Original Articles
Long-term follow-up of home mechanical ventilation in young children with spinal cord injury and neuromuscular conditions

https://doi.org/10.1067/mpd.2003.47Get rights and content

Abstract

Objectives To provide outcomes of two decades of experience in home ventilation of children with spinal cord injury and neuromuscular conditions. Study design Data were collected through chart review and interviews on 39 children who had become ventilator-dependent before their 6th birthday; 23 children had neuromuscular diseases and 16 had spinal cord injuries. Results Patients required an average of 0.7 rehospitalizations per year. There were 8 deaths. Survival rates were 97% at 1 year, 97% at 3 years, 84% at 5 years, and 71% at 10 years. Thirty children attended school, 13 were in regular school (1 at university level), 5 were home-schooled, 5 were in special education schools, and 5 were in regular school with some special education classes. One graduated high school, and another graduated university and received a graduate degree. Three children had progressive weakness. Two gained significant muscle strength. Conclusions Our experience showed that these patients can be discharged to home with low morbidity and mortality rates and successful reintegration into the community. (J Pediatr 2003;142:476-80)

Section snippets

Methods

All pediatric patients seen at RLANRC between 1980 and 2000 were included in the study if mechanical assisted ventilation had been initiated before the 6th birthday. Of the 46 patients, 7 were lost to follow-up. Of the 39 remaining patients, data were collected through retrospective chart reviews and telephone interviews. Data were collected for the sole purpose of this study. One of the physicians personally participated in the care of each patient. All patients were discharged from the

Results

A total of 39 children (28 boys) were included in the study; 23 children had neuromuscular diseases, including 7 with spinal muscular atrophy (SMA), 4 with nemaline rod myopathy, 3 with polio, 3 with congenital muscular dystrophy, 2 with demyelinating neuropathy, and 1 each with mitochondrial myopathy and myotubular myopathy. Two children had myopathies of unknown type. Sixteen children had spinal cord injuries (SCI): 14 C2 injuries, 1 C3, and 1 C4,5.

Of the surviving 31 children, the average

Discussion

Patients who were ventilator-dependent from the polio epidemics of the 1940s and 1950s began the era of home care for ventilator patients. Since that time, home care has flourished.19, 20 By 1987, it was estimated that 2000 ventilator-dependent children resided at home in the United States.21

Studies have been performed to determine the long-term outcome of ventilating infants with BPD.1, 2, 3, 4, 5, 6, 7, 8, 9 Studies also exist of older children with SCI and neuromuscular diseases,11, 12, 15

Acknowledgements

We thank the following people, who contributed significantly to the care of the patients in this study: William Boelter, MD, Jan Ensley, RN, Mary Janson, LVN, Thomas Keens, MD, Sheila Kun, RN, Eithne MacLaughlin, MD, Monique Margetis, MD, and Sally Davidson Ward, MD. We also thank William S. Linn and John Ortega for their help in preparation of the manuscript.

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