Phoenix Children Research Articles

Initiation of family-centered bedside rounds at Phoenix Children's Hospital Center for Cancer and Blood Disorders.

170 Background: There has been a national trend towards a family/patient-centered care model in an effort to achieve many goals: earlier discharges, enhanced collaboration and patient safety, and improved satisfaction among medical care teams, patients and families. While most of the services at Phoenix Children’s Hospital (PCH) utilized a family-centered rounding model, the hematology/oncology service used table rounds. In December 2017, the Center for Cancer and Blood Disorders (CCBD) initiated family-centered rounds (FCR) on the inpatient service. Methods: FCR participants include patient, parent(s), bedside nurse, clinical supervisor, residents, fellow, attending, nurse practitioners, pharmacist, dietitian and child life specialist. One unique aspect of our FCR model is using scheduled rounds, whereby each patient is allotted a specific length of time and each family is given a time range within which to expect the team. Patients are scheduled every 10 minutes and if more time is needed, the attending/fellow return later for discussion. Staying on a schedule allows for improved nursing workflow and for consulting teams and interpreters to join rounds for specific patients. Outcome measures include patient, family and employee satisfaction. Results: Patient satisfaction is measured using NRC Real-time electronic survey. Scores have increased from a baseline of 70% (Oct/Nov 2017) to 87.5% (April 2018) after the initiation of FCR. From a qualitative standpoint, families have expressed gratitude for the new rounding model. Staff have expressed increased satisfaction at the efficiency and thoroughness of communication on rounds and the multidisciplinary team allows for real-time collaboration. Conclusions: FCR have led to increases in patient satisfaction both quantitatively and qualitatively. Ongoing initiatives include adding family-centered night rounds and a more extensive evaluation of FCR’s impact on employee satisfaction, patient safety, length of stay and lower overall costs. We are continuously evaluating the structure and efficacy of FCR to achieve the best possible care for our patients.

Pediatric and Gynecologic Rates of Documentation of Last Menstrual Period in Female Adolescents.

The American Academy of Pediatrics and American College of Obstetricians and Gynecologists have identified the menstrual cycle as essential in assessing overall health of adolescent girls. Menses should be considered a "vital sign" and documentation of last menstrual period (LMP) is recommended at every patient encounter. The aim of this study was to estimate and compare LMP documentation among adolescent and pediatric health care providers. A retrospective cohort study identified a random sample of 50 female patients seen in the general pediatrics (PEDS), adolescent medicine (AM), and pediatric and adolescent gynecology (PAG) ambulatory clinics at Phoenix Children's Hospital between January 1, 2016 and March 31,2016. Rates of LMP documentation were compared between clinics using the Pearson χ2 test. Multivariable logistic regression analyses quantified the association of clinic and LMP documentation, with adjustments for visit reason, provider gender, and age at presentation. Rates of LMP documentation were 100%for adolescent gynecologists, but significantly lower for general pediatricians (14%) and AM (60%) providers (P<.0001). These findings were confirmed in multivariable analyses (PAG vs PEDS: odds ratio [OR], 280; 95% confidence interval [CI], 32-2331; PAG vs AM: OR, 34; 95% CI, 4.4-270; AM vs PEDS: OR, 3.8; 95% CI, 1.3-11.0). Adolescent gynecologists document LMP routinely. In contrast, LMP documentation rates in AM, and especially in PEDS, were significantly lower. Quality improvement methods such as incorporation of an LMP section into the vitals portion of an electronic medical record could help improve rates of compliance.

