sickle cell disease and single ventricle pathway palliatiion

What is it about?

Sickle cell disease child tolerates prolonged obligatory hypoxemia that was associated with surgery

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Why is it important?

This report is about a child with sickle cell disease that predisposes the child to a crisis when exposed to low oxygen levels in blood [hypoxemia]. Such a child had a cardiac condition that could be repaired in stages spread over a period of several years. During the initial stages of this surgical pathway, there was obligatory hypoxemia which the child tolerated without developing a sickle cell crisis. This was surprising and intriguing. We presume that the protective mechanism in this child that helped the child avoid developing a crisis is the high fetal hemoglobin levels that were found in the child. Elevated fetal hemoglobin levels are usually found in people originating from the same geographical area as the child. So salutary effect of elevated fetal hemoglobin helped the child tide over hypoxemia during the prolonged surgical pathway successfully

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