Support and rehabilitation of patients with pulmonary expansion deficit by using game therapy.

Patients suffering from hypoventilation and pulmonary expansion deficit are at increased risk of developing pulmonary complications such as atelectasis, pneumonia or pleural effusion. These complications can increase the length of stay and spending on health, and generate long-term functional impairment. This study aims to produce a therapeutic alternative to the traditional method of lung re-expansion through incentive spirometry (IS) using the game therapy to build an innovative system. This system makes use of infrared and Bluetooth communication technology to associate the game therapy to EI. At the end of the system implementation, we expect to obtain good adhesion of the patient and the physiotherapists.

Hypoventilation improvement in an adult non-invasively ventilated patient with Rapid-onset Obesity with Hypothalamic Dysfunction Hypoventilation and Autonomic Dysregulation (ROHHAD).

Rapid-onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) is a rare disease of unknown etiology, characterized by rapid-onset obesity in young children, hypoventilation, hypothalamic and autonomic dysfunction. Patients between the ages of 2 and 4 present with hyperphagia and weight gain, followed by neuro-hormonal dysfunction and central hypoventilation months or years later. Cardiac arrest may represent the fatal complication of alveolar hypoventilation and early mechanical ventilation is essential for the patient's life. In this paper, we describe a 22-year-old patient with ROHHAD syndrome who had an acute respiratory failure during nocturnal non-invasive ventilation (NIV).

A historical perspective on expiratory muscle aids and their impact on home care.

Mechanically assisted coughing is the combination of mechanical insufflation-exsufflation to the airways in conjunction with an exsufflation-timed abdominal thrust. It has permitted in-home long-term survival of respirator-dependent users of continuous noninvasive intermittent positive pressure ventilatory support in the United States since shortly after it became available in 1952. By contrast, the first European patients to benefit from continuous noninvasive intermittent positive pressure ventilatory support were not described until institutionalized continuous noninvasive intermittent positive pressure ventilatory support users with Duchenne muscular dystrophy were described in Belgium in 2006, 2 yrs after mechanically assisted coughing was approved for sale by the European Union. Domiciliary management with continuous noninvasive intermittent positive pressure ventilatory support and mechanically assisted coughing has subsequently been described in Europe. This work describes the historical development of mechanically assisted coughing in the framework of aiding the respiratory muscles to prevent respiratory failure and avoid invasive airway intubation. Some current mechanical in-exsufflators include oscillation as a surrogate for physiologic mucociliary transport along with their assisted coughing function. Noninvasive management prolongs life while promoting cost containment and preserving quality-of-life for patients with respiratory muscle impairment.

[Exercise testing by spiroergometry in a patient with sarcoidosis stage II. An unusual combination of functional impairments]. / Spiroergometrie bei einem Patienten mit Sarkoidose II. Eine aussergewöhnliche Kombination von Funktionsstörungen.

Sarcoidosis is a multisystem disease of unknown origin characterised by epitheloid cell granulomas in the lung and many other organs. The measurement of the alveolo-arterial O(2)-gradient (AaDO(2)) during exercise is the most sensitive method to detect the involvement of the lung with impairment of gas exchange. The method is suitable for follow-up observations to make the indication for a steroid therapy in the case of deterioration. In this case presentation using the 9-panel display proposed by Wasserman we diagnosed besides a slight impairment of the gas exchange a cardiocirculatory dysfunction with a reduction of the oxygen pulse and a reduced minute ventilation with a decrease of the alveolar ventilation. The hypoventilation could be explained by a weakness of the respiratory muscles or a reduction of the central respiratory drive. The functional disturbances could be diagnosed by spiroergometry without expensive or invasive methods. The findings should be examined more specifically with regard to therapeutic consequences.

Effect of non-invasive ventilation on survival, quality of life, respiratory function and cognition: a review of the literature.

Symptoms of nocturnal hypoventilation may negatively influence the quality of life (QoL) of ALS patients long before respiratory failure ensues. Non-invasive mechanical ventilation (NIV) is considered a treatment option for nocturnal hypoventilation. The primary objective of NIV is improving quality of life (QoL). It may also prolong life by several months. A systematic review of the literature was performed to analyse what is known of the effect of NIV on survival, QoL and other outcome measures. A computerized literature search was performed to identify controlled clinical trials and observational studies of treatment of ALS-associated nocturnal hypoventilation from 1985 until May 2005. Twelve studies fulfilled the inclusion criteria. Four studies were retrospective, seven prospective and in one study randomization was used. All studies reported beneficial effects of NIV on all outcome measures. In seven studies NIV was associated with prolonged survival in patients tolerant for NIV, and five studies reported an improved QoL. In conclusion, studies on the use of NIV in ALS differ in study design and endpoint definitions. All studies suggest a beneficial effect on QoL and other outcome measures (Evidence level Class II-III). Well-designed randomized controlled trials comparing the effect on QoL and survival have not been performed.

Kyphoscoliosis ventilatory insufficiency: noninvasive management outcomes.

OBJECTIVE: To determine the effects on symptoms, pulmonary function, sleep, and other clinical variables of treating kyphoscoliosis-associated chronic alveolar hypoventilation with nocturnal nasal ventilation. DESIGN: Sixteen patients with kyphoscoliosis were treated with nocturnal nasal ventilation delivered by volume-cycled (seven patients) and pressure-cycled (nine patients) ventilators. Dyspnea, morning headaches, fatigue, hypersomnolence, and perceived sleep quality were assessed. RESULTS: All pretreatment symptoms improved significantly with nasal ventilation. Likewise, PaO2 (mm Hg), PaO2/FlO2, PaCO2 (mm Hg), pH, and forced vital capacity (in milliliters and as a percentage of predicted normal) significantly improved with treatment. Maximum inspiratory pressures and maximum expiratory pressures also significantly increased. Tidal volumes increased significantly and breathing frequency decreased (not significant). Although perceived sleep quality improved, as well as sleep oxyhemoglobin saturation, there was no significant change in sleep architecture. Hospitalization days for respiratory difficulties also decreased from 10.9 +/- 13.3 days in the 6 mo before intermittent positive-pressure ventilation to 0 days during the first 6 mo of treatment. CONCLUSIONS: Although not apparently affecting sleep architecture, nocturnal nasal ventilation can significantly improve nocturnal and daytime blood gases, pulmonary function, and symptoms of hypoventilation for patients with severe kyphoscoliosis.

[Noninvasive intermittent self-ventilation as a palliative measure in amyotrophic lateral sclerosis]. / Nichtinvasive intermittierende Selbstbeatmung (ISB) als Palliativmassnahme bei amyotropher Lateralsklerose.

Almost all patients with amyotrophic lateral sclerosis (ALS) experience symptoms of nocturnal hypoventilation during the course of the illness. These symptoms can develop years before death and may severely affect quality of life. Non-invasive intermittent home mechanical ventilation (HMV) via mask is a possible palliative measure for these symptoms, which is not often used in Germany. We report on our experience with HMV in 24 patients with ALS. Our data show a good palliative effect in 17 of 24 treated patients. Severe complications did not occur. The mean ventilation time at present is 14 months. Available options and their consequences need to be discussed in detail with patients and relatives before HMV is initiated.