improving care for children with sickle cell disease/acute chest...

DOI: 10.1542/peds.2010-3099; originally published online January 17, 2011; 2011;127;e480Pediatrics

Gladstone Airewele and Marilyn J. HockenberryTanya J. Hilliard, Julie P. Katkin, Kathy McCarthy, Mireya Paulina Velasquez,

Elizabeth A. Crabtree, M. Michele Mariscalco, Joy Hesselgrave, Suzanne F. Iniguez,Improving Care for Children With Sickle Cell Disease/Acute Chest Syndrome

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of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 2011 by the American Academy published, and trademarked by the American Academy of Pediatrics, 141 Northwest Pointpublication, it has been published continuously since 1948. PEDIATRICS is owned, PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly

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Improving Care for Children With Sickle CellDisease/Acute Chest Syndrome

abstractBACKGROUND: Acute chest syndrome (ACS) is a leading cause of hos-pitalization and death of children with sickle cell disease (SCD). Anevidence-based ACS/SCD guideline was established to standardizecare throughout the institution in February 2008. However, by the sum-mer of 2009 use of the guideline was inconsistent, and did not seem tohave an impact on length of stay. As a result, an implementation pro-gram was developed.

OBJECTIVE: This quality-improvement project evaluated the influenceof the development and implementation of a clinical practice guidelinefor children with SCD with ACS or at risk for ACS on clinical outcomes.

METHODS: Clinical outcomes of 139 patients with SCD were evaluatedbefore and after the development of the implementation program. Out-comes included average length of stay, number of exchange transfu-sions, average cost per SCD admission, and documentation of the clin-ical respiratory score and pulmonary interventions.

RESULTS: Average length of stay decreased from 5.8 days before im-plementation of the guideline to 4.1 days after implementation (P �.033). No patients required an exchange transfusion. Average cost perSCD admission decreased from $30 359 before guideline implementa-tion to $22 368. Documentation of the clinical respiratory score in-creased from 31.0% before implementation to 75.5%, which is an im-provement of 44.5% (P� .001). Documentation of incentive spirometryand positive expiratory pressure increased from 23.3% before imple-mentation to 50.4%, which is an improvement of 27.1% (P� .001).

CONCLUSIONS: Implementation of a guideline for children with SCDwith ACS or at risk for ACS improved outcomes for patients with SCD.Pediatrics 2011;127:e480–e488

AUTHORS: Elizabeth A. Crabtree, MPH,a M. MicheleMariscalco, MD,b,c,d Joy Hesselgrave, MSN, RN,e

Suzanne F. Iniguez, BS,f Tanya J. Hilliard, MSN, BSN, MHA,e

Julie P. Katkin, MD,d,g,h Kathy McCarthy, BSN,i MireyaPaulina Velasquez, MD,d Gladstone Airewele, MD, MPH,e

and Marilyn J. Hockenberry, PhD, PNP, RNd,j,k

aEvidence Based Outcomes Center, bCritical Care Medicine,eCancer Center, Departments of fRespiratory Care andhPulmonary Medicine, iCenter for Clinical Research, andjDepartment of Nursing, Texas Children’s Hospital, Houston,Texas; and Divisions of cCritical Care Medicine and gPulmonaryMedicine, dDepartment of Pediatrics, and kDepartment ofHematology and Oncology, Baylor College of Medicine, Houston,Texas

KEY WORDSquality improvement, implementation, guideline development,acute chest syndrome, sickle cell disease

ABBREVIATIONSACS—acute chest syndromeSCD—sickle cell diseaseIS—incentive spirometryPEP—positive expiratory pressureCRS—clinical respiratory scorePDSA—plan-do-study-act

www.pediatrics.org/cgi/doi/10.1542/peds.2010-3099

doi:10.1542/peds.2010-3099

Accepted for publication Nov 29, 2010

Address correspondence to Elizabeth A. Crabtree, MPH, TexasChildren’s Hospital; 1102 Bates Ave, Feigin Center, Suite 1490,Houston, TX 77030. E-mail: [email protected]

PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).

