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Summary of i2b2 Data: Spirometry in Pediatric Sickle Cell Populations

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Summary of i2b2 Data: Spirometry in Pediatric Sickle Cell Populations
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Loscalzo, Melissa
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Comorbidity of sickle cell disease (SCD) and asthma puts individuals at increased risk of acute chest syndrome, the leading cause of premature death in patients with Sickle Cell Disease (Miller, 2011). Prompt diagnosis via pulmonary function testing (PFT) may reduce the incidence of acute chest syndrome (ACS). The purpose of this study is to analyze i2b2 data obtained from four major academic medical centers (Harvard, Texas Medical Center, Indiana State, and the University of Florida) to observe the incidence of children comorbid with sickle cell disease, asthma, and acute chest syndrome. Data revealed that among the four centers, only 30.9% of patients with sickle cell, asthma, and acute chest syndrome received spirometry treatment. Additionally, we can begin to explore the care provided to patients comorbid with these conditions and determine if revision of the standard of care is necessary. The scope of this study pertains to pediatric sickle cell patients comorbid with asthma and is limited to identifying whether there is reason to pursue further research on this topic and develop alternative standard care measures. Prompt management of asthma can improve quality of life and life expectancy for this population and is therefore imperative to their care. ( en )
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Awarded Bachelor of Science in Nursing, cum laude, on May 8, 2018. Major: Nursing
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College or School: College of Nursing
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Advisor: Laurie Duckworth. Advisor Department or School: College of Nursing

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Copyright Melissa Loscalzo. Permission granted to the University of Florida to digitize, archive and distribute this item for non-profit research and educational purposes. Any reuse of this item in excess of fair use or other copyright exemptions requires permission of the copyright holder.

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Running Head: SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 1 Summary of i2b2 Data: Spirometry in Pediatric Sickle Cell Populations Melissa Sue Loscalzo University of Florida College of Nursing

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 2 Table of Contents Results and Discussi Ref

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 3 Abstract Comorbidity of sickle cell disease (SCD) and asthma puts individuals at increased risk of acute chest syndrome, the leading cause of pr emature death in patients with sickle cell d isease (Miller, 2011). Prompt diagnosis via pulmonary function testing (PFT) may reduce the incidence of acute chest syndrome (ACS). The purpose of this study is to analyze i2b2 data obtained from four major academic medical centers (Harvard, Texas Medical Center, Indiana State, and the University of Florida) to observe the inciden ce of children comorbid with sickle cell disease, asthma, and acute chest syndrome. Data revealed that among the four centers, only 30.9% of patients with sickle cell, asthma, and acute chest syndrome received spirometry treatment. Additionally, we can beg in to explore the care provided to patients comorbid with these conditions and determine if revision of the standard of care is necessary. The scope of this study pertains to pediatric sickle cell patients comorbid with asthma and is limited to identifying whether there is reason to pursue further research on this topic and develop alternative standard care measures. Prompt management of ast hma can improve quality of life and life expectancy for this population and is therefore imperative to their care. Introduction Within the United States, approximately 100,000 individuals have been diagnosed wit h sickle cell disease (Hassell 2010). Sickle cell disease is a common genetic disorder primarily affecting African American populations, with 1 in 500 of these individuals being diagnosed with the disease (Hass ell, 2010). According to the Centers for Disease Control and Prevention, approximately 25 million US individuals have been diagnosed with asthma, a condition that is influenced by both genetic and environmental factors (2018). A common complication for sickle cell patients is acute chest syndrome, which is characterized by chest pain, tachypnea fever,

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 4 respiratory distress, and lung infiltrate s (Gladwin & Rodgers, 2000). Acute chest syndrome is caused by sickling within the microvasculature and subsequent vaso occlusion. These vaso occlusive episodes result from the sickled cells characteristic of the disease process their rigid and sticky nature can cause them to block small vessels, thus impairing the flow of blood, which carries vital oxygen and nutrients throughout the body (Gladwin & Rodgers, 2000). Without supportive care, acute chest synd rome can quickly progress to death, and has actually been identified as the most common cause of death in sick le cell patients (Quinn, Rogers & Buchanan, 2004). With this being said, early identification and rapid management of this condition can be a matt er of life or death within this patient population. A correlation has been identified between age and pulmonary function, with younger children being more predisposed to deteriorating lung function and acute chest syndrome episodes (Greenough & Knight Madd en, 2018). This interrelationship places even greater emphasis on implementing treatment measures as early as A relationship has been identified among patients comorbi d with asthma and sickle cell disease, with pediatric patients being five times more likely to experience respiratory symptoms during a vaso occlusive episode if asthma is present as well (Jain, Bakshi & Krishnamurti, 2017). With a correlation this strong, appropriate interventions in regards to the asthma are imperative to not only managing, but also preventing pathologic progression condition. The prognosis of pediatric populations is characteristically unpredictable and at times fragile, so the coexistence of an asthmatic condition in a sickle cell patient greatly increases their likelihood of complications arising. Although more emphasis has b een placed on the importance of adequate management in patients comorbid with these conditions in recent years, a gap still persists in relation to proper screening, diagnosis, and appropriate intervention.