Editorial introductions

Editorial introductions

Asthma treatment and outcomes for children in the emergency department and hospital

ABSTRACTObjective: To describe and compare the treatment of acute asthma exacerbations in children given in the emergency department (ED) and admitted to acute care floor in the hospital or intensive care unit (ICU). Methods: A retrospective chart review of visits for acute exacerbation of asthma treated at Phoenix Children's Hospital between January 1, 2014 and December 31, 2016. Results: A total of 287 asthma exacerbation cases were identified including 106 (37%) ED visits, 134 (47%) hospital floor and 47 (16%) ICU admissions. A history of a previous ED visit (ED 88%, Floor 60% and ICU 68%; p < 0.0001) and prior pulmonology inpatient consultation (ED 30%, Floor 19% and ICU 15%; p = 0.05) varied significantly. Pulmonology inpatient consultations were performed more frequently in the ICU than on the hospital floor (54% versus 8%; p < 0.0001). Although overall 145 (51%) of the cases were already on inhaled corticosteroids (ICS) at the time of visit with no differences across locations, ICS initiation/step-up was greater in the ICU (72%) than on the hospital floor (54%) and ED (2%) (p < 0.0001). A recommendation given to the family for follow-up with pulmonology was more frequent for patients who had been admitted to the ICU (68%) as compared to those only admitted to the floor (31%) or ED (4%) (p < 0.0001). Readmission rates were similar for patients previously admitted to the hospital (Floor 42%; ICU 40%), but significantly higher for previous ED visits (77%) (p < 0.0001). Conclusions: Physicians in the ED have an opportunity to provide preventative care in the acute care setting and should be encouraged to initiate treatment with ICS. Consideration should be given to develop a program or clinical pathway focused on long-term asthma management and maintenance to reduce readmissions and long hospital stays.

Clinical Presentation and Outcomes Associated with Different Treatment Modalities for Pediatric Bark Scorpion Envenomation.

Scorpion envenomation is potentially life-threatening and affects children in the Southwestern USA. An FDA-approved antivenom is available, but its high cost has led to use of off-label antivenom dosing or supportive care alone as alternatives to FDA-recommended dosing. This study sought to determine whether treatment approach influences outcomes in envenomated children. A retrospective cohort study of children with grade III or IV scorpion envenomation evaluated in Phoenix Children's Hospital ED between September 1, 2011, and March 31, 2014. Patients were grouped based on treatment: group 1, supportive care only; group 2, FDA-recommended dosing (3-vial initial dose); group 3, "off label" dosing (1-2 vial initial dose). Primary outcomes were ED length of stay and hospital admission. Secondary outcomes were mechanical ventilation and aspiration pneumonia. One hundred fifty-six patients were included with 58 patients in group 1, 16 patients in group 2, and 82 patients in group 3. Group 1 was significantly older than the antivenom groups (p<0.001), and group 2 was younger than group 3 (p=0.024). Envenomation grade was also different, with group 1 having fewer grade IV then groups 2 and 3 (p<0.001). Three percent of group 1, 56% of group 2, and 28% of group 3 had respiratory distress (p<0.001). ED LOS was not significantly different between groups. Hospital admission occurred in 3.4% group 1, no group 2, and 8.5% group 3 patients. Two intubations and two aspirations occurred in group 3. In this study, clinical presentation appeared to influence treatment. Groups that received antivenom had a higher envenomation grade than the group that received supportive care. The FDA-recommended dosing group was younger and had more respiratory distress than those treated with initial doses of 1-2 vials. Outcomes were not significantly different between groups. Prospective studies may identify the ideal population for each treatment approach.

