Amy Sobota

Corticosteroids for acute chest syndrome in children with sickle cell disease: Variation in use and association with length of stay and readmission†

Acute chest syndrome (ACS) causes significant morbidity and mortality in sickle cell disease. The role of corticosteroids is unclear. The objectives of our study were to examine the variation between hospitals in their use of corticosteroids for ACS, describe characteristics associated with corticosteroids, and investigate the association between corticosteroids, length of stay, and readmission. We performed a retrospective examination of 5,247 hospitalizations for ACS between January 1, 2004, and June 30, 2008, at 32 hospitals in the Pediatric Health Information System database. We used multivariate regression to examine the variability in the use of corticosteroids adjusting for hospital case mix, identify factors associated with corticosteroid use, and evaluate the association of corticosteroids with length of stay and 3‐day readmission rates controlling for propensity score. Corticosteroid use varied greatly by hospital (10–86% among all patients, 18–92% in patients with asthma). Treatment with corticosteroids was associated with comorbid asthma (OR 3.9, 95% CI: 3.2–4.8), inhaled steroids (OR 1.4, 95% CI: 1.1–1.7), bronchodilators (OR 3.2, 95% CI: 2.5–4.2), nitric oxide (OR 2.4, 95% CI: 1.2–5.0), oxygen (OR 2.3, 95% CI: 1.8–2.9), ICU (OR 1.7, 95% CI: 1.3–2.3), ventilation (OR 2.0, 95% CI: 1.4–2.8), APR‐DRG severity level (OR 1.4, 95% CI: 1.2–1.6), and discharge year (OR 0.86, 95% CI: 0.80–0.92). Corticosteroids were associated with an increased length of stay (25%, 95% CI: 14–38%) and a higher 3‐day readmission rate (OR 2.3, 95% CI: 1.6–3.4), adjusted for confounding. Hospitals vary greatly in the use of corticosteroids for ACS, even in patients with asthma. Clear evidence of the efficacy and toxicity of corticosteroid treatment in ACS may reduce variation in care. Am. J. Hematol. 2010.

Thirty‐day readmission rates following hospitalization for pediatric sickle cell crisis at freestanding children's hospitals: Risk factors and hospital variation

Readmission within 30 days after hospitalization for sickle cell crisis was developed by The National Association of Childrens Hospitals (NACHRI) to improve hospital quality, however, there have been few studies validating this.

Transition from pediatric to adult care for sickle cell disease: Results of a survey of pediatric providers†

As recently as the 1970s, children born with sickle cell disease (SCD) were unlikely to survive into adulthood. With advances in medical care, most patients now survive childhood and live into their forties or beyond [1,2]. A better transition from pediatric- to adult-focused care is therefore increasingly important [3]. Despite recent awareness of the importance of transition by the SCD community, little is known about existing transition programs. We conducted a survey of pediatric sickle cell clinics to describe current transition practices and identify areas for improvement. Survey topics included program demographics, transition logistics, assessment of patient transition readiness and independence, transition preparation, and program evaluation. Twenty-three clinics (77%) report having a transition program, although half have been in place for under 2 years. There is wide variation in specific transition practices. Most centers (97%) have an identified accepting adult provider, however, only 60% routinely transfer their patients to an adult hematologist specializing in SCD. Although there has been a recent effort to establish transition programs in pediatric sickle cell clinics, specific practices vary widely. As anticipated, lack of an accepting adult hematologist with an interest in SCD emerged as a common barrier to transition.

Improving the Management of Vaso-Occlusive Episodes in the Pediatric Emergency Department.

OBJECTIVES: Vaso-occlusive episodes (VOEs) account for the majority of emergency department (ED) visits for children with sickle cell disease (SCD). We hypothesized that addressing key barriers to VOE care would improve receipt of analgesics and outcomes. METHODS: A quality improvement (QI) initiative was conducted from September 2010 to April 2014 to streamline VOE care in an urban pediatric ED. Four interventions were used: a standardized time-specific VOE protocol; intranasal fentanyl as the first parenteral pain medication; an SCD pain medication calculator; and provider and patient/family education. Data were collected for 3 outcome measures (mean time from triage to first parenteral opioid and admission/discharge decision, and proportion discharged from the ED); 1 process measure (mean time from triage to initiation of patient-controlled analgesia); and 4 balancing measures (mean time from triage to second intravenous opioid dose, 24-hour ED readmission, respiratory depression, and length of stay). RESULTS: There were 289 ED visits in the study period. Improvements were seen in mean time to: first dose of parenteral opioid (56 to 23 minutes); second opiate intravenous dose (106 to 83 minutes); admission and discharge decisions (163 to 109 minutes and 271 to 178 minutes, respectively); and initiation of patient-controlled analgesia (216 to 141 minutes). The proportion discharged from the ED increased from 32% to 48% (χ2 = 6.5402, P = .01). No increase in 24-hour readmission, respiratory depression, or inpatient length of stay was observed. CONCLUSIONS: Using VOE-specific interventions, we significantly improved VOE care for children. Studies are needed to determine if these results can be replicated.

