Implementing and Infantile Spasms Guideline and Order Set for Inpatient Healthcare Providers Scholarly Paper Draft Johns Hopkins School of Nursing

SCHOLARLY PAPER DRAFT 2 Background: In 2010, a Task Force of the Commission of Pediatrics developed a consensus document focused on the treatment of children with infantile spasms (Wilmshurst, Gaillard, Vinayan, Tsuchida, Plouin, Van Bogaert, & Nordli, 2015). This report suggested that evidence‐ based guidelines, or recommendations, for the management of infants with seizures are lacking and that evidence‐based guidelines that clarify the optimal management of seizures in the infantile period are essential for all healthcare providers treating children with infantile spasms. Misdiagnosis of infantile spasms is potentially catastrophic, with unsuccessful treatment associated with devastating neurodevelopmental arrest and enduring epilepsy. Purpose: The overall purpose of this QI project was to implement a guideline and electronic medical record order set for inpatient neurology providers caring for patients with infantile spasms, to determine provider knowledge of the evaluation and treatment of these patients prior to and after an educational intervention, and to determine provider adherence to the guidelines using an audit tool. Methodology: This quality improvement project utilized a pre-post test design to determine the effect of the educational intervention, a guideline review meeting for inpatient healthcare providers, on provider knowledge. Provider knowledge on treating patients with infantile spasms was expected to increase after the educational meeting as evidenced by a pre-post knowledge test. In addition, a retrospective chart review was completed to evaluate provider adherence to evidence based practice care of patients with infantile spasms. It was hypothesized that provider adherence would increase after the implementation of the clinical intervention. Results: The educational intervention of the guideline review meeting for inpatient neurology providers increased provider knowledge on current evidence based treatment for infants with SCHOLARLY PAPER DRAFT 3 infantile spasms by a significant value. In addition, there a statistically significant increase in provider adherence to the implemented guideline. Implications for Practice: Throughout the U.S. there is a lack of current evidence based guidelines available for providers in an inpatient setting for specific syndromes. This can lead to greater variability in treatment and higher risks for patients due to inaccurate management. As a result, health care providers may not be adequately prepared to care for vulnerable populations. Establishing current evidence based practice guidelines for inpatient specialty settings and choosing an effective implementation strategy to educate inpatient healthcare providers are first steps toward improving the care of vulnerable patient populations such as those with infantile spasms. Introduction Infantile spasms (IS), also known as West syndrome, is a severe epilepsy diagnosis defined by a triad infantile spasm seizures, abnormal rhythm on electroencephalogram (EEG) of hypsarrhythmia, and developmental regression (Wheless, Gibson, Rosbeck, Hardin, O’Dell, Whittemore, & Pellock, 2012). Misdiagnosis of infantile spasms is potentially catastrophic, with unsuccessful treatment associated with devastating neurodevelopmental arrest and continued epilepsy (Hussain, Lay, Cheng, Weng, Sankar, & Baca, 2017). IS therefore is the epitome of an epileptic encephalopathy where urgent and accurate diagnosis, evaluation, and treatment is essential, as poor developmental outcome is correlated with increased length of time to treatment and inadequate seizure control (Wheless et al., 2012). Suspected cases of infantile spasms should be evaluated by acute care neurologists immediately so that a timely diagnosis can be established and correct treatment initiated. In 2010, a Task Force of the Commission of Pediatrics developed a consensus document focused on SCHOLARLY PAPER DRAFT 4 the treatment of children with infantile spasms (Wilmshurst, Gaillard, Vinayan, Tsuchida, Plouin, Van Bogaert, & Nordli, 2015). This report suggested that evidence‐based guidelines, or recommendations, for the management of infants with seizures are lacking and that evidence‐ based guidelines that clarify the optimal management of seizures in the infantile period are essential for all healthcare providers treating children with infantile spasms. Subsequently, there has been a significant focus on promoting adherence to evidence based evaluation and treatment guidelines aimed at healthcare providers treating patients with infantile spasms. Even with the exponential growth of publicly available clinical practice guidelines, providers often have difficulty in equipping themselves with the evidence necessary to provide the standard of care. As healthcare providers caring for patients with this devastating diagnosis, use of evidence based treatment recommendations to guide treatment