Asthma and sickle cell disease: two distinct diseases or part of the same process?

Abstract

A physician diagnosis of asthma in children and adults with sickle cell disease (SCD) has been associated with increased rates of pain and acute chest syndrome (ACS) episodes and premature death. Despite the clinical significance of a doctor’s diagnosis of asthma in individuals with SCD, the criteria for a physician diagnosis of asthma are not well defined. Many features of asthma are common in individuals with SCD, including symptoms of wheezing, obstructive lung disease and airway hyper-responsiveness. However, it is not clear if these signs and symptoms of asthma reflect a physician diagnosis of asthma, or if these asthma features are related to SCD. Further complicating the diagnosis of asthma in children with SCD is the significant overlap in clinical manifestations between an asthma exacerbation and an ACS episode. Evidence supporting the concept that asthma and SCD are separate co-morbid conditions includes a similar prevalence of asthma between children with SCD and those in the general population and the observation that asthma is inherited in a familial pattern in the families of children with SCD. In contrast, there is significant evidence that asthma-like features may be associated with SCD without a diagnosis of asthma, including a higher than expected prevalence of airway hyper-responsiveness and obstructive lung disease. Regardless of whether SCD and asthma are distinct or overlapping co-morbid conditions, we recommend a systematic and complete evaluation of asthma when the diagnosis is suspected or when patients have multiple episodes of pain or ACS.

Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

“…The largest increase in treatment was attributed to the group started on both hematologic and respiratory medications. Studies on SCD children additionally diagnosed with asthma have suggested a beneficial role of treatment with albuterol and ICS [29][30][31][32][33][34][35]. In our study, approximately 35.9 % of the study population had a concurrent diagnosis of asthma, but an improvement in FEV1% of predicted was seen in all groups started on medications regardless of a concurrent diagnosis.…”

Pulmonary Complications of Sickle Cell Disease

“…The prevalence of asthma among pediatric patients with SCD appears to be similar to that in children of African descent in the general population (57,60). As mentioned earlier, obstructive lung disease in adults with SCD is less common, occurring in less than 3% of patients (56).…”

Considerations for Care: Management of Asthma in the Child with Sickle Cell Disease

“…5 There is ample evidence that ''asthma'' is a commonly occurring comorbidity in children with sickle cell disease [6][7][8][9][10][11][12] with a reported prevalence varying from 2% to 45%. [6][7][8][9][10][11]13,14 However, it is not clear what respiratory characteristics in the child with sickle cell disease are appropriately attributed to underlying asthma as defined in the guidelines 15 as opposed to being caused by the pathology that contributes to sickle cell disease associated pulmonary dysfunction. 16 It is clear that those children with asthma or asthma-like symptoms are at increased risk of morbidity associated with higher rates of acute chest syndrome, vaso-occlusive events, stroke, pain, as well as death.…”

“…15 The reader is referred to the guidelines for in depth coverage of these issues (www.nhlbi.nih.gov/ guidelines/asthma/index.htm). No prospective controlled trials evaluating drug treatments for asthma symptoms in patients who have both sickle cell disease and asthma have been published, 13,16,21 and there are certain caveats to consider when prescribing these medications to children with sickle cell disease that are described in this update.…”