Transcript Trisomy 18
Trisomy 18-Referrals to a Children’s Palliative Care Service Breffni Hannon1, Valerie Jennings1, Eleanor J Molloy2, Marie Twomey1, Maeve O’Reilly1. 1Department of Palliative Medicine, Our Lady’s Children’s Hospital Crumlin. 2Department of Neonatology, Our Lady’s Children’s Hospital Crumlin & National Maternity Hospital, Holles Street, Royal College of Surgeons of Ireland. 1 Why Trisomy 18? • 2011:10 years Children’s Palliative Care service OLCHC • Newly appointed full-time consultant • Most common congenital anomaly referred • GOS presentation Cardiff 2010 • Limited published data regarding palliative care needs 2 What is Trisomy 18? 3 Aims & Methods • Quantify trisomy 18 referrals to service • Identify the unique palliative & end-of-life care needs of this group • Consider methods of improving care delivery to this group • Retrospective chart review spanning 10 years • Permission sought from referring hospitals 4 Results Number Referrals per year Gender Male Female 04 (19%) 17 (81%) Birth weight 2.1kg (1.5-2.7) Apgar Scores 1 minute 5 minutes 5.0 (1-9) 7.3 (3-9) Maternal age 35.9 (19-44) Antenatal diag. Yes No number Demographic Data 6 5 4 3 2 1 0 '02 '03 '04 '05 '06 '07 '08 '09 10 year 03 (15) 18 (85) 5 Reasons for Referral Problem Number (%) Feeding difficulties 17 (85) Breathing difficulties Discharge planning Irritability +/- pain 16 (76) 10 (50) 04 (20) 6 Medications prescribed 7 Outcomes Outcomes Number Place of death Home Hospital 12 (57%) 08 (38%) Cause of death Respiratory distress/sepsis 11 (58%) Trisomy 18 04 (21%) Age at death (days) 52 (03-217) Discharge to death (days) 46 (02-193) 8 Multidisciplinary Input 9 Conclusions • Infants born with trisomy 18 have significant palliative care needs • 1st study of its type to specifically identify these needs • 1st study to report place of death • Highlights current deficits in delivery of care to this group 10 Future Work • • • • • Co-ordinated approach to discharge planning Community outreach nurses Children's hospice Level(s) of palliative care input Neonatal pathways for babies with palliative care needs (ACT) • Database 11 Acknowledgements • Special thanks to: • Valerie Jennings CNS OLCHC • Medical records staff • Dr Martin White • Prof Eleanor Molloy 12 References 1.Edwards JH, Harnden DG, Cameron AH, Crosse VM, Wolff OH, 1960. A new trisomic syndrome. Lancet 1: 787-789. 2.Carey JC, 2001. Trisomy 18 and trisomy 13 syndromes. In: Cassidy SB, Allenson JE, editors. Management of genetic syndromes. 2nd Edition, New York: Wiley-Liss. P417436. 3.http://emedicine.medscape.com/article/943463-overview Trisomy 18:eMedicine Pediatrics: Genetics and Metabolic Disease. 4.Rasmussen SA, Wong LYC, Yang Q, May KM, Friedman JM. Population-Based Analyses of Mortality in Trisomy 13 and Trisomy 18. Pediatrics 2003; 111; 777-784. 5.Support Organization for Trisomy 18, 13 and Related Disorders (SOFT). 6.Lin HY, Lin SP, Chen YJ, Hung HY, Kao HA, Hsu CH, Chen MR, Chang JH, Ho CS, Huang FY, Shyur SD, Lin DS, Lee HC. Clinical Characteristics and Survival of Trisomy 18 in a Medical Center in Taipei, 1988-2004. American Journal of Medical Genetics Part A 140A: 945-951 (2006). 7.Kosho T, Nakamura T, Kawame H, Baba A, Tamura M, Fukushima Y. Neonatal Management of Trisomy 18: Clinical Details of 24 Patients Receiving Intensive Treatment. American Journal of Medical genetics Part A 140A: 937-944 (2006). 8.Goc B, Walencka Z, Wloch A, Wojciechowska E, Wiecek-wlodarska D, KrzystolikLadzinska J, Bober K, Swietlinski J. Trisomy 18 in neonates: prenatal diagnosis, clinical features, therapeutic dilemmas and outcome. Journal of Applied Genetics 47(2), 2006, pp 165-170. 13