Transcript Palliative Care for Children with Developmental

Integrating Palliative Care Into
The Care Of Children With
Developmental Disabilities
Mike Harlos MD, CCFP, FCFP
Professor and Section Head, Palliative Medicine, University of Manitoba
Medical Director, WRHA Adult and Pediatric Palliative Care
Erin Shepherd RN, MN
Clinical Nurse Specialist, WRHA Pediatric Palliative Care
The presenters have no
conflicts of interest to
disclose
Objectives
• To explore where palliative care may fit into the spectrum
of care for children with developmental disabilities
• To consider potential barriers to the involvement of
palliative care
• To review common issues of communication in palliative
and end-of-life care
• To explore an approach to health care decision-making
• To review common symptoms at end-of-life and their
management
http://palliative.info
WHO Definition of
Palliative Care for Children
• Palliative care for children is the active total care of the
child's body, mind and spirit, and also involves giving
support to the family.
• It begins when illness is diagnosed, and continues
regardless of whether or not a child receives treatment
directed at the disease.
• Health providers must evaluate and alleviate a child's
physical, psychological, and social distress.
• Effective palliative care requires a broad multidisciplinary
approach that includes the family and makes use of
available community resources; it can be successfully
implemented even if resources are limited.
• It can be provided in tertiary care facilities, in community
health centres and even in children's homes.
“Thank you for giving
me aliveness”
Jonathan – 6 yr old boy terminally ill boy
Ref: “Armfuls of Time”; Barbara Sourkes
Common Trajectory Of Decline In
Progressive Life-Limiting Illness In Children
From presentation by Joanne Wolfe at the 16th International Congress on the Care of The
Terminally Ill
Functional
Status
Decline
Crises
(“Scary Dips”)
Time
Death
Prognostic Uncertainty
“I don’t know whether to buy a coffin or a tricycle”
Comment from a father during prenatal palliative
care consult
•
Addressing the comfort of children must require
prognostic certainty or the acknowledgment of
the likelihood of dying
•
Palliative Care can follow in parallel with
aggressive, cure-focused care, as one member of
the team supporting patient and family
Palliative Care… The “What If…?” Tour Guides
• What would things look like?
• Time frame?
“What if…? • Where care might take place
• What should the patient/family
expect (perhaps demand?)
regarding care?
• How might the palliative care
team help patient, family,
health care team?
Disease-focused Care
(“Aggressive Care”)
Palliative Care for Children with
Developmental Disabilities
• Range of conditions
• Prognostication difficult
– These children would typically not meet the criteria required
for adult programs => pediatric criteria required
• Specific needs are varied
– Will depend on care environment, momentum of decline,
needs of the family +/or health care providers
•
•
•
•
•
Resource for symptom management or crises
Assist with care planning and decision making
Inform the range of options and possibilities
Resource for the family in dealing with serious illness
Bereavement resources, follow-up +/or support
Barriers to Integration of Curative and Palliative Care
Among the Population of Children with Severe
Neurodevelopmental Disabilities
1. Professional Attitudes Towards Severe Cognitive
Disability
–
May inappropriately downplay the burden of
technological intervention
–
May under treat pain
–
May limit the spectrum of care options
–
If working in an inpatient setting may not
recognize their assessment of QOL is based on
an incomplete clinical picture
Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for
Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365
Continued…
2. Reconfigured Parental Roles Towards Children with
Complex Medical Care Needs
–
–
–
–
–
Special relationship with their child – intertwined
identity
Feelings of guilt evoked at prospect of child’s death
Reluctance to plan for end of life care
Optimal palliative care may be as important as any care
decision made throughout the child’s life
Integration of palliative care goals into the usual
chronic care regime
Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for
Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365
Continued…
3. The Uncertainty of Prognosis and the Pressure of Past
–
Struggle to identify outcome indices and valid
comparison group on which to base accurate
prognostication
–
Without faith in prognostication, planning for end of
life care can be postponed or ignored
–
Clinicians must find a way to overcome their sense of
discomfort with being previously wrong and counter
parents’ unrealistic expectations for the endless
restorative prowess of medical care
Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for
Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365
A Model for End-of-Life Care in Children with
Neurodevelopmental Disabilities
1. Acknowledge that children with disabilities have
quality-of-life
2. Acknowledge that children with disabilities are entitled
to end-of-life care
3. Begin discussions regarding interventions, treatment
options, quality-of-life, and end-of-life care early and
as part of a medical home model
4. When considering technology support options, present
both benefits and potential difficulties, while also
acknowledging that technology is not irrevocable
Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for
Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365
Continued…
5. Utilize a multidisciplinary approach, including physicians,
nurses, social workers, psychologists, teachers, friends and
others
6. Engage non-acute care providers at times of medical crisis
7. Equate end-of-life care with quality care that is not
inconsistent with chronic care
8. Acknowledge the parent-child relationship, expertise, and
longitudinal perspective
9. Model effective and compassionate care of children with
neurodevelopmental disabilities for trainees and
colleagues
Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for
Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365
Silence Is Not Golden
 Don’t assume that the absence of question reflects
an absence of concerns
 As families watch a loved one decline, it would be
very unusual for them not to wonder what to expect,
and over what time frame
 Waiting for such questions to be posed may result in
missed opportunities to address concerns; consider
exploring preemptively
Be Clear
“The single biggest problem in communication is the
illusion that it has taken place.”
