The Times They Have Changed:
30 years of Cardiac Care for Children
Stollery Children’s Hospital, October, 2013
Patricia O’Brien, MSN, CPNP-AC
Nurse Practitioner, Pediatric Cardiology
I have no disclosures
Introduction
Why Study History?
• Understand who we are and how we got to
where we are now
• Appreciate our history and proud heritage
• Shared identity as community
• Better understand change and discovery
“We study the past to understand the present,
we understand the present to guide the
future”
William Lund
Why Study History?
Founded in 1869
Opened 2001
New Building, 1988
Outline
• Using Tetralogy of Fallot as a theme,
discuss some important advances in the
care of children with cyanotic heart disease
• Discuss some important changes in nursing
over the course of my career
• Appreciate the varied paths to change and
discovery
• Brief look to the future
Tetralogy of Fallot
•
•
Most common
cyanotic heart defect
Described in 1888
1. Infundibular Pulmonic
Stenosis
2. Right ventricular
hypertrophy
3. Conoventricular VSD
4. Dextroposition of the Aorta
– Overrides VSD
Tetralogy of Fallot
• Several types:
– TOF with Pulmonic Stenosis
– TOF with Pulmonary Atresia
– TOF with Absent Pulmonary Valve
– TOF with Complete AV Canal
Tetralogy of Fallot
Blalock Taussig Shunt
Johns Hopkins, 1945
Blalock Taussig Shunt
Assigned to head pediatric
cardiology clinic,
Johns Hopkins, 1930
Became interested in
congenital heart disease
“Blue Baby syndrome”
Problem was lack of blood
flow to the lungs
Congenital Malformations of the
Heart,1960
Dr. Helen Taussig
1898-1986
Blalock Taussig Shunt
Dr. Blalock: chief of
surgery, Johns Hopkins
Vivien Thomas, surgical
Dr. Alfred Blalock
Vivien Thomas
assistant who advanced to
supervisor of the surgical
research labs
Together, they developed
the techniques and
instruments to perform the
Blalock Taussig shunt, first
performed in 1944
Blalock Taussig Shunt
Tetralogy of Fallot
• First complete repair, Lillehei, 1954
• 2 Stage repair
– BT shunt in infancy (not neonates)
– Complete repair at 3 years or older
• Primary repair in infancy
– 1970’s Barratt Boyes, Castaneda
• Modified BT shunt: using tube graft
– Described in 1962, not in wide use until 1980’s
Prostaglandins
Early 1970’s State of Cardiac Care
– Diagnostic tools: CXR, EKG, catheterization
– Cyanotic infants identified by appearance and
blood gases (as PDA closing)
– Emergent catheterization:
• Balloon atrial septostomy (1966) for TGA
• Shunt for obstructed PBF in hyperbaric chamber
– Some survivors
Prostaglandins
• Lipid compound derived from fatty acids
• Found in most tissues and organs
• Regulate contraction and relaxation of smooth
muscle
• Many uses: induce childbirth
– Prevent and treat peptic ulcers
– Pulmonary hypertension
– Glaucoma
– Promotion and resolution of inflammation
Prostaglandin E2
1973: Coceani and Olley
(Hosp. for Sick Kids, Toronto)
: PGE2 relaxed the PDA in
fetal lambs
: Published results in 4 neonates
used in the cath lab, all successful
(Circulation, 53, 1976)
England : Elliot (Lancet, 2, 1975)
: Rapid adoption for sick
neonates before publications
Prostaglandin E1
• Lifesaving for many infants
–
–
–
–
Increase pulmonary blood flow
Improve tissue oxygenation
Correct metabolic acidosis
Improve chance of successful surgery
• Best response in younger infants, lower PaO2
• Quickly tested in infants with IAA, CoA and
d-TGA
Prostaglandin E1
Rapid clinical use:
Alternative had high mortality
Dramatic clinical improvement
Easy to use
No barriers to obtaining drug
Clinical trial published after it
was used nationwide in 492
infants (Freed et al, Circulation 64, 1981)
Close Ductus Arteriosus
If you could open a PDA, you
could also close it!
