Adolescents and young adults (A/YA) with sickle cell disease (SCD) are hospitalized in both children’s and general hospitals. We determined the effect of hospital type and provider specialty on outcomes of hospitalized A/YA with SCD and acute chest syndrome (ACS).
This retrospective cohort study used the 2007–2009 Premier Database, a large multi-institutional database, to identify 1476 patients ages 16 to 25 years with 2299 admissions with SCD and ACS discharged from 256 US hospitals from 2007 to 2009. Multilevel logistic regression and zero-truncated negative binomial regression were performed after adjustment for patient demographic, clinical, and hospital characteristics to test the association of hospital type and provider specialty on death, endotracheal intubation, simple or exchange transfusion, length of stay (LOS), and 30-day readmission.
Of all admissions, 14 died and 45% were intubated. General hospitals had 13 deaths and were associated with higher intubation rates (predicted probability [PP], 48% [95% confidence interval (CI), 43%–52%]) and longer LOS (predicted mean LOS, 7.6 days [95% CI, 7.2–7.9]) compared with children’s hospitals (PP of intubation, 24% [95% CI, 5%–42%]; and predicted mean LOS, 6.8 days [95% CI, 5.6–5.8]). There was no difference by hospital type or provider specialty in PP of simple or exchange transfusion, or 30-day readmission.
General hospitals carry higher intubation risks for A/YA with SCD and ACS compared with children’s hospitals. We need to better understand the drivers of these differences, including the role of staff expertise, hospital volume, and quality of ongoing SCD care.
Dear Editor,
We applaud the efforts of Jan et al (1) to bring to light the morbidity and mortality risks of adolescents and young adults with sickle cell disease and acute chest syndrome. However, as general academic pediatricians who work in a community hospital setting we were concerned by the broad generalization regarding the care of these patients at "general hospitals". There are many community hospitals with robust pediatric services and to essentially lump all general hospitals together does not appropriately capture the care that is given at these institutions.
Further, it is unclear whether the differences in outcomes between children's hospitals and general hospitals are related to quality of care or the natural history of the disease. The authors conclude that general hospitals were associated with longer length of stay and higher intubation risk compared with children's hospitals. They also reported a non- statistically significant trend toward higher mortality in general hospitals, with 13 deaths in their cohort occurring at general hospitals and one at a children's hospital. They posit that these differences may be due to expertise at children's hospitals that lead to better outcomes. That conclusion may not be warranted, however. One important confounder is that patients at general hospitals were significantly older than those at children's hospitals with 64% aged 21-25 at general hospitals versus 37% at children's hospitals. All the mortality occurred in patients older than 18. Other studies have confirmed an increase in mortality rate in these young adults (2,3). So, is the higher mortality rate in the 20-25 year old group related to the quality of their care or the natural history of sickle cell disease in which older patients are also sicker? Studies in Africa may shed some light on this. Makani et al (4) followed patients in Tanzania who had limited access to resources. These patients also had an increase in mortality rate (1.4 deaths per person year for 5-19 year olds vs. 1.8 deaths per person year for those >20), but this change could not be explained by a sudden loss of resources. So it well may be that patients with sickle cell in their 20s are sicker than their younger counterparts whether they get care at children's hospitals or general hospitals.
There is much work to be done to decrease morbidity and mortality for patients with sickle cell. We hope that Jan's results are not misconstrued and these young patients are not shunted away from hospitals that may provide excellent care within their local community.
References:
1. Jan, S Slap G, Smith-Whitley K, Dai D, Keren R, Rubin D. Association of Hospital and Provider Types on Sickle Cell Disease Outcomes. Pediatrics. 2013; 132 (5): 854-861
2. Hamideh D, Alvarez O. Sickle Cell Disease Related Mortality in the United States (1999-2009). Pediatr Blood Cancer. 2013; 60 (9): 1482-1486
3. Lanzkron S, Carroll CP, Haywood C. Mortality Rates and Age at Death from Sickle Cell Disease: U.S., 1979-2005. Public Health Reports. 2013; 128: 110-116
4. Makani J et al. Mortality in Sickle Cell Anemia in Africa: A Prospective Cohort Study in Tanzania. PLoS ONE. 2011; 6(2): e14699. doi:10.1371/journal.pone.0014699
Conflict of Interest:
None declared