Caring for Children with Life-Threatening

Illness: Can we do better?

Joanne Wolfe, MD, MPHDivision Chief, Pediatric Palliative Care

Department of Psychosocial Oncology and Palliative CareDana-Farber Cancer Institute

Director, Pediatric Palliative CareChildren’s Hospital Boston

Disclosures

• No financial interests to disclose.

Objectives

Part IScope of Pediatric Palliative Care (PPC)Scope of SufferingPart IIThe Boston PPC ExperiencePart IIIAdapting the PPC Model to Low and Middle Income Countries

Part I

15-19 years25.3%

10-14 years7.6%

5-9 years6.4%

1-4 years9.6%

Postneonatal16.9%

Neonatal34.3%

In the US, 500,000 children live with complex chronic conditions

55,000 children ages 0 to 19 die annually

IOM report 2003, NEJM 2004

Percentage of total childhood deaths by major causes

Unintentional Injuries22%

Homicide & Suicide8%

Other33%

Cancer4%

Respiratory Distress2%

Heart Disease2%

SIDS5%

Short Gestation8%

Complications of Pregnancy

2%

Congenital Anomalies12%

Placental Cord Membranes

2%

IOM report 2003

Diagnoses in patients receiving PPC

0

5

10

15

20

25

30

35

40

45

Genet

ic/Con

genita

l

Neurom

uscula

r

Cancer

Respira

tory

Other

Gastro

intes

tinal

Cardiov

ascu

lar

Renal

Imm

unolog

ic

Per

cen

t

Feudtner et al, Pediatrics 2011

Survival of patients receiving PPC

Feudtner et al, Pediatrics 2011

Feudtner et al, Pediatrics 2011

Signs and symptoms of patients receiving PPC services

Drugs received by patients receiving PPC

Feudtner et al, Pediatrics 2011

Technology in patients receiving PPC

0

10

20

30

40

50

60

70P

erce

nt

Feudtner et al, Pediatrics 2011

Hope for cure, life extension, a miracle…

Hope for comfort, meaning…

Individualized blending of care directed

at underlying illness

and

physical, emotional, social, and spiritual

needs of child and family

with continuous reevaluation and adjustment

End-of-life care

Bereavement care

Pediatric Palliative Care

Suffering

Suffering is a specific state of distress that occurs when the intactness or integrity of the person is threatened or disrupted. It lasts until the threat is gone or integrity is restored.

The meanings and the fear are personal and individual, so that even if two patients have the same symptoms, their suffering would be different.

Eric Cassel, MD

“Visible” threatsLife-threatening illnessSymptomsEmotional Factors

“Invisible” threatsDisruptions from "normal life”Emotional factorsExistential concernsSocio-demographic concerns

SUFFERING

A Framework of Suffering

PATIENT

PARENTS

Family’s Self

Integrity

SIBLINGS

PainFatigueDrowsy

IrritabilityNausea

Anorexia Sleep

DiarrheaVomiting

NervousnessSadness

CoughWorrying

ConcentrationItching

Skin issuesConstipation

DyspneaImage

Dry mouthNumbness

SweatingDysphagia

Dysuria

0.0% 10.0% 20.0% 30.0% 40.0% 50.0% 60.0%

Child self-reported symptoms 20 weeks of follow upPrevalence and proportion with moderate to severe distress

(n=704 surveys)

Prevalence

• Study to detail the everyday occurrence of pain in non-communicating children with cognitive impairment

• 34 parents completed daily pain diaries for two weeks, each day for 5 defined periods rating whether their child had been in pain and its duration and severity.