George Bernard Shaw
Make sure you’re both talking about the same thing
There’s a tendency to use euphemisms and vague
terms in dealing with difficult matters such as death &
dying… this can lead to confusion
20
Planning For Predictable Challenges
• functional decline occurs
• food/fluid intake decrease.;
feeds may not be tolerated
• enteral medication route may
be ineffective
• symptoms develop:
dyspnea, congestion, delirium
• family will need support &
information
Connecting
• A foundational component of effective communication
is to connect / engage with that person… i.e. try to
understand what their experience might be
– If you were in their position, how might you react or
behave?
– What might you be hoping for? Concerned about?
• This does not mean you try to take on that person's
suffering as your own
• Must remain mindful of what you need to take
ownership of (symptom control, effective
communication and support), vs. what you cannot (the
sadness, the unfairness, the very fact that this person
is dying)
Responding To Difficult Questions
1. Acknowledge/Validate and Normalize
“That’s a very good question, and one that we should talk about. Many
people in these circumstances wonder about that…”
2. Is there a reason this has come up?
“I’m wondering if something has come up that prompted you to ask this?”
3. Gently explore their thoughts/understanding
• “Sometimes when people ask questions such as this, they have an idea
in their mind about what the answer might be. Is that the case for you?”
• “It would help me to have a feel for what your understanding is of your
condition, and what you might expect”
4. Respond, if possible and appropriate
• If you feel unable to provide a satisfactory reply, then be honest about
that and indicate how you will help them explore that
The Perception of the “Sudden Change”
When reserves are depleted, the change seems sudden
and unforeseen.
However, the changes had been happening.
That
was
fast!
Melting ice = diminishing reserves
Day 1
Day 2
Day 3
Final
Helping Families At The Bedside: Physical Changes
 physical changes of dying can be upsetting to those at the
bedside:
– skin colour – cyanosis, mottling
– breathing patterns and rate
– muscles used in breathing
 reflect inescapable physiological changes occurring in the
dying process.
 may be comforting for families to distinguish between who
their loved one is - the person to whom they are so
connected in thought and spirit - versus the physical
changes that are happening to their loved one's body.
Decisions
Helping Family And Other
Substitute Decision Makers
 In situations where death will be an inescapable
outcome, family may nonetheless feel that their
choices about care are life-and-death decisions
(treating infections, hydrating, tube feeding, etc.)
 It may be helpful to say something such as:
“I know that you’re being asked to make some very difficult
choices about care, and it must feel that you’re having to make
life-and-death decisions. You must remember that this is not a
survivable condition, and none of the choices that you make
can change that outcome.
We are asking for guidance about how we can ensure that we
provide the kind of care that he would have wanted at this
time.”
An Approach To Decision Making
• The health care team has a key role in providing
information related to technical or medical issues, and
physiological outcomes…
 reviewing/explaining details about the condition, test
results, or helping explore treatment options
 indicating when a hoped-for outcome or treatment option
is not medically possible
• Patient/family must have a central role in
considerations relating to value/belief systems (such as
whether life is worth living with a certain disability) or to
experiential outcomes (such as energy, well-being,
quality of life)
Goal-Focused Approach To Decision Making
Regarding effectiveness in achieving its goals, there are 3 main
categories of potential interventions:
1. Those that will work: Essentially certain to be effective in
achieving intended physiological goals (as determined by the
health care team) or experiential goals (as determined by the
patient) goals, and consistent with standard of medical care
2. Those that won’t work: Virtually certain to be ineffective in
achieving intended physiological goals (such as CPR in the
context of relentless and progressive multisystem failure) or
experiential goals (such as helping someone feel stronger, more
energetic), or inconsistent with standard of medical care
3. Those that might work (or might not): Uncertainty about the
potential to achieve physiological goals, or the hoped-for goals
are not physiological/clinical but are experiential
Goal-Focused Approach To Decisions
Goals unachievable, or
inconsistent with standard of
medical care
• Discuss; explain that the
intervention will not be offered
or attempted.
