One important factor omitted from your otherwise excellent editorial and associated report and review of the ethics of long-term ventilation of infants with spinal muscular atrophy is that death at 1–2 years of age is not accompanied by the patient's comprehension of dying. Dying in infancy is likely to be much less distressing for the patient than when dying is accompanied by awareness later in life. Compassion, which must underlie all ethical decisions, dictates that a less distressing death in infancy is preferable to dying later, after a few years of distressing life. I personally believe it was not compassionate, and therefore was unethical, to offer long-term ventilation initially to the subject of the case report. Doctors are not required to provide treatment which they regard as unethical and the decision to offer ventilation would have involved management of the parents, not the patient.
Now that the child is eight years old, and apparently has reached an age of conscious awareness of her predicament, her potential distress caused by awareness of imminent death is a strong argument against a compassionate withdrawal of treatment. The situation now is very different from the situation in infancy when it would have been better not to have commenced ventilation. Nevertheless, the same compassionate approach can still be used to arrive at an ethical answer to the question of ‘is this life worth living?’ Decision-making is ethical only if it is based on compassion. However, compassion-based ethical decisions are sometimes very personal and individual, and different people can place more or less importance on the amount of compassion in two different courses of action. Provided the decisions are based on compassion, both courses of action are ethical. The parents, who are the final decision-makers, can compassionately and ethically decide either to withdraw treatment or alternatively, to continue ventilation and perhaps end up with another person like Stephen Hawkins. If a decision is based on compassion, it is right for the person who makes the decision and who has to live with it for the rest of their life. We cannot measure compassion objectively so personal ethics based on the parents' compassion, need to be respected, and not the ethics of a committee or judge.