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

Abstract

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 < 0.002). The number of spikes/maneuver was low when compared with reference (2.0 ± 0.8 versus 6–12 spikes) and cough vital-capacity was lower than expiratory vital capacity (0.95 ± 0.43 versus 1.03 ± 0.47; p < 0.01). Inefficient C-PF was more prevalent in patients suffering from recurrent respiratory illness. The length of wheelchair confinement duration mostly influenced the C-VC level. 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.

• Ataxia Telangiectasia
• cough
• pneumonia
• spirometry

Declaration of interest

None of the authors have any conflict of interest to declare or do any financial and personal relationships with other people or organizations that could inappropriately influence (bias) their work. The study was supported by the J. Baum Foundation of Israel Lung Association, Tel-Aviv Israel.