• If needed, provide a process for
conflict resolution:
 Mediated discussion
 2nd medical opinion
 Ethics consultation
 Transfer of care to a
setting/providers willing to
pursue the intervention
Uncertainty RE: Outcome
Consider therapeutic trial, with:
1. clearly-defined target
outcomes
2. agreed-upon time frame
3. plan of action if ineffective
Goals achievable and
consistent with standard of
medical care
• Proceed if desired by patient
or substitute decision maker
Considerations In The Final Hours
• Are there preexisting medical conditions need attention in
the final hours?
 not typically necessary to continue ongoing medical
management of underlying illnesses, with the possible
exception of seizure disorder
• What new symptoms exists of might arise (typically
dyspnea, congestion, agitated delirium), and related
medication needs?
 Uncommon for pain to arise as a new symptoms in
final hours
 What are the available routes of medications
administration?
• Anticipated concerns of family
Prevalence of Symptoms In Final Days
• Dyspnea: 80% +
• Congestion: reported as high as 92%
• Delirium: 80% +
• Families who would be grateful for support and
information: must be near 100%
When these issues arise at end-of-life, things
haven’t “gone wrong”… they have gone as they
are inclined to.
Role of the Health Care Provider
• Anticipate changes in functional status, loss of
oral route, the development of symptoms
• Develop a care plan that prepares for predictable
issues – LAD, ACP
• Preemptively address communications issues:







food/fluid intake
sleeping too much
are medications causing the decline?
how do we know he/she is comfortable?
can he/she hear us?
don’t want to miss being there at time of death
how long can this go on? what will things look like?
Management of Symptoms
Symptom
Drug
Non-Oral Route(s)
Dyspnea
opioid
• sublingual (SL) – small volumes of high
concentration; same dose as oral
• subcutaneous – supportable in most
settings; same dose as IV = ½ po dose
• IV – limited to hospital settings
• intranasal – fentanyl – lipid soluble
opioid; use same dose as IV to start.
Pain
opioid
see above
Secretions
scopolamine
• subcutaneous
• transdermal (patches; compounded gel)
glycopyrrolate
• subcutaneous
Agitated
Delirium
neuroleptic
• SL– use same dose for all routes
(methotrimeprazine • subcutaneous (most settings); IV
; haloperidol)
(hospital)
lorazepam
• SL – generally use with neuroleptic
Medications Needed
1. Opioid: pain, dyspnea
2. Antisecretory: congestion
3. Sedative (neuroleptic +/- benzodiazepine):
agitated delirium
Plus
whatever condition-specific medications are
needed (e.g. anticonvulsants)
Opioid Use in Final Days/Hours
• specific doses will depend on the degree of distress and
existing opioid tolerance
• usually need to use short-acting opioid in order to respond
quickly to changing symptoms (dyspnea)
• if patient is on long-acting morphine or hydromorphone, switch
to equivalent daily dose of short-acting divided q4h (½ the oral
dose if switching to subcutaneous or IV)
• if patient is on transdermal fentanyl, consider leaving this as is,
and adding a q4h dose of morphine or hydromorphone,
starting low and titrating up as needed.