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 5 In this study, numerical statistics have been ex tracted from four major academic medical centers, evaluating the number of patients with sickle cell disease, asthma, acute chest syndrome, and those who received spirometry treatment. This data can be used to compare and contrast the frequency of asthma s creening and diagnosis among sickle cell populations and the development of acute chest syndrome. The information provided by this study can present the reality of the frequency of asthma screening and subsequent symptom management within this patient popu lation. By identifying relevant relationships and contributing factors to this potentially fatal condition, this study can advance the care that is provided to this patient population. By evaluating the effectiveness of spirometry interventions, this study can provide health care professionals with a greater unde rstanding of how to manage care for these individuals. The purpose of this study is The objectives of this study are as follows: to analyze the coexistence of asthma diagnosis among this patient population and its relationship to the development of acute chest syndrome, to determine the frequency of interventional spirometry utilization among patients with sickle cell, asthma, and acute chest syndrome, and to explore the reasoning behind our findings. Narrative Data from four large academic medical centers has been gathered and evaluated for the prevalence of sickle cell disease (SCD), asthma, and acute chest syndrome (ACS) among patients 5 34 years of age spanning from December 2010 through December 2015 using i2b2, an IRB app roved de identified database. The four academic medical centers include Harvard, Texas Health Center, Indiana University and UF Health Shands at the University of Florida. These

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 6 medical centers are labeled A, B, C, and D, respectively, throughout the stud y. The data collected from the four centers includes total sickle cell patients, those patients comorbid with asthma, those patients who developed acute chest syndrome, and those who received interventional spirometry treatment. Data was then organized int o a table according to the following criteria: (1) total sickle cell patients (2) sickle cell and asthma (3) sickle cell and acute chest syndrome (4) sickle cell, asthma, and acute chest syndrome (5) sickle cell, asthma, acute chest syndrome, and spiromet ry treatment. Queries to cross reference those patients with the current procedural terminology (CPT) code for pulmonary function testing (PFT) was then completed The gathered information is organized into data tables that are referenced throughout the s tudy and can be located within Appendices A, B, and C. From the collected data, we are able to compare and contrast the incidence of acute chest syndrome development among these patients as it relates to asthma diagnosis and the implementation of spirometr y treat ment measures. This data has been manipulated to yield percentages regarding the following: (i) sickle cell patients presenting with asthma (ii) sickle cell patients who developed acute chest syndrome (iii) sickle cell patients with both asthma and acute chest syndrome (iv) sickle cell patients with asthma, acute chest syndrome, and received spirometry treatment for each academic medical center.

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 7 From this data and the identified trends, we can begin to explore the processes behind the statistics. W e can begin to question the reasoning behind our findings, and whether the results are due to inadequate screening measures, a gap in documentation, or a variety of other contributing factors. Understanding the contributing factors that led to the statisti cs at hand is the first step to deriving meaning from this data set. The project described in this study was initiated as a University of Florida College of Nursing Honors research project by honors student Melissa Loscalzo (traditional BSN senior) along w ith research preceptor Dr. Laurie Duckworth, PhD, ARNP in Gainesville, Florida The project includes the analysis of i2b2 data obtained from four academic medical center organizations: Harvard, Texas Health Center, Indiana University, and UF Health Shands at the University of Florida. Meetings between the honors student and research preceptor took place from May 2017 through April of 2018 within the University of Florida College of Nursing in regard to the scope of the project, organization, data acquisitio n, research design, stakeholder analysis, and review of the finished project. Rough drafts of the project have been provided to the mentor for feedback and guidance. Additionally, the final draft of the thesis and e poster have been submitted to the mentor for approval prior to submission/presentation for the UF honors course as well as the 2018 University of Florida Nursing Research Summit and Malasanos Lectureship on Friday, April 13 th 2018 This study required a great deal of collaboration among multiple parties, and several key stakeholders can be identified. Stakeholder involvement includes Dr. Laurie Duckworth, PhD, ARNP, who gathered and organized the data from the i2b2 de identified data base constructed the initial data table along with accompanying calculations, conducted literature review, and presented findings at the 2017 Sigma Theta Tau International Nursing Research Congress held in

