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Prodromal illness before acute chest syndrome in pediatric patients with sickle cell disease
Susan E Creary1, Lakshmanan Krishnamurti
1*Division of Pediatric Hematology/Oncology, Nationwide Children's Hospital, Columbus, OH †Division of Pediatric Hematology/Oncology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.
Insights
Nearly one-third of children with sickle cell disease experience a distinct prodromal illness before acute chest syndrome (ACS). Identifying this early illness may help predict and prevent severe ACS episodes in pediatric patients.
Area of Science:
- Pediatric Hematology
- Sickle Cell Disease Research
- Acute Chest Syndrome
Background:
- Acute chest syndrome (ACS) significantly contributes to morbidity and mortality in pediatric sickle cell disease (SCD) patients.
- A distinct prodromal illness preceding ACS episodes in children with SCD is hypothesized.
Purpose of the Study:
- To investigate the characteristics of a potential prodromal illness in children with sickle cell disease leading to acute chest syndrome.
Main Methods:
- Retrospective chart review of pediatric hospitalizations for ACS between 2005 and 2010.
- Defined prodromal visits as acute care encounters within two weeks prior to ACS hospitalization.
- Analyzed documented history, physical examination findings, laboratory results, and radiographs of prodromal visits.
Main Results:
- Of 196 ACS episodes, 29% had documented prodromal visits.
- Common prodromal reasons included painful vaso-occlusive crisis (61%), often in the chest or back (81%).
- Observed prodromal symptoms included hypoxia (53%), tachypnea (29%), history of asthma (39%), prior ACS (80%), and winter seasonality (38%).
Conclusions:
- A significant proportion of pediatric ACS cases are preceded by a distinct prodromal illness.
- Further research is warranted to confirm and define this prodromal illness.
- Identifying prodromal illness may aid in early detection and risk stratification for ACS in children with SCD.
Background:
Acute chest syndrome (ACS) is associated with morbidity and mortality in children with sickle cell disease. We hypothesize that children with sickle cell disease have a distinct prodromal illness before their ACS episode.
Materials And Methods:
We performed a chart review of ICD-9-CM identified ACS episodes at a pediatric hospital from 2005 to 2010. Prodromal visits were defined as acute visits that resulted in a discharge from care and occurred within 2 weeks of a hospitalization that included ACS. We reviewed the documented history, examination, laboratory studies, and radiographs for each prodromal visit.
Results:
We identified 196 ACS episodes. Children received prodromal care in 29% of the ACS episodes. Painful vaso-occlusive crisis was a common reason for seeking this care (61%) and was commonly located in the chest or back (81%). We also observed that patients were hypoxic (53%), tachypneic (29%), had a history of asthma (39%) or ACS (80%), and presented during the winter months (38%).
Conclusions:
These data suggest that nearly one third of patients who develop ACS seek care for a prodromal illness. Further research is needed to confirm and better define an ACS prodromal illness that may help to identify patients at high risk for developing ACS.
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