Multi‐modal intervention for the inpatient management of sickle cell pain significantly decreases the rate of acute chest syndrome

Abstract

INTRODUCTIONSickle cell-related pain is the most common morbidity experienced by children and adults with sickle cell disease (SCD). Acute chest syndrome (ACS), though second to SCD-pain in frequency, contributes significantly to both morbidity and mortality in children with SCD. ACS is responsible for up to one-quarter of sickle cell-related mortality [1], and almost half of the deaths due to ACS are in children <20 years of age [2]. Furthermore, ACS during childhood negatively impacts long-term lung function [3], and predisposes children to subsequent episodes [4,5]. A report from the Cooperative Study of Sickle Cell Disease found that three-quarters of patients who died from ACS initially presented with lower extremity pain due to sickle cell vaso-occlusion, and that death from ACS primarily occurred within 48 hr of symptom onset. For patients who did not die, 50% of adults and 11% of children were presented with SCD-pain prior to the onset of ACS [2]. Consequently, a close temporal relationship exists between ACS and SCD-pain.The pathophysiology of ACS is not fully understood, yet multiple risk factors have been identified including splenectomy [6], abdominal surgery [7], asthma [4], infection [8], fat emboli and pain [9]. Therapies to prevent the development of ACS are limited and they are risk factor dependent. For example, pre-operative blood transfusion reduces the rate of ACS in the surgical setting [10] as does the use of laparascopic rather than open abdominal procedures [11]. The use of controller medications in patients with asthma [12] and hydroxyurea in patients with frequent episodes of ACS reduces ongoing risk, but these therapies are not used to treat an acute episode [13]. Additionally, the use of incentive spirometry reduces the development of pulmonary complications in children admitted with pain [14]. Lastly, in a retrospective study that examined the frequency of ACS development, patients who received nalbuphine, a mixed opioid agonist-antagonist, compared to morphine had a lower rate of ACS [15].In this study, we evaluated the impact of a multi-modal intervention combining standardized orders and education for the inpatient management of SCD-pain on the rate of subsequent ACS episodes. The use of incentive spirometry substantially decreases the rate of ACS among individuals hospitalized for pain [15], though we observed that many patients are not compelled to consistently use incentive spirometry while hospitalized. We, therefore, tested the hypothesis that a standard treatment for pain episodes including specific orders to nursing staff regarding monitoring incentive spirometry would decrease the rate of ACS in patients admitted with SCD. METHODSThe protocol was approved by the Washington University School of Medicine Human Research Protection Office. A prospective cohort study was performed to assess the effect of a multi-modal intervention aimed at reducing 30-day hospital readmission for children admitted with SCD-pain. Secondary outcome measures obtained in the cohort study included ...

The intersection between asthma and acute chest syndrome in children with sickle-cell anaemia

“…Another strategy to prevent acute chest syndrome includes standardising hospital medical care and educating physicians and nursing staff to ensure patients receive optimum doses of opioids to relieve pain (but not cause sedation leading to atelectasis) and adequate intravenous and oral fluids. 19 When an inpatient multimodal strategy was implemented to manage vaso-occlusive pain, rates of acute chest syndrome decreased from 25% to 12% (p=0·003). 19 …”

“…19 When an inpatient multimodal strategy was implemented to manage vaso-occlusive pain, rates of acute chest syndrome decreased from 25% to 12% (p=0·003). 19 …”

Association of Hospital and Provider Types on Sickle Cell Disease Outcomes

“…These guidelines typically call for fluid resuscitation, pain control, antibiotics, bronchodilators, transfusion, and aggressive respiratory support. 5,6 Historically, SCD-associated ACS was considered a disease of early childhood. However, comprehensive care for infants and children with SCD has doubled survival to adulthood and increased the numbers of adolescents and young adults (A/YA) with SCDrelated complications such as ACS.…”

Pulmonary Complications of Sickle Cell Disease

“…In the Cooperative Study of Sickle Cell Disease, a 29% incidence of ACS was reported in 3,751 subjects over a 2-year period, representing 12.8 episodes per 100 patient-years for HbSS disease (16). Before the recognition of PH, ACS was considered the leading cause of premature death, accounting for 25% of sickle cell-related mortality in early registry trials (8,(15)(16)(17). The associated mortality has decreased in the modern era of hydroxyurea therapy and early, more aggressive transfusion therapy (18).…”

“…Therapies reported to prevent ACS in at-risk patients include preoperative blood transfusion (42), maintenance asthma therapy for children with asthma (43), maintenance hydroxyurea therapy (14,44), and the use of incentive spirometry during acute VOC hospitalizations (14,17,45).…”