Respiratory problems have a significant impact on morbidity and mortality in patients with cerebral palsy (CP). In particular, recurrent aspiration, impaired airway clearance, spinal and thoracic deformity, impaired lung function, poor nutritional status, and recurrent respiratory infections negatively affect respiratory status. Bronchopulmonary dysplasia may contribute to pulmonary problems, but asthma is not more common in CP than in the general population. We discuss treatment options for each of these factors. Multiple coexisting and interacting factors that influence the respiratory status of patients with CP should be recognized and effectively addressed to reduce respiratory morbidity and mortality. What this paper adds Respiratory problems are a significant cause of morbidity in patients with cerebral palsy (CP). Respiratory status in patients with CP is influenced by recurrent aspiration and impaired airway clearance. Spinal and thoracic deformity, impaired lung function, poor nutrition, and respiratory infections also negatively affect respiratory status. These factors should all be addressed to reduce respiratory problems in patients with CP.

IntroductionNoninvasive ventilation (NIV) is a well-established treatment for acute-on- chronic respiratory failure in hypercapnic COPD patients. Less is known about the effects of a long-term treatment with NIV in hypercapnic COPD patients and about the factors that may predict response in terms of improved oxygenation and lowered CO2 retention.MethodsIn this study, we randomized 15 patients to a routine pharmacological treatment (n = 5, age 66 [standard deviation ± 6] years, FEV1 30.5 [±5.1] %pred, PaO2 65 [±6] mmHg, PaCO2 52.4 [±6.0] mmHg) or to a routine treatment and NIV (using the Synchrony BiPAP device [Respironics, Inc, Murrsville, PA]) (n = 10, age 65 [±7] years, FEV1 29.5 [±9.0] %pred, PaO2 59 [±13] mmHg, PaCO2 55.4 [±7.7] mmHg) for 6 months. We looked at arterial blood gasses, lung function parameters and performed a low-dose computed tomography of the thorax, which was later used for segmentation (providing lobe and airway volumes, iVlobe and iVaw) and post-processing with computer methods (providing airway resistance, iRaw) giving overall a functional image of the separate airways and lobes.ResultsIn both groups there was a nonsignificant change in FEV1 (NIV group 29.5 [9.0] to 38.5 [14.6] %pred, control group 30.5 [5.1] to 36.8 [8.7] mmHg). PaCO2 dropped significantly only in the NIV group (NIV: 55.4 [7.7] → 44.5 [4.70], P = 0.0076; control: 52.4 [6.0] → 47.6 [8.2], NS). Patients actively treated with NIV developed a more inhomogeneous redistribution of mass flow than control patients. Subsequent analysis indicated that in NIV-treated patients that improve their blood gases, mass flow was also redistributed towards areas with higher vessel density and less emphysema, indicating that flow was redistributed towards areas with better perfusion. There was a highly significant correlation between the % increase in mass flow towards lobes with a blood vessel density of >9% and the increase in PaO2. Improved ventilation–perfusion match and recruitment of previously occluded small airways can explain the improvement in blood gases.ConclusionWe can conclude that in hypercapnic COPD patients treated with long-term NIV over 6 months, a mass flow redistribution occurs, providing a better ventilation–perfusion match and hence better blood gases and lung function. Control patients improve homogeneously in iVaw and iRaw, without improvement in gas exchange since there is no improved ventilation/perfusion ratio or increased alveolar ventilation. These differences in response can be detected through functional imaging, which gives a more detailed report on regional lung volumes and resistances than classical lung function tests do. Possibly only patients with localized small airway disease are good candidates for long-term NIV treatment. To confirm this and to see if better arterial blood gases also lead to better health related quality of life and longer survival, we have to study a larger population.

Studies that provide solid evidence of the effectiveness of different airway clearance techniques in patients with COPD are rather scarce. The available evidence indicates that active breathing techniques, such as active cycle of breathing techniques, autogenic drainage and forced expiration, can be effective in the treatment of COPD. The evidence for passive techniques such as postural drainage and percussion is low. Supporting techniques such as intrapulmonary percussive ventilation, positive expiratory pressure and non-invasive ventilation have little evidence because of the small number of studies. Little evidence is found for the combined use of active techniques and supporting techniques such as (oscillating) positive expiratory pressure, postural drainage and vibration in COPD patients. There is clearly a need for well-powered controlled clinical trials on the long-term effects of (combined) airway clearance techniques in COPD.

The addition of whole body vibration to both static and dynamic exercises appears to significantly increase oxygen uptake in overweight and obese women. More research is needed to determine the physiological pathway and clinical relevance of this increase.