Copyright © 2011 by the American Academy of Pediatrics

FINANCIAL DISCLOSURE: Dr Katkin serves on research advisorycommittees for 2 studies conducted by MedImmune, neither ofwhich has any relationship to sickle cell disease or qualityimprovement; the other authors have indicated they have nofinancial relationships relevant to this article to disclose.

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Acute chest syndrome (ACS) usuallypresents in patients with sickle celldisease (SCD) as a rapidly occurringpulmonary disease recognized bylower respiratory tract symptoms, hy-poxemia, and a new infiltrate shown ona chest radiograph.1 ACS causes a highnumber of hospitalizations and deathsof children with SCD, and by some es-timates, it accounts for as many as25% of SCD-related deaths.1–4

Almost half of the patients diagnosedwith ACS develop the respiratory com-plication during hospitalization for an-other diagnosis such as pain crisis.Factors such as young age, low base-line hemoglobin level, and low fetal he-moglobin level place patients at ahigher risk of developing ACS.3–7 Opioidanalgesics, postoperative atelectasis,and asthma exacerbations or respira-tory viral infections may promote thedevelopment of an ACS episode.1–9 Al-though the etiology is undetermined inmany cases, pulmonary infarction, fatembolism, and infection are now rec-ognized as important causes of ACS.Chlamydia pneumoniae, Mycoplasmapneumoniae, Streptococcus pneu-moniae, Staphylococcus aureus, andparvovirus are pathogens commonlyisolated from patients with ACS.2

Management of ACS is primarily sup-portive care and includes respiratorytherapy (eg, incentive spirometry [IS],positive expiratory pressure [PEP],and bronchodilators), antibiotics, andred-cell transfusions (J. Myers, unpub-lished PowerPoint presentation, 2006,and refs 10–22). There have been fewpublished studies proving the effec-tiveness of thesemeasures in theman-agement of ACS. In their study, Bellet etal13 documented the efficacy of ade-quate analgesia in combination with ISin preventing ACS in patients admittedfor vaso-occlusive crisis. Pain controlprevented splinting and resulted in im-proved aeration and prevention of at-electasis. Other treatments either are

empiric or their effectiveness hasbeen inferred from studies that in-volved other complications of SCD (ie,transfusions and exchange transfu-sions after stroke).1–7 At our own insti-tution, we found that more than half ofall children who received red-cell ex-change transfusion for ACS did notshow evidence of ACS at presentation,and the median time to exchangetransfusion was 1 day.23 In addition, wecould detect no consistent criteria forperforming an exchange transfusion.

In this article we discuss the develop-ment and, more importantly, the im-plementation of an evidence-basedguideline for children and adolescentswith SCD with ACS or at risk for ACS.The guideline was based on interven-tions to potentially decrease the devel-opment of ACS in those at risk, height-ened vigilance for ACS detection, andaggressive treatment to prevent sig-nificant morbidity and mortality. Weembarked on an implementation pro-gram, because data in the pilotstudy indicated that average length ofstay had not been affected and pulmo-nary interventions and clinical respi-ratory score (CRS) assessments werenot being administered according tothe guideline. Outcome measures de-signed to evaluate success of theguideline included length of stay, num-ber of exchange transfusions, averagecost per SCD admission, and adher-ence to respiratory interventions.These outcomes indirectly measuredmorbidity and the efficiency of ourpractice. Implementation of this guide-line demonstrated that even when evi-dence is limited, standardization ofcare can produce significant improve-ment in clinical outcomes.

METHODS

Guideline Development

Physicians from hematology, emer-gency medicine, critical care, and pul-monary medicine along with nursing

and respiratory care served as con-tent experts to establish the clinicalpractice guideline. Guideline develop-ment followed the standards estab-lished by the hospital’s evidence-basedoutcomes center, which included re-view preparation, exploring existing in-ternal and external guidelines, search-ing for relevant evidence from the past15 years, critically analyzing researchstudies, and summarizing the evidenceby preparing the guideline, order sets,and an interdisciplinary plan of care.Three systematic reviews from the Co-chrane Database, 5 randomized clini-cal trials, 29 nonrandomized studies,and 16 review articles were used in thedevelopment of the guideline. Contentexperts met monthly over the courseof 1 year to explore the evidence andestablish critical components of theguideline.

