Affiliations: Nemours/Alfred I. duPont Hospital for Children,
Wilmington, DE, USA
Note: [] Corresponding author: L. Holmes, Jr., Orthopedic Department,
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USA.
Tel.: +1 302 377 3550; Fax: +1 302 651 5951; E-mail: lholmes@nemours.org
Abstract: OBJECTIVES: It is not uncommon for children with cerebral palsy
(CP) to be discovered dead during sleep (DDDS); however, the factors associated
with this pattern of mortality remain unknown. The current study aims to
describe the mortality associated with children with CP from a single, tertiary
care center who were DDDS. METHODS: A retrospective (case-only) design
to examine proportionate mortality and patient characteristics and
co-morbidities that may be related to children DDDS between 1993 and 2011. RESULTS: There were 177 patients with CP whose deaths were reported
to our institution during the study period, of which 19 were DDDS at home. The
period proportionate mortality (PPM) was 114.5 per 1000. The average age at
time of death was 17 years and 6 months (minimum, 6 years; maximum, 25 years).
All but one of the DDDS patients had gastrointestinal feeding tubes, seizure
disorders, respiratory disorders, and were non-ambulatory. Very importantly,
our DDDS patients manifested clusters of respiratory disorders, namely
recurrent aspiration pneumonia (10/19), asthma pneumonitis (4/19),
food/vomitius inhalation (6/19), reactive airway disease (16/19), respiratory
failure (14/19), chronic bronchitis (7/19), chronic obstructive lung disease
(9/19), and nocturnal respiratory insufficiency (16/19). CONCLUSIONS: Respiratory disorders, severe motor disability, seizures, and intellectual
status are possible co-morbidities that may be associated with DDDS. There is a
need for further study in order to understand what type of monitoring and care
(if any) may help prevent DDDS related to these co-morbidities and sleep
disorders/abnormalities.
