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Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably Staphylococcus aureus. CF is a rare genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. The hallmark feature of CF is the accumulation of thick mucus in different organs. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males. Different people may have different degrees of symptoms.
A cough is a sudden expulsion of air through the large breathing passages which can help clear them of fluids, irritants, foreign particles and microbes. As a protective reflex, coughing can be repetitive with the cough reflex following three phases: an inhalation, a forced exhalation against a closed glottis, and a violent release of air from the lungs following opening of the glottis, usually accompanied by a distinctive sound.
Bronchiectasis is a disease in which there is permanent enlargement of parts of the airways of the lung. Symptoms typically include a chronic cough with mucus production. Other symptoms include shortness of breath, coughing up blood, and chest pain. Wheezing and nail clubbing may also occur. Those with the disease often get lung infections.
Mucus is a slippery aqueous secretion produced by, and covering, mucous membranes. It is typically produced from cells found in mucous glands, although it may also originate from mixed glands, which contain both serous and mucous cells. It is a viscous colloid containing inorganic salts, antimicrobial enzymes, immunoglobulins, and glycoproteins such as lactoferrin and mucins, which are produced by goblet cells in the mucous membranes and submucosal glands. Mucus serves to protect epithelial cells in the linings of the respiratory, digestive, and urogenital systems, and structures in the visual and auditory systems from pathogenic fungi, bacteria and viruses. Most of the mucus in the body is produced in the gastrointestinal tract.
Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive genetic ciliopathy, that causes defects in the action of cilia lining the upper and lower respiratory tract, sinuses, Eustachian tube, middle ear, fallopian tube, and flagella of sperm cells. The alternative name of "immotile ciliary syndrome" is no longer favored as the cilia do have movement, but are merely inefficient or unsynchronized. When accompanied by situs inversus the condition is known as Kartagener syndrome.
Atelectasis is the partial collapse or closure of a lung resulting in reduced or absent gas exchange. It is usually unilateral, affecting part or all of one lung. It is a condition where the alveoli are deflated down to little or no volume, as distinct from pulmonary consolidation, in which they are filled with liquid. It is often referred to informally as a collapsed lung, although more accurately it usually involves only a partial collapse, and that ambiguous term is also informally used for a fully collapsed lung caused by a pneumothorax.
Cystic fibrosis transmembrane conductance regulator (CFTR) is a membrane protein and anion channel in vertebrates that is encoded by the CFTR gene.
Chest physiotherapy (CPT) are treatments generally performed by physical therapists and respiratory therapists, whereby breathing is improved by the indirect removal of mucus from the breathing passages of a patient. Other terms include respiratory or cardio-thoracic physiotherapy.
Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterised by an exaggerated response of the immune system to the fungus Aspergillus. It occurs most often in people with asthma or cystic fibrosis. Aspergillus spores are ubiquitous in soil and are commonly found in the sputum of healthy individuals. A. fumigatus is responsible for a spectrum of lung diseases known as aspergilloses.
Obstructive lung disease is a category of respiratory disease characterized by airway obstruction. Many obstructive diseases of the lung result from narrowing (obstruction) of the smaller bronchi and larger bronchioles, often because of excessive contraction of the smooth muscle itself. It is generally characterized by inflamed and easily collapsible airways, obstruction to airflow, problems exhaling, and frequent medical clinic visits and hospitalizations. Types of obstructive lung disease include asthma, bronchiectasis, bronchitis and chronic obstructive pulmonary disease (COPD). Although COPD shares similar characteristics with all other obstructive lung diseases, such as the signs of coughing and wheezing, they are distinct conditions in terms of disease onset, frequency of symptoms, and reversibility of airway obstruction. Cystic fibrosis is also sometimes included in obstructive pulmonary disease.