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 8 Dublin, Ireland and served as the UF CON honors research preceptor for this project. Additionally, Melissa Loscalzo, a University of Florida College of Nursing Honors student completed summary of the numerical i2b2 data to covert the quantitative data into descriptive statistics. Significant c orrelation s among the data have been identified and significant findings have been summarized. The reasoning behind the findings of this study have been explored and given thoughtful consideration in order to interpret the data and derive meaning. Lastly, the four a cademic medical centers (Harvard, Texas Health Center, Indiana University, and UF Health Shands at the University of Florida) from which i2b2 data has been extracted are key stakeholders for this study as well A potential barrier to this research study w ould be rejection from the academic medical centers in regard to participating in the study. Without the data obtained from these facilities, we would have either needed to explain in more detail why participation is so vital to the study in order to persu ade the centers or seek participation from different facilities entirely. Another barrier to this study is the ability to account for unmeasurable factors and how they have contributed to the extracted data for each medical center within their unique geogr aphical location s Some of these factors include weather/environment, lifestyle factors, diet, family history, patient noncompliance, or health literacy level. An actual barrier related to this study involved the initial delineation of what aspect of the s tudy for the honors student to focus on. This barrier was resolved by reviewing materials provided by the honors research mentor, study (refer to appendix D as follows: Exploring Spirometry Use to Measure Asthma Control in Patients with Sickle Cell Disease: A Multicenter Study (IRB 201601125). The honors student and preceptor met several

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 9 times within the UF College of Nursing throughout the course of the mentorship in order to determine the scope of the nursing honors thesis and provide assistance as needed. Results and Discussion The following i2b2 data was obtained for academic medical c en ters labeled A, B, C, and D. Refer to Appendix A to view the data table for the following criteria: (1) T otal sickle cell patients: A=428, B=378, C=1202, D=74 (2) S ickle cell and asthma: A=79, B=57, C=377, D=64 (3) S ickle cell and acute chest syndrome: A=72, B=78, C=142, D=117 (4) S ickle cell, asthma, and acute chest syndrome: A=37, B=43, C=146, D=23 (5) S ickle cell, asthma, acute chest syndrome, and spirometry treatment: A=1, B=18, C=52, and D=6. Using this data, we can yield the following quantitative data (refer to Appendices B and C and to compare and contrast these values within a bar graph): (i) P ercentage of sickle cell patients comorbid with asthma, which can be obtained by dividing the values from item (2) by item (1): A=18.5%, B= 15.1%, C=31.4%, D =8.6% (ii) Sickle cell patients who developed acute chest syndrome, obtained by dividing item (3) by item (1): A=16.8%, B=20.6%, C=11.8%, D=15.8% (iii) Sickle cell patients with both asthma and acute chest syndrome, obtained by dividing item (4) by item (1): A=8.6%, B=11.4%, C=12.1%, D=3.1% (iv) Sickle cell patients with asthma, acute chest syndrome, and received spirometry treatment, obtained by dividing item (5) by item (4): A=2.7%, B=41.9%, C=35.6%, D=26.1%.

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 10 Among the four medical centers, a total of 249 patients fell under item (4) sickle cell, asthma, and acute chest syndrome, and of those patients, only 77 received pulmonary function testing (item 5), therefore only 30.9% of patients with SCD, asthma, and ACS utilized pulmonary function testing. A notable correlation among the data is that medical center C had the highest value for item (i) with a value of 31.4% of sickle cell patients diagnosed with asthma, which is almost double the value of any other center that was evaluated Additionally, medical center C had th e lowest value for item (ii) out of the four centers with only 11.8% of sickle cell patients who had developed acute chest syndrome The highest percentage of patients comorbid with sickle cell, asthma, and ACS receiving spirometry treatment was found wi thin medical ce nter B with a value of 41.9%, which is not even half of the patient population for this particular center. Also, the percentage of patients receiving interventional spirometry varied greatly, with the highest value being 41.9% (medical cente r B), and the lowest being 0.2% (medical center A). Based on these findings, it raises the question of how this data came to be: is there a lack of pulmonary and asthma evaluation among this patient population, or are these patients indeed being evaluated for this condition and there is just a gap in documentation? Additionally, we can explore the reasoning as to why only 30.9% of patients with sickle cell, asthma, and acute chest syndrome received interventional spirometry, or if this reveals yet another gap between implementation of interventions and proper documentation. Another notable finding is seen within medical center C, in which there was the highest percentage of sickle cell patients diagnosed with asthma but also the lowest percentage of acute c hest syndrome development among this patient population This leaves us with the question of what measures have been set in place within this particular facility to ensure asthma screening and diagnosis takes place, and

