pearls

1
Pearls in Pediatric Palliative Care
All You Really Need to Know
2
My Background

• Pediatric Residency, Maine Medical Center
• Pediatric Oncology Fellowship, UCONN
• Maine Childrens Cancer Program, 1985-1998
• General Pediatrics, 1998-Present
• ABP, EPEC, ABHPM Certified
• Medical Director, JP, 1999-present
• SMMC Chief of Pediatrics, 2001-present

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Outline

• Medical
• What is palliative care?
• The right to be free from pain
• The right dose is the dose that works
• When all else fails
• Two roads to death - be prepared
• Newborns feel pain, too
• Psychosocial
• Breaking bad news
• Hope
• Miracles
• Listen to the children

4
The Broad Need for Services

• Statistics pediatric deaths from chronic
  non-malignant diseases (CDC, US DHHS, NCHS, OAE)
• USA total 1999 33,089 (2517 Cancer)
• Maine total 1999 84 (14 Cancer)
• USA total 1979-1998 45,030/yr (3069 Ca)
• Maine total 1979-1998 151/yr (14 Cancer)

From CDC Wonder (wonder.cdc.gov) WONDER
provides a single point of access to a wide
variety of reports and numeric public health
data.
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Cure vs. Palliation

• Cure
• fundamental hope is eradication of disease
• assumes cure is worth a sacrifice
• Palliation
• fundamental hope is comfort
• consequences of any intervention that relieves
  suffering are acceptable
• Skills gap

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A Better Viewpoint
Curative / Life-Prolonging Therapy
Presentation
Death
Relieve Suffering - Palliative Care
7
Integrating Curative Palliative Care It Can
Be DoneGail Austin Cooney, MD, FAAHPM

• A vision for best practices would include the
  concept of integrated care that would provide
  these ideals
• Cure when possible
• Disease modification and management
• Informed decision making
• Coordinated care
• Symptom control a priority
• Optimize quality of life
• End-of-life care

8
Pain Philosophy

• Freedom From Pain A Matter of Rights?
• T. Patrick Hill, M.A.
  Ca. Invest., 12 (4), 1994
• Pain Isolates We are probably never more alone
  than when severe pain invades us.
• Pain is Elusive Despite the fact that it is the
  result of biochemical processes, it is also ... a
  subjective experience, felt only within the
  confines of our individual minds.

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Pain Philosophy

• Pain is unlike disease, and that to treat its
  symptoms clinically, physicians need above all to
  understand how the ravages of pain can reach
  beyond the body to the soul of the person,
  assaulting its very integrity.
• There exists a principle on which rests the
  human right to be free of pain and the
  corresponding obligation of health-care
  professionals to honor it. All patients are
  vulnerable, but none is more vulnerable than the
  patient in severe pain. The measure of medicine
  in general and of a physician in particular is
  ultimately their respect for the patients right
  to be free of pain.

10
Barriers to Effective Opioid Use
- Weissman, David E. Home Health Care Consultant
9/1995

• ... the most pervasive and difficult to
  overcome relate to the fears among patients,
  families, and health professionals of opioid
  analgesics, which are the cornerstone of drug
  therapy for moderate to severe pain.
• These fears include an exaggerated estimation of
  opioid addiction and tolerance, fear of opioid
  side effects -- most notably respiratory
  depression -- and ethical and regulatory concerns
  about using opioids.

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The Right Dose - Legal

• Maine Board of Licensure in Medicine
• Chapter 11Use of Controlled Substances for
  Treatment of Pain
• -- March 22, 1999
• Preamble The Boards recognize that principles of
  quality medical practice dictate that the people
  of the State of Maine have access to appropriate
  and effective pain relief.
• The Boards encourage physicians to view effective
  pain management as a part of quality medical
  practice for all patients with pain, acute or
  chronic, and as especially important for patients
  who experience pain as a result of a terminal
  illness.

12
State of the Art

• Federal State Model Policy for the Use of
  Controlled Substances for the Treatment of Pain
  -- May, 2004All physicians should become
  knowledgeable about effective methods of pain
  treatment, as well as statutory requirements for
  prescribing controlled substances. Accordingly,
  this policy have been developed to clarify the
  Boards position on pain control and to
  alleviate physician uncertainty and to encourage
  better pain management.Appropriate pain
  management is the treating physicians
  responsibility. As such, the Board will consider
  the inappropriate treatment of pain to be a
  departure from standards of practice and will
  investigate such allegations, recognizing that
  some types of pain cannot be completely relieved,
  and taking into account whether the treatment is
  appropriate for the diagnosis.