is critical to patient safety. For this reason, both an evidence based infantile spasms treatment guideline and electronic medical record order set were implemented on the inpatient neurology unit at Ann and Robert H. Lurie Children’s Hospital. Background and Significance Wheless et al (2012) reports IS affects infants between 4 and 12 months old, accounts for as much as 5% of all childhood-onset epilepsy, and accounts for up to 20% of new-onset epilepsy cases in children under 2 years of age. IS is diagnosed in about 1.6–4.5/10,000 live births, about 1,200 infants diagnosed in the United States each year (Wheless et. al, 2012). Epilepsy and seizures may affect more than 3 million Americans of all ages, at an estimated annual cost of $15.5 billion in direct and indirect costs (Kurth, Lewis, & Walker, 2010). Kurth, Lewis, & Walker (2010) also reported the population of patients with infantile spasms is reported to have the highest utilization of health care costs as compared to other epilepsy diagnoses and a large SCHOLARLY PAPER DRAFT 5 societal burden. Overall patients with infantile spasms averaged about 10 physician visits a year, although only two of them were coded to epilepsy and some 24 diagnostic tests or procedures annually according to Kurth, Lewis, & Walker (2010). In addition, patients averaged more than 30 drug dispensing’s yearly, about one‐fourth of these being for anticonvulsant medications. In one study of epilepsy cost burden, Kurth, Lewis, & Walker (2010) reported that the small group of patients with infantile spasms had the highest mean values of almost all classes of healthcare resources utilized. This included outpatient physician visits, days hospitalized, diagnostic procedures, and medical supplies. At the Ann and Robert H. Lurie Children’s Hospital, an average of 32.2 patients are admitted and diagnosed with infantile spasms each year, approximately 2.6 patients each month. Despite distinctive presentation and a common procedure for confirming the diagnosis (EEG), the identification of infantile spasms can be challenging because spasms are often mistaken for gastroesophageal reflux or “normal” infant movements (Hussain, Lay, Cheng, Weng, Sankar, & Baca, 2017). The diagnostic challenge of infantile spasms is the urgency of treatment, as even a brief delay (as little as one week) has been associated with poor long-term neurodevelopmental outcomes (Hussain et. al, 2017). Hussain et. al (2017) also reports that approximately 30% of children with infantile spasms, those treated successfully and those with “mild” underlying causes of infantile spasms, can become developmentally normal and seizurefree. However, due to to either treatment delay and/or severe underlying causes of infantile spasms, the majority of children experience continued seizures and/or poor cognitive outcomes, and nearly 10% will die by their second birthday. After seizures are treated, the infant’s brain has a longer period to recover and gain developmental ground that may have been lost while the seizures occurred (Saemundsen, SCHOLARLY PAPER DRAFT 6 Ludvigsson, & Rafnsson, 2008). Thus, a thorough etiological work up and immediate evidence based treatment with medication is essential to improving neurological and developmental outcomes. In addition, monitoring and follow up is essential for patients with IS, as over 40% of patients do not respond to the first treatment therapy (Saemundsen, Ludvigsson, & Rafnsson, 2008). Guidelines for the evaluation, treatment, monitoring, and follow up of patients with infantile spasms are currently available for utilization by pediatric epilepsy providers. Problematically, a multitude of pediatric epilepsy providers are not utilizing or adhering to the present guidelines established by several consensus reports for management of infantile spasms. The primary benefit of the quality improvement project is improving quality of care by increasing provider adherence to a standardized guideline on the evaluation and treatment of patients with new onset infantile spasms. Review of Literature A search was conducted to evaluate effective guideline implementation strategies for inpatient units using PubMed, the Cochrane Library database, Cumulative Index to Nursing and Allied Health Literature (CINAHL), and EMBASE. An initial search was conducted in October 2017, and the publication time frame included studies published between January 2008 and October 2017. MeSH terms utilized during the search included ‘implementation,’ 'strategies,' ‘inpatient,’ and ‘guideline.’ A total of 325 titles were identified and reviewed. Of these, 108 studies were considered to be relevant with 14 total studies meeting the inclusion and exclusion criteria. The inclusion criteria for the review were articles written or translated in the English language, included at least one guideline implementation strategy, and implementation strategies SCHOLARLY PAPER DRAFT 7 targeted at inpatient healthcare providers or units. Exclusion criteria included those articles that focused solely on barrie