Effects of inspiratory muscle training on lung volumes, respiratory muscle strength, and quality of life in patients with ataxia telangiectasia

Pediatr Pulmonol. 2014 Mar;49(3):238-44. doi: 10.1002/ppul.22828. Epub 2013 Aug 19.


Background: Ataxia telangiectasia (AT) is a genetic syndrome caused by a mutation of chromosome 11. The clinical features are cerebellar ataxia, telangiectasia, and progressive loss of muscular coordination, including an inefficient cough secondary to progression of neurological disease.

Objective: To evaluate the effects of inspiratory muscle training (IMT) on ventilation, lung volume, dyspnoea, respiratory muscle strength, and quality of life in patients with AT.

Methods: A longitudinal study was conducted with 11 AT patients and nine healthy volunteers. Ventilometry, subjective sensation of dyspnoea, maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and quality of life were assessed before and after a 24-week IMT program. The IMT load used was set at 60% of the MIP, and the training was performed for 20 min daily.

Results: Patients with AT had lower height and weight and also had lower respiratory muscle strength and lung volume compared with healthy volunteers. Furthermore, patients with AT showed a significant improvement when pre- and post-IMT were compared for ventilatory pattern: Vt (476.5 ± 135 ml vs. 583.3 ± 66 ml, P = 0.015) and f (23.3 ± 6 rpm vs. 20.4 ± 4 rpm, P = 0.018), and VC (1,664 ± 463 ml/kg vs. 2,145 ± 750 ml/kg, P = 0.002). IMT also significantly improved the sensation of dyspnoea (median 0.5; minimum 0; maximum 1.0; P = 0.022) and respiratory muscle strength: MIP (-22.2 ± 2 cmH2O vs. -38 ± 9 cmH2O, P < 0.001) and MEP (29 ± 7 cmH2O vs. 40 ± 8 cmH2O, P = 0.001). The health and vitality domains of the SF-36 also showed significant improvement (P = 0.009 and P = 0.014, respectively) post-IMT.

Conclusion: IMT was effective in improving ventilatory pattern, lung volume, respiratory muscle strength, and the health and vitality domains for quality of life in patients with AT. IMT may be an effective adjunct therapy to drug treatment for patients with AT.

Keywords: ataxia telangiectasia; immunodeficiency syndromes; inspiratory muscle training; physical therapy modalities; respiratory function tests.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Ataxia Telangiectasia / physiopathology
  • Ataxia Telangiectasia / therapy*
  • Breathing Exercises / methods*
  • Case-Control Studies
  • Child
  • Female
  • Humans
  • Inspiratory Capacity*
  • Longitudinal Studies
  • Male
  • Muscle Strength*
  • Quality of Life*
  • Respiratory Muscles / physiopathology*
  • Spirometry
  • Treatment Outcome
  • Vital Capacity