Restrictive thoracic disease

Nevertheless, IPPV is preferred for most patients who require continuous support and in those unable to protect their upper airway, as well as those who tolerate NTV poorly (7). A recent study suggested that some patients with restrictive thoracic disease (RTD) who received IPPV, enjoyed a better quality of life than those receiving NTV (8). Therefore, the choice of ventilation strategy should consider the preference of the patient and the caregiver as well as the environment (7). 

Nickol AH, Hart N, Hopkinson NS, et al. Mechanisms of improvement of respiratory failure in patients with restrictive thoracic disease treated with non-invasive ventilation. Thorax 2005 60 754-760. 

Domenach-Clar R, Nauffal-Manzu D, Perpina-Tordera, et al. Home mechanical ventilation for restrictive thoracic diseases effects of patient quality-of-life and hospitalizations. Respir Med 2003 97(12) 1320-1327. 

A musculoskeletal injury that arises gradually as a result of repeated microtrauma. CTDs are characterized by injuries to the tendons, nerves, or neurovascular system. Muscles and joints are stressed, tendons are inflamed, nerves are pinched, or the flow of blood is restricted. Examples of CTDs include tendinitis, tenosynovitis, carpal tunnel syndrome, thoracic outlet syndrome, and Raynaud s phenomenon (white finger disease). 

Musculoskeletal changes occur during the course of the disease. The chest assumes a barrel shape in which the anteroposterior (AP) diameter equals the transverse diameter. The accessory muscles of respiration gradually hypertrophy. Hypertrophic scalene muscles may impinge on neurovascular structures passing between or near them. Rib motion is markedly restricted and eventually contributes to the dyspnea. The thoracic spine becomes kyphotic and immobile. Motion of the diaphragm is restricted. 

Patients with thoracic restriction or advanced parench)mial diseases, who develop respiratory failure, represent a heterogeneous group. For such patients, nocturnal noninvasive ventilation is the preferred alternative. The results are very encouraging among those with stable thoracic restriction and less clear among those with advanced parenchymal diseases. The development of specialized facilities for long-term ventilation is described elsewhere in this book. 

NIV is often an effective treatment for patients with acute respiratory failure, especially when secondary to exacerbation of chronic obstructive pulmonary disease (COPD) (24). NIV is also utilized for long-term ventilation (LTV) of patients with chronic respiratory failure due to thoracic restriction or NMD. It is used less frequently for COPD as there is only limited evidence of its long-term effectiveness in this condition (25). In stable patients, NIV is affected by the type of interface used (26). 

Chest wall abnormalities such as severe scoliosis, kyphosis, or thoracic dystrophy may cause restrictive diseases severe enough to require long-term NPPV. 

The greatest impact on quality of life and survival has been documented in those patients afflicted with neuromuscular diseases (NMD), spinal cord injury (SCI), thoracic restriction (TR), and sleep-disordered breathing (4,5). This is likely due to the nature of the disease process and the relative ease with which a ventilatory steady state is reached. In this group of diseases patients can be ventilated with simple modes and little monitoring. 

By 1994, fees paid by medical insurance increased to cover medical services provided by the hospital, clinic, or home care nurse as well as the costs of medical equipment, such as the ventilator rental. This led to rapid growth in the population of patients receiving HMV (2). In April 1995, of the 536 HMV cases 65% had NMD, 20% had parenchymal disease (PD), such as sequelae of tuberculosis and chronic obstructive pulmonary disease (COPD), and 15% had thoracic restriction or central hypoventilation syndrome (3,4). In June 1995, of the 1006 patients undergoing LTV for at least three months, 215 (21%) could have been discharged to a home care setting if an appropriate public assistance program had been established (3,4). By January 1997, there were 1250 patients receiving HMV of whom 461 (1.2 people/million) used noninvasive positive pressure ventilation (NIPPV) (5). 

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