13
Progress?

• In August 2004, the Drug Enforcement
  Administration (DEA) published on its Office of
  Diversion Control Web site a document entitled
  "PRESCRIPTION PAIN MEDICATIONS Frequently Asked
  Questions and Answers for Health Care
  Professionals and Law Enforcement Personnel." The
  document contained misstatements and has
  therefore been removed from the DEA Web site. DEA
  wishes to emphasize that the document was not
  approved as an official statement of the agency
  and did not and does not have the force and
  effect of law.DEA recognizes that the proper
  use of controlled substances in the treatment of
  pain remains an extremely important issue.
  Accordingly, DEA intends to address this matter
  in the future.-- Drug Enforcement
  Administration, 2004

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Who is Helping the DEA?

• 21 Health Organizations
• University of Wisconsin Comprehensive Cancer
  Center
• American Academy of Pain Medicine
• Federation of State Medical Boards
• Jason Program Website

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Bottom Line

• Do what is right for your patient (and you)
• Communicate effectively
• Assess the medical situation carefully
• Provide whatever is needed for pain relief
• Consider abusive personalities
• Consider signing a contract
• Arrange follow-up appropriately

All in 15 minutes
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The Right Dose - Medical

• Opioids The right dose is the dose that works
• Pain and the Reticular Activating System The
  respiratory depressant effect of opioid agonists
  can be demonstrated easily in volunteer studies.
  When the dose of morphine is titrated against a
  patients pain, however, clinically important
  respiratory depression does not occur. This
  appears to be becausepain acts as a
  physiological antagonist to the central
  depression effects of morphine.
• Wall, R.D., ed. Textbook of Pain. Churchill
  Livingstone
• Naive Pts. vs. Tolerance

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Parenteral Narcotics

• Morphine
• 0.1 mg/kg IV bolus, Q 1-2hr
• 0.05 mg/ kg/hr, CI - IV or SQ
• Hydromorphone (Dilaudid)
• Approximately 6 times stronger than morphine
• Fentanyl
• Approximately 10 times stronger than morphine
• Wide dosing range
• 1-2 mcg/kg IV slow push
• 0.5-1.0 mcg/kg/hr, CI - IV or SQ
• Total hourly dose as a transderm patch

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Patient-Controlled Analgesia

• Age gt 4 years (if able to play computer games)
• Home or Hospital
• Adequate observation

Medication Base Rate Bolus Dose
Lockout Max/Hr Morphine .03 mg/kg
Same 6-10 min .15 mg/kg Dilaudid
5 mcg/kg Same 6-10 min
25 mcg/kg Fentanyl 1 mcg/kg Same
6-10 min 4 mcg/kg
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Equianalgesic Narcotic DosingSource McCaffery
M, Pasero C. PAIN Clinical Manual, 2nd
Edition, Harcort Health Sciences Website, 2000.
www.harcourthealth.com/PAIN/index.html
Oral/Rectal Dose (mg) Analgesic Parenteral Dose (mg)
3 Morphine 1
20 Codeine 12
3 Hydrocodone --
0.75 Hydromorphone 0.15-(0.3 w/ PCA)
2 Oxycodone --
2 Methadone 1
25 mcg/hr Fentanyl Patch 1 mg/hr IV MSO4
-- Fentanyl 10-20 mcg
XX Meperidine XX
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When All Else Fails

• 16 year old girl, 50 kg, with far advanced
  abdominal malignancy and intestinal obstruction.
  Receiving morphine at 100 mg/hr without relief.
  Her parents would like her to be awake for the
  arrival of a relative tomorrow, but dont want
  her to suffer.

21
(continued)

• You have increased the morphine, and the pain is
  well controlled, but severe nausea and dark,
  bilious vomiting begins. You have placed an NG
  tube to drainage, and tried Zofran, Trilafon, and
  Octreotide, all without relief. Pain escalates
  despite morphine at 1000 mg/hr, with further
  increases ineffective.
• What are your options?

