When Adolescent and Parents Disagree on Medical Plan, Who Gets to Decide?

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked autosomal recessive disease affecting 16 to 20 per 100 000 live births.1,2 It is characterized by progressive muscle weakness due to a defect in the dystrophin gene. It typically leads to loss of ambulation by age 8 to 14 years,1 followed by cardiomyopathy and respiratory failure. Historically, adolescents with DMD have died at ∼20 years of age.1–3 As respiratory compromise occurs, patients are supported with noninvasive ventilation (eg, nasal bilevel positive airway pressure).3–6 When this becomes unsuccessful, patients may be candidates for tracheostomy; this often happens in the second or third decade of life.7 The decision of whether to proceed with tracheostomy is complicated and is most often left to the patient and family. Family members do not always agree. We present a case in which acute illness forced a minor and his family to face this decision earlier than is typical. The adolescent desired a tracheostomy to extend his life. The parents did not believe that a tracheostomy was in his best interest and felt that comfort care was the most appropriate approach. Experts comment on the ethical issues raised by medical decision-making in cases involving adolescents and life-and-death decisions.