Cough ability measurements and recurrent respiratory symptoms in individuals with Ataxia Telangiectasia

Objectives: Ataxia-Telangiectasia (A-T) individuals often present with respiratory muscle weakness, causing recurrent respiratory system infections, asthma-like symptoms, and chronic cough life-threatening events. The cough flow volume maneuver may reveal powerless airflow needed for efficient cough. The study aims to explore cough ability in relation to the flow/volume maneuver. Methods: Data collected retrospectively from clinical charts of 35 A-T patients (age 12.7?±?4.9 years) included forced expiratory and cough flow/volume maneuvers performed on the same day. Analysis compared among the maneuvers matching indices, numbers of cough-spikes, flow rate decay, and the reference data of similar ages. Adjusted to age, BMI, and number of hospitalizations prior to the tests, values were correlated with the cough indices. Results: Cough peak-flow (C-PF) was propagated within 90?±?20?ms compared with peak expiratory flow (PEF?>?200?ms). C-PF measured values were higher than expiratory peak-flow measured values (3.27?±?1.53?L/s versus 3.02?±?1.52?L/s, respectively, but C-PF (%predicted) values were significantly lower than expiratory peak-flow (%predicted) (46?±?15 versus 68?±?20 %predicted, respectively, p?p?Conclusions: The cough flow–volume curve can be applied as a method to follow cough ability in patients with A-T who showed a significantly reduced cough capacity. Further studies are needed to establish if the findings may aid decisions regarding cough assistance.