Long-term ventilator support in patients with Werdnig-Hoffmann disease

Withholding and withdrawing life-sustaining treatment for patients with Werdnig-Hoffmann disease (WHD) have been accepted as standard medical practice in most Western countries. However, a number of Japanese pediatricians are providing ventilator care for patients with this otherwise fatal disorder. We investigated the attitude of physicians in Japan who are providing ventilator care for patients with WHD.

A postal questionnaire was sent to 40 hospitals where pediatricians were taking care of 55 ventilator-assisted patients with WHD. Their views were sought on aspects of the care of these patients.

Thirty-three pediatricians from 31 hospitals responded to the questionnaire. Mechanical ventilation was initiated as an emergency measure in one-third (12/32) of the patients before obtaining full informed consent from the parents. Two-thirds (19/32) of parents asked the physicians to start ventilator care for the patients, while only three parents asked for the life-sustaining treatment to be withheld. Although 80% (24/30) of the physicians thought that the quality of life of the ventilator-dependent patients with WHD was inadequate, about half (17/30) answered that they would start ventilator assistance if they had a new patient with WHD.

Strong familial endorsement for the prolongation of a patient's life, the secure national insurance and general pro-life beliefs could have affected physicians' decisions in favor of providing life-sustaining treatments for patients with WHD.