Article Text

“A GOOD DEATH”: PALLIATIVE SURGERY IN TRISOMY 18
  1. C Almeida Fernandes1,
  2. S Melo Gomes2,
  3. C Neves3,
  4. H Ramos4,
  5. E Fernandes3,
  6. M Santos3,
  7. M A Nunes5,
  8. J P Magalhaes6,
  9. O Nascimento3,
  10. J Marques Valido3
  1. 1Pediatrics Department, Centro Hospitalar de Setubal, EPE, Setubal, Portugal
  2. 2Pediatrics Department, Centro Hospitalar Das Caldas Da Rainha, Caldas Da Rainha, Portugal
  3. 3Pediatrics Department, Dr Alfredo Da Costa Maternity, Lisbon, Portugal
  4. 4Pediatrics Department, Espírito Santo Hospital, Evora, Portugal
  5. 5Pediatric Cardiology Department, Cruz Vermelha Portuguesa Hospital, Lisbon, Portugal
  6. 6Cardio-Thoracic Surgery Department, Cruz Vermelha Portuguesa Hospital, Lisbon, Portugal

Abstract

Palliative care serves to enhance quality of life, relieve symptoms, and provide support to families.

A “good death” is one that is free from avoidable distress and suffering for patients, families, and care-givers.

Various severe congenital malformations, profound mental retardation as well as a high rate of infant mortality are characteristic features of trisomy18. Congenital cardiopathy occurs in 60–90%. Since it is a condition of poor prognosis, a “minimal care” policy is usually adopted.

The authors present a child who was born with a phenotype compatible with trisomy 18, confirmed later by cariotype. She presented with a large ventricular septal defect (VSD), formanen ovale and patent ductus arteriosus, which resolved with indomethacin. Because of VSD there was clinical deterioration with congestive cardiac failure (CHF) and respiratory distress that made it impossible to send her home, which was the parent’s desire.

After discussion between neonatologists, the pediatric cardiologist, cardio-thoracic surgeon and the parents, a decision for palliative cardiac surgery was made. Pulmonary artery banding in the 37th day of life allowed for a better clinical condition and release to go home on the 44th day of life.

On movement she revealed no signs of CHF or respiratory distress. She died on 77th day probably from central apnoea. The family was thankful for the chosen option. Cardiac surgery seems to be a palliative option to consider in cases of trisomy 18, since it can ease significantly the pain in these patients. The purpose of neonatal palliative care is to add life to the infant’s time, not to add time to the infant’s life.

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