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

Background: Withholding and withdrawing life-sustaining treatment for patie nts with Werdnig-Hoffmann disease (WHD) have been accepted as standard medi cal practice in most Western countries. However, a number of Japanese pedia tricians are providing ventilator care for patients with this otherwise fat al disorder. We investigated the attitude of physicians in Japan who are pr oviding ventilator care for patients with WHD. Methods: A postal questionnaire was sent to 40 hospitals where pediatrician s were taking care of 55 ventilator-assisted patients with WHD. Their views were sought on aspects of the care of these patients. Results: Thirty-three pediatricians from 31 hospitals responded to the ques tionnaire. Mechanical ventilation was initiated as an emergency measure in one-third (12/32) of the patients before obtaining full informed consent fr om 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 l ife-sustaining treatment to be withheld. Although 80% (24/30) of the physic ians 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. Discussion: Strong familial endorsement for the prolongation of a patient's life, the secure national insurance and general pro-life beliefs could hav e affected physicians' decisions in favor of providing life-sustaining trea tments for patients with WHD.