22
(continued)

• Discuss goals of care
• Try a different opioid
• Add an anxiolytic
• Explore psychologic religious issues
• Surgical and radiotherapy consults to relieve
  obstruction
• Pain consult
• Inform patient family there are no further
  options

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Comfort Sedation

• Barbiturates in the Care of the Terminally
  IllTruog, Robert D., et. al. NEJM, Vol. 327, No.
  23, 1678-81

• Barbiturates
• Reliably produce sedation and unconsciousness
• Are used in the execution of prisoners by lethal
  injection
• Ethical Considerations
• The Principle of Double Effect --
  Distinction between intended effects and
  unintended although foreseen effects.
• Commonly called terminal sedation

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Barbiturates Are Justified

• To relieve physical suffering when all reasonable
  alternatives have failed
• To produce unconsciousness before terminal
  extubation
• Produce deep sedation and unconsciousness as a
  means of relieving nonphysical suffering

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Terminal Sedation Opinions

• Terminal sedation ethical implications in
  different situations - Hallenbeck JL. J Palliat
  Med. 2000 Fall3(3)313-20
• Sedation is supported in a case of severe
  distress in a patient very close to death, but
  not in a case of of psychic distress
• In providing sedation the physician's primary
  intent should be to alleviate suffering -
  Sedatives should be titrated to observable signs
  of distress
• Not all suffering is appropriately treated with
  sedation

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Terminal Sedation Opinions

• Family experience with palliative sedation
  therapy for terminally ill cancer patients -
• J Pain Symptom Manage. 2004 Dec28(6)557-65
• Although the majority of families were
  comfortable with this practice, clinicians should
  minimize family distress by regular monitoring of
  patient distress and timely modification of
  sedation protocols, providing sufficient
  information, sharing the responsibility of the
  decision, facilitating grief and providing
  emotional support

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On Our Own Terms- Bill Moyers

• In rare cases some patients who are very ill do
  not respond to pain medications or may be
  suffering in other ways that make comfort
  impossible. In these circumstances there is a
  last resort therapy that can be used terminal
  sedation. With terminal sedation, a patient will
  be given medications that induce sleep or
  unconsciousness until such time as death occurs
  as a result of the underlying illness or disease.
  These measures are often accompanied by the
  withholding of artificial life supports like
  intravenous feeding and artificial respiration.
  Like the use of medications that cause a "double
  effect," the intention with terminal sedation
  must be to relieve suffering only, not to cause
  death.

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Last Days of Living - Medical Aspects

• Weakness Fatigue
• Dehydration
• Respiratory Distress
• Temperature Changes
• Increased Secretions
• Pain May Increase
• Anxiety
• Two Roads to Death

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Two Roads to Death
Difficult
Confused
Tremulous
Restless
Hallucinations
Usual
Delirium
Myoclonic Jerks
Sleepy
Lethargic
Seizures
Obtunded
Comatose
Death
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Last Days of Living - Social Aspects

• Family Preparation
• DNR
• Letting Go
• MD Presence at Time of Death
• Mechanism of Death
• Autopsy
• Follow-up

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Pain in Newborns -- Compassion Common Sense
Yeah, Baby!
32
State of the Art
Prevention and Management of Pain Stress in the
Neonate -- American Academy of
Pediatrics Committee on Fetus and
NewbornCommittee on DrugsSection on
AnesthesiologySection on Surgery -- Pediatrics
Volume 105, Number 2 February 2000, pp 454-461
33
Conclusion

• Management of pain must be considered an
  important component of the health care provided
  to all neonates, regardless of their gestational
  age or severity of illness.

34
Fundamentals

• Pain in newborns is often unrecognized and
  undertreated.
• If a procedure is painful in adults, it should be
  considered painful in newborns, even if they are
  preterm.
• Compared with older age groups, newborns may
  experience a greater sensitivity to pain and are
  more susceptible to the long-term effects of
  painful stimulation.
• Adequate treatment of pain may be associatedwith
  decreased clinical complicationsand decreased
  mortality.

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Neonatal Pain Scales

• Validated and Reliable Scales Exist
• Measure Physiologic Parameters
• Heart rate, resp rate, BP, O2 sats,
  sweating,vagal tone, plasma cortisol catechols
• Measure Behavioral Parameters
• Facial expressions, body movements, crying
• Examples

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CRIES Scale

• Crying
• Requirement for oxygen (to keep SaO2 gt95)
• Increased heart rate and BP
• Expression
• Sleeplessness
• Inter-rater reliability gt.72

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CRIES Scale
0 1 2
Crying No High PitchConsolable Inconsolable
FiO2 No lt 30 gt 30
Increased Heart Rate BP No 11-20 ? gt 20 ?
Expression Calm Grimace Grimace Grunt
Sleepless No Frequent Awakening Constantly Awake
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PIPP Scale -Premature Infant Pain Profile

• Facial Actions
• Brow bulge
• Eye squeeze
• Nasolabial furrow
• Physiological Indicators
• Heart rate
• Oxygen saturation
• Context
• Gestational age
• Behavioral state
• Inter-rater reliability gt.93