• the interval between prn (as-needed) doses should not exceed
one hour, regardless of route. The effect of a prn dose will be
evident by 1h, and a longer interval will cause needless
suffering
Medications Needed
1. Opioid: pain, dyspnea
2. Antisecretory: congestion
3. Sedative (neuroleptic +/- benzodiazepine):
agitated delirium
Plus
whatever condition-specific medications are
needed (e.g. anticonvulsants)
Opioids in Dyspnea
● Uncertain mechanism
● Comfort achieved before resp compromise; rate
often unchanged
● Often patient already on opioids for analgesia; if
dyspnea develops it will usually be the symptom
that drives the need for titration
● Dosage should be titrated empirically
● May need rapid dose escalation in order to keep
up with rapidly progressing distress
Recommended Opioid And Sedative Doses For Dyspnea (> 6 Months Age)*
* For infants < 6 months start with ¼ of the pediatric starting dose and titrate
Agent
Intermittent Dose
Parenteral Infusion Dose
Codeine
Enteral
0.5 – 1.0 mg/kg q4h
Not recommended parenterally
Morphine
Sulfate
Enteral
0.2 – 0.3 mg/kg q 4h
IV/SQ
0.05 – 0.2 mg/kg q 2-4h
0.05 mg/kg IV load over 10 min then 0.01 –
0.03 mg/kg/hr
Enteral
30 – 80 micrograms/kg q4h
IV/SQ
15 micrograms/kg q 2 – 4h
Hydromorphone
Oxycodone
0.05 – 0.15 mg/kg po q4h
Fentanyl Citrate
0.5 – 2 micrograms/kg IV
Lorazepam
0.05 mg/kg IV/SL
Midazolam
Methotrimeprazine
IV
0.025 – 0.05 mg/kg titrated
carefully, with 2-3 min. between
fractions
Nasal
0.1 mg/kg in each nostril
po/SL
Child 1 month–18 years 0.5
micrograms/kg (max. 15 mg)
30–60 minutes before procedure
0.025 - 0.1 mg/kg q6h po/SQ
10 – 20 micrograms/kg IV load over 10 min
then 2 – 8 micrograms/kg/hr
N/A
0.5 – 2 micrograms/kg/hr IV
• Infusion would be guided by prn doses
• “…neither surgical anesthesia nor fatal
intoxication is produced by benzodiazepines
in the absence of other drugs with CNSdepressant actions; an important exception
is midazolam, which has been associated
with decreased tidal volume and respiratory
rate” (Goodman & Gilman)
0.1 - 0.4 mg/kg/24 hr IV/SQ
Common Concerns About Aggressive Use
of Opioids at End-Of-Life
• How do you know that the aggressive use
of opioids for dyspnea doesn't actually bring
about or speed up the patient's death?
• “I gave the last dose of morphine and he
died a few minutes later… did the
medication cause the death?”
1. Literature: the literature supports that opioids
administered in doses proportionate to the degree
of distress do not hasten death and may in fact
delay death
2. Clinical context: breathing patterns usually seen in
progression towards dying (clusters with apnea,
irreg. pattern) vs. opioid effects (progressive
slowing, regular breathing; pinpoint pupils)
3. Medication history: usually “the last dose” is the
same as those given throughout recent hours/days,
and was well tolerated
Typically, with excessive opioid dosing one
would see:
• pinpoint pupils
• gradual slowing of the respiratory rate
• breathing is deep (though may be shallow) and regular
COMMON BREATHING PATTERNS
IN THE FINAL HOURS
Cheyne-Stokes
Rapid, shallow
“Agonal” / Ataxic
Palliative Management
of Secretions
Managing Secretions in Palliative Patients
 Factors influencing approach management:
 Oral secretions vs. lower respiratory
 Level of alertness and expectations thereof
 Proximity of expected death
 “Death Rattle” – up to 50% in final hours of life
 At times the issue is more one of creating an environment less
upsetting to visiting family/friends
 Suctioning: “If you can see it, you can suction it”
Suctioning
Increased
Secretions
Mucosal
Trauma
Secretions - Prevalence At Study Entry And In Last Month Of Life
UK Children’s Cancer Study Group/Paediatric Oncology Nurses Forum Survey
Goldman A et al; Pediatrics 2006; 117; 1179-1186
Atropine Eye Drops
For Palliative Management Of Secretions
• Atropine 1% ophthalmic preparation
• Local oral effect for excessive salivation/drooling
• Dose is usually 1 – 2 drops SL or buccal q6h prn
• There may be systemic absorption… watch for
tachycardia, flushing
Glycopyrrolate
For Palliative Management Of Secretions
• Less sedating than scopolamine (doesn’t cross the blood-brain
barrier), longer acting, however not as effective
• Useful where patient is still alert; scopolamine will cause sedation
and delirium in awake patients
Enteral: 40 – 100 micrograms/kg 3 – 4 times daily
Refs:
• 2006 British National Formulary For Children
• IWK Health Centre (Halifax) Formulary
Parenteral: 4 – 10 micrograms/kg 3 – 4 times daily (1/10th the enteral dose)
Ref:
IWK Health Centre (Halifax) Formulary
Scopolamine
For Palliative Management Of Secretions
Ref: 2007 British National Formulary For Children
Transderm-V ® (Scopolamine)
Age
Dose
1 month – 3 yrs
250 micrograms every 72 hours (1/4 patch)
3 – 10 yrs
500 micrograms every 72 hours (1/2 patch)
10 – 18 yrs
1 mg every 72 hours (one patch)
Intermittent SQ/IV: 6-10 micrograms/kg (max. 600 micrograms) q 4h
Continuous SQ/IV: 40-60 microgram/kg/day (1.67 – 2.5 microgram/kg/h)
Ref: 2006 Rainbow Hospice Guidelines