Critical Components of theGuideline

Clinical history, physical examination,oxygen saturation level, and chest ra-diography were used to diagnoseACS.1–5,7–9 Herewe used a broad clinicaldefinition of ACS (ie, lower respiratorysymptoms including hypoxemia or newlung infiltrate) rather than the nar-rower definition used for epidemio-logic or clinical studies. Essential com-ponents of the history and examinationare listed in Table 1. To achieve stan-dardization of respiratory assess-

TABLE 1 History and Physical Examination:ACS

History: assess forLower respiratory symptoms (cough, wheezing,tachypnea, chest pain)FeverPrevious history of ACS episodePhysical examination:Assess respiratory rate, use of accessorymuscles, colorAuscultate lungsObtain pulse oximetry; detect new-onsethypoxemiaRate severity of ACS according to CRS (seeTable 2)

QUALITY-IMPROVEMENT REPORTS

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ment, we integrated a CRS to measurethe severity of respiratory distress.The CRS ranged from 0 to 12 and incor-porated evaluation of the patient’s re-spiratory rate, ausculation of the lungfields, use of accessory muscles, men-tal status, room-air oxygen saturation,and skin/mucosal color (Table 2). Reli-ability of the CRS was first establishedwith 314 children, aged 1 to 18 years,who presented to the emergency cen-ter with symptoms that suggestedasthma.24 Before implementing thenew guideline, the CRS was examinedin 73 children who presented to theemergency center with a diagnosis ofSCD. The mean age of this group was12 years. The Respiratory Distress As-sessment Instrument (RDAI) was usedto obtain a score of respiratory sever-ity for comparison with the CRS eachtime it was assessed. The RDAI is awell-published scale that is similar inassessment categories to the CRS andmeasures adventitious lung sounds onexpiration and inspiration and retrac-tions observed in the supraclavicular,intercostal, and subcostal spaces. Inthe 73 children with SCD, there was asignificant (P � .004) association be-tween the CRS and RDAI and the finaldisposition of the patients (being hos-pitalized or sent home). The receiveroperating characteristic (ROC) curvewas computed to evaluate accuracy ofthe CRS. The CRS at time of dischargefrom the emergency center was moresensitive in predicting discharge (ROC

curve: 0.63) compared with the RDAI(ROC curve: 0.51). Test-retest reliabilityof the CRS instrument was determinedby having a second health care pro-vider rate a subset of patients withSCD independently within 15 minutesof each CRS measurement and thencomputing the correlation analysis.CRS correlations among 2 respiratorytherapists who rated children at thetime of emergency-center admission(P � .001), before treatment (P �.001), and after treatment (P � .001)were significant, which supports thescale as an effective clinical tool thatcan be used consistently by healthcare providers.

Respiratory therapists and physiciansused the CRS to evaluate children withSCD who presented to the emergencycenter with respiratory illnesses andthose at risk for developing ACS (fever,pain, vaso-occlusive disease). The CRSwas used to guide treatment for all pa-tients with SCD (Table 2). All patientsadmitted on the ACS/SCD guideline re-ceived IS alternating with PEP (anairway-clearance device) therapy ev-ery 2 hours from 8 AM until 10 PM eachday. Therapy continued beyond 10 PM ifthe patient was awake (Table 3). Pa-tients also received supplemental oxy-gen, if needed, to maintain oxygen sat-urations of�94% (Table 3). The IS andPEP were coordinated between nurs-ing and respiratory care. Every 4 hoursthe patient was assessed by using the

CRS, and the score was documentedon the flow sheet (Table 3). Respiratoryinterventions and the trigger for theirimplementation are outlined in theclinical algorithm (Fig 1) and arebased on patient CRS.

In addition to CRS assessment and ad-ministration of respiratory interven-tions, the clinician’s role includedprompt interventions based on theclinical algorithm. Ensuring that painwas adequately managed, monitoringintake and output to prevent fluid over-load, administering antibiotics as indi-cated, monitoring laboratory results,alerting the physician team if the he-moglobin transfusion threshold hadbeen reached, and ambulating pa-tients at least twice daily were impor-tant care strategies (Table 3). Nursesand respiratory therapists were re-sponsible for communicating with themedical team when CRSs escalatedand assisting with patient transfers toa higher level of care as needed. Chil-dren with wheezing or rales/cracklespresent received specific attention, in-cluding steroid administration (Fig 1).Discharge criteria also were estab-lished to avoid unnecessary variationsin length of stay (Table 4). All patientswith recurrent or severe ACS were re-ferred to a SCD pulmonary clinic.

