The A-T Children’s Project and the A-T Society in the United Kingdom have jointly awarded a grant to Emma Ross PhD, from the University of Brighton in England, entitled “Inspiratory muscle training in patients with Ataxia-Telangiectasia.”

Lung disease is a frequent and often serious problem in patients with A-T. A 2010 paper in the journal Pediatric Pulmonology, which focused on the evaluation and management of lung disease in A-T, suggested that exercises that can maintain or strengthen respiratory muscles may help patients with this disease.

Dr. Ross will examine the effects of inspiratory muscle training (IMT) on muscle strength, productive cough and quality of life in individuals with A-T. Just like arm or leg muscles, the muscles used for breathing can be trained to improve performance. Study participants will use a special device to improve the performance of muscles involved in the inhalation process.

Each of the 15 participants in this study will use a hand held IMT training device for 15 minutes, twice a day, for 12 weeks. In addition to the IMT, all participants will also receive mock or sham training as a placebo control for a portion of the study.

Dr. Ross’ team will evaluate how well participants’ breathing muscles responded to the IMT training by measuring their strength, the strength of cough and overall lung function. Quality of life will be assessed using a questionnaire.

If results from this study are positive, IMT may offer a non-pharmacological, inexpensive and accessible method to improve respiratory muscle function and quality of life in people with A-T.

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