1. “Old Truths” & New Realities: Re-examining futility & “lethal” diagnoses Brenda Barnum, MA BSN RN bbarnum@chla.usc.edu

2. Objectives: Discuss the ethical challenges inherent in caring for Trisomy 13 & 18 patients. Describe how clinician perspective plays a role in understanding parental perspective and decision making with this unique patient population. Identify the complexity of futility and how it might apply to complex cases in the NICU.

3. What is Trisomy 13? – Patau Syndrome – Extra chromosome on 13 – Very Rare – Fewer than 20,000 cases per year – Classic features: Cleft lip/palate Small head/absent eyebrows Cutis Aplasia Clenched fists/Polydactyly Cardiac defects Mental retardation

4. What is Trisomy 18? – Edwards Syndrome – Extra copy of 18 – 1 in 3,000 to 8,000 births – Full, Partial, Mosaic – 80% more common in females – Classic features: IUGR Rocker Bottom feet Clenched hands/overlap fingers Cardiac defects Renal anomalies Mental retardation

5. “Old truths” Lethal – “lethal fetal anomalies” – “incompatible w/ life” 2 OPTIONS: – Terminate the pregnancy – Carry to term & allow the baby to die

6. Luke’s Story 6

7. New Realities = New Options  “life-limiting conditions”  Valuation for life  Prenatal & Neonatal ACPs  More options: - Trach - GT - palliative cardiac repairs - long term NG/ND feedings - O2  Home w/ or w/out hospice

9. The good of the patient: Which good? – Physical Life – Quality of Life As determined by whom? Obligations: – The family unit – Other patients – Society – Professional Integrity

10. Quality of Life Can the quality of a life be objectively measured? No single definition exists Hundreds of different assessment tools have been developed Factors in determining QOL: – Disease state and physical symptoms – Functional status – Psychological functioning – Social functioning – Other (academic achievement, neuropsychological functioning, overall satisfaction with life)

11. A life not worth living? 11

12. The Futility Debates “We want everything done” Often a disagreement over quality of life or best interest for the child Often over resource allocation & the impact of these decisions on society

13. Futile Care Attempts to define medical futility have mostly floundered (too vague or too specific) Definitions of futility differ depending on who you ask: 1. Futility is when the burdens of the treatment grossly outweigh benefits. 2. Futility is when the patient will never survive outside the ICU. 3. Futility is when the patient is permanently unconscious. 4. Futility is when the treatment cannot achieve the patient’s goals. 5. Futility is when death is imminent. General consensus from case law: – Courts will not decide to withdraw against the family – MDs who exercise professional integrity are generally supported

14. Reframing Futility Discussion starts with understanding the goals of treatment – To achieve a complete return to baseline health? – To provide a few more days or weeks of life? The goals of treatment are values-driven, and ought to be defined by the patient/patient family The next question is about the likelihood that treatment can achieve those goals – Determined by the physician – Often uncertain – lack of data, wide confidence intervals – Lack of Consensus

15. Widening our ethical gaze… Looking up from our patient’s bedside Ask “what is the bigger picture”? Navigating the Parent Narrative Sharing our angst, our voices matter

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