Abstract

Mucopolysaccharidosis (MPS) is a rare metabolic disorder with a wide spectrum of symptoms affecting the bone, skeletal system, and organs of affected individuals. The severity of symptoms varies widely. The objective of this study is to examine drivers of hospital utilization in MPS patients. A retrospective descriptive study was conducted on a cross-section of MPS discharges in the MedAssets health system data for inpatient (N=527) and outpatient (N=10450) visits from 2009 through 2013. Negative binomial multivariable regression was used to identify significant drivers of inpatient and outpatient visits and inpatient length of stay (LOS). The sample included 1454 unique patients from 216 hospitals. More than half of thedischarges (66.7%) were male with 87.3% less than 18 years old and 8.7% between the ages of 18 and 29. The most common comorbid conditions in inpatients were chronic obstructive pulmonary disease (27.1%), respiratory failure (24.1%), pneumonia (20.5%), heart valve disorders (18.6%), epilepsy and seizures (17.8%), and hypertension (16.1%). In outpatients the most common comorbidities were delayed mental development (7.9%), musculoskeletal disorders (6.9%), hypertension (6.9%), and heart valve disorders (5.7%). The average number of outpatient visits was 7.1 with primary drivers including esophageal reflux (IRR=1.73, p<.001), lack of development (IRR=1.69, p<.001), gastrointestinal issues (IRR=1.47, p<.01), and hydrocephalus (IRR=1.52, p<.05). While the average number of inpatient visits was less than 1 the average LOS was 11.47. Respiratory failure (IRR=1.58, p<.001) and vein disorders (IRR=1.71, p<.01) were associated with inpatient visits. In addition to teaching status and region drivers of LOS were hypertension (IRR=1.64, p<.001), respiratory failure (IRR=1.50, p<.01), cardiovascular disorders (IRR=3.91, p<.01), pneumonia (IRR=1.40, p<.05), and vein disorders (IRR=1.71, p<.05). MPS patients average more than one outpatient visit per year and when hospitalized have long LOS. Drivers of utilization vary in the inpatient and outpatient setting.

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