A 2-day-old girl is in the neonatal intensive care unit due to respiratory failure. The infant was born vaginally at term to a 19-year-old primigravid woman who had limited prenatal care. Ultrasound at 22 weeks gestation revealed severe limb shortening and small thoracic circumference, suggestive of severe skeletal dysplasia. The parents were distraught by the information and did not return for additional imaging or counseling. The infant had profound respiratory distress and hypoxia immediately after delivery and was intubated. Currently, the infant is sedated and ventilated. The upper and lower extremities are extremely short, and the abdomen is protuberant. Chest radiograph reveals small ribs, a narrow thoracic cavity, and severe pulmonary hypoplasia. After consultation with a genetic specialist, the diagnosis is determined to be thanatophoric dysplasia, a skeletal dysplasia that is virtually always lethal in the newborn period. The findings and prognosis are discussed with the family during a multidisciplinary meeting. Multiple specialists are present and agree that tracheostomy will not change the newborn's course. Redirection of care to end-of-life comfort is recommended by the medical team. However, the parents continue to demand surgery. Which of the following is the most appropriate next step in management?

Question

A 2-day-old girl is in the neonatal intensive care unit due to respiratory failure.  The infant was born vaginally at term to a 19-year-old primigravid woman who had limited prenatal care.  Ultrasound at 22 weeks gestation revealed severe limb shortening and small thoracic circumference, suggestive of severe skeletal dysplasia.  The parents were distraught by the information and did not return for additional imaging or counseling.  The infant had profound respiratory distress and hypoxia immediately after delivery and was intubated.  Currently, the infant is sedated and ventilated.  The upper and lower extremities are extremely short, and the abdomen is protuberant.  Chest radiograph reveals small ribs, a narrow thoracic cavity, and severe pulmonary hypoplasia.  After consultation with a genetic specialist, the diagnosis is determined to be thanatophoric dysplasia, a skeletal dysplasia that is virtually always lethal in the newborn period.  The findings and prognosis are discussed with the family during a multidisciplinary meeting.  Multiple specialists are present and agree that tracheostomy will not change the newborn's course.  Redirection of care to end-of-life comfort is recommended by the medical team.  However, the parents continue to demand surgery.  Which of the following is the most appropriate next step in management?

Quizplus · Accepted Answer

Explanation: 11ec2b69_c1e3_e103_8f63_3bf153967544_MD0005_00 End-of-life care may involve discontinuation of ongoing therapies and/or shift to palliative therapies. This phase of treatment may begin when curative therapy is likely futile. Futility may be physiologic if the treatment is extremely unlikely to allow survival, or qualitative if it offers only minimal benefit at the cost of significant pain and suffering. When this point is reached, the primary goals of therapy shift toward minimizing discomfort, anxiety, and distress for the patient and family. When the patient's family and medical team disagree about futility, a stepwise approach toward agreement should be implemented. First, family meetings, in which treatment and prognosis are discussed, are scheduled to promote joint decision-making. Second, for ongoing disagreement, a palliative care consultation or referral to the hospital ethics committee can help with arbitration. If resolution is still not reached, transferring the patient to another provider, either within the institution or to a different facility, can be considered (Choice E). Once the hospital ethics committee is involved and all providers have agreed that additional care is futile, then-and only then-should providers cease futile interventions in the absence of family agreement (Choice D). (Choice A) When a family is adamantly opposed to the medical team's plan, additional family meetings without gaining additional information or perspectives (eg, from a palliative care consult or the ethics committee) will likely prolong futile care and are unlikely to lead to an agreement. (Choice C) If additional procedures are deemed to be futile, it is unethical for providers to pursue that care under the principle of nonmaleficence (ie, do no harm). Educational objective: End-of-life care is focused on minimizing discomfort, anxiety, and distress for the patient and family once efforts to cure or modify disease become futile. If there is disagreement about whether further care is futile, providers should seek agreement through a stepwise approach that includes involvement of consultants and the hospital ethics committee. References: Medical futility: a new look at an old problem. (http://www.ncbi.nlm.nih.gov/pubmed/25451353)

A 2-Day-Old Girl Is in the Neonatal Intensive Care Unit