Mortality, Asthma, Smoking and Acute Chest Syndrome in Young Adults with Sickle Cell Disease

Knight‐Madden, Jennifer; Barton-Gooden, Antoinette; Weaver, Steve; Reid, Marvin; Greenough, Anne

doi:10.1007/s00408-012-9435-3

Cited by 46 publications

(51 citation statements)

References 26 publications

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“…The aforementioned studies focused on subjective report of exposure in children's home environments and may not have sufficiently captured other places in which children are frequently exposed to tobacco smoke (such as regular childcare arrangements, the homes of close friends and relatives, or split-parent households) and likely underestimate the effect of tobacco smoke exposure. Further, smoking (also by self-report) has been associated with fivefold increased risk of death in young adults with SCD [24].…”

Section: Discussionmentioning

confidence: 99%

Secondhand smoke is associated with more frequent hospitalizations in children with sickle cell disease

et al. 2016

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Tobacco smoke exposure has been associated with more frequent hospitalizations in children with sickle cell disease (SCD), but previous studies have not quantified the exposure by objective methods. We enrolled 50 children and young adults with SCD in a retrospective and prospective cohort study and quantified tobacco smoke exposure by objective (salivary cotinine) and survey measures. We used a multivariable negative binomial regression model to evaluate the association between salivary cotinine and hospital admissions. Forty-five percent (22/49) of participants had significant elevation of salivary cotinine ( 0.5 ng/ ml). The incidence risk ratio (IRR) for hospital admission for those with elevated cotinine was 3.7 (95% CI 1.8-8). Those exposed to secondhand smoke but not primary smokers (cotinine between 0.5 and 10 ng/ml) had a similarly increased risk of hospitalization [IRR 4.3 (95% CI 1.8-10)]. We show that an objective measure of tobacco smoke exposure, salivary cotinine, is strongly associated with the rate of hospital admissions in children and young adults with SCD. This association underscores the importance of screening for tobacco smoke exposure in people with SCD. Further investigation is warranted to determine the mechanisms of and to evaluate interventions to decrease tobacco smoke exposure.

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Section: Discussionmentioning

confidence: 99%

Secondhand smoke is associated with more frequent hospitalizations in children with sickle cell disease

et al. 2016

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“…The strong association between lung disease and SCA-related mortality has been well-established [3][4][5]13,26,27 ; however, what is not known is whether pulmonary function results are associated with earlier death. For the first time, we have demonstrated that FEV 1 % predicted can be used to predict earlier death in individuals with SCA.…”

Section: Discussionmentioning

confidence: 99%

“…In the largest prospective cohort study investigating the natural history of sickle cell disease (SCD) in children and adults, the Cooperative Study of Sickle Cell Disease (CSSCD), Platt et al 1 noted an increased risk of earlier death in patients with SCA was associated with acute chest syndrome (ACS), renal failure, seizures, an elevated baseline white cell count, and a low level of fetal hemoglobin (Hb). Since that report, end-stage renal disease requiring dialysis, 2 the presence of asthma [3][4][5] and/or wheezing, 6 smoking, 5 N-terminal (NT)-pro brain natriuretic peptide levels, 7 right heart catheter-confirmed pulmonary hypertension along with the physiological severity of the precapillary pulmonary hypertension, 8,9 and severity of hemolysis [10][11][12] have all been associated with earlier death among individuals with SCA.…”

Section: Introductionmentioning

confidence: 99%

“…14 Using this new reference and based on the strong association between forced expiratory volume in 1 second percent (FEV 1 %) predicted measurements and risk of earlier death in the general [15][16][17] and cystic fibrosis populations, 18,19 coupled with the observation that lung disease is associated with mortality in SCA, [3][4][5]13 we tested the hypothesis that FEV 1 % predicted would be associated with earlier death in adults with SCA after adjustment for known predictors of death in SCA. 9 …”

Section: Introductionmentioning

confidence: 99%

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Low forced expiratory volume is associated with earlier death in sickle cell anemia

Kassim

Payne

Rodeghier³

et al. 2015

Blood

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• Among adults with SCA, decreased FEV 1 percent predicted was associated with earlier death.• Obstructive and restrictive pulmonary function patterns do not predict earlier death in adults with SCA.Pulmonary complications result in mortality in adults with sickle cell anemia (SCA). We tested the hypothesis that abnormal pulmonary function was associated with earlier death. A prospective cohort of adults with SCA, followed in the Cooperative Study for Sickle Cell Disease, was constructed using the first pulmonary function test at >21 years of age. Spirometry measures: forced expiratory volume in 1 second (FEV 1 ), forced vital capacity, and total lung capacity were categorized based on age, gender, height, and race. Pulmonary function patterns were categorized based on the American Thoracic Society guidelines using both spirometry and lung volumes. A cohort of 430 adults with SCA, mean age 32.6 6 9.5 (range, 21.0-67.8) years at time of first pulmonary function test, and a median follow-up of 5.5 years, was evaluated. A total of 63 deaths occurred. At baseline, 47% had normal, 29% restrictive, 8% obstructive, 2% mixed, and 14% nonspecific lung function patterns. In the final multivariable model, lower FEV 1 percent predicted was associated with increased hazard ratio of death (HR per % predicted 1.02; 95% confidence interval [CI] 1.00-1.04; P 5 .037), as was older age (HR 1.07; 95% CI 1.04-1.10; P < .001), male sex (HR 2.09; 95% CI 1.20-3.65; P 5 .010), higher lactate dehydrogenase levels (HR per mg/dL 1.002; 95% CI 1.00-1.003; P 5 .015), and higher acute chest syndrome incidence rate (HR per event/year 10.4; 95% CI 3.11-34.8; P < .001). Presence of obstructive (HR 1.18; 95% CI: 0.44-3.20; P 5 .740) and restrictive (HR 1.31; 95% CI: 0.64-2.32; P 5 .557) pulmonary function patterns were not associated with earlier death. Understanding the pathophysiology of a low FEV 1 percent predicted in individuals with SCA is warranted, enabling early intervention for those at risk. (Blood. 2015;126(13):1544-1550

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“…In individuals with SCD, asthma is associated with increased frequency of pain episodes and ACS [96], as well as significantly higher mortality [94,97]. This was most clearly demonstrated in data from the CSSCD cohort where 1,963 individuals with SCA who were classifiable for asthma were followed for a total of 18,495 patientyears.…”

Section: Pulmonary Complicationsmentioning

confidence: 99%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

2015

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Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimistic that the next 40 years will be focused on meeting current challenges in SCD by addressing chronic complications of SCD to reduce mortality and improve quality of life in a growing population of adults with SCD in high-income countries, while simultaneously decreasing the disparity of medical care between high and low-income countries.

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Mortality, Asthma, Smoking and Acute Chest Syndrome in Young Adults with Sickle Cell Disease

Abstract: Our results indicate that young adults with SCD should be discouraged from smoking and their asthma aggressively treated.

Cited by 46 publications

References 26 publications

Secondhand smoke is associated with more frequent hospitalizations in children with sickle cell disease

Secondhand smoke is associated with more frequent hospitalizations in children with sickle cell disease

Low forced expiratory volume is associated with earlier death in sickle cell anemia

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

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