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 11 if this is the reason for the low in cidence of acute chest syndrome among the patients within this facility. Given the results found in this single study, it is important to evaluate our findings compared to current literature that relates to the topics we explored According to the Center of Disease Control and Prevention, approximately 12.7% of children had received an asthma diagnosis within their lifetime ( Akinbami, 200 6). Another study conducted by the American Society of Hematology, found that 17% of the participants with sickle cell d isease were comorbid with asthma ( Boyd, Macklin, Strunk, & Debaun, 2006). Within our study, the percentage of sickle cell patients with asthma is not too far off from these statistics. The exception to this would be within academic medical center C, which we have already pointed out as having the highest rate of asthma diagnosis among these sample populations. This leaves us to further consider how this statistic came to be is there a significant abundance of environmental or genetic factors within the geo graphic region of medical center C, or is it related to prompt screening and documentation within this facility? Another study found that there is an association between sickle cell children who both currently have asthma or have ever experienced asthma wi th recurrent acute chest syndrome episodes ( Knight Madden, Forester, Lewis & Greenough, 2005). The correlation of these two conditions furthers the argument of this study, that sickle cell patients with asthma require prompt pulmonary in tervention measures for symptom management and in order to prevent exacerbation of their condition. Although t he amount of research expl oring the use of spirometry in sickle cell patients is limited, a study conducted by Scott. T. Miller (2011) has shown that the implementat ion of spirometry prescribed for ten times every two hours while awake has shown to reduce the occurrence of acute chest sy ndrome for sickle cell patients

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 12 pulmonary function testing and tre atment can indeed manage the symptoms of patients presenting with sickle cell disease, as well as prevent the onset of more serious pulmonary complications. Summary and Conclusion By conducting this study, we aimed to quantify the comorbidity of asthma an d acute chest syndrome among pediatric sickle cell populations. Among these groups, we examined the utilization of interventional spirometry treatments for these patients. Lastly, we were left to explore the reasoning behind such findings, and how the data came to be. By extracting i2b2 data from four academic medical centers, we were able to identify trends among the data and possible correlations. It was discovered that among the four centers, less than one third (30.9%) of sickle cell patients comorbid w ith asthma and acute chest syndrome received interventional spirometry. It was also discovered that the medical center with the highest incidence of asthma had the lowest incidence of acute chest syndrome, causing us to question the interventions in place that seems to be saving these patients from the exacerbation of their pulmonary s tatus Percentages of patients receiving spirometry varied greatly from one center to the next. The information gathered from this study can help us to learn more about this p atient population and the proper interventions to be included in their care. development of acute chest syndrome, one of the leading causes of fatality among this patient population. Utilization of PFT's in patients with SCD may aid in early diagnosis and subsequent symptoms management of asthmatic symptoms. Early diagnosis facilitates effec tive management of symptoms, can reduce exacerbations and, consequently, the develop ment of acute chest syndrome. From this data, we can infer that medical center C is actively screening and

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 13 diagnosing sickle cell patients for asthma and following through with subsequent treatment measures for their pulmonary status. As a result, the occ urrence of acute chest syndrome is been set in place could be beneficial for other medical providers and this patient population as a whole. From the data obt ained from the four medical centers, we can infer that there is a gap between adequate pulmonary screening measures for sickle patients and/or proper documentation. An increased level of attention should be brought to this issue, and a revised standard of practice implemented in order to ensure adequate care of this patient population and prevention of complications. This can alter the way that nursing professionals and other healthcare providers care for sickle cell patients, which can lead to an entirely new standard of care for those diagnosed with having asthma or other pulmonary symptoms. This can lead to a decrease in mortality among this patient population, as well as an overall improv ement in their quality of life and overall prognosis. As an aspiri ng pediatric nurse, the findings of this study will impact my professional development as I encounter children with sickle cell throughout my career. Due to my experience with and the knowledge I have gained through this research study, I will have a heigh tened awareness in regard to identifying pulmonary symptoms and now that I know the impact of such comorbidities in these individual s I will be able to make conscientious, prudent decisions in regard to their care. I hope to pursue my doctorate in nursing practice here at the University of Florida in the near future, and during that time I will have the opportunity to continue my participation in this study and expound upon the knowledge that has been identified with in this project

