1. 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

2. I have no disclosures

3. Introduction

4. 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

5. Why Study History?
Founded in 1869
Opened 2001
New Building, 1988

6. 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

7. Tetralogy of Fallot Most common cyanotic heart defect
Described in 1888
1. Infundibular Pulmonic Stenosis
Right ventricular hypertrophy
Conoventricular VSD
4. Dextroposition of the Aorta
Overrides VSD

8. Tetralogy of Fallot Several types: TOF with Pulmonic Stenosis
TOF with Pulmonary Atresia
TOF with Absent Pulmonary Valve
TOF with Complete AV Canal

9. Tetralogy of Fallot

10. Blalock Taussig Shunt
Johns Hopkins, 1945

11. 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

12. 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 Blalock
Vivien Thomas

13. Blalock Taussig Shunt

14. 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

15. 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

16. 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

17. 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

18. 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

19. 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)

20. 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)

21. My Career in Pediatric Cardiology
Graduated university, BSN, 1977
CNS in pediatric cardiac surgery, UCLA, 1982
Nurse practitioner, Boston Children’s Hospital, 1987

22. 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

23. 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

24. Technology in 1980 Still an analog world Paper records No cell phones
Computer technology
Microsoft, 1975
Apple, 1976
Personal computers coming on the market

25. Pulse Oximetry
What is your O2 Saturation??

26. Pulse Oximetry
Measurement of transmitted light through a translucent measuring site to determine oxygen saturation
Oxygen rich hemoglobin absorbs more infrared light

27. 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

28. Pulse Oximetry 1983: Evaluation of Pulse Oximetry. 1988:
(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)

29. 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

30. JET Junctional Ectopic Tachycardia
1980
Electrophysiology
had few tools:
EKG’s
Pacemakers
Medications:
Digoxin
Beta blockers
Quinidine

31. 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

32. JET Junctional Ectopic Tachycardia
Multifaceted Treatment Strategy ( )
Fever control
Cooling
Procainamide
Later, use of Amiodarone, 1993
Deaths now uncommon

33. What was Happening in Nursing?
Importance of Patient Safety
Advanced Nursing Practice
Nursing Research

34. Patient Safety
Creating Safe Passage

35. as the very first requirement in a hospital
“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

36. 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

37. 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

38. 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

39. Patient Safety
Infections were the cost of doing business

42. Patient Safety

43. Advanced Nursing Practice
Master’s Prepared Nurses in US
4 groups
Nurse practitioners
Clinical nurse specialists
Nurse Midwives
Nurse Anesthetists

44. 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

45. 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

46. 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

47. 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

48. Growth of NP Practice Changing Workforce needs Lower cost of NP’s
Decreasing resident and fellow hours
Decreasing number of fellows
Increased clinical demand
Increased specialization
Lower cost of NP’s
Less costly education
Lower pay

49. 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

50. 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

51. 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

52. 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

53. 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

54. Pediatric Pressure Ulcer Scale Braden Q
It began with a bed
It began with a bed

55. Pressure Ulcer Assessment Scale
Bergstrom, Braden, Laguzza, Holman, Nursing Research, 1987

56. 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

57. 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

58. 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 ( )
11 item (12 point) scale
Objective items, easily integrated into practice
Fewer items than previous scales, twice daily

59. 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

60. Pediatric CV Nursing Research
Pediatric Nursing Research, AHA, 2008
Literature Review, English,
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

61. Pediatric CV Nursing Research

62. Pediatric CV Nursing Research

63. 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

64. 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:
Alton, G: Functional Outcomes after neonatal surgery
Ellinger, MK: Parental Decision Making about HLHS
Shearer, K: Adolescents with CHD
Dr. Gwen Rempel

65. What Hasn’t Changed
It is still about the children and families

67. 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

68. 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

69. 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
???????????????

70. “When I want to understand what is happening today
Or try to decide what will happen tomorrow,
I look back”
Omar Khayyem

71. 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

72. Thank you!