In addition to the guideline, physi-cian order sets were developed foruse in the emergency center and out-patient hematology clinic and on the

TABLE 2 Clinical Respiratory Score

Assess CRS

0 1 2

Respiratory rate (�12 mo) �30 30–40 �40Auscultation Good air movement, scattered wheezing

(only expiratory), loose cracklesDepressed air movement, inspiratory andexpiratory wheezes

Diminished or absent breath sounds,severe wheezing, or markedprolonged expiration

Use of accessory muscles Mild to no use of accessory muscles; mildto no retractions or nasal flaring oninspiration

Moderate intercostals retractions, mild-to-moderate use of accessory muscles,nasal flaring

Severe intercostals and substernalretractions, nasal flaring

Mental status Normal to mildly irritable Irritable, agitated, restless LethargicRoom-air oxygen saturation, % �95 90–95 �90Color Normal Pale to normal Cyanotic, dusky



acute care floors. The essential com-ponents of the diagnoses and man-agement of children at risk for ACSand those with ACS are outlined inTable 3. Note that those children atrisk for ACS were monitored fordevelopment of ACS and received inter-ventions to potentially decrease itsdevelopment.

Guideline Implementation

The specific aim of implementationwas to improve utilization and compli-

ance with the ACS/SCD guideline. Animplementation team, comprisingmembers of the initial content expertguideline development team, wasformed. The team first met to assessbarriers to implementation and estab-lish outcome measures. Assessing po-tential barriers allowed the team todevelop more targeted, effectiveinterventions. Barriers identified in-cluded (1) lack of physician awarenessof the clinical guideline, (2) nonen-gagement among the specialties and

disciplines, (3) lack of availability ofthe paper physician order set, and(4) uncertainty regarding what ser-vice was responsible for administer-ing respiratory interventions. Mea-sures identified for gauging thesuccess of implementation included(1) average length of stay, (2) num-ber of exchange transfusions, (3) av-erage cost per SCD admission, and(4) documentation of the CRS andpulmonary interventions. The imple-mentation team used the Institute

TABLE 3 Essential Management Components for Children With SCD at Risk for ACS and Those With ACS

Recommend Consider

Monitoring Vital signs (blood pressure, heart rate, respiratory rate) every 4 h —Pulse oximetry every 12 h or continuous (encouragingambulation)Weigh dailyStrict charting of fluid intake/outputPain-severity rating, minimum every 4 hMonitor CRS at least every 4 h (see Table 1)

Diagnostics Complete blood count, differential, platelet count, and reticulocytecount (initially and daily until improved; compare with patient’sbaseline values)

If history of recent fever, consider blood culture

Chest radiograph if cough, chest pain, hypoxemia, or anyrespiratory symptoms are present or develop after admission

If severe illness or hemoglobin level�1 g/dLbelow baseline hemoglobin level, stronglyconsider type and cross-matchIf severe illness, consider obtaining serum ureanitrogen, creatinine, alanineaminotransferase, aspartateaminotransferase, and bilirubin levelsIf severe abdominal pain, consider abdominalultrasound

Fluids, nutrition, general care Total fluid intake (intravenous plus oral) at 1� maintenance If patient is dehydrated or insensible losses areincreased as with persistent fever,administer more fluids

Encourage ambulation, activity If patient is�5 y old: consider introducing theuse of IS and PEP depending on cooperation

If patient is�5 y old, use IS as follows: supervised IS, 10 breaths Consider making the patient blow soap bubblesevery 2 h between 8 AM and 10 PM, and alternate the use of PEP; If signs of fluid overload: intravenousIS should be documented alternately by respiratory therapist furosemide 0.5 mg/kg�1and nursing services