Ketogenic diet treatment for pediatric super-refractory status epilepticus

PurposeWe aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm. MethodA retrospective review was performed at Phoenix Children’s Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015. ResultsTen children with super-refractory status epilepticus, ages 2–16 years, were identified. 4/10 patients had immune mediated encephalitis, including Rasmussen encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, and post-infectious mycoplasma encephalitis. Other etiologies included Lennox Gastaut Syndrome, non-ketotic hyperglycinemia, PCDH19 and GABRG2 genetic epilepsy, New Onset Refractory Status Epilepticus, and Febrile Infection-Related Epilepsy Syndrome. 4/10 patients’ EEG features suggested focal with status epilepticus, and 6/10 suggested generalized with status epilepticus. Median hospital length was 61days and median ICU length was 27days. The median number of antiepileptic medications prior to diet initiation was 3.0 drugs, and the median after ketogenic diet treatment was 3.5 drugs. Median duration of status epilepticus prior to KD was 18days. 9/10 patients had resolution of super-refractory status epilepticus in a median of 7days after diet initiation. 8/9 patients were weaned off anesthesia within 15days of diet initiation, and within 1day of achieving ketonuria. 1/10 patients experienced side effects on the diet requiring supplementation. ConclusionMost patients achieved resolution of status epilepticus on KD therapy, suggesting it could be an effective therapy that can be utilized early in the treatment of children with super refractory status epilepticus.

Abstracts from The 34(th) Annual National Neurotrauma Symposium June 26-29, 2016 Lexington, Kentucky.

Abstracts from The 34(th) Annual National Neurotrauma Symposium June 26-29, 2016 Lexington, Kentucky.

Symptoms of cobalamin activation disorders in relation to ethnicity and Native American tribal affiliation: An exploratory study

The Cobalamin activation disorders (CblA‐CblG) are deleterious inborn errors of Vitamin B12 activation, resulting in severe intellectual disability, vision impairment, and seizures. Although cobalamin activation disorders are exceedingly rare, typically occurring 1 in every 200,000 births, Phoenix Children's Hospital has an unusually high number of patients (n=21). We conducted a retrospective, exploratory examination of medical records to identify symptom trends in individuals clinically diagnosed with CblC (n=16) in relation to race, ethnicity, and tribal affiliation. This study is unique due to our direct access to a large population of children with this condition, the majority of whom are Native American (n=6) or Hispanic (n=8), while the remaining 2 are of Caucasian descent. We also examined the symptoms of two Native American children believed to have a novel mutation related to cobalamin metabolism. Of the 16 individuals clinically diagnosed with CblC, those with the most severe eye abnormalities tended to be of Athabascan descent. In addition, growth delays were present in 5 children, with no apparent connection to race or ethnicity. Finally, two children believed to exhibit a novel mutation related to cobalamin activation, who are both of the same Native American tribal (non‐Athabascan) descent, display no ophthalmological symptoms. Findings from this study will expand the clinical understanding of this disease and will shed light on the impact of these conditions in minority populations. In addition, these results lay the foundation for future research examining associations between symptoms, symptom severity, and genetic analysis data. The ultimate goal of this research is to contribute to the Cobalamin activation section of a gene chip that addresses genetic disorders in Native Americans.Support or Funding InformationPhoenix Children's Hospital

Impact of Conditioning Regimen in Allogeneic Stem Cell Transplant for Acute Myeloid Leukemia: The Phoenix Children's Hospital Experience

Impact of Conditioning Regimen in Allogeneic Stem Cell Transplant for Acute Myeloid Leukemia: The Phoenix Children's Hospital Experience

Outcomes of thoracoscopic thymectomy in patients with juvenile myasthenia gravis

IntroductionMyasthenia gravis (MG) is an autoimmune disorder of the postsynaptic neuromuscular junction resulting in fatigability of voluntary muscles. There has been increasing evidence supporting thymectomy for MG in adults, and evidence for the role of surgery in pediatric age groups is increasing. The purpose of this study is to describe the outcomes of our patients with juvenile MG undergoing thoracoscopic thymectomy. Material and methodsAll patients with juvenile MG who underwent thoracoscopic thymectomy at Phoenix Children's Hospital between 1999 and 2014 were included. Patients were diagnosed by their treating neurologist. An Osserman and Genkins criterion was used to classify the severity of the disease and DeFilippi classification was used to assess remission. ResultsTwelve patients underwent thoracoscopic thymectomy for juvenile MG during the time frame studied. Nine (75%) patients had an Osserman stage of IIB, with only two patients with ocular disease. There were no conversions to an open procedure. Seven (59%) patients had normal thymic histology, 4 (33%) had evidence of follicular hyperplasia and one (8%) had involutional changes. The median length of hospital stay was 2days (range 1–5days). There was no 30-day postoperative morbidity, reoperations or mortality. The median length of follow-up was 31months (range, 4–91months) and at the time of their last follow-up; all 12 (100%) patients had a DeFilippi Classification of 3 or better. ConclusionSurgery for MG in children is indicated for antibody-receptor-positive patients with moderate to severe disease. Thoracoscopic thymectomy is a safe and acceptable treatment for juvenile MG with good disease control. The low morbidity and shorter hospital duration make it an excellent option for consideration.

