Implementation of a Bleeding Risk Screening Tool and Hematology Referral Process Prior to Pediatric Endoscopy.

OBJECTIVES: Gastrointestinal (GI) endoscopic procedures are considered low risk with an overall bleeding risk for upper and lower endoscopies of 0.11%. However, a certain population of patients may have a higher risk for bleeding, and there is not a standardized process for screening patients to determine who these patients are. METHODS: At Children's Wisconsin, our gastroenterology and hematology divisions adapted an abbreviated version of a validated, history-based bleeding risk screening tool and implemented a hematology referral process to identify those at risk for bleeding prior to their first endoscopy. Provider compliance with the bleeding screen, referral to hematology, time to be seen in hematology clinic, new diagnoses of bleeding disorders, and bleeding complications were assessed from 2019 to 2021 across 3 phases. RESULTS: Provider compliance with the bleeding screen improved throughout our study from 48% (120/251) to 75% (189/253). For those who screened positive, compliance with referral to hematology ranged from 38% to 74% across our phases. The overall time to be seen by hematology decreased from 30 days to 7.5 days. Eighteen patients ultimately screened positive and were seen in hematology clinic, of whom 22% (4/18) were diagnosed with a new bleeding disorder. No bleeding complications were seen in our study population. CONCLUSIONS: Our quality improvement project provided a standardized screening tool to assess preoperative bleeding risk and reinforced the value of a history-based screening tool. This modified screening tool identified those with an undiagnosed bleeding disorder and preventative measures were undertaken to prevent procedural bleeding complications.

Endoscopic balloon dilation for management of stricturing Crohn's disease in children.

BACKGROUND: Crohn's disease (CD) has a multitude of complications including intestinal strictures from fibrostenotic disease. Fibrostenotic disease has been reported in 10%-17% of children at presentation and leads to surgery in 20%-50% of cases within ten years of diagnosis. When symptoms develop from these strictures, the treatment in children has primarily been surgical resection. Endoscopic balloon dilation (EBD) has been shown to be a safe and efficacious alternative to surgery in adults, but evidence is poor in the literature regarding its safety and efficacy in children. AIM: To evaluate the outcomes of children with fibrostenosing CD who underwent EBD vs surgery as a treatment. METHODS: In a single-center retrospective study, we looked at pediatric patients (ages 0-18) who carry the diagnosis of CD, who were diagnosed after opening a dedicated Inflammatory Bowel Disease clinic on July 1, 2012 through May 1, 2019. We used diagnostic codes through our electronic medical record to identify patients with CD with a stricturing phenotype. The type of intervention for patients' strictures was then identified through procedural and surgical billing codes. We evaluated their demographics, clinical variables, whether they underwent EBD vs surgery or both, and their clinical outcomes. RESULTS: Of the 139 patients with CD, 25 (18%) developed strictures. The initial intervention for a stricture was surgical resection in 12 patients (48%) and EBD in 13 patients (52%). However, 4 (33%) patients whom initially had surgical resection required follow up EBD, and thus 17 total patients (68%) underwent EBD at some point in their treatment process. For those 8 patients who underwent successful surgical resection alone, 4 of these patients (50%) had a fistula present near the stricture site and 4 (50%) had strictures greater than 5 cm in length. All patients who underwent EBD had no procedural complications, such as a perforation. Twenty-two (88%) of the treated strictures were successfully managed by EBD and did not require any further surgical intervention during our follow up period. CONCLUSION: EBD is safe and efficacious as an alternative to surgery for palliative management of strictures in selected pediatric patients with CD.

Defining natural history: assessment of the ability of college students to aid in characterizing clinical progression of Niemann-Pick disease, type C.

Niemann-Pick Disease, type C (NPC) is a fatal, neurodegenerative, lysosomal storage disorder. It is a rare disease with broad phenotypic spectrum and variable age of onset. These issues make it difficult to develop a universally accepted clinical outcome measure to assess urgently needed therapies. To this end, clinical investigators have defined emerging, disease severity scales. The average time from initial symptom to diagnosis is approximately 4 years. Further, some patients may not travel to specialized clinical centers even after diagnosis. We were therefore interested in investigating whether appropriately trained, community-based assessment of patient records could assist in defining disease progression using clinical severity scores. In this study we evolved a secure, step wise process to show that pre-existing medical records may be correctly assessed by non-clinical practitioners trained to quantify disease progression. Sixty-four undergraduate students at the University of Notre Dame were expertly trained in clinical disease assessment and recognition of major and minor symptoms of NPC. Seven clinical records, randomly selected from a total of thirty seven used to establish a leading clinical severity scale, were correctly assessed to show expected characteristics of linear disease progression. Student assessment of two new records donated by NPC families to our study also revealed linear progression of disease, but both showed accelerated disease progression, relative to the current severity scale, especially at the later stages. Together, these data suggest that college students may be trained in assessment of patient records, and thus provide insight into the natural history of a disease.