Oxygen Maintain pulse oxygenation at�94% —Notify care providers of increased fraction of inspired oxygenrequirement or increase in CRS

Pain management Acetaminophen: 15 mg/kg orally every 4 h or as needed fortemperature at�38°C; maximal daily dose: 75 mg/kg per d

—

Morphine: 0.05–0.15 mg/kg intravenously every 2 h or 0.05–0.10mg/kg per h continuous infusion or patient-controlledanalgesiaIbuprofen (if no contraindications): 10 mg/kg orally every 6–8 h;limit frequent dosing to 72-h duration

Antibiotics Discontinue prophylactic penicillin while on wide-spectrumantibiotics

If febrile, cefotaxime: 50 mg/kg per dose every8 h (maximum: 1–2 g per dose)

Add azithromycin: 10 mg/kg orally on day 1 (maximum: 500 mg If patient is allergic, substitute clindamycinper dose), then 5 mg/kg orally once daily (maximum: 250 mgper dose) for 4 d (erythromycin may be substituted)

If severe illness, Consider addition of (maximumdose: 1 g) every 8 hintravenous vancomycin at 10–15 mg/kg(maximum dose: 1 g) every 8 h





for Healthcare Improvement’s plan-do-study-act (PDSA) model to docu-ment a test of change with the ACS/SCD guideline.25,26 PDSA is a systematicapproach to problem-solving and a4-step process for continuous qualityimprovement. Interventions and datafrom 2 PDSA cycles (phases 1 and 2)are summarized below. Pilot datawerecollected before the onset of the first

PDSA cycle. Pilot data provided theimplementation team with baselinemeasures to compare with future as-sessments after the guideline was im-plemented. As a result of the pilot data,specific barriers to implementationwere identified, and an education ini-tiative was launched to address theseissues.

Education

Phase 1/PDSA 1

The ACS/SCD guideline was designedfor use by multiple specialtiesthroughout the hospital. All special-ties routinely caring for patientswith SCD were identified from the pi-lot data, and education sessionswere developed for each specialty.Individualized in-services were heldfor staff from the emergency center,inpatient hematology-oncology unit,general medicine, float pool staff,progressive care unit, outpatient he-

matology clinic, and respiratorycare. Physicians, fellows, and resi-dents who work in these specialty ar-eas were educated on the ACS/SCDguideline through noon conferences,education modules, lectures, educa-tional lunches, and grand rounds.Presentations provided a brief de-scription of ACS and its complica-tions, reasons for implementing theguideline, and bullet points withinformation pertinent to the physi-cian, nurse, and respiratory thera-pist roles in caring for patients withSCD. Case scenarios were presentedto allow staff to apply the guidelineto real-life situations. A frequently-asked-questions hand-out was givento use as a quick reference guide (Fig2). In addition, a video describing pul-monary pathophysiology, and the ra-tionale for the guideline’s pulmonaryinterventions, was developed andplaced on the hospital’s intranet site toaugment live in-services.

FIGURE 1Clinical algorithm for ACS respiratory supportmanagement. PO indicates bymouth; IV, intravenous; SPO2, oxygen saturation; IPV, intrapulmonary percussiveventilation; BID, twice daily; TID, three times daily; GI, gastrointestinal; Hb, hemoglobin; BiPAP, bilevel positive airway pressure.

TABLE 4 ACS/SCD Discharge Criteria

Improved pulmonary symptoms anddocumentation of adequate oxygenation onroom air

Afebrile for�24 h and negative cultures for�24–48 h, if applicable

Stable hemoglobin/hematocrit levelsTaking adequate oral fluids and able to take oralmedications, if applicable

Adequate pain relief, if needed, with oralanalgesics

Slow wean from steroids (over 7–10 d)Parent understandsDischarge careWhen hematology/oncology and pulmonarySCD clinic follow-ups are scheduled

Home care agencies notified as needed



Phase 2/ PDSA 2

Initial evaluation of guideline usage af-ter implementation of the educationinitiative revealed a deficit in docu-mentation of both the CRS and pulmo-nary interventions. The second PDSAcycle focused on improving documen-tation of these 2 components. Smallgroup meetings with nursing and re-spiratory care representatives fromthe implementation team were held.Data from the first PDSA cycle wereshared with nursing and respiratorycare educators and managers, whothen disseminated the data to their col-leagues, highlighting opportunities forquality improvement. The guideline-implementation coordinator also pre-sented the data and solicited feedbackfrom nurses at shift report on all inpa-tient units. In addition, CRS badge cardswith the CRS scale and bulleted pointsessential to the ACS/SCD guideline weregiven to nurses on the 3 units that re-

ceived the highest volumeof children ad-mitted with SCD.