• Results– 74% of children experienced pain on at least one day– 68% with moderate or severe pain on at least one

day– 12% with mod-severe pain lasting greater than 30

minutes on 5 or more days– NO CHILD WAS RECEIVING PAIN MEDICATION

Distress in cognitively impaired, non-communicating children

Stallard et al Arch Dis Child 2001

Additional Domains of Family Suffering• Spiritual distress (Robinson, Pediatrics 2006)

• Psychological distress (Rosenberg, JAMA Pediatrics 2013)

• Financial distress (Dussel, JCO 2011)

Parental Psychological Distress

Rosenberg et al, JAMA Pediatrics 2013

Family Financial Distress

Bona et al, J Pain Sx Mgmt 2013

“Visible” threatsLife-threatening illnessSymptomsEmotional Factors

“Invisible” threatsDisruptions from "normal life”Emotional factorsExistential concernsSocio-demographic concerns

SUFFERING

Targeted Interventions(e.g. symptom treatment trials)

Global Interventions(e.g. MA PPC Network)

A Framework for Easing Suffering

PATIENT

PARENTS

Family Integrity

PATIENT

SIBLINGSPARENTS

“NEW”Family

Integrity

SIBLINGS

Part II

Joanne Wolfe, MD, MPH

The Boston Pediatric Palliative Care

Experience

Core Ideals

Meaningful Experiences

Open Communication

Intensive Symptom

Management

Timely Access to Care

Flexibility

Psychosocial and Spiritual

Support

Patient Oriented,

Family Oriented

Any place, Any time

Interdisciplinary Team

Patient and

Family

Physician

Nurse Practitioner

Nurse

ChaplainSocial Worker

Pharmacist

Child-life Therapist

Primary vs Subspecialty PPC

• Primary– Team provides interdisciplinary support

• SubspecialtyClinical: more complex care

Education: enhance knowledge, skills, behaviors and attitudes

Innovation and research: advancing the field

Advocacy: system wide changes

PACT Beginnings

• 1997: Demonstration project in pediatric oncology

• 2001: Expanded to care for children with non-cancer diagnoses

• 2003: Improved outcomes in children with cancer, greater institutional support

• 2003: Fellowship program

PACT Strategies• Consults• Education/Training

– Across all disciplines– Interdisciplinary fellowship

programs– Visitor program

• Interventions– Direct admission policy– Emergency medication work sheet– Inpatient orders for uncontrolled

pain, dyspnea and/or agitation– “Intervention orders for persons

with advanced Illness” – Comfort Corner

• Bereavement programs– Family– Caregivers

• Research program

PACT Physician Fellows

Jenny Mack Fellowship Director, Pediatric Hematology/Oncology DFCHCC, MA

Craig Hurwitz Director, Palliative Care, Dell Children’s Hospital, Austin, TX

Todd Pearson Medical Director, Pediatric Palliative Care, Cook Children’s Medical Hospital, Fort Worth TX

Christina Ullrich PACT and Stem Cell Transplant Physician, DFCHCC

Julie Hauer Medical Director, Seven Hills Pediatric Center, MA

Bradd Hemker Palliative Care and Heme/Onc Attending, Helen DeVos Children's Hospital, Grand Rapids, MI

Tamara Vern-Gross Proton Beam Fellow, Wake Forest, Baptist Health, NC

Jennifer Linebarger Medical Director, Pediatric Palliative Care, Children’s Mercy Hospitals and Clinics, Kansas City, MO

Kate Nelson Health Services Research Fellow, Hospital for Sick Children, Toronto, ON

Kevin Weingarten Palliative Care Physician and Medical Ethics Fellow, Hospital for Sick Children, Toronto, ON

Elisha Waldman Medical Director, Pediatric Palliative Care, Hadassah Hospital, Jerusalem, Israel

Todd Barrett Adult and Pediatric Palliative Care, Cedars-Sinai, Los Angeles, CA

PACT Social Work Fellows

Kristen Caminiti Social Worker, Children's National Medical Center, Washington DC

Katie Sweeney Social Worker, Connecticut Hospice, CT

Kate Lindy Social Worker, Intermediate Care Unit, BCH, MA

Arden O’Donnell Social Worker, Adult Palliative Care, DFCI/BWH, MA

Kate Evans Social Worker, Pediatric Hospice, Denver ,CO

Nicholas Purol Social Worker, Merrimack Valley Hospice, MA

Jordan Miraglia Social Worker and Bereavement Coordinator, Hands of Hope, Hospice Care of South Carolina, SC