Indomethacin:
Prostaglandin inhibitor
Effects of indomethacin in
premature infants with PDA
Studied in one of the first
multicenter trials in cardiology
(Gersony et al, J Pediatr 102, 1983)
My Career in Pediatric Cardiology
Graduated university,
BSN, 1977
CNS in pediatric cardiac
surgery, UCLA, 1982
Nurse practitioner,
Boston Children’s
Hospital, 1987
Pediatric Cardiology, 1980
•
•
•
•
Echocardiography in it’s infancy, 1980
No Arterial Switch procedure, 1982
No Stage 1 Norwood for HLHS, 1981
No interventional catheterizations
– First balloon dilation of PS, 1983
• No MRI, late 1990’s
• No ECMO, 1984
• No pediatric heart transplants, 1984
Hospitals, 1980
Large rooms, open wards
Much less technology
Paper based charts
IV pumps being developed
Limited parent visiting
Nurses did not round with
doctors
Technology in 1980
Still an analog world
Paper records
No cell phones
Computer technology
– Microsoft, 1975
– Apple, 1976
Personal computers
coming on the market
Pulse Oximetry
What is your O2 Saturation??
Pulse Oximetry
Measurement of transmitted
light through a translucent
measuring site to
determine oxygen
saturation
Oxygen rich hemoglobin
absorbs more infrared light
Pulse Oximetry
• 1930’s Germany ear oxygen meter
• 1940’s “oximeter”: light through a red filter
was oxygen sensitive
– Used in aviation and research
• 1970’s Aoyage, Japan
– First patent on pulse oximeter
• 1980’s Biox and Nellcor developed first
commercial machines in clinical use
Pulse Oximetry
• 1983: Evaluation of Pulse Oximetry.
(Yelderman and New, Anesthesia 59, 1983)
• 1988:
– Accuracy of Pulse Oximetry in Neonates
– Reliability in Hypoxic Infants
• By 1997: Pulse Oximetry recommended
as a 5th pediatric vital sign
(Mower et al, Pediatrics 99, 1997)
Pulse Oximetry
• Increased safety of anesthesia
– WHO now trying to have pulse oximeters in
every OR in the world
• Screening newborns for congenital heart
disease
– Now recommended in the US (Kemper, 2011)
– Measure right hand and one foot on DOL #2
• Home monitoring programs (Ghanayem, 2003)
– Decrease interstage mortality for single
ventricle infants
JET Junctional Ectopic Tachycardia
1980
Electrophysiology
had few tools:
EKG’s
Pacemakers
Medications:
Digoxin
Beta blockers
Quinidine
JET Junctional Ectopic Tachycardia
Uncommon form of SVT
Low cardiac output and
death in 20-50% of pts.
Described in 1980’s
Transient postop issue in
infants with surgeries
near the AV junction
Ex: TOF, VSD
Difficult to manage, no
medications
JET Junctional Ectopic Tachycardia
Multifaceted Treatment Strategy (1985-95)
Fever control
Cooling
Procainamide
Later, use of Amiodarone, 1993
Deaths now uncommon
What was Happening in Nursing?
• Importance of Patient Safety
• Advanced Nursing Practice
• Nursing Research
Patient Safety
Creating Safe Passage
“It might seem a strange principle to enunciate
as the very first requirement in a hospital
that it should do the sick no harm.
It is quite necessary nevertheless to lay down
such a principle.”