Methods of Evaluation

Pilot Study

A retrospective chart review was per-formed for all guideline-eligible pa-tients (all patients from 1 to 21 yearsof age admitted with SCD) from Feb-ruary through March of 2009. Dataon length of stay, number of ex-change transfusions, and documen-tation of pulmonary interventionsand CRS assessments for the first 3days of admission were collected foreach SCD admission.

Phase 1/PDSA 1

A data-collection form was developedto capture outcome measures estab-lished by the implementation team forthe 2 subsequent PDSA cycles. A retro-spective chart review was performedfor all guideline-eligible patients from

October through November of 2009.Data on length of stay and number ofexchange transfusions over the entireinpatient stay for each patient withSCD were also collected. Data on theaverage cost per patient admission forSCD were obtained from a financial an-alyst who searched a database by us-ing All-Patient Refined Diagnosis-Related Groups codes.

Data on documentation of CRS and pul-monary interventions for the first 3 in-patient days for every patient with SCDwere collected. As indicated in theguideline, the CRS was to be assessedevery 4 hours and supervised IS/PEPtherapy administered every 2 hoursbetween the hours of 8 AM and 10 PM.These responsibilities were shared be-tween nursing and respiratory care.The number of opportunities for nurs-es/respiratory therapists to documentthe CRS and pulmonary interventionsadministered was compared with thenumber of times there was actual doc-umentation of the CRS and pulmonaryinterventions performed.

Phase 2/PDSA 2

Identical methods of evaluation wereused for the second PDSA cycle, be-cause the same outcome measureswere monitored. Data on patient ad-missions for SCD from June throughAugust of 2010 were collected for thesecond PDSA cycle.

In addition to the data collected in the 2PDSA cycles, data on average length ofstay for patients with SCD were evalu-ated in fiscal years 2007, 2008, and2009 and the first 6 months of fiscalyear 2010. These data were obtainedfrom a financial analyst who searcheda database by using All-Patient RefinedDiagnosis-Related Groups codes.

We did not obtain institutional reviewboard approval for this retrospectiveanalysis, because it was a quality-improvement exercise, not a researchstudy.

FIGURE 2Frequently-asked-questions (FAQ) hand-out provided to inpatient nurses caring for patients with SCDwith ACS or at risk for ACS.





Analysis

Minitab statistical software (MinitabInc, State College, PA) was used to an-alyze the data. A 2-sample t test wasrun to evaluate the difference in lengthof stay between the pilot data and eachof the 2 PDSA cycles. A 2-proportionstest was run to assess if there was adifference in documentation of the CRSand pulmonary interventions betweenthe initial pilot data and the data col-lected after the interventions in eachof the 2 PDSA cycles.

RESULTS

The guideline was developed and in-stituted in February 2008 after infor-mal education interventions withnursing, respiratory care, and physi-cian groups. The average length of staydid not appreciably change from pre–guideline development in 2007 to up to22 months after institution of theguideline. The formal implementationteam was initiated in the summer of2009.

Three measurement periods wereused to evaluate the formal implemen-tation of the ACS/SCD guideline. In thepilot study conducted from January toFebruary of 2009, 1 year after theguideline was completed and insti-tuted, 59 patients older than 1 yearwith SCD and at risk for ACS or withACS were admitted. Fifty-five chartswere available for review; 1 patientwith a prolonged length of stay of 38days was removed from analysis, be-cause the extended hospitalizationwas unrelated to ACS. Sixty-two pa-tients older than 1 year were admittedfrom October to November of 2009; 61charts were available for review forthe first PDSA cycle evaluation, be-cause 1 patient had a prolonged lengthof stay of 25 days and was not includedbecause the extended hospitalizationwas unrelated to ACS. Twenty-four pa-tients older than 1 year were admittedfrom June through August of 2010, and

all were reviewed and included in theanalysis of the second PDSA cycle.

