1. Quality of Life in Pediatric VAD- How are we doing? INTERMACS Eighth Annual Meeting May 5, 2014 Karen Uzark, PhD, CPNP University of Michigan Congenital Heart Center

2. Definitions HEALTH = “not only the absence of disease and infirmity, but the presence of physical, mental, and social well-being” QUALITY OF LIFE = “the individual’s perception of their position in life, in the context of culture and value systems in which they live and in relation to their goals, expectations, standards and concerns” Quality of Life ≠ Physical Health Status or Abilities Quality of life cannot be simply inferred from clinical and laboratory findings or objective criteria. 1948 WHO 1991 WHO

3. Dimensions of Quality of Life Physical functioning (Disease state and physical symptoms) Psychological/Emotional functioning Social functioning School/role functioning (Neurodevelopmental functioning)

4. Ventricular Assist Devices –Goals: Survival to Transplant or Recovery Improved cardiac output → improvement in end-organ function Physical rehabilitation Extubation Mobilization/Ambulation Enteral nutrition Neurological evaluation-rehabilitation → Improved psychosocial quality of life

5. Quality of Life (QOL) in Children Bridged to Transplant with VADs No significant difference in QOL between 21 children who had VAD and 42 without VAD at a median of 4.2 years post-transplant (Ezon et al, J Thorac Cardiovasc Surg 2014) Limitations: small sample size, wide age range, parent survey only, type of VAD support varied, survivor bias Children who required VAD/ECMO (n=26) reported QOL similar to children without MCS (n=58) at a median of 3 years post transplant * (Wray et al, J Heart Lung Transplant 2012) Limitations: small sample size, wide age range, CHD dx exclusion, type of support varied, parent survey in younger patients, survivor bias * Longer period of heart failure in MCS was associated with poorer child ratings of social QOL.

6. Experience of Children with VAD as Bridge to Transplant* Qualitative study with semi-structured interviews with children (n=4) over 13 years old. Child responses : Frightened, scared of clots and strokes, appreciative/relieved (would die without it) Not prepared for “big washing machine”, big hoses. *Gilmore and Newall, Pediatr Cardiol 2011.

7. PediMACS QOL indicators

8. PedsQL

9. VAD-specific QOL

10. Peds QL and VAD Qol Completion Status for Parents/Children by Patient Age N PedsQL - ChildPedsQL - ParentPedsOL - either Completed% % % Age at Follow-Up (24)N/A 00.0%0 <2 years 2-4 years 11N/A 19.1%1 5-7 years 900.0%111%1 8-12 years 3126.5%723%7 13-18 years 38616%1026%1026% TOTAL 1138/78(10%)1917%19 17%* Pre-Implant *21% excluding <2 years

11. Peds QL and VAD Qol Completion Status for Parents/Children by Patient Age 3 month Follow-up N Peds QL - ChildPedsQL - ParentPedsQL - eitherVAD QOL - ChildVAD QOL - ParentVAD QOL - either Complete% % % % % % Age at Follow-Up (9)N/A 00.0%0 0 111%1 <2 years 2-4 years 4N/A 125%1 00.0%125%1 5-7 years 300.0%0 0 0 0 0 8-12 years 10220%2 2 2 1 10%220% 13-18 years 20735%525%735% 4 20% 3 15%420% 19+ years 200.0%0 0 0 0 0 TOTAL 489/30(30%)_817%10 21% 613%6 8 17%* *21% excluding <2 years

12. Peds QL – Reasons For Incompletion Reasons for Peds QoL Incompletion ParentChild N%N% Too Sick 18 21.23940.6 Urgent Implant, No Time 13 15.366.3 Coordinator Too Busy or Forgot 33.522.1 Unable to Contact Patient 22.4.. Other Reason, Specify 49 57.6 4951.0 TOTAL 8510096100 Pre-implant Reasons for Peds QoL Incompletion ParentChild N%N% Too Sick 312311.5 Urgent Implant, No Time 1413.8 Coordinator Too Busy or Forgot 8 32 830.8 Other Reason, Specify 13 52 1453.8 TOTAL 2510026100 3 month Follow-up

13. OTHER Reasons Form Incomplete Pre-implant (n=43) Forms/IRB not approved -17 (40%) (? license pending -2) Age too young -10; 1 too old (26%) Not consented -12 (28%) Other: Patient autistic -1 No coordinator -2 Post-implant (n=13) Forms/IRB -4 (31%) Age -2 (15%) No Consent -3 (23%) Transplanted -2 (15%) Reason Missing -35

14. VAD QOL – Parent Report n=4-6

15. VAD QOL – Parent Report (continued) (n=4-6)

16. VAD QOL – Child Self-Report (n=6)

17. VAD QOL – Child Self-Report (continued) (n=6)

18. VAD QOL – Child Self-Report Differences between 3 and 6 months (n=6 at 3 mos, 5 at 6 mos)

19. THANK YOU!