Question
A 15-year old girl with cystic fibrosis is rushed to the hospital for an acute respiratory infection and suspected pneumonia. The admission chest x-ray showed
A 15-year old girl with cystic fibrosis is rushed to the hospital for an acute respiratory infection and suspected pneumonia. The admission chest x-ray showed extensive bronchiectasis and persistent complete atelectasis in the lower left lobe. Despite aggressive therapy including IV antibiotics, the patient’s pulmonary function remained poor and level of consciousness was diminished with no verbal responses but eyes open to voice. Sputum samples were collected via airway suctioning, arterial blood gasses were draw and pH was determined to be 7.32 (normal pH: 7.4). As a radical therapy for this patient an infusion of fetal hemoglobin is suggested to potentially increase oxygen perfusion. Fetal hemoglobin (HbF) has different oxygen binding kinetics when compared to the oxygen binding displayed by adult hemoglobin (illustrated below). This difference is in part due to the inability of HbF to interact with 2,3 bisphosphoglycerate (2,3 BPG).
Based on this information would the use of HbF as a potential therapy in this patient be successful?
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