The discharge criteria facilitateddecision-making at the time of dis-charge and decreased length of stayby standardizing the approach to dis-charge. Average length of stay was re-duced from 5.8 days during the pilotstudy before implementation to 4.1days after the second PDSA cycle,which is a reduction of 1.7 days (29%)(95% confidence interval for differ-ence: 0.1–3.2) (Table 5). No patients inthe pilot study or the 2 PDSA-cycleevaluations received an exchangetransfusion. Average cost per SCDadmission decreased from $30 359in fiscal year 2008 before implemen-tation of the guideline to $22 368 inthe first 6 months of 2010, which is areduction of $7991 (26.3%). On aver-age, this hospital admits 357 pa-tients per year for SCD. The reducedaverage cost per admission wouldresult in an annual savings of morethan $2 850 000.

Documentation of the CRS increasedfrom 31% before implementation ofthe guideline to 75.5% after the secondPDSA cycle, which is an improvementof 44.5% (95% confidence interval fordifference: 38.7%–50.2%). Documenta-tion of IS/PEP increased from 23% be-fore implementation of the guideline to�50% after the second PDSA cycle,which is an improvement of 27% (95%confidence interval for difference:21.3%–33.0%) (Table 5). Patients witha higher CRS in the emergency center

were more likely to receive a simpleblood transfusion while hospitalized(P� .001). CRS in the emergency cen-ter was not related to the patient’slength of stay or transfer to the criticalcare unit.

Average length of stay for patients withSCD decreased from 5.4 days in 2007,2008, and 2009 to 4.1 days in the first 6months of fiscal year 2010.

DISCUSSION

The mainstay of successful treatmentfor children with SCD with ACS or atrisk for ACS is high-quality supportivecare. Implementation of a standard-ized approach to care for children atrisk for ACS and those with ACS re-vealed significant changes in clinicaloutcomes. Standardizing fluid man-agement, maintaining oxygenation, ad-ministering respiratory interventions,and ensuring pain control were essen-tial elements of quality care for thesepatients. We also instituted an aggres-sive respiratory monitoring and ther-apy program. In addition, using theCRS afforded us a common languagefor describing the condition of thesechildren to each other and drivingtreatment and triage. The use of theCRS as a standardizedmethod for eval-uating clinical respiratory statusplayed an important role in minimizingvariation in assessment as well asmanagement for these children.

The decision whether to institute an ex-change transfusion, and determinantsof the need for acute respiratory moni-

TABLE 5 Outcome Measures at Baseline, Phases I and II

Outcome Measures Baseline, Jan–Feb2009 (N� 54)

Phase I, Oct–Nov2009 (N� 61)

Phase II, June–Aug2010 (N� 24)

Average length of stay, d 5.8 4.3 4.1Exchange transfusions, n 0 0 0Hospitalization cost, US $ 30 359 — 22 368Opportunities to document CRS, n 831 866 310CRS documented, n (%) 258 (31.0) 491 (56.7) 234 (75.5)Improvement in CRS documentation, % (P) — 25.7 (�.001) 18.8 (� .001)Opportunities to document IS, n 974 1197 353IS documented, n (%) 227 (23.3) 418 (34.9) 178 (50.4)Improvement in IS documentation, % (P) — 11.6 (�.001) 15.5 (�.001)



toring, were inconsistent and undefinedbefore the guideline’s development. Onthe basis of results from our earlierstudy,23 it is remarkable that no ex-change transfusions were needed forchildren with ACS during the 3 monitor-ing periods. Thus, we believe a standard-ized approach to exchange transfusionresulted indecreaseduseof this therapyduring the initial roll-out of the protocoleven before the guideline was formallyimplemented. One explanation is thefairly rapid adoption of the guideline byour critical care service.

It is important to recognize the criticalrole of implementation in improving pa-tient outcomes. The guideline was for-mally implemented in the summer of2009, more than 1 year after the guide-line’s development and dissemination. Itwas only after implementation that pa-tient outcomes improved. Data showedthe step-wise improvement in outcomesfrom the pilot data collected before im-plementation through each of the 2 im-plementation phases (Table 5).

