Abstract Plastic bronchitis (PB) is a rare complication seen most commonly in children with congenital cardiac defects, asthma, or following respiratory infections. This condition has been reported in a few case reports and case series in children with sickle cell disease (SCD). While almost all of the cases reported have been in children with HbSS phenotype, there was one case series that included a patient with the HbSC variant. Studies show that clinical presentation of SCD is milder in those with HbSC variants, with significantly fewer episodes of acute chest syndrome than in those with the homozygous variant, HbSS. While the actual pathophysiology of PB in SCD is unclear, it is likely secondary to an interplay of multiple factors causing airway inflammation and impaired muco-ciliary clearance. In addition, impaired lymphatic drainage has been hypothesized to contribute to the pathogenesis of plastic bronchitis secondary to pulmonary hypertension attributable to pulmonary microvascular occlusion, hypoxic vasoconstriction or fat embolism during vaso-occlusive crisis. We aim to show the importance of identifying PB as a complication in children with HbSC variants where early initiation of aggressive airway clearance can minimize invasive procedures like bronchoscopy and potentially long-term morbidity. We report on a 10-year-old boy with HbSC SCD and recurrent episodes of plastic bronchitis with a restrictive pattern of lung disease on pulmonary function testing. He had his first episode of acute chest syndrome at 16 months of age with collapse of the right upper and middle lobe requiring a therapeutic bronchoscopy. Spirometry showed a restrictive pattern at 5 years of age. He was readmitted with another episode of acute chest when he was 10 years old with a collapse of the right middle and lower lobes, clinically worsening despite starting early antibiotics and aggressive airway clearance with chest physiotherapy, albuterol, hypertonic saline, and pulmozyme. Addition of mucomyst, incentive spirometry, cough assist along with high frequency chest wall oscillation and intrapulmonary percussive ventilation for 48 hours were able to clear mucus plugs, resulting in significant improvement on imaging. Lung function testing at follow-up showed a persistent restrictive lung pattern. Identifying these children can inform monitoring of their pulmonary function, ultimately helping to identify those at higher risk of organ damage and lung function decline. These patients could potentially benefit from treatment with drugs like hydroxyurea for which we have existing extensive data on to support its use and efficacy in the HbSS disease process. This abstract is funded by: None
Dayalan et al. (Fri,) studied this question.