Self-Reported Transition Readiness among Young Adults with Sickle Cell Disease

Background: A growing body of literature addresses the need for transition programs for young adults with sickle cell disease (SCD); however, studies assessing transition readiness are limited and there are few validated instruments to use. Objective: To conduct a pilot study to assess transition readiness of patients with SCD in our transition program and to evaluate a SCD-specific assessment tool that measures 5 knowledge skill sets (medical, educational/vocational, health benefits, social, and independent living), and 3 psychological assessments (feelings, stress, and self-efficacy). Results: Of the 47 SCD patients between the ages of 18 and 22 seen in our facility, 33 completed the assessment tool. The majority of patients reported good medical knowledge of SCD and said they were motivated to pursue a career despite the burden of living with the disease. We identified knowledge gaps in the area of independent living and health benefits skills sets. A majority of patients reported being worried that their SCD would prevent them from doing things in their life; however, few respondents said they were worried or anxious about their transition to adult care. Conclusions: Adolescents beginning a transition intervention program reported a high level of knowledge of their disease and demonstrated a positive attitude toward transition with good self-efficacy.

Young Adult Perspectives on a Successful Transition from Pediatric to Adult Care in Sickle Cell Disease.

Objective This qualitative study sought to learn from young adults with sickle cell disease (SCD) about their experience leaving pediatric care and perspective on what makes a successful transition. Methods Fifteen young adults with SCD who had left pediatric care within the previous five years participated in focus groups led by a trained moderator. Transcripts were analyzed using grounded theory. Results Four main themes emerged from the analysis: facilitators of transition (meeting the adult provider prior to transfer, knowing what to expect, gradually taking over disease self-management and starting the process early), barriers to transition (negative perceived attitude of adult staff, lack of SCD specific knowledge by both patients and staff, and competing priorities interfering with transition preparation), what young adults wished for in a transition program (opportunities to meet more staff prior to transfer, more information about the differences between pediatric and adult care, learning from a peer who has been through the process, more SCD teaching, and flexibility in transition preparation) and how they define a successful transition (gradually assuming responsibility for self-management of their SCD). Conclusion Our findings present unique opportunities to learn from young adults with SCD about ways to improve current transition programs.

How we prevent and manage infection in sickle cell disease

Sickle cell disease (SCD) affects approximately 100 000 people in the US, 12 500 in the UK, and millions worldwide. SCD is typified by painful vaso‐occlusive episodes, haemolytic anaemia and organ damage. A secondary complication is infection, which can be bacterial, fungal or viral. Universal newborn screening, routine use of penicillin prophylaxis, availability of conjugated vaccines against S. pneumoniae and comprehensive care programmes instituted during the past few decades in industrialized countries have dramatically reduced childhood mortality and improved life expectancy. Yet patients with SCD remain at increased risk of infection. Unfortunately, the treatment of most bacterial infections that are common in SCD is not based on the results of randomized controlled clinical trials. In their absence, treatment decisions are based on consensus guidelines, clinical experience or adapting treatment applied in other diseases. This leads to wide variation in treatment among institutions and even between treating physicians in a single institution. Prevention of infection, when possible, is most important and we focus on prevention through targeted prophylaxis and vaccination. We will share our management strategies for managing the more common infections in SCD and provide the rationale for our recommendations.

Improvement in influenza vaccination rates in a pediatric sickle cell disease clinic.

Children with sickle cell disease (SCD) are at increased risk of complications from influenza. However, despite widespread recommendations that these patients receive an annual influenza immunization, reported vaccination rates remain very low at under 50%.

APHON/ASPHO Policy Statement for the Transition of Patients With Sickle Cell Disease From Pediatric to Adult Health Care.

Summary: With advances in medical care, the majority of children with sickle cell disease are surviving to adulthood. Patients, families, and providers now face the need for this growing population to move from pediatric- to adult-focused care. In order to facilitate a successful transfer to adult health care, and prepare young adults for greater independence, it is recommended that all pediatric patients with sickle cell disease receive transition preparation. Association Position: As the professional voice of pediatric hematology/oncology healthcare practice, the Association of Pediatric Hematology/Oncology Nurses (APHON) and the American Society of Pediatric Hematology Oncology (ASPHO) recommends that the discussion of transition begin early and is presented as part of the natural process of becoming an adult; that patients, providers, and families are all involved in creating a transition plan and assessing transition preparedness annually; and that transfer of care involve direct communication between the pediatric team and the accepting adult provider.

Development of quality indicators for transition from pediatric to adult care in sickle cell disease: A modified Delphi survey of adult providers

Transition from pediatric to adult care is a vulnerable time for young adults with sickle cell disease (SCD); however, improvements in transition are limited by a lack of quality indicators. The purpose of this study was to establish quality indicators for transition in SCD and to determine the optimal timing between the final pediatric visit and the first adult provider visit.