The change in average cost per SCDadmission is impressive. This de-crease ismost likely attributable to thedecrease in average length of stay androom charges for SCD admissions af-ter implementation of the guideline.

One of the resources required for im-plementation included amaster’s leveltrained implementation coordinator.This person coordinated implementa-tion programs for several evidence-based guidelines and devoted approx-imately one-third of her time to thisproject. In addition, the implementa-tion teamwas vital to the project’s suc-cess. The team comprised �15 mem-bers representing various medicalspecialties, nursing, respiratory care,and other allied health professions.Team members attended severalmeetings to assess barriers and de-fine outcome measures, participatedin the education initiative, and servedas champions for the project by engag-

ing their colleagues in discussionsabout the guideline.

Although our institution serves one ofthe largest populations of people withSCD in the country and has a highlycomprehensive SCD center, we feelthat one of the exciting things aboutthis project is its ability to be repli-cated. The success of the programrested heavily on education and creat-ing awareness. None of the interven-tions implemented were terribly so-phisticated or required a great deal ofresources. The guideline and CRS pro-vided the framework for standardizingcare. A smaller, more resource-constrained institution should also seeimprovements in clinical outcomes forpatients if they have engaged provid-ers who are willing to implement sucha framework. It may be easier, in fact,for smaller institutions to implementsuch a program, because their sizewould enable them to develop moretargeted interventions.

Although we saw improvements in out-comes for patients with SCD, we iden-tified a number of barriers to and op-portunities for quality improvementafter the second and final PDSA cycle.Documentation of the CRS and IS waspoorer on those units that received alower volume of admissions for SCD. Inaddition, nurses documented less thanrespiratory care providers on all units,which may have been, in part, becauseof the chart design. Nurses now arerequired to document in 2 differentplaces. As our institution goes live withcomputerized physician order entryand electronic chart documentationthis year, one of our next PDSA cyclesmay include examining ways to im-prove documentation by modifying thenursing flow chart in our institution’selectronic medical record. These is-sues highlight the fact that quality im-provement is an ongoing process.

There are limitations to this quality-improvement project. The number of

charts reviewed in the second phase ofimplementation was significantly lessthan the number reviewed in the pilotstudy and the first phase of implementa-tion. There were fewer SCD admissionsduring this period. We could not detectthespecificmechanismsbywhich imple-mentation improved outcomes; thus, wedo not knowwhich portions of the guide-line may be critical to its success.

It is unlikely that the outcomes of thisquality-improvement project were in-fluenced by other systematic changesduring the implementation period. Theinstitution’s practices concerning dis-charge did not change after the guide-line was developed. In addition, therewas no difference in severity of ACSpresentations over the course of theguideline’s development and imple-mentation. We do not have data on theuse of hydroxyurea during this period.It is possible that an increase in theuse of this drug in the population ofpatients with SCD had an impact onclinical outcomes, but we feel that thereduction in average length of stay canbe attributed to both improvementsmade in the administration and docu-mentation of the CRS and pulmonaryinterventions and the avoidance of ex-change transfusions.

CONCLUSIONS

Standardization of care improves clin-ical outcomes for patients with SCDwho are at risk for ACS or have ACS. Itis even more important to note thatstandardization of care did not resultfrom guideline development alone butalso from implementation efforts. Weadvocate for, and encourage otherproviders of patients with SCD toadopt, an evidence-based model ofcare and to devote the resources nec-essary for implementation. The resultsof this project indicate that both com-ponents are vital for improving theclinical outcomes of patients with SCD.





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DOI: 10.1542/peds.2010-3099; originally published online January 17, 2011; 2011;127;e480Pediatrics

Gladstone Airewele and Marilyn J. HockenberryTanya J. Hilliard, Julie P. Katkin, Kathy McCarthy, Mireya Paulina Velasquez,

Elizabeth A. Crabtree, M. Michele Mariscalco, Joy Hesselgrave, Suzanne F. Iniguez,Improving Care for Children With Sickle Cell Disease/Acute Chest Syndrome

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