2 Complex Case Studies in UK Organ Donation

1. 2 Complex Case Studies in UK Organ Donation Dr Dale Gardiner Deputy N-CLOD www.clodlog.com

2. Session Outline A red flag case The most complicated ethical case of my career, to date… South Central Regional Collaborative, May 2014

3. Essential Medical Facts • Female patient in her late 40’s • Admitted to a DGH via ED with T2 Resp Failure • PMHx: myotonic dystrophy, no admissions • Short stay ICU then discharged • In hospital asystolic cardiac arrest • ICU: sedated fentanyl + propofol ≈ 54hours • 100 hours off sedation, GCS 3/15, myoclonic jerks, pupils 4 sluggish, mandatory ventilation with no spontaneous respirations. • Normal bloods. South Central Regional Collaborative, May 2014

4. Essential Medical Facts • CT scan revealed: "Evidence of global hypoxic brain injury.” • On return from CT scan, she had no signs of brainstem function – no cough, no gag, apnoea on ventilator, fixed pupils 5mm. • Clinically appeared to cone. • Death confirmed using neurological criteria 4 hours later. South Central Regional Collaborative, May 2014

5. What transpired next? • Family consented for organ donation (on ODR) • Coroner agreed 1a hypoxic brain injury 1b cardiac arrest 1c myotonic dystrophy • Scout team attended • Five hours after testing, bronchoscopy on the ICU for exploration of lung donation stimulated a patient cough. This was repeatable. … the diagnosis of death was withdrawn. South Central Regional Collaborative, May 2014

6. What did they do now? • Family contacted • Coroner re-contacted • DCD progressed the next day • Breathed • Following withdrawal, 45 mins to asystole South Central Regional Collaborative, May 2014

7. Myotonic dystrophy as a confounder to testing • Myotonic dystrophy is a rare and progressive neuromuscular disease that causes weakness. • Respiratory complications are secondary to respiratory and bulbar involvement and obstructive sleep apnoea. • Myotonic dystrophy patients are exquisitely sensitive to opiates, with reports of respiratory depression days after opiates. Anesthesiology 1993, 79:881–892. South Central Regional Collaborative, May 2014

8. Myotonic dystrophy as a confounder to testing • We could not find any case reports of brain death testing in myotonic dystrophy patients, reflecting the rarity of the condition and the rarity of brain death. • There is a generic warning about profound neuromuscular weakness as a confounder to brainstem death testing in the AoMRC (2008) guidance. Conclusion • Despite 100 hours off sedation and seven days from the asystolic cardiac arrest, we believe it was unsafe to carry out brainstem death testing at the time performed, owing to the confounder that myotonic dystrophy brings to the diagnosis of death using neurological criteria. South Central Regional Collaborative, May 2014

9. Recommendations • Routinely waiting a minimum of six hours before testing from the loss of the last brainstem function, in ALL cases. • Additional caution is required in any patient with pre-existing or concomitant neuromuscular disorders. • A more prolonged time for first testing (24 hours) • The use of reversal agents should be considered • The use of clinical ancillary tests, such as atropine, should be considered. • Consideration that testing cannot be safely carried out in patients with pre-existing neuromuscular disorders, without ancillary (blood brain flow) testing. • Open and transparent discussion with families, even in these extreme circumstances, meant that relationships with the family and the care of the patient were not compromised. South Central Regional Collaborative, May 2014

10. Diagnostic caution is advised in the following ‘Red Flag’ patient groups. (Based on the literature and unpublished case reports.) For advice in difficult circumstances contact the local or regional Clinical Lead for Organ Donation or the regional neuro-intensive care unit. Testing < 6 hours of the loss of the last brain-stem reflex Testing < 24 hours where aetiology primarily anoxic damage Therapeutic hypothermia (24 hour observation period following re-warming to normothermia recommended) Patients with pre-existing neuromuscular disorders Steroids given in space occupying lesions such as abscesses Prolonged fentanyl infusions Aetiology primarily located to the brain-stem or posterior fossa South Central Regional Collaborative, May 2014

11. Session Outline Myotonic dystrophy as a confounder to the diagnosis of death using neurological criteria, a red flag case The most complicated ethical case of my career, to date… South Central Regional Collaborative, May 2014

12. Essential Medical Facts • Female patient in her 20’s • Admitted with vomiting and altered behaviour • Cardiac arrest that evening • CT scan: right frontal lobe mass and brain shift • Urgent neurosurgery to debulk tumour • Death confirmed using neurological criteria (brain death) 4 days after admission 14 weeks pregnant South Central Regional Collaborative, May 2014

13. Challenges • ‘Brain dead’ • Pregnant • Eastern European • Partner from a different religion • Organ Donation South Central Regional Collaborative, May 2014

14. Ethical challenges • Who has rights over the deceased body? • Could the foetus be viable? • If the foetus is potentially viable, who can decide its fate? • Is organ donation still appropriate to offer? • How will the foetus die during organ donation? South Central Regional Collaborative, May 2014

15. JahiMcMath USA

16. Ethical challenges • Who has rights over the deceased body? • Could the foetus be viable? • If the foetus is potentially viable, who can decide its fate? • Is organ donation still appropriate to offer? • How will the foetus die during organ donation? South Central Regional Collaborative, May 2014

17. 1993 South Central Regional Collaborative, May 2014

18. (Feldman et al, 2000) 24 weeks legal for abortion 26 weeks viable just 28 weeks viable 34 weeks healthy UK

19. Ethical challenges • Who has rights over the deceased body? • Could the foetus be viable? • If the foetus is potentially viable, who can decide its fate? • Is organ donation still appropriate to offer? • How will the foetus die during organ donation? South Central Regional Collaborative, May 2014

20. Marlise Munoz USA Mother as incubator? South Central Regional Collaborative, May 2014

21. Ethical challenges • Who has rights over the deceased body? • Could the foetus be viable? • If the foetus is potentially viable, who can decide its fate? • Is organ donation still appropriate to offer? • How will the foetus die during organ donation? South Central Regional Collaborative, May 2014

22. X The family approach: 3 key stages Planning Confirming understanding and acceptance of loss As a standard of best practice, as a marker of quality practice, the family approach should be a collaborative effort between senior clinical staff and the SN-OD. Discussing donation 22 Organ Donation Past, Present and Future

23. Ethical challenges • Who has rights over the deceased body? • Could the foetus be viable? • If the foetus is potentially viable, who can decide its fate? • Is organ donation still appropriate to offer? • How will the foetus die during organ donation? South Central Regional Collaborative, May 2014

24. Ethical challenges • Who has rights over the deceased body? • Could the foetus be viable? • If the foetus is potentially viable, who can decide its fate? • Is organ donation still appropriate to offer? • How will the foetus die during organ donation? South Central Regional Collaborative, May 2014