Exhaled Carbon Monoxide as a Marker of Hemolysis in Sickle Cell Disease- an Exploratory Study

Sickle Cell Disease

carbon mono oxide, sickle cell, hemolysis

The investigators propose to evaluate etCO in patients with HbSS, HbSC, and HbS-beta thalassemia during routine clinic visits, and longitudinally. Our goal is to know whether etCO differs amongst subjects with different sickle cell syndrome genotypes, and whether it is a stable marker of hemolytic rate, as reflected in routine labs obtained for clinical care (including total hemoglobin, reticulocyte count, lactate dehydrogenase, and, when sampled, total and direct bilirubin). We hope to establish whether this inexpensive and non-invasive test faithfully reflects hemolytic parameters in sickle cell syndromes.

This is an observational study of sickle cell patients with the aim to measure exhaled carbon monoxide levels and to correlate with genotype and standard clinical markers of hemolysis. Upon enrollment and at each subsequent visit, if acceptable to the patient, each subject will undergo measurement of exhaled carbon monoxide utilizing a hand held carbon monoxide monitor, MicroCO/Smoke check. Subsequent evaluations may be obtained during a routine visit, while hospitalized for a vasocclusive crises (VOC) episode, or when evaluated in the acute care clinic. The principal study objective is to assess if exhaled carbon monoxide levels are a non-invasive marker of hemolysis in subjects with sickle cell disease (SCD). The secondary study objective is to assess exhaled carbon monoxide levels serially in subjects with SCD, relative to vasocclusive crises, transfusion requirements, and pain syndromes. The one inclusion criteria is for all adult sickle patients presenting for follow up at the outpatient sickle cell clinic who are capable of following simple instructions. The exclusion criteria are a recent (2 week) history of lung infection, asthma, acute chest syndrome, or COPD exacerbation and/or a significant pulmonary dysfunction in the recent (3-6 month) past.

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