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 • • • • • • • • • 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!