Talking with Parents about a Positive Critical Congenital Heart Disease (CCHD) Screen or Test. Emily Drake, PhD RN May 20, 2014.

Transcription

1 Talking with Parents about a Positive Critical Congenital Heart Disease (CCHD) Screen or Test Emily Drake, PhD RN May 20, 2014 Disclosure I have no financial or other conflicts of interest 1

2 Learning Objectives 1. Differentiate between screening and testing 2. Discussing the results with parents 3. Vulnerable child syndrome 4. Ensuring follow-up Most Common Birth Defect Lee,

3 The face of CCHD Cute! BACKGROUND How many deliveries/month do you have? How many babies/month with CHD do you have? 3

4 BACKGROUND The 7 Targets -Heart Defects 1. hypoplastic left heart syndrome (HLHS) 2. pulmonary atresia 3. transposition of the great arteries 4. truncusarteriosus 5. tricuspid atresia 6. tetralogy of Fallot 7. total anomalous pulmonary venous return (TAPVR) 4

5 GENETICS ENVIRONMENT RISK FACTORS Often asymptomatic Signs & Symptoms 5

6 Signs & Symptoms Pale or bluish skin color Fast breathing/fast heartbeat Difficulty feeding Tires easily during feedings Trouble breathing during feedings slow weight gain Sweating (especially on the forehead or during feeding) Sleeps a lot, lethargic Irritable, difficult to console Puffy face, handsor feet Repeated lung infections What happens when they screen positive? 6

7 The proposed pulse-oximetry monitoring protocol based on results from the right hand (RH) and either foot (F). Kemper A R et al. Pediatrics 2011;128:e1259-e by American Academy of Pediatrics Physical Exam Echocardiogram X-ray, EKG, Labs DIAGNOSIS Interpreting results 7

8 TREATMENTS Cardiac Catheterization Surgery Meds Devices (e.g., artificial valves, pacemakers) Outcomes 5 year survival rates > 85% 8

9 Heart Defect Treatment Typicalage at intervention HLHS 3-step surgery (Norwood) or transplant Pulmonary atresia Prostaglandin Surgical correction Tet TAPVR TGA Tricuspidatresia Complete surgical repair Complete surgical repair Balloon+ surgical repair Sequence of surgical repairs Survival Rate (approximate) 1 st year/infancy 55-65%at 10 y.o. 1 st week of life 65-80% at 2 y.o. 4-6 months 94% at 25 y.o. 1 st year/infancy 65-95% at 14 y.o. Soon after birth 88% at 15 y.o. 1 st year/infancy 86% at 5 y.o. Truncus arteriosus Surgical repair neonatal 83-90% at 15 y.o. Evidence Review Report, 2010 Baby not screened Story of Baby G. 9

10 a good outcome Story of Baby S. Screening vs. Testing A positive screen does not necessarily mean the baby has a CCHD. 10

11 False positives Issues Other dx (pneumonia, sepsis) 75, screen 30 echos 3 CCHD 17 other dx 10 Vulnerable Child Syndrome Anxiety Parenting stress Increased hospitalization & frequent visits to HCP 11

12 False negatives Staying vigilant Issues A "passed" screen does not rule out the possibility of a CCHD or other heart defect. For every 3 children detected with a CCHD by screening, 1 child with a CCHD will still be missed. Right to decline... Follow-up Follow-up is so important Reporting & Tracking 12

13 What to say to parents Don t scare them to death! Provide factual information & resources Make sure they understand importance of follow-up Refer to parent support groups you are not alone Emotional support Scripts We are screening now to determine what testing your baby may need to have done later, if any The cardiologist is going to take a look before you go home to see if there s anything we should follow-up on. Excellent results Long healthy life There is hope You are not alone Talking to other parents who have been through this can be extremely helpful Your baby s CCHD is not your fault, and as of today there is no known way to prevent CCHD. You and/or you partner didn t do anything wrong. 13

14 Resources for Clinicians & Parents Virginia Department of Health (VDH) Handouts for patients and families ildhealth/gns/cchd.htm -Virginia Newborn Screening Education Program - On-demand CCHD Webinars Resources for Clinicians & Parents American Academy of Pediatrics (AAP) March of Dimes (MOD) NewSteps(APHL) Center for Disease Control & Prevention (CDC) 14

15 Resources for Clinicians & Parents National Newborn Screening & Global Resource Center STAR-G CCHD.html Save Babies Through Screening Genetic Alliance Baby s First Test Parent Support Mended Little Hearts Children s Heart Foundation Children s Heart Association Little Hearts Newborn Coalition One in One Hundred 15

16 Emerging Technology You are going to SAVE LIVES! 16

17 Thank you Q & A 17

18 How to Claim Credit Log in to: The following window will pop up complete the registration information requested, take the quiz and course evaluation and print certificate. 18