PURPOSE: The ethics of invasive mechanical ventilation for children with the progressive neuromuscular disease Spinal Muscular Atrophy Type I (SMA I) is highly debated. We conducted this international survey to identify regional factors and physician characteristics associated with recommendation for tracheostomy and ventilation for SMA I.
METHODS: A cross-sectional international online survey was distributed to 1736 pediatric pulmonologists and intensivists from online membership directories of ATS, ACCP, and ERS. Questions explored physician demographics, attitudes and experience with SMA and end-of-life care, knowledge of consensus guidelines, and recommendations for respiratory care of SMA I. Predictor variables were analyzed with Chi-square testing and entered into a logistic regression model.
RESULTS: 363 (21%) physicians completed the survey; 84% were pediatric pulmonologists and 16% pediatric intensivists. 70% of respondents were from the U.S. Physicians averaged 16 years of experience in their field of practice. 50% of physicians were aware of SMA consensus guidelines. 60% agreed invasive ventilation is acceptable for SMA I patients with chronic respiratory failure. In univariate analysis, physicians from Commonwealth countries (U.K., Canada, Australia, etc.) were significantly less likely to recommend tracheostomy/ventilation than U.S. physicians (7% vs. 25%, p = 0.011). Logistic regression modeling identified years of experience, agreement with a pro-life statement, and recommendation for noninvasive ventilation as predictive of recommendation for long-term invasive ventilation for SMA I.
CONCLUSION: In the largest international survey on this topic, we identified regional differences in physician recommendation for tracheostomy/mechanical ventilation for SMA I. We found that only half of physicians caring for children with SMA I were aware of consensus guidelines. Logistic regression modeling identified physician experience and attitudes as predictive of recommendation of long-term invasive ventilation.
CLINICAL IMPLICATIONS: Key goals for SMA I include improving awareness of treatment options, reducing variability of care, reducing costly invasive interventions, and minimizing suffering of patients and families. Our data demonstrate a need for increased awareness of consensus guidelines and will hopefully serve to stimulate physician dialogue to further these goals.
DISCLOSURE: Renee Benson, No Financial Disclosure Information; No Product/Research Disclosure Information