End stage pulmonary disease

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End stage pulmonary disease
Other namesEnd stage lung disease (ESLD) [1]
Specialty Pulmonology

End stage pulmonary disease (ESPD) [2] is the result of chronic progressive lung diseases like COPD, [3] idiopathic pulmonary fibrosis, or systemic progressive diseases that affect the lungs such as cystic fibrosis or granulomatosis with polyangiitis. It is defined as when the lungs can no longer or barely remove enough carbon dioxide or supply enough oxygen to meet the body's basic needs. Treatment incorporates a lung transplant. [4]

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Shortness of breath Feeling of difficulty breathing

Shortness of breath (SOB), also medically known as dyspnea (AmE) or dyspnoea (BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity", and recommends evaluating dyspnea by assessing the intensity of its distinct sensations, the degree of distress and discomfort involved, and its burden or impact on the patient's activities of daily living. Distinct sensations include effort/work to breathe, chest tightness or pain, and "air hunger". The tripod position is often assumed to be a sign.

Alpha-1 antitrypsin deficiency Medical condition

Alpha-1 antitrypsin deficiency is a genetic disorder that may result in lung disease or liver disease. Onset of lung problems is typically between 20 and 50 years of age. This may result in shortness of breath, wheezing, or an increased risk of lung infections. Complications may include chronic obstructive pulmonary disease (COPD), cirrhosis, neonatal jaundice, or panniculitis.

Bronchiectasis Disease of the lungs

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 frequent lung infections.

Pulmonary heart disease Medical condition

Pulmonary heart disease, also known as cor pulmonale, is the enlargement and failure of the right ventricle of the heart as a response to increased vascular resistance or high blood pressure in the lungs.

Nail clubbing Deformity of the finger or toe nails associated with a number of diseases

Nail clubbing, also known as digital clubbing or clubbing, is a deformity of the finger or toe nails associated with a number of diseases, mostly of the heart and lungs. When it occurs together with joint effusions, joint pains, and abnormal skin and bone growth it is known as hypertrophic osteoarthropathy.

Fontan procedure

The Fontan procedure or Fontan–Kreutzer procedure is a palliative surgical procedure used in children with univentricular hearts. It involves diverting the venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries without passing through the morphologic right ventricle; i.e., the systemic and pulmonary circulations are placed in series with the functional single ventricle. The procedure was initially performed in 1968 by Francis Fontan and Eugene Baudet from Bordeaux, France, published in 1971, simultaneously described in 1971 by Guillermo Kreutzer from Buenos Aires, Argentina, and finally published in 1973.

Interstitial lung disease Group of diseases

Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of respiratory diseases affecting the interstitium of the lungs. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process is disrupted, and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The disease presents itself with the following symptoms: shortness of breath, nonproductive coughing, fatigue, and weight loss, which tend to develop slowly, over several months. The average rate of survival for someone with this disease is between three and five years. The term ILD is used to distinguish these diseases from obstructive airways diseases.

Non-invasive ventilation

Non-invasive ventilation (NIV) is the use of breathing support administered through a face mask, nasal mask, or a helmet. Air, usually with added oxygen, is given through the mask under positive pressure; generally the amount of pressure is alternated depending on whether someone is breathing in or out. It is termed "non-invasive" because it is delivered with a mask that is tightly fitted to the face or around the head, but without a need for tracheal intubation. While there are similarities with regard to the interface, NIV is not the same as continuous positive airway pressure (CPAP), which applies a single level of positive airway pressure throughout the whole respiratory cycle; CPAP does not deliver ventilation but is occasionally used in conditions also treated with NIV.

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Right ventricular hypertrophy Medical condition

Right ventricular hypertrophy (RVH) is a condition defined by an abnormal enlargement of the cardiac muscle surrounding the right ventricle. The right ventricle is one of the four chambers of the heart. It is located towards the lower-end of the heart and it receives blood from the right atrium and pumps blood into the lungs.

Aspergillosis Medical condition

Aspergillosis is a fungal infection of usually the lungs, caused by the genus Aspergillus, a common mold that is breathed in frequently from the air around, but does not usually affect most people. It generally occurs in people with lung diseases such as asthma, cystic fibrosis or tuberculosis, or those who have had a stem cell or organ transplant, and those who cannot fight infection because of medications they take such as steroids and some cancer treatments. Rarely, it can affect skin.

