Behavioural changes
Loss of mobility, Feeding, Speech, Cognitive
abilities
Psychosocial Impact Shock, Optimism for a
cure, Grief, Concern over genetic implications
Psychosocial impact Shock, Grief, Concern about
future.
Role change Grief at each decline
Increasing routine of care
Parents become carer Home
modifications
Practical impact Hospital visits Time off work
Care for siblings
Appearance changes Behavioural changes Loss of
mobility, Feeding, Speech, Cognitive abilities
Condition Milestones Symptoms, Seeking help,
Tests, Diagnosis
Practical Impact Hospital visits Time
out of school Time off work
Care for siblings
Condition Milestones Symptoms Seeking help
Tests Diagnosis
ACT/RCPCH III
ACT/RCPCH IV
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Results 3 - Thematic analysis of milestones

• 1. Relationship with care professionals
• Relationship seen as a battle by most families.
  
• Only three families felt they had received enough
  support.
• Difference between fighting the disease and
  fighting the system

Mother its just that everything is a fight,
which it shouldnt be. Its hard enough having
somebody whos got a life-limiting disability and
like weve said I go to bed tonight, I dont know
if shes going to wake up in the morning. You
have that, yet everything else which you
shouldnt have to be worrying about, you still
have to fight for. (Group 4)
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• 2. Information needs
• Parents search internet and contact support
  groups for information.
• Danger of inaccurate information and extra
  distress.
• Impact of a delayed diagnosis.

Mother - I spent hours in on the internet. That
was, that was all. Though the person, the
registrar who gave us the news clearly didnt
know anything about the condition. He told us
all sorts of things that werent actually true.
So wed basically written them off as not being
anyone we were able to talk to. (Group
3) Mother - She went for a check and said its
funny that the left leg is stiffer than the
right and (child) must have been about 7 months
old then and she said oh yes, thatll be the
spasticity coming through. Well I didnt even
know what spasticity was. (Laughs) The word was
just spinning round in my head. It was
devastating. (Group 4)
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3. Access to provision

• Problems are not complex care routines (normal
  for individual child) but access to services.
• Felt to be more problematic than the changing
  condition itself.

Its, as I say, looking after (foster child) is
easy. Hes the easy bit. Its all the hassle
that comes with him thats the problem.
(Infantile Battens, Group 3)
Sister - its always the cross over Once hes
actually got to that stage, its fine but as hes
going through that stage when you actually get
the help and support once hes reached that stage
Oh, right now he needs to be fed its
fine (Duchenne Muscular Dystrophy, Group 2)
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4. Nature of milestones/transitions

• Many transitions are gradual and overlapping and
  families find it difficult to assign a time scale
  to the changes.
• Families constantly adapting to the routines of
  care
• Dates for diagnosis, leaving school, operations,
  sudden declines, DNR discussions, and death
  remembered most clearly.

Mother - You know it was a gradual thing really.
Fast but gradual if you know what I mean. He
changed from day to day with things. He declined
from day to day really. (ALD, Group 3)
Foster carer - You dont see it. When youre
with them all the time, you dont really notice
as much as if youre in and out. But everything
just went really. It hasnt really affected day
to day life, because its been so gradual youve
just adjusted without it realising. (Infantile
Battens, Group 3)
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Specific question Defining Palliative Care

• Participants asked When do you think palliative
  care started for your child?
• Understood as treatment when no cure available,
  respite/hospice care, care when close to dying or
  co-ordinated care.

Interviewer What do you think marked the
beginning of palliative care for son? Mother
What do you mean in? Whats that? Ive neverI
dont know what, I dont understand. (Duchenne
Muscular Dystrophy Group 2) Mother Palliative
care? We havent really had much palliative care
because he hasnt really needed it, has he?
Interviewer Has he seen the Symptom Control
Team at the hospital? Mother I didnt know there
. Whats that exactly? Interviewer A team in
Cardiff, Heath Hospital Dr Hain? Mother Oh
yes, Dr Hain, he was great, wasnt he? Father
Yeah, but there wasnt really a lot he could do.
He just explained more to us what was happening,
wasnt it? (Battens Group 3)
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So what have we achieved so far ?

• Identified milestones on disease journey
• Preliminary timings - basis for trajectory
  modelling and comparison
• Thematic analysis of qualitative data
• Professionals
• Families

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Where next ?

• Pilot finishes Jan 2008
• Main study will
• Refine trajectories using greater numbers (whole
  UK around 800 families)
• Validate ACT groups by comparing trajectories
• Show how much of trajectory of any LLC can be
  predicted by knowing ACT group
• Use of models to streamline funding ?

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Acknowledgements

• WORD (funding EB)
• WAG (funding RH)
• Prof David Fone (CHSR)
• Contact a Family
• Families and patients
• Participants in Focus Groups