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?

Opened 2001

New Building, 1988

Founded in 1869

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 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

Dr. Alfred BlalockVivien Thomas

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’sTransient postop issue in

infants with surgeries near the AV junctionEx: 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 ProfessorUniversity 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

1980-89 1990-99 2000-080

10

20

30

40

50

60

70

80

# of studies

Pediatric CV Nursing Research

1980-89 1990-99 2000-080

5

10

15

20

25

30

35

CV riskCHDTx

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 ResearchersFirst 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:

Alton, G: Functional Outcomes after neonatal surgery

Ellinger, MK: Parental Decision Making about HLHS

Shearer, K: Adolescents with CHD

Dr. Gwen Rempel

What Hasn’t Changed

It is still about the children and families

1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 20120

200

400

600

800

1000

1200

1400

0%

1%

2%

3%

4%

5%

6%

CICU MortalityCY 1992-2012

Total # Patients Percentage

To

tal A

dm

iss

ion

s

% M

ort

alit

y

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 todayOr try to decide what will happen tomorrow,I look back”

Omar Khayyem

Thank You

• 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!