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 14 This study has the set the stage for further research to take place in order to examine the effect of pulmonary function testing on this patient population. currently devising a pilot study to evaluate the use of interventional spirometry trea tment in pediatric sickle cell populations, which is expected to span over six months for data collection The plan for this study includes the establishment of a cohort divided into two groups: a control group receiving standard of care asthma treatment a nd an experimental group participating in interventional spirometry treatments with regular follow up and evaluation Collection of data regarding symptoms, peak flow, quality of life, and frequency of hospitalizations can reveal the effectiveness of inter ventional pulmonary treatment in comparison to the standard of care treatments in place currently.

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 15 References Akinbami L. J., The state of childhood asthma, United States, 1980 2005. Advance data, no. 381,pp. 1 24, 2006. Boyd, J. H., Macklin, E. A., Strunk, R. C., & Debaun, M. R. (2006). Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood, 108 (9), 2923 2927. doi:10.1182/blood 2006 01 011072 Gladwin, M. T., & Rodgers, G. P. (2000). Pathogenesis and treatment of acute chest syndrome of sickle cell anaemia. The Lancet, 355 (9214), 1476 1478. doi:10.1016/s0140 6736(00)02157 7 Greenough, A., & Knight Madden, J. (2018). Kendigs disorders of the resp iratory tract in children (9th ed.). Philadelphia: Saunders. doi:10.1016/B978 0 323 44887 1.00062 6 Hassell K Population estimates of sickle cell disease in the U,S. American Journal of Preventive Medicine, vol.38,no. 4,pp. S512 S521, 2010. CDC.gov. (2018 ). CDC Asthma Most Recent Asthma Data. [online] Available at: https://www.cdc.gov/asthma/most_recent_data.htm [Accessed 16 Feb. 2018]. Jain, S., Bakshi, N., & Krishnamurti L. (2017). Acute Chest Syndrome in Children with Sickle Cell Disease. Pediatric Allergy, Immunology, and Pulmonology, 30 (4), 191 201. doi:10.1089/ped.2017.0814 Knight Madden, J. M., Forester, T. S., Lewis, N. A., & Greenough, A. (2005). Asthma in childre n with sickle cell disease and its association with acute chest syndrome. Thorax,60 (3), 206 210. doi:10.1136/thx.2004.029165

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 16 Miller S T How I treat acute chest syndrome in children with sickle cell disease. Blood 2011;117:5297 305. Quinn, C. T., Rogers Z. R., & Buchanan, G. R. (2004, June 01). Survival of children with sickle cell disease. Retrieved March 13, 2018, from http://www.bloodjournal.org/content/103/11/4023?ijkey=22a19e18ce978f5342dc8eb4b1 00df8cea911175&keytype2=tf_ipsecsha

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 17 Appendix A SCD Comorbidi ty with Asthma and/or ACS vs. Those Receiving Spirometry Appendix B

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 18 Appendix C Appendix D Use of Spirometry to Measure Asthma Control in Patients with Sickle Cell Disease Purpose The purpose of this study is to determine the use of spirometry testing to monitor and evaluate asthma control in patients with asthma, sickle cell disease and acute chest syndrome, ages 5 to 34 years. Background Sickle Cell Disease (SCD) is a common inherited genetic disorder and affects approximately 100,000 people in the United States annually and one in 400 African American births (CDC, 201 6). Asthma affects 23 mil lion people in the US alone. African American