Bryce Moffet Social Worker, Boston Health Care for the Homeless, MA

PACT Nurse Practitioner Fellows

Ashley Atkins Pediatric NP, Joslin Diabetes Center, Boston, MA

Gaby Harrison Pediatric NP, Merrimack Valley Hospice, MA

Meghan Tracewski Pediatric NP, Pediatric Advanced Care team, Children’s Health Care of Atlanta, GA

Anna Roche Pediatric NP, DFCHCC, MA

Macallagh McEvoy Pediatric NP, Seattle Children’s Hospital, WA

Core Team MembersPhysicians (2.6 FTE) Nurse practitioner (2.0 FTE)Social worker (1 FTE)Administrators (2.0 FTE)Trainees:

Physician Palliative care fellow (2 FTE)Nurse practitioner fellow (1 FTE)Social work fellow (1 FTE)

PACT Interdisciplinary TeamCommittee MembersICU, NICU & BMT nursesAmbulatory clinic nursesPatient Care CoordinatorParent representativesPain Team SpecialistIntensivist/Coordinated Care physicianPsychiatristPhysical TherapistPsychologistSocial workerChaplainChild-life specialistsPharmacistCommunication enhancement

specialistGenetic Counselor

DFCI/CHB PACT Clinical Activity

FY 01

FY 02

FY 03

FY 04

FY 05

FY 06

FY 07

FY 08

FY 09

FY10

FY11

FY12

13A0

100

200

300

400

500

600

700

New Consults On-Going

Total Patient Census Deaths

Referring Services FY 2012 173 New Patients

Onc; 47; 27%

Pulm; 5; 3%

Gen & Metab; 22; 13%

Cardio; 30; 17%

Neuro; 63; 36%

Other; 7; 4%

Patient/ family and provider satisfaction

Data source: Family satisfaction survey as of 2/10/12, n=39

Aspect of Care Weighted Average (1-5, 5 is highest score)

Concern and caring to child 4.9

Symptom management 4.3

Emotional support to child 4.6

Concern and caring to family 4.9

Emotional support to family 4.4

Included in decision-making 4.7

Recommend PACT 3.9 (1-4, 4 is highest score)

• Example scores from the patient/ family satisfaction survey

• Example scores from the provider satisfaction survey

Aspect of Care Weighted Average

How valuable is it to have PACT 4.82 (1-5, 5 is highest score)

Would you recommend PACT to a colleague 3.87 (1-4, 4 is highest score)

Aspect of Care Weighted Average (1-5, 5 is highest score)

Concurrent consultation by PACT No PACT consultation

Management of physical symptoms such as pain, nausea, dyspnea 4.33 3.88

Management of psychosocial distress of patients and families 4.36 3.92

FatiguePain

Dyspnea

Poor Appetite

Nausea/vomiting

ConstipationDiarrh

ea0

20

40

60

80

100

Per

cen

t of

Ch

ildre

n

Symptom Prevalence Suffering

Wolfe et al. NEJM 2000.

Before

After…

PPC Saves Resources!Results of Pre-Post Analysis

Rates Pre-Service  Post-Service  p Value 

Inpatient Stays/year (95% CI) 4.64 (4.3, 5.0) 3.72 ( 3.4, 4.1) <0.001

ED visits/year (95% CI) 0.89 ( 0.7, 1.1) 0.57 ( 0.4, 0.7) 0.003

Bed Days/year (95% CI) 44.17 ( 43.1, 45.3) 42.37 ( 41.3, 43.4) 0.019

Inpatient Stays/year

ED Visits/year0.0

1.0

2.0

3.0

4.0

5.0

Pre-Service Service

Rate

/yea

r

Bed Days/year40.0

42.0

44.0

46.0

48.0

50.0

Pre-Service Service

Rate

/yea

r

PACT Pearls…

What are the five cardinal questions?

Goals of Care Discussion

1. Tell us about your child, what is s/he like as a person?

2. What is your understanding of your child’s illness?

3. In light of your understanding, what’s most important to you?

4. What are your hoping for? What are your worries?

5. Where do you find your strength?

Choose your words carefully

“Similar to scalpels for surgeons, words are the palliative care clinician’s greatest tools. Surgeons learn to use their tools with extreme precision, because any error can be devastating. So too should clinicians who rely on words”

Examples of Forbidden LanguageFrequently used phrases:

What would you like us to do?