Florence Nightingale
Patient Safety
• 1980’s: focus on mortality, less on morbidity
• Reluctant to admit errors
– Errors thought to be individual mistake, not a
system problem
• Quality Assurance, not Improvement
• Concern about “cookbook” medicine
– Too many protocols, not enough thought, not
individualized to the patient
Patient Safety
Institute for HealthCare Improvement, 1980’s
“To Err is Human” Institute of Medicine, 1999
Leapfrog Group, business group, 2000
Changed focus to prevention:
Systems issues, not individual errors
Assumed people would make mistakes
Make it harder to make an error
Best practices, evidenced based medicine
Patient Safety
• Hospital System changes:
– Timeouts for procedures
– Surgical checklists
– Computerized medication order entry
– Infection Control strategies:
• Hand washing, line placement, pneumonia prevention
– Improve communication
• Handoffs, shift report, teamwork
– Involve patients and parents
Patient Safety
Infections were the
cost of doing
business
Patient Safety
Advanced Nursing Practice
• Master’s Prepared Nurses in US
• 4 groups
– Nurse practitioners
– Clinical nurse specialists
– Nurse Midwives
– Nurse Anesthetists
Clinical Nurse Specialists
• Prominent in the 1980’s, now returning
• Hospital based
• Focus on improving nursing care of
specific patient population
• Clinical practice, education, consultant,
research
Nurse Practitioners
• Clinical management of acute and chronic
illnesses
• Initially in primary care in pediatrics, 1965
• Neonatal NP’s, 1970’s
• Prescriptive authority since 1990’s
• Expansion into hospital settings, late 1980’s
– ACC Task Force on Workforce, 1994
– NAPNAP recognized acute care PNP’s, 2004
Nurse Practitioners
• Boston Children’s Hospital
– First nurse practitioners, ambulatory, 1980’s
– First inpatient NP, cardiac surgery, 1987
• Boston Children’s Hospital, 2013
– NP’s in Cardiology: 40
– Total number of NP’s: 250
• PNP’s practicing in the US: 13, 384
– Based on national certification data 2008-09
Growth of NP Practice
• Safe effective clinicians within their scope
of practice
• Collaborative model: MD/NP teams
• Different skill sets:
– MD- Diagnosis, procedures
– NP-Clinical management, patient counseling,
care coordination
• Fewer legal and administrative barriers to
practice
Growth of NP Practice
• Changing Workforce needs
– Decreasing resident and fellow hours
– Decreasing number of fellows
– Increased clinical demand
– Increased specialization
• Lower cost of NP’s
– Less costly education
– Lower pay
Nursing Research
Florence Nightingale
active practice 1853-1875
• Infection Control
• Asepsis
• Cohorting sickest
patients together near
the nurses station
• Use of data, statistics,
outcome data
Nursing Research
• 1952 Nursing Research
Journal established
• 1960’s only 14 graduate
programs in nursing in the
US
• 1964 Nurse Training Act
spurred development of
graduate nursing programs
Nursing Research
• Growth in nursing research:
– Increased numbers of PhD prepared nurses
– Master’s prepared clinicians at the bedside
– Nurse Scientists on hospital staff
– Increase in funding
• US: National Center for Nursing Research, NIH,
1986
– Computer technology
Nursing Research
• Pediatric Nursing Research
– Martha Curley, PhD
• Pediatric CV Nursing Research
– Survey
– Researchers:
• Karen Uzark, PhD: QOL
• Kathy Mussatto, PhD: Family adaptation
• Gwen Rempel, PhD: Parental decision making
Pediatric Clinical Research
• Pediatric Critical Care
research
– RESTORE multicenter trial
• Development of the Braden Q
scale for skin assessment
• Development of the
Withdrawal Assessment Tool
Dr. Martha Curley
Professor
University of Pennsylvania SON
Pediatric Pressure Ulcer Scale
Braden Q
It began with a bed
Pressure Ulcer Assessment Scale
Bergstrom, Braden, Laguzza, Holman, Nursing Research, 1987
Pediatric Pressure Ulcer Scale:
Braden Q
• Braden Scale has 6 subscales, scored from 1
(high risk) to 3 or 4 (low risk),
– Less than 16: risk for pressure ulcers
• Quigley and Curley (1996) adapted the scale for
pediatric use
–
–
–
–
Accounted for developmental differences