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 19 children are disproportionally affected having a greater prevalence rate for asthma compared to Caucasians, in addition to a higher rate of hospitalization and higher mortality rate. SCD, when combined with a diagnosis of asthma, increases the risk of Acute Chest Syndrome (ACS), which can lead to significant morbidity and mortality. Findings from The National Heart Lung and Blood Institute (NHLBI) funded Sickle Asthma Cohort (SAC) study confirm that asthma is a risk factor for ACS in patients with SCD and that one early life ACS episode was a significant predictor for future ACS events (DeBaun et al., 2014). It has been reported that as many as 28% of children with SCD may have asthma {Strunk et al., 2014). Whil e there seems to be increasing recognition of the importance of co morbid asthma and SCD, asthma continues to be underdiagnosed and undertreated (DeBaun, Strunk, 2016). NHLBI Guidelines for the Diagnosis and Management of Asthma should be followed for pati ents with SCD and asthma (EPR 3, 2007). This would include routine follow up by a pulmonology provider to ensure proper management of asthma. Part of this routine care would include Spirometry testing to evaluate effectiveness of asthma medications and su btle changes in pulmonary function. Early detection of a decrease in pulmonary function may lead to changes in management, which may decrease the incidence of an asthma exacerbation and possibly prevent an occurrence of ACS. The number of spirometry proc edures conducted may be indicative of how many patients with Asthma and SCD are receiving routine pulmonology management. Data gleaned from the EMR database may show that although a significant number of patients with SCD and ACS carry the diagnosis of as thma, very few have spirometry testing to evaluate the effectiveness of asthma treatment or to provide early detection of worsening lung function. Methods

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 20 A descriptive cross sectional study design was utilized to identify the number of patients with SCD, ACS, Asthma and the number of spirometry procedures performed. The Electronic Medical Record (EMR) using i2b2, (a de identified data repository) for 4 Academic Medical Centers (AMC) was queried for the count of patients 5 34 years of age seen between 12 /01/2010 and 12/01/2015 having co morbid diagnoses of Asthma, Sickle Cell Disease and Acute Chest Syndrome. Queries to cross reference those patients with the CPT code for spirometry was then performed. The i2b2 query included; (1) Number of patients with SCD + ACS + Asthma + Spirometry, (2) Number of patients with SCD, (3) Number of patients with SCD + Asthma, (4) Number of patients with SCD + ACS, and (5) Number of patients with SCD + ACS + Asthma. The percentage of SCD + ACS+ Asthma patients having spir ometry testing was calculated by dividing SCD + ACS + Asthma +Spirometry by the number of patients with SCD + ACS+ Results The combined total number of patients for 4 AMC (A,B,C,D) for categories (1) SCD+A CS+Asthma+Spirometry,(2) SCD,(3) SCD+Asthma, (4) SCD+ACS, (5) SCD+ACS+Asthma are respectively; (1)77, (2)2749, (3) 577, (4) 409, and (5) 249. Number of patients for each AMC for these five categories; (1) Number of patients with SCD + ACS + Asthma + Spirom etry, A =<10, B= 18, C= 52, D= <10. (2) Number of patients with SCD, A=428, B=378, C=1202, D=741. (3) Number of patients with SCD+Asthma, A=79 (18.4%), B=57 (15.0%), C=377 (31.3%), D=64 (8.6%). (4) Number of patients with SCD+ACS, A=72 (16.8%), (B) =78 ( 20.6%), C=142 (11.8%), D=117 (15.7%). (5) SCD+ACS+Asthma, A=37 (8.6%), B=43 (11.4%), C=146 (12.1%), D=23(3.1%). The percent of patients with SCD+ACS+Asthma who also had spirometry performed for each AMC was calculated

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SPIROMETRY IN PEDIATRIC SICKLE CELL POPULATIONS 21 (SCD+ACS+Asthma+Spiro/SCD+ACS+Asthma = %); A=<10%, B=41.8%, C=35.6%, D=26%. The total number of patients with SCD+ACS+Asthma for all 4 AMC= 249, those having spirometry = 77, thus (77/249) 30.9% of patients with SCD+ACS+Asthma had spirometry performed. Conclusion Asthma is prevalent in childr en with SCD and may result in episodes of ACS. Across 4 AMC only 30.9% of patients with SCD, ACS, and asthma received spirometry testing. In addition there is considerable variation among the 4 AMC ranging from <10% to 41.8% receiving spirometry. Despit e the NHLBI recommendations for children with asthma it appears that only a third of these patients with co morbid SCD, ACS, and Asthma receive this procedure. Very few are being routinely tested for changes in pulmonary function, or those tests are not b eing coded and documented appropriately. Better coding and documentation practices for spirometry testing in those with sickle cell disease and asthma will allow better access for providers to utilize those test results for asthma management. If the tes ting is not being done routinely, further research should be done to determine if there are barriers to obtaining appropriate pulmonology evaluation and management.