Idiopathic pulmonary fibrosis Medical condition

Idiopathic pulmonary fibrosis (IPF), or (formerly) fibrosing alveolitis, is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue. It is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function. The tissue in the lungs becomes thick and stiff, which affects the tissue that surrounds the air sacs in the lungs. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired, and abnormally large and dome shaped finger and toenails. Complications may include pulmonary hypertension, heart failure, pneumonia or pulmonary embolism.

Bronchitis Inflammation of the large airways in the lungs

Bronchitis is inflammation of the bronchi in the lungs that causes coughing. Symptoms include coughing up sputum, wheezing, shortness of breath, and chest pain. Bronchitis can be acute or chronic.

Obstructive lung disease Category of respiratory disease characterized by airway obstruction

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.

Roflumilast

Roflumilast, sold under the trade name Daxas among others, is a drug that acts as a selective, long-acting inhibitor of the enzyme phosphodiesterase-4 (PDE-4). It has anti-inflammatory effects and is used as an orally administered drug for the treatment of inflammatory conditions of the lungs such as chronic obstructive pulmonary disease (COPD).

FEV1/FVC ratio Ratio used in the diagnosis of lung disease

The FEV1/FVC ratio, also called Tiffeneau-Pinelli index, is a calculated ratio used in the diagnosis of obstructive and restrictive lung disease. It represents the proportion of a person's vital capacity that they are able to expire in the first second of forced expiration (FEV1) to the full, forced vital capacity (FVC). The result of this ratio is expressed as FEV1%.

Pulmonary rehabilitation, also known as respiratory rehabilitation, is an important part of the management and health maintenance of people with chronic respiratory disease who remain symptomatic or continue to have decreased function despite standard medical treatment. It is a broad therapeutic concept. It is defined by the American Thoracic Society and the European Respiratory Society as an evidence-based, multidisciplinary, and comprehensive intervention for patients with chronic respiratory diseases who are symptomatic and often have decreased daily life activities. In general, pulmonary rehabilitation refers to a series of services that are administered to patients of respiratory disease and their families, typically to attempt to improve the quality of life for the patient. Pulmonary rehabilitation may be carried out in a variety of settings, depending on the patient's needs, and may or may not include pharmacologic intervention.

Chronic obstructive pulmonary disease Lung disease involving long-term poor airflow

Chronic obstructive pulmonary disease (COPD) is a type of progressive lung disease characterized by long-term respiratory symptoms and airflow limitation. The main symptoms include shortness of breath and a cough, which may or may not produce mucus. COPD progressively worsens with everyday activities such as walking or dressing becoming difficult. While COPD is incurable, it is preventable and treatable.

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 Medical condition

Emphysema, or pulmonary emphysema, is a lower respiratory tract disease, characterised by air-filled spaces (pneumatoses) in the lung, that can vary in size and may be very large. The spaces are caused by the breakdown of the walls of the alveoli and they replace the spongy lung parenchyma. This reduces the total alveolar surface available for gas exchange leading to a reduction in oxygen supply for the blood. Emphysema usually affects the middle aged or older population because it takes time to develop with the effects of smoking, and other risk factors. Alpha-1 antitrypsin deficiency is a genetic risk factor that may lead to the condition presenting earlier.

References

  1. Currow, David C; Ward, Alicia; Clark, Katie; Burns, Catherine M; Abernethy, Amy P (1 December 2008). "Caregivers for people with end-stage lung disease: Characteristics and unmet needs in the whole population". International Journal of Chronic Obstructive Pulmonary Disease. 3 (4): 753–762. doi:10.2147/COPD.S3890. PMC   2650595 . PMID   19281090.
  2. Pople, Josephine (2 October 2017). "Palliative care for people living with end-stage pulmonary disease". International Journal of Palliative Nursing . 23 (10): 486–495. doi:10.12968/ijpn.2017.23.10.486. PMID   29087752.
  3. "COPD and death: End stage symptoms and how to cope". 29 October 2021.
  4. "End-Stage Lung Disease". 2016-02-02. Archived from the original on 2017-12-21. Retrieved 2017-12-21.