Prevalence of tube feedings
Availability of lab values and O2 saturations
Added 7th Subscale: Tissue Perfusion and Oxygenation
• Excluded unrepaired CHD, intracardiac shunting
Pediatric Pressure Ulcer Scale
Braden Q
• Curley and others established predictive values
(Nursing Research, 52, 2003)
• Adopted in many pediatric settings
• The work goes on:
– Predicting Immobility-related and Medical
device-related Pressure Ulcer Risk in Pediatric
Patients (Curley, Quigley, Noonan, McCabe, Wypij)
– Funded study in 6 U.S. children’s hospitals
– Includes the cardiac population and extends
assessment to injury related to medical devices
Withdrawal Assessment Tool:
WAT -1
• Accurate assessment of withdrawal is
necessary for prevention and treatment
• Lack of adequate measures for pediatrics
– Most used was neonatal abstinence score
– Franck studying opioid withdrawal (1998-2004)
• 11 item (12 point) scale
– Objective items, easily integrated into practice
– Fewer items than previous scales, twice daily
Withdrawal Assessment Tool:
WAT-1
• Instrument:
–
–
–
–
Record review (temp, vomiting, loose stools)
2 minute pre-stimulation observation
1 minute stimulus observation,
Recovery
Score 0-12
• Score > 3 correlated with clinical evidence
for opioid withdrawal
– High sensitivity (0.87) and specificity (0.88)
( Franck, et al, Peds Crit Care Med, 2008) (Franck, et al, Pain, 2012)
• Widely adopted
Pediatric CV Nursing Research
Pediatric Nursing Research, AHA, 2008
Literature Review, English, 1980-2008
CINHAL and MEDLINE databases
Search Terms: heart disease, congenital,
heart, cardiac, cardiovascular
Qualifiers: Children, nursing, research
156 studies identified
Limiting factors: only nurse as primary author,
may include reviews, miss research on narrow topics
Pediatric CV Nursing Research
80
70
60
50
# of studies
40
30
20
10
0
1980-89
1990-99
2000-08
Pediatric CV Nursing Research
35
30
25
CV risk
CHD
Tx
20
15
10
5
0
1980-89
1990-99
2000-08
Pediatric CV Nurse Researchers
Karen Uzark, PhD
One of first PhD’s
Co-Director, Heart Center
Research, U. Michigan
Research:
Quality of Life
Heart Transplantation
Psychosocial responses
Kathleen A.Mussatto, PhD
Research coordinator
Recent PhD
Now Nurse Scientist,
Children’s Hospital of
Wisconsin
Research:
Quality of Life
Developmental Outcomes
Pediatric CV Nurse Researchers
First study on parent decision making after
antenatal diagnosis of CHD (JOGNN, 2004)
Multiple studies on parenting children with
complex CHD
Current studies:
School age children with complex CHD:
stories of everyday life
Strengthening family resilience
Collaborative studies:
Dr. Gwen Rempel
Alton, G: Functional Outcomes after neonatal
surgery
Ellinger, MK: Parental Decision Making about
HLHS
Shearer, K: Adolescents with CHD
What Hasn’t Changed
It is still about the
children and families
CICU Mortality
CY 1992-2012
1400
6%
1200
5%
1000
800
3%
600
2%
400
1%
200
0
0%
1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012
Total # Patients
Percentage
% Mortality
Total Admissions
4%
Lessons from the Past
• We can always do better
• Some advances take years of persistent work
– Braden Q, Treatment of JET
• New discoveries and technologies can create
rapid change
– Prostaglandins, pulse oximetry
• Collaboration and teamwork
– All examples
Future Challenges
Our patients will live longer
Currently more adults with
CHD than children
Have to think really long term
Other co-morbidities:
HTN, coronary disease, obesity
Other organ system disease
Future Challenges
New Technologies, New Treatments
– Genetics and genomics
– Tissue engineering
– Stem cell research
– Catheter interventions replacing surgery
– Increased emphasis on prevention
– Continued efforts to reduce morbidity and
improve quality of life
– ???????????????
“When I want to understand what is happening today
Or try to decide what will happen tomorrow,
I look back”
Omar Khayyem
Thank You
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Dr. Michael Freed
Dr. Barry Keane
Dr. Martha Curley
Sandy Quigley, CPNP
Debra Morrow, RN
Elizabeth Tong, MSN, CPNP
Julie Rehman, RN
Dr. Gwen Rempel
Google
Thank you!