The Animal-Assisted Therapy Program at Phoenix Children’s Hospital: A Journey Towards Evidence

ABSTRACTAnimals have positive effects on humans. The effect of human-animal interaction has been rigorously researched, showing a wide variety of benefits. The animal-assisted therapy (AAT) program at Phoenix Children’s Hospital has recorded over 55,000 therapy dog visits as of 2014. Until recently, most AAT research has been qualitative. The AAT Program Coordinator desired to measure the effects of the program quantitatively. Literature searches probed for validated studies to replicate, and a viable clinical question was identified with librarian assistance. This institutional review board–approved protocol measures and studies physiological variables and stress biomarkers in children before and after an AAT visit.

Review of Prevention for Pediatric and Adolescent Migraine.

Authors from the Barrow Neurological Institute at Phoenix Children's Hospital present a narrative overview of preventive treatment for pediatric and adolescent migraine.

Effecting Change in an Evidence-Based Medicine Curriculum: Librarians’ Role in a Pediatric Residency Program

Librarians for the joint Phoenix Children's Hospital/Maricopa Medical Center Pediatric Residency Program were asked to assist on the Evidence-Based Medicine (EBM) Subcommittee for the program. Faculty was open to recommendations for revising and improving the curriculum and desired librarian assistance in completing the task. The annual program review and conference evaluations revealed a gap between the objectives of the EBM curriculum and the residents’ perceived abilities to integrate knowledge into meaningful literature searches. This column demonstrates how librarians can collaborate with their residency programs to revise and improve processes to effect change in their program's EBM curriculum.

Results from First Year Experience of Dedicated Pediatric DBS Dystonia Program at Barrow Neurological Institute at Phoenix Children’s Hospital

To review and quantify changes in a cohort of pediatric patients with primary or secondary dystonia following bilateral Deep Brain Stimulation (DBS) to the Globus Pallidus Internus (GPi).