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PIPP Scale - part 1
Indicator Finding Points
Gestational age gt 36 weeks 0
32 weeks to 35 weeks 6 days 1
28 weeks to 31 weeks 6 days 2
lt 28 weeks 3
Behavioral state active/awake eyes open facial movements 0
quiet/awake eyes open no facial movements 1
active/sleep eyes closed facial movements 2
quiet/sleep eyes closed no facial movements 3
Maximum Heart Rate 0-4 beats per minute increase 0
5-14 beats per minute increase 1
15-24 beats/minute 2
gt 25 beats/minute 3
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Indicator Finding Points
Minimum O2 sat 0-2.4 decrease 0
2.5-4.9 decrease 1
5-7.4 decrease 2
gt 7.5 decrease 3
Brow bulge lt 9 of time 0
10-39 of time 1
40-69 of time 2
gt 70 of time 3
Eye Squeeze lt 9 of time 0
10-39 of time 1
40-69 of time 2
gt 70 of time 3
Nasolabial furrow lt 9 of time 0
10-39 of time 1
40-69 of time 2
gt 70 of time 3
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PIPP Scoring
PIPP Score SUM (points for all 7
indicators) Interpretation minimum score 0
maximum score 21 The higher the score the
greater the pain behavior Reference Stevens B
Johnston C et al. Premature Infant Pain Profile
Development and initial validation. Clinical
Journal of Pain. 1996 12 13-
42
COMFORT Scale

• Developed at Erasmus MC-Sophia Netherlands
• Designed for post-surgical infants 0-3 yrs old
• Complicated but Effective
• Assessment form
• Treatment Algorithm

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Barriers

• Indifferent medical staff who regard pain
  assessment as a nursing issue only. Medical staff
  commitment is essential, however, because they
  have to develop an analgesic algorithm linking
  pain assessment to pain treatment. Furthermore,
  they should have a genuine interest in patients
  pain scores during daily rounds.
• No time or money to appoint one or more contacts
  on the ward, i.e. staff taking care of training
  of all nurses, and reaching a common decision
  with regards to frequency and patient population
  when pain assessment should take place.

44
NonpharmacologicTreatment 0f Procedural Pain in
Infants
45
Avoid Painful Procedures

• Painful procedures should be minimized and, when
  appropriate, coordinated with other aspects of
  the neonates care.
• Obtaining blood by venipuncture may be less
  painful than heel lancing.56-58
• Consider implanted catheters

46
Endogenous Analgesia

• Generalized tactile
• Touch ( swaddling)
• Orotactile
• Touch suckling
• Orogustatory
• Touch feeding

47
Tactile skin-skin contact
Contact
Percent of time
Control
Grimace Cry
Gray, et al, Pediatrics 2000
48
Non-nutritive sucking

• Tested during heelstick procedure
• Heelstick caused no effect on respiratory rate
  and oxygen saturations
• Sucking reduced time of crying and heart rate
  increases
• --Corbo, et al. Biol Neonate, 2000

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Effect of Oral Sucrose Solutionon Venipuncture
Pain
Abad, et al, Acta Paediatr, 1996
Time crying (sec)
50
Breastfeeding is Analgesic in Healthy Newborns
-- Gray, et.al, Pediatrics Vol. 109,
No. 4, April 2002
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Analgesic Medications
52
(No Transcript)
53
Outline

• Medical
• The right to be free from pain
• The right dose is the dose that works
• When all else fails
• Two roads to death - be prepared
• Newborns feel pain, too
• Psychosocial
• Breaking bad news
• Hope
• Miracles
• Listen to the children

54
Breaking Bad News

• From How to Break Bad News, by Robert Buckman,
  MD, 1992
• Bad news is any information whose consequences
  are worse than the patients expectations
• Why this topic is important

55
Should We Tell the Truth?

• 1672 -- Samuel de SorbiereTruth is a good idea
  that wont catch on.
• 1960s -- 90 of MDs preferred not to inform
  their patients of a diagnosis of cancer. Methods
  of deceitwere published. Truth destroys hope.
• 1980s --50-97 of pts want to know the truth.
• Now --Patients have absolute rights to their
  medical information. Physician practices have
  changed. How to tell is the debate.

56
Empathy - Respond to patients feelings

• Steps
• Identify the emotion open-ended questions
• Identify its cause
• Respond in a way that shows you understand the
  connection between 1 and 2
• ? Example

57
Planning

• A reasonable plan the patient will follow is
  better than an ideal plan the patient will
  ignore.
• Any plan is far better than no plan.

