Primary ciliary dyskinesia is a rare disease that affects the tiny, hair-like structures that line the airways. Cilia move together in wavelike motions. That motion carries mucus toward the mouth or nose to be coughed or sneezed out of the body.
Kovesi and Shapiro are co-senior authors of a paper published in the journal Pediatric Pulmonology that reports on seven Inuit patients with PCD identified through genetic testing. Diagnosing PCD is important, Kovesi said, because treatments — similar to therapies to treat cystic fibrosis — are available. Treatment includes daily airway clearance and the use of antibiotics to prevent infections, among other tactics.
“We see so much lung disease in the Inuit and it is so severe, it is disproportionate,” said Shapiro.Article content Shapiro noted that out of 40 children being followed at Montreal Children’s Hospital for severe chronic lung disease, the majority do not have PCD.