Jeffrey B. Anderson, MD MPH The Heart Institute Cincinnati Children’s Hospital Medical Center

1. Variation in interstage weight gain among surgical centers in single ventricle infants:Identification of strategies to improve growth Jeffrey B. Anderson, MD MPH The Heart Institute Cincinnati Children’s Hospital Medical Center

2. Introduction • Harvey Hamrick, MD

3. Introduction • Pediatric Academic Society 2006

4. Competition has been shown to be useful up to a certain point and no further, but cooperation, which is the thing we must strive for today, begins where competition leaves off. ~ Franklin D. Roosevelt

5. Outline • Definition of rare diseases • Cooperation to understand these patients • Specific example of how this works • Call to arms for working together

6. Introduction • Chronic kidney disease requiring dialysis ~5,600 • Duchene Muscular Dystrophy ~15,000 • Cystic fibrosis ~ 30,000 • Complex congenital heart disease ~ 180,000 and rising • Most of the patients we care for fall under the classification of rare disease

7. Where can we find solutions? • Traditional clinical research limited by small numbers • We often rely on the findings from case reports or case series to guide our management decisions • Because of the rarity of many complex pediatric problems it is difficult for any one center to see enough cases to adequately study and determine best care • Organized systems of care help alleviate this problem

8. What is an organized system? Organized systems of care are groups that allow for collaborative, integrated care among a group of caregivers who are accountable for the quality, cost and overall care of a defined population of patients.

9. Collaborative Care and Improvement Organized systems of care that have resulted in profound patient improvements: • Children’s Oncology Group • Northern New England Cardiovascular Group • End Stage Renal Disease Network • Neonatology (Vermont Oxford Network, California Perinatal Quality Care Collaborative, others) • National Health Services primary care collaborative • Cystic Fibrosis Collaborative • NACHRI Catheter Related Blood Stream Infections Collaborative

10. Models for Collaborative Improvement • Successful system models of improving care have common features: • Multicenter shared data collection with transparency • Multidisciplinary involvement, including patients and parents • Definition and implementation of standardized care practices • Systems to support sharing evidence/knowledge • Collaborative learning across practice sites

11. Acute Lymphoblastic Leukemia 5 year survival rate Simone J., Lyons, J: J Clin Oncology 1998 Sep;16(9):2904-5

12. It is instructive to learn that the cure rate for childhood acute lymphoblastic leukemia rose from about 40% in the early-1970’s to about 70% in the mid-1990’s without a single new frontline therapeutic agent. In leukemia and other cancers, improvements came largely from trial-and-error adjustments of therapeutic dosages and schedules made possible by the large pool of patients participating in clinical trials. Joseph Simone, MD Children’s Oncology Group In report to Institute of Medicine

13. Where are we in cardiology? Area under the curve represents number of practitioners using Innovation. Diffusion of Innovations, 1962, Everett Rogers

14. How do Cardiologists make decisions? • 10 pediatric cardiologists • Reasons for every clinical decision • Variety of clinical situations Darst et al. Cong Heart Dis. 2011

15. How do we make medical decisions? • Experience/anecdote 37.1% • Arbitrary/Instinct 14.7% • Trained to do it 14.6% • General study 12.3% • First principles/physiology 12.3% • Limited study 5.1% • Specific study 2.9% • Parenteral preference 0.5% • For research 0.3% • Avoid a lawsuit 0.2% Darst et al. Cong Heart Dis. 2011

16. How do we do this better?

17. Background: Hypoplastic left heart syndrome

18. Palliative surgical procedures Norwood procedure Bidirectional Glenn Fontan completion INTERSTAGE

19. Background • Infants with a single ventricle have poor growth prior to their bidirectional Glenn procedure (Stage 2) • Lower preoperative weight-for-age z-score is associated with increased hospital length of stay following BDG procedure Anderson, JACC 2008; 51(10 Suppl A): A83-97

