Sickle Cell Disease (SCD) primarily affects the African-American population, in whom the newborn frequency is about 1 in 600. Major clinical manifestations of SCD are caused by the abnormal sickle hemoglobin (HbS), which, when de-oxygenated, polymerizes in the red cell and causes t to become rigid and deformed (""""""""sickled""""""""). This process is thought to be responsible for one of the major clinical manifestations of SCD, recurrent vaso-occlusive painful episodes (VOPEs). Despite the knowledge that sickling can be caused in vitro by de-oxygenation of hemoglobin, the role of common lung diseases which result in hypoxemia, such as reactive airway disease (RAD), in the pathogenesis of VOPEs is unknown. This knowledge is crucial for the following reasons: a) patients with recurrent VOPEs have a shorter life span than those without, b) patients with SCD may have a higher risk of RAD than the normal population, and c) RAD is easily treated with inhaled bronchodilators and anti-inflammatory agents.
The specific aims of this study are therefore: 1) to test the hypothesis that children with SCD have a higher prevalence of RAD than a matched population of children without SCD, and to determine the risk factors for, and mechanisms whereby, children with SCD might be more prone to the development of RAD, 2) to test the hypothesis that SCD children with RAD are hypoxemic compared with children without RAD, 3) to test the hypothesis that children with RAD experience more severe and frequent sickle cell pain, and more admitted more frequently for treatment of VOPEs than children without RAD, and that this susceptibility to the development of VOPEs is related to hypoxemia, and 4) to test the hypothesis that treatment of RAD can reduce the frequency and severity of sickle cell pain, and reduce admissions of VOPEs.
These aims will be accomplished by measuring lung function. oxygen saturation and bronchial responsiveness in 160 children with SCD and 60 control patients. A longitudinal analysis will then be performed to determine the relationship between lung function (measured by home spirometry and oximetry), and the number and intensity of pain episodes (quantitated by pain diaries) before and during optimal treatment for RAD.

National Institute of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Comprehensive Center (P60)
Project #
Application #
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
Fiscal Year
Total Cost
Indirect Cost
Thomas Jefferson University
United States
Zip Code
Dampier, Carlton; Ely, Beth; Brodecki, Darcy et al. (2014) Pain characteristics and age-related pain trajectories in infants and young children with sickle cell disease. Pediatr Blood Cancer 61:291-6
Setty, B N Yamaja; Key, Nigel S; Rao, A Koneti et al. (2012) Tissue factor-positive monocytes in children with sickle cell disease: correlation with biomarkers of haemolysis. Br J Haematol 157:370-80
Barakat, Lamia P; Patterson, Chavis A; Daniel, Lauren C et al. (2008) Quality of life among adolescents with sickle cell disease: mediation of pain by internalizing symptoms and parenting stress. Health Qual Life Outcomes 6:60
Barakat, Lamia P; Patterson, Chavis A; Weinberger, Beverley Slome et al. (2007) A prospective study of the role of coping and family functioning in health outcomes for adolescents with sickle cell disease. J Pediatr Hematol Oncol 29:752-60
Ely, Beth; Alexander, Leslie B; Reed, Monica (2005) The working alliance in pediatric chronic disease management: a pilot study of instrument reliability and feasibility. J Pediatr Nurs 20:190-200
Dampier, Carlton; Setty, B N Yamaja; Eggleston, Barry et al. (2004) Vaso-occlusion in children with sickle cell disease: clinical characteristics and biologic correlates. J Pediatr Hematol Oncol 26:785-90
Dampier, Carlton; Ely, Elizabeth; Eggleston, Barry et al. (2004) Physical and cognitive-behavioral activities used in the home management of sickle pain: a daily diary study in children and adolescents. Pediatr Blood Cancer 43:674-8
Sharan, K; Surrey, S; Ballas, S et al. (2004) Association of T-786C eNOS gene polymorphism with increased susceptibility to acute chest syndrome in females with sickle cell disease. Br J Haematol 124:240-3
Stuart, Marie J; Nagel, Ronald L (2004) Sickle-cell disease. Lancet 364:1343-60
Setty, B N Yamaja; Stuart, Marie J; Dampier, Carlton et al. (2003) Hypoxaemia in sickle cell disease: biomarker modulation and relevance to pathophysiology. Lancet 362:1450-5

Showing the most recent 10 out of 18 publications