The parents signed a DNR

The patient is DNR

Narcotics

Alternatives:

Based on my understanding of your goals, I would recommend…

A resuscitation status

order was placed in

the chart

Pain medications such

as morphine, opioids

Strategies to Ease Distress

Live as long as possible

Live as long as possible and as well as possible

Live as comfortably as possible

Differential diagnosis Differential diagnosis Differential diagnosis

Intensive assessment Targeted assessment Limited-target assessment

Intensive treatment Targeted treatment Flexible and at times empiric treatment

Intensive follow-up Intensive follow-up Intensive follow-up

Guided by Goals of Care

Partnering with Community

Massachusetts Pediatric Palliative Care Legislation May 2006

Monday, February 26, 2007

Help and hope

New palliative program offers ‘total approach to care’ for children being treated for serious illnesses – and their families…

PACT Advocacy – Massachusetts Pediatric Palliative Care Program

7

4

22

13

42

50

18

3512

18 5

Always maintain hope!

Part III

Knapp et al, Pediatric Blood Cancer 2011

Level 1 No known ppc activityLevel 2 Capacity building activity identifiedLevel 3 Localized provision of PPCLevel 4 Integration with main stream service providers

Levels of PPC provision around the world

Levels of PPC provision around the world

Knapp et al, Pediatric Blood Cancer 2011

Availability of palliative care services for children with cancer in economically diverse regions of the world

• 262 completed questionnaires from 58 countries (response rate, 59.8%)

• Regular users of Cure4Kids (St. Jude)

Delgado et al European J of Cancer 2010

Physician-reported availability of specialized services to their patients

Physician rated quality of palliative care elements

Physician responses to the following statements

Comfort care medications available to more than 50% of respondents patients

Study Question

1. Available resources.2. Availabiliy and access to pediatric specialists3. Availability and access to drugs (opioids and other

drugs).4. Degree of integration of palliative care practices into

pediatric cancer care• Symptom Control • Medical decision making processes• Care provided to families that need to migrate• Advanced care planning and care at the end of life• Location of death• Bereavement

5. Palliative care specialist’s role

Assessed Domains

Is palliative care provided to children with advanced cancer in Argentina?

Dussel et al

PPC Providers PPC Teams (n=8) PPC Specialists (n= 6) PPC providers with less than 2

years of experience (n= 7) Adult PC providers (n=3) “Obliged” PPC providers (n=5) No PPC provider identified (n=7)

Available Resources

Oncology teams PPC teams NGOs

Insufficient staff

Low interaction between oncology teams

(hematologists/oncologists)

Interaction with other teams:

is lower with pain specialists, PPC providers and social service

is higher with psychological area

High regional and institutional variability

High staff turnover (lack of funding, emotional impact of the task, etc.)

Low interaction between PPC teams across the country

Wide range of servicesDifferent agendas between

NGOs and health providers

Networking between NGOs and health providers improves their interaction:

> interaction> equity in access to services provided by NGOs

Resistence to NGOs presence in hospitals

Under utilization of NGOs resources

Available Resources

Opioid Availability

• Intravenous morphine was available in all institutions while oral morphine was available in 88% of visited centers and methadone in only 35% of institutions.

• Other opioid drugs useful for cancer pain treatment such as oxycodone were not available in any of the visited centers.

• Key informants identified at least two provinces that do not have opioids at all (not included in this sample).

• Specialists reported problems with treatment options, e.g. opioid rotation.

“We have few tools, here we have morphine, or morphine, and we have morphine as well ... or morphine ... In other words, I have nothing! If I have to rotate opioids, I can’t. At this moment I have one [patient] who suffers from hallucinations [secondary to morphine], she lives 70 km away and I don’t know how to help her; [silence] with the few tools we have, we do a lot.” (PPC specialist)

Summary

• PPC involves an extra layer of interdisciplinary support

• Simple strategies can go a long way• Internationally, there is much work to be

done to ensure that every child with a life-threatening illness has access to basic PPC.

Thank you

A Mission of Accompaniment

“It’s all very sad, but there’s a lot of collateral beauty along the way.”

Loorie Moore (Mother)