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Long-term ventilator support in patients with Werdnig-Hoffmann disease
Y Sakakihara1, M Kubota, S Kim
1Department of Pediatrics, Faculty of Medicine, University of Tokyo, Japan. sakakihr@ped.h.u-tokyo.ac.jp
Pediatrics International : Official Journal of the Japan Pediatric Society
|September 15, 2000
Summary
Japanese pediatricians often provide ventilator care for infants with Werdnig-Hoffmann disease (WHD), a fatal condition. Despite concerns about quality of life, many physicians would initiate mechanical ventilation for new WHD patients.
Area of Science:
- Pediatric critical care medicine
- Neonatology
- Bioethics
Background:
- Withholding/withdrawing life-sustaining treatment is standard in Western countries for Werdnig-Hoffmann disease (WHD).
- Japanese pediatricians are increasingly providing ventilator care for WHD patients.
- This study investigates the attitudes of Japanese physicians managing WHD patients on ventilators.
Purpose of the Study:
- To explore the perspectives of Japanese physicians on providing mechanical ventilation for WHD patients.
- To understand the factors influencing decisions regarding life-sustaining treatment for WHD in Japan.
Main Methods:
- A postal questionnaire was distributed to 40 hospitals.
- Pediatricians caring for 55 ventilator-assisted WHD patients were surveyed.
- Views on patient care aspects were collected.
Main Results:
- 33 pediatricians from 31 hospitals responded.
- Mechanical ventilation was initiated emergently in 1/3 of cases before full parental consent.
- 2/3 of parents requested ventilator care, while only 3 requested withholding treatment.
- 80% of physicians believed WHD patients' quality of life was inadequate.
- However, ~50% would initiate ventilation for a new WHD patient.
Conclusions:
- Strong family support for life prolongation influences treatment decisions.
- Secure national health insurance may facilitate provision of life-sustaining care.
- General pro-life beliefs likely contribute to favoring mechanical ventilation for WHD patients in Japan.