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WISKOTT ALDRICH SYNDROME -- Is transplant a better option than supportive care?
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  • Nikki Agarwal,
  • Divyaswathi Citla Sridhar,
  • Sindhoosha Malay,
  • Nirav Patil,
  • Sanjay Ahuja,
  • jignesh dalal
Nikki Agarwal
MetroHealth Medical Center
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Divyaswathi Citla Sridhar
UH Rainbow Babies and Children's Hospital
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Sindhoosha Malay
Case Western Reserve University
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Nirav Patil
Case Western Reserve University
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Sanjay Ahuja
UH Rainbow Babies and Children's Hospital
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jignesh dalal
UH Rainbow Babies and Children's Hospital
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Abstract

Background : Wiskott Aldrich syndrome (WAS) is a rare disease and hematopoietic stem cell transplant (HCT) is considered the treatment modality of choice for WAS. Methods : We conducted a cross-sectional analysis on the Kids’ inpatient database and compared hospitalization rates, complications and healthcare utilizations in the transplant and non-transplant arms. Results. : Of the 396 patients with WAS admitted between 2006-2012, 114 underwent transplant and 269 did not. The non-transplant arm included older children, female patients and more African Americans. Death rates, income and payer source were similar in both arms, however the total charge for each admission was higher in the transplant arm. ED visits were similar but elective admissions were more in the transplant arm. Length of stay was prolonged in the transplant arm. When comparing morbidities, lymphomas, ulcerative colitis and autoimmune complications of WAS were seen only in the non-transplant arm. Conclusions : Our study shows that transplant is the largest contributor to healthcare utilization in WAS patients. We noted a change in practice moving away from splenectomy in WAS patients. We identified healthcare disparities based on race and socioeconomic status and found that only a few centers across the nation manage WAS, suggesting need for wider resources for this very rare disease.