LDL Discordance in Pediatric Survivors of Acute Lymphoblastic Leukemia

Background: Survivors of childhood acute lymphoblastic leukemia (ALL) have a four-fold excess risk of mortality secondary to cardiovascular events compared to the general population. While it is well-known that LDL has a role in atherogenesis and subsequent cardiac events, recent studies consistently show that LDL particle number (LDLp) is a stronger, independent predictor of coronary heart disease in adults compared to LDL-cholesterol (LDLc), especially when the values are discordant. There is emerging evidence that LDL discordance also occurs in children. We studied this phenomenon in pediatric ALL survivors and identify associated risk factors.Methods: Complete data were available from 64 patients enrolled in the Phoenix Children’s Hospital pediatric cancer survivorship registry. Patients were 12.9±3.5 years of age (range: 7.7-22.7 years) with a mean time off therapy of 5.5±2.6 years (range 2.3-11.4 years). LDLc and LDLp were assessed by NMR spectroscopy. Patients were assessed by a registered dietician for calcium, fat, and fruit intake as well as physical activity. Ideal LDLc and LDLp were defined as <160 mg/dL and <1600 nmol/L, respectively, based on American Heart Association guidelines for people with standard cardiac risk. Discordance was classified based on ideal vs. not-ideal status for each parameter.Results: Mean BMI was 22.7±5.7 kg/m2 (range 14-38 kg/m2); mean BMI percentile was 73.1±26.1% (0.9-99.5%) with 42% of patients considered overweight or obese at the time of evaluation. Mean LDLc and LDLp were 79±25.5 mg/dL (29-164 nmol/L) and 1124±517 nmol/L (411-2837 nmol/L), respectively, with 89% (n=56) of survivors exhibiting ideal LDLc and 47% (n=30) of survivors exhibiting ideal LDLp. In a subgroup of patients with ideal LDLc, 40.6% (n=26) exhibited not-ideal LDLp and were classified as discordant. Regression analysis showed that after adjusting for age and gender, BMI (p<0.00001) and LDLc (p<0.00001) were significant independent predictors of LDLp while lifestyle factors and time off therapy were not significant predictors of LDLp. When patients were grouped as concordant (ideal LDLc and LDLp, n=30) or discordant (ideal LDLc and elevated LDLp, n=25), BMI was 17.6% higher in the discordant group (25.0±5.4 kg/m2 vs. 20.6±5.0 kg/m2, p=0.003).Conclusion: In this population of pediatric ALL survivors, LDLc measurements may not provide a complete assessment of cardiovascular disease risk. Given that LDLp is an early predictor of cardiovascular outcomes, conventional lipid testing may underestimate the true cardiovascular disease risk in ALL survivors. Survivors may benefit from early, expanded screening and targeted intervention. Further studies should focus on the broader pathophysiology of cardiovascular disease in pediatric ALL survivors. DisclosuresNo relevant conflicts of interest to declare.

PierianDx Partner Network Welcomes the U of Arizona and Phoenix Children's

PierianDx Partner Network Welcomes the U of Arizona and Phoenix Children's

Is Change in Regional (Somatic) Near-Infrared Spectroscopy Not a Useful Indictor of Clinically Detectable Low Cardiac Output in Children After Surgery for Congenital Heart Defects?

Department of Critical Care and Cardiology, Phoenix Children’s Hospital, Phoenix, AZ. The author has not disclosed any potential conflicts of interest.

Establishing Measurement Invariance

Registered nurses and nurse researchers often use questionnaires to measure patient outcomes. When questionnaires or other multiple-item instruments have been developed using a relatively homogeneous sample, the suitability of even a psychometrically well-developed instrument for the new population comes into question. Bias or lack of equivalence can be introduced into instruments through differences in perceptions of the meaning of the measured items, constructs, or both in the two groups. To explain measurement invariance and illustrate how it can be tested using the English and Spanish versions of the Paediatric Asthma Quality of Life Questionnaire (PAQLQ). A sample of 607 children from the Phoenix Children's Hospital Breathmobile was selected for this analysis. The children were of ages 6-18 years; 61.2% completed the PAQLQ in Spanish. Testing measurement invariance using multiple-group confirmatory factor analysis, a series of hierarchical nested models, is demonstrated. In assessing the adequacy of the fit of each model at each stage, both χ2 tests and goodness-of-fit indexes were used. The test of measurement invariance for the one-factor model showed that the English and Spanish versions of the scale met the criteria for measurement invariance. The level of strict invariance (equal factor loadings, intercepts, and residual variances between groups) was achieved. Confirmatory factor analysis is used to evaluate the structural integrity of a measurement instrument; multiple confirmatory factor analyses are used to assess measurement invariance across different groups and to stamp the data as valid or invalid. The PAQLQ, a widely used instrument having evidence to support reliability and validity was used separately in English- and Spanish-speaking groups. Traditional methods for evaluating measurement instruments have been less than thorough, and this article demonstrates a well-developed approach, allowing for confident comparisons between populations.

Infantile hemangiomas treated with propranolol: Description of the Phoenix Children's Hospital experience

Infantile hemangiomas treated with propranolol: Description of the Phoenix Children's Hospital experience

It is what it was*

Phoenix Children's Hospital; University of Arizona School of Medicine; Phoeniz, AZ The author has not disclosed any potential conflicts of interest.