BackgroundSickle Cell Disease (SCD), which is characterized by a mutation in the gene
encoding beta hemoglobin, causes bodily dysfunctions such as impaired
pulmonary function and reduced functional capacity. ObjectiveTo assess changes in pulmonary function and functional capacity in patients
with SCD and to identify the relationships between these variables.MethodWe evaluated sociodemographic, anthropometric, lung function (spirometry),
respiratory (manovacuometer), peripheral muscle strength (Handgrip strength
- HS) and functional capacity (i.e., the six-minute walk test) parameters in
21 individuals with SCD (average age of 29±6 years). Shapiro-Wilk,
paired Student's, Wilcoxon, Pearson and Spearman correlation tests were used
for statistical analyses, and the significance threshold was set at
p<0.05. ResultsA total of 47.6% of the study subjects exhibited an altered ventilation
pattern, 42.8% had a restrictive ventilatory pattern (RVP) and 4.8%
exhibited a mixed ventilatory pattern (MVP). The observed maximal
inspiratory pressure (MIP) values were below the predicted values for women
(64 cmH2O), and the maximum expiratory pressure (MEP) values, HS
values and distance walked during the 6MWT were below the predicted values
for both men (103 cmH2O, 39 Kgf and 447 m, respectively) and
women (64 cmH2O; 27 Kgf; 405 m, respectively). Positive
correlations were observed between maximum voluntary ventilation (MVV) and
MEP (r=0.4; p=0.046); MVV and BMI (r=0.6; p=0.003); and between HS and MIP
(r=0.7; p=0.001), MEP (r=0.6; p=0.002), MVV (r=0.5; p=0.015), distance
walked in the 6MWT (r=0.4; p=0.038) and BMI (r=0.6; p=0.004). ConclusionsSCD promoted changes in lung function and functional capacity, including RVPs
and a reduction in the distance walked in the 6MWT when compared to the
predictions. In addition, significant correlations between the variables
were observed.