Asthma in Sickle Cell Disease: Implications for Treatment

Abstract

Objective. To review issues related to asthma in sickle cell disease and management strategies. Data Source. A systematic review of pertinent original research publications, reviews, and editorials was undertaken using MEDLlNE, the Cochrane Library databases, and CINAHL from 1947 to November 2010. Search terms were [asthma] and [sickle cell disease]. Additional publications considered relevant to the sickle cell disease population of patients were identified; search terms included [sickle cell disease] combined with [acetaminophen], [pain medications], [vitamin D], [beta agonists], [exhaled nitric oxide], and [corticosteroids]. Results. The reported prevalence of asthma in children with sickle cell disease varies from 2% to approximately 50%. Having asthma increases the risk for developing acute chest syndrome , death, or painful episodes compared to having sickle cell disease without asthma. Asthma and sickle cell may be linked by impaired nitric oxide regulation, excessive production of leukotrienes, insufficient levels of Vitamin D, and exposure to acetaminophen in early life. Treatment of sickle cell patients includes using commonly prescribed asthma medications; specific considerations are suggested to ensure safety in the sickle cell population. Conclusion. Prospective controlled trials of drug treatment for asthma in patients who have both sickle cell disease and asthma are urgently needed.

Pulmonary Hypertension, Tricuspid Regurgitant Velocity Screening, and the Nitric Oxide Pathway

“…28 Airway hyperreactivity (AHR) is reported in 40%-77% of SCA patients, and the clinical diagnosis of asthma in 2%-50%. 29 Recent investigation suggests that AHR and clinical asthma may not be strongly correlated in children with SCA. 30 Further studies are needed to understand whether AHR associated with elevated lactate dehydrogenase levels 30 represents a unique and independent pulmonary process in children with SCA.…”

“…Obstructive changes on PFTs and a clinical diagnosis of asthma are associated with a higher rate of hospitalization for acute chest syndrome and vasoocclusive crisis and a 2.36-fold increased risk of death compared with children without these findings. 28,29 Segregation analysis suggests that asthma, which disproportionately affects African-American children, is a comorbidity rather than a consequence of SCD. 31 Features of AHR and asthma, including elevation of inflammatory mediators (leukotrienes, interleukins, and soluble vascular adhesion molecules) and transient hypoxia, ventilation-perfusion mismatch, and reperfusion injury, may contribute to the development of vasculopathy and chronic lung injury.…”

“…31 Features of AHR and asthma, including elevation of inflammatory mediators (leukotrienes, interleukins, and soluble vascular adhesion molecules) and transient hypoxia, ventilation-perfusion mismatch, and reperfusion injury, may contribute to the development of vasculopathy and chronic lung injury. 28,29 Thromboembolic disease. Autopsy series demonstrate acute and chronic thrombi in the macro-and/or microvascular pulmonary circulation of 24%-38% of SCD patients.…”

“…27,39 Small cohorts of SCA adult patients have undergone RHC. 4,6,15,32,40 Even with a TRV Ն 3.0 m/s, RHC most often demonstrates modest elevations in mPAP (28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40) and PVR in the setting of an increased CO. In ϳ 40%, an elevated PCWP is detected, indicating left-sided cardiac dysfunction.…”

Transfusion‐related adverse events are decreased in pregnant women with sickle cell disease by a change in policy from systematic transfusion to prophylactic oxygen therapy at home: A retrospective survey by the international sickle cell disease observatory

“…Patients with baseline hypoxemia or asthma have been reported to experience more severe SCA complications and (in some studies) pain crises . Interestingly, SDB (including snoring and OSA) has been linked to adverse pregnancy outcomes (such as gestational hypertension and gestational diabetes) and potential adverse fetal outcomes (such as preterm birth and growth restriction) …”