Pulmonary function studies in Kuwaiti children with sickle cell disease and elevated Hb F.

Area of Science:

• Pediatric Pulmonology
• Hematology
• Genetics

Background:

• Conflicting reports exist regarding ventilatory defects in pediatric sickle cell disease (SCD).
• Kuwaiti SCD patients exhibit milder disease, possibly due to the Arab-Indian haplotype and elevated fetal hemoglobin (Hb F).
• No prior studies have investigated pulmonary function in SCD patients with this specific haplotype.

Purpose of the Study:

• To assess pulmonary function in children with sickle cell disease (SCD) in Kuwait.
• To investigate potential differences in pulmonary function based on SCD genotype and clinical complications.
• To compare pulmonary function in SCD patients with healthy controls and those with HbH disease.

Main Methods:

• Pulmonary function tests (PFTs) were conducted on 28 children with SCD (21 SS, 7 Sβ0-thalassemia) in steady state.
• Two control groups were included: 17 age- and sex-matched healthy children and 10 children with HbH disease.
• Patient charts were reviewed for acute chest syndrome and vaso-occlusive crisis frequency.

Main Results:

• Significantly lower forced vital capacity (FVC) and vital capacity (VC) were observed in SS patients compared to healthy controls.
• SS patients showed significantly lower FEV1 and VC compared to children with HbH disease.
• No significant differences in PFT parameters were found between SCD patients with or without acute chest syndrome, or based on crisis frequency.

Conclusions:

• Steady-state children with SCD exhibit early restrictive and obstructive pulmonary function patterns.
• Pulmonary function changes in SCD are not solely attributable to anemia, as evidenced by HbH disease patients.
• This study underscores the early onset of pulmonary involvement in sickle cell disease, even in milder forms.