58
Hope Never Leaves

• What is Hope?
• Why Hope is Important
• Helping Your Patients

59
Definitions

• To look forward to with confidence or
  expectation
• "Beware how you take away hope from another
  human being."- Oliver Wendell Holmes, 1809-1894

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Hope Its Spheres Dimensions
-- Dufault, K. and Martocchio, B. NursClinNA. 1985
Affective
Contextual
General
Particular
Cognitive
Temporal
Behavioral
Affiliative
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The Neurobiology of Hope
-- Gottschalk, L.A., et.al., The Cerebral
Neurobiology of Hope and Hopelessness.
Psychiatry, V.56, Aug 1993
Activity Regions of Hope Memory - Frontal Cortex
Language - Temporal Perception - Parietal
Vision - Occipital Emotions Hippocampus ?
Psychopathology -
62
Hope Persists

• different stages that people go through when
  they are faced with tragic news --coping
  mechanisms to deal with extremely difficult
  situations. These means will last for different
  periods of time and will replace each other.
• The one thing that usually persists through all
  these stages is hope.
• Just as children in the concentration camp of
  Terezin maintained their hope years ago, although
  out of a total of about 15,000 childrenonly
  around 100 came out of it alive.
• Elisabeth Kubler-Ross, MD On Death and Dying,
  1969

63
Ever-Present Hope

• we were always impressed that even the most
  accepting, the most realistic patients left the
  possibility open for some cure.It is this
  glimpse of hope which maintains them through
  days, weeks, or months of suffering. It is the
  feeling that this all must have some
  meaning.(or) that this is just like a nightmare
  and not trueIt gives the terminally ill a sense
  of a special mission in life which helps them
  maintain their spirits for others it remains a
  form of temporary but needed denial.
• Elisabeth Kubler-Ross, MD On Death and Dying,
  1969

64
Ever-Present Hope

• Everybody has it
• It allows them to live
• Hope is required to experience life during the
  process of dying
• It has two forms
• Provides meaning in illness death
• Provides temporary denial

65
Helping Your Patients

• Remember that there is no greater loss than the
  loss of a child
• Allow for Hope and Share It
• Tell the truth (Miracles Do Exist)
• Hope Shifts
• Timing
• Embrace Life

66
Allow for Hope

• we found that all our patients maintained a
  little bit of it (hope) and were nourished by it
  in especially difficult times. The showed the
  greatest confidence in the doctors who allowed
  for such hope realistic or not an appreciated
  it when hope was offered in spite of bad
  news.While we maintained hope with them, we did
  not reinforce hope when they finally gave it up,
  not with despair but in a stage of final
  acceptance.
• Elisabeth Kubler-Ross, MD On Death and Dying,
  1969

67
Miracles Do Exist - I

• JS, 4 year old boy with stage IV neuroblastoma,
  considered incurable. Chemotherapy achieves a
  partial response. Bone marrow transplant
  performed, and an abdominal mass is found only
  three weeks later. Biopsy shows neuroblastoma. No
  further therapy. JS is alive and well without
  disease 5 years later, past his Collins risk
  period for relapse.

68
Miracles Do Exist - II

• CB, 11 year old boy with ALL. Induction therapy
  fails. Told he was incurable. He receives
  intensified, investigational treatment, which
  fails. Told he was incurable. Palliative steroids
  were given, and he enters remission, but develops
  a systemic aspergillos infection. Told it was
  incurable. Receives an investigational antifungal
  agent and clears the infection. He then receives
  a bone marrow transplant, but 6 months later
  relapses. Told it was incurable. He was treated
  with palliative therapy, and enters remission. He
  then relapses after 6 months of more treatment.
  We decided we wouldnt tell him he was incurable,
  because he might think we were stupid. He lives
  another year with good quality of life before
  dying of his disease.
• --
  Listen to the Children

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Hope Shifts

• We May Assume
• Hope for Survival
• Western Medicine
• Life is not worth living if one is bedridden
• Comatose people experience nothing

• What Can Be
• Hope For Survival
• Alternative Medicine
• Miracles
• Comfort
• Peace for self others
• Events
• Babys Birth, Wedding
• Hope for Hope

70
Timing

• Be aware that life is dynamic
• Stages of Facing Tragic News
• Denial and Isolation
• Anger
• Bargaining
• Depression
• Acceptance
• I dont feel like talking now
• Managing Unrealistic Hope

71
Embrace Life

• Just Living
• Building Memories
• No Regrets
• Relationships Strengthening, Creating, Mending

72
Thanks for Listening
The Jason Program13 Industrial Park RoadSaco,
Maine 04072 Phone [phone]Fax (207)
294-8257
Visit us on the web at www.jasonprogram.org Files
available at www.jasonprogram.org/teaching
73
Thanks for Listening

• Be the practitioner you would want if you were
  sick