Chronic obstructive pulmonary disease (COPD)

COPD is considered a preventable and treatable chronic condition characterized by progressive airflow limitation resulting from a response of the lung to harmful gases or particles. Causes include smoking, occupational hazard, and genetic predisposition. Assessment of COPD is based on spirometric testing in which a person takes a maximum inhalation and exhales as forcefully and quickly as possible. This test provides measures of forced expiratory volume and forced vital capacity based on age, sex, and height norms (see below for more information). There are currently four stages according to spirometric testing, ranging from “mild” to “very severe” qualifications of disease progression.

Neuromuscular disease

Neuromuscular conditions (e.g., amyotrophic lateral sclerosis) and spinal cord injury/disorder may lead to progressive respiratory muscle weakness. As a result, supportive ventilation may be required to assist with breathing. Interventions range with regard to level of invasiveness, with less invasive approaches available for people who maintain adequate bulbar muscle strength. Many individuals are able to gradually wean from ventilator support. However, this process can be challenging in the setting of recurrent respiratory muscle weakness or fatigue. Re-intubation and even tracheostomy for long-term support may be required if weaning is unsuccessful.

Cystic fibrosis (CF)

An inherited autosomal recessive disorder, CF is characterized by production of viscous mucus that ultimately leads to susceptibility to lung infection. Viscous mucus results from degenerating neutrophils (e.g., infection-fighting white blood cells). CF can be classified as a severe combined obstructive-restrictive pulmonary disease. Life expectancy of children with CF has increased in recent years, bringing the importance of rehabilitative interventions and quality of life measures clearly into focus.