Dilemma to chronic ventilation in Werdnig-Hoffmann disease: study of 16 patients. C.E. Kim, P. Lecussan, E. Casella, L. Albano, G. Oselka. Pediatrics, Instito da Crianca, Sao Paulo, Brazil.

   Werdnig-Hoffmann disease, the most common lethal autosomal recessive disorder and its frequency is 1 per 10 000 births. Affected patients usually die before 2 years of age, due to the respiratory involvement. In Brazil, due to cultural,religious and legal problems, almost all patients are submitted to chronic ventilation and have their lives actively prolonged for unlimitated time.Therefore,iniciation of ventilator use in this condition is raising an important ethical issue and high medical costs. We studied 16 patients, all under permanent mechanical ventilation, started from 2 to 19 months (mean age=6,6 mo). Ten patients are maintained in intensive care units of hospitals and 6 patients are at home care. The duration of ventilation varied from 7 months to 13 years. The average time of ventilation was 6 years in patients at home and 2 years and 9 months in patients at hospital.Two among the patients in hospital died after 4 and 21 months of ventilation. The current ages of alive patients range from 1 year and 5 months to 14 years. Tracheostomy and gastrostomy were performed in all patients. interestingly, the parents of all patients prefere their child to be submitted to ventilation, even thoughsuffering emotional stress and poor quality of the life style without any pespectives of improvement.