20. Mean -1.3 Number of patients Results: Weight distribution (n=100) Mean -0.2 Number of patients

23. Growth of the NPC-QIC

24. NPC-QIC Participating Sites Seattle Children’s Hospital University of Chicago Comer Children’s Hospital Advocate Hope Children’s Hospital Wisconsin Cleveland Clinic Children’s Memorial Mayo Clinic, Rochester Doernbecher Children’s Hospital Children’s Hospital Boston Yale New Haven Children’s Hospital Nationwide Montefiore Children’s Hospital and Research Center, Omaha NYU Riley Children’s Hospital Cohen Children’s Primary Children’s Medical Center CHOP Penn State Hershey Children’s Children’s Hospital - Denver University of Maryland Inova Fairfax UC Davis Children’s Hospital Cincinnati Children’s Hospital Medical Center Johns Hopkins University of Louisville Children’s National St. Louis Children’s Hospital Children’s Hospital and Research Center, Oakland University of Virginia Children’s Hospital Arkansas Children’s Hospital Monroe Carrell Jr. Children’s Hospital at Vanderbilt Le Bonheur Children's Hospital Arizona Pediatric Cardiology Consultants Duke University Medical Center Lucile S. Packard Children’s Hospital at Stanford Levine Children’s Hospital Mattel Children’s Hospital UCLA Medical University of South Carolina Children’s Healthcare of Atlanta Children’s Hospital, Los Angeles Arnold Palmer Children’s Hospital Methodist Children’s Hospital Texas Children’s Hospital All Children’s Hospital CHRISTUS Santa Rosa Children's Hospital Miami Children’s Hospital Children’s Medical Center Dallas

25. Purpose • Identify variation in growth outcomes among NPC-QIC centers • Identify nutritional practices that are associated with better interstage growth • Use this evidence to spread these practices to institutions within the collaborative

26. Methods • Retrospective analysis of patients in the NPC-QIC registry • Inclusion: • Patients who had presented for stage 2 (S2) surgical repair • From centers who had enrolled > 4 patients who had presented for S2

27. Methods: Nutritional processes • Registry information regarding nutritional practices • Blinded structured interviews to gain more detailed information • Designed and reviewed by • Cardiologist • Two separate registered dieticians • Epidemiologist with survey expertise

28. Methods: Outcomes • Primary outcome • Change in weight-for-age z-score (WAZ) between discharge following neonatal Norwood (S1) and presentation for Bidirectional Glenn (S2), ie during the interstage

29. Analysis • Variation in WAZ among centers was identified • Centers with a median increase in WAZ were selected • Nutritional processes were identified that were associated with an increase in WAZ between S1 and S2

30. Results: patient characteristics (n=132)

31. Results: variation among centers

32. Results • Nutritional processes common to centers with a positive median WAZ change • use of home scales for interstage weight monitoring • specific weight gain/loss “red flags” to identify patients with growth failure in the interstage period • regular phone contact with families during the interstage period regarding nutrition and growth • dietitian available for each cardiology outpatient visit during the interstage period • standard post-Norwood feeding evaluation

34. Conclusions • There is considerable variation in growth of infants with HLHS among sites caring for these infants • There are specific nutritional practices used at centers with better infant growth • A combination of these “best practices” is associated with an effective increase in weight for age z-score of 0.98

35. What next?? • Prospectively implement these best nutrition practices • Next week, at the NPC-QIC fall learning session, we will begin enrolling centers who commit to implement these nutritional practices and follow their patient’s growth over time

36. Strengths and Limitations • This type of work could not be done by a single individual or institution • We learn incrementally more as we share methods and experiences among centers • This specific work is limited by the data we gather • Yet to be determined whether these findings are generalizable beyond the very small number of infants with a single ventricle

37. Conclusions • Infants and children have rare diseases • Individuals and even individual institutions do not care for enough patients to allow for adequate understanding of disease processes or treatment effectiveness • Collaboratives/Registries a powerful tool to moving forward in our understanding of these rare problems.

38. Incentives to participate in collaboration • Allow unique approaches to problem solving • Exposure to different ways to treat patients • ABP stance on collaborative work • TheAmerican Board of Pediatrics (ABP) was created to advance the science, study, and practice of pediatrics by a series of credentialing and certifying activities. Requirements for maintenance of certification now emphasize assessing quality of care and demonstrating systematic improvement of care for children

39. Character is like a tree and reputation like its shadow. The shadow is what we think of it; the tree is the real thing. ~Abraham Lincoln

41. Cincinnati, the Queen City

42. Results: Daily weight gain (n=100) Median 16.4 g/day CDC recommendation

43. Small multiple tables

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45. Small multiples tables