Lung function, haemoglobin and irreversibly sickled cells in sickle cell patients.

This study seeks to show whether there is a positive relationship between HbF, irreversibly sickled cells and several lung function parameters. Percentage haemoglobin F (%HbF), percent irreversibly sickled cells (%ISC), blood pressure (BP, mm Hg), heart rate (HR, b/min), forced vital capacity (FVC,I), timed vital capacity (FEV1, I) FEV1% and peak expiratory flow rate (PEFR, I/min) were measured in 10 sickle cell (in the steady state) and 15 non-sickle cell subjects matched by age and sex. Results showed that although the ages in both groups of subjects were similar, sickle cell anaemia (SCA) subjects had smaller stature. Diastolic BP was significantly lower while pulse pressure, %Hbf and %ISC were significantly higher in SCA subjects (P < 0.001 in each case). Predicted FVC, FEV1, and PEFR were significantly higher (P < 0.01, P < 0.01, P < 0.001 in each case) than observed values obtained from SCA subjects. Similarly, measured FVC, FEV1, and PEFR in SCA subjects were significantly lower (P < 0.001 respectively) than values obtained from non-sickle cell subjects. In SCA subjects, %HbF or %ISC correlated negatively with each of the ventilatory parameters and with each other. Similar results (except for PEFR) were obtained in the non-sickle cell subjects. The results suggest that %HbF and %ISC correlate negatively with lung function parameters in sickle cell sufferers in the steady state.