Peribronchial cuffing, also referred to as peribronchial thickening or bronchial wall thickening, is a radiologic sign which occurs when excess fluid or mucus buildup in the small airway passages of the lung causes localized patches of atelectasis. This causes the area around the bronchus to appear more prominent on an X-ray. It has also been described as donut sign, considering the edge is thicker, and the center contains air.
Mucociliary clearance (MCC), mucociliary transport, or the mucociliary escalator describes the self-clearing mechanism of the airways in the respiratory system. It is one of the two protective processes for the lungs in removing inhaled particles including pathogens before they can reach the delicate tissue of the lungs. The other clearance mechanism is provided by the cough reflex. Mucociliary clearance has a major role in pulmonary hygiene.
Pulmonary hygiene, also referred to as pulmonary toilet, is a set of methods used to clear mucus and secretions from the airways. The word pulmonary refers to the lungs. The word toilet, related to the French toilette, refers to body care and hygiene; this root is used in words such as toiletry that also relate to cleansing.
Warren J. Warwick was an American pediatrician, notable for co-inventing a chest wall oscillation device called the Vest Airway Clearance System, or "The Vest", a mechanical vest for clearing the lungs of children with cystic fibrosis. He was a professor of pediatric pulmonology at the University of Minnesota, where he was a faculty member for more than 50 years. He served as director of the Cystic Fibrosis Center at the University of Minnesota 1962 to 1999, recognized by peer institutions as among the best in the United States.
Denufosol (INN) is an inhaled drug for the treatment of cystic fibrosis, being developed by Inspire Pharmaceuticals and sponsored by the Cystic Fibrosis Foundation. It was tested in two Phase III clinical trials, TIGER-1 and TIGER-2. Initially, in the first Phase III trial, TIGER-1, the compound showed significant results as compared with placebo. In the second Phase III trial, TIGER-2, the compound did not meet the primary endpoint, a significant change in baseline FEV1 at the week 48 endpoint as compared to placebo. As of 2011, no additional clinical studies are being conducted with the compound.
Physiotherapists treating patients following uncomplicated coronary artery bypass surgery surgery continue to use interventions such as deep breathing exercises that are not supported by best available evidence. Standardised guidelines may be required to better match clinical practice with current literature.
Emphysema is any air-filled enlargement in the body's tissues. Most commonly emphysema refers to the permanent enlargement of air spaces (alveoli) in the lungs, and is also known as pulmonary emphysema.
Plastic bronchitis (PB) is a disorder in which branching casts of the airways are expectorated. PB is not a single disease with a defined mechanism that explains the cast formation in all conditions. Examples of diseases associated with expectoration of casts, and which sometimes are labeled PB include tuberculosis, atypical mycobacterial disease, allergic bronchopulmonary aspergillosis, and asthma.
Airway clearance therapy is treatment that uses a number of airway clearance techniques to clear the respiratory airways of mucus and other secretions. Several respiratory diseases cause the normal mucociliary clearance mechanism to become impaired resulting in a build-up of mucus which obstructs breathing, and also affects the cough reflex. Mucus build-up can also cause infection, and inflammation, and repeated infections can result in damage to the airways, and the lung tissue.
Underrepresented populations, especially black and hispanic populations with cystic fibrosis are often not successfully diagnosed. This is in part due to the minimal dissemination of existing data on patients from these underrepresented groups. While white populations do appear to experience a higher frequency of cystic fibrosis, other ethnicities are also affected and not always by the same biological mechanisms. Thus, many healthcare and treatment options are less reliable or unavailable to underrepresented populations. This issue affects the level at which public health needs are being met across the world.
References
- ↑ When 'Amazing' Is No Longer Enough. By Barnaby J. Feder. Published: April 6, 1995. The New York Times.
- ↑ Device and method for measuring a spirogram, Warren J. Warwick et al. Patent number: 6050953. Filing date: May 21, 1998.
- ↑ Chest compression apparatus, Warren J. Warwick et al. Application number: 11/204,547. Publication number: US 2006/0036199 A1.