Cystic Fibrosis: Fact Sheet Articles 2022
Added on 2022-09-22
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CYSTIC FIBROSIS: FACT SHEET
Introduction
Cystic fibrosis (CF) is a condition associated with the production of mucous in the lungs and pancreas. It
blocks the mucous leading to lung damage. The symptoms may vary for different individuals and it can include the
following:
Persistent coughs with phlegm
Wheezing or shortness of breath
Diarrhoea or severe constipation
Acute bronchitis
Fatigue
Pulmonary hypertension
Bulky stools
Salty skin
Normal physiology and homeostasis of the system
CF is a condition that affects the normal physiology of the lungs. Other parts of the which are affected by CF
includes respiratory system, digestive system and reproductive system. The lung is the fundamental organ of the
respiratory system and the CF patient present with many changes in the physiological structure of the lungs. CF is
characterized by viscous secretion of the exocrine glands and most of the severe pathological changes occur in tthe
lungs (Collawn & Matalon, 2014). In normal scenario, lung plays a role in facilitating gas exchange and
transporting oxygen through the alveoli into the capillaries. The lung is subdivided into several alveoli, which is the
primary organ for gas exchange. Restrictive lung disease like CF restricts lung expansion and result in decreased
lung volumes (Haddad & Sharma, 2019).
Introduction
Cystic fibrosis (CF) is a condition associated with the production of mucous in the lungs and pancreas. It
blocks the mucous leading to lung damage. The symptoms may vary for different individuals and it can include the
following:
Persistent coughs with phlegm
Wheezing or shortness of breath
Diarrhoea or severe constipation
Acute bronchitis
Fatigue
Pulmonary hypertension
Bulky stools
Salty skin
Normal physiology and homeostasis of the system
CF is a condition that affects the normal physiology of the lungs. Other parts of the which are affected by CF
includes respiratory system, digestive system and reproductive system. The lung is the fundamental organ of the
respiratory system and the CF patient present with many changes in the physiological structure of the lungs. CF is
characterized by viscous secretion of the exocrine glands and most of the severe pathological changes occur in tthe
lungs (Collawn & Matalon, 2014). In normal scenario, lung plays a role in facilitating gas exchange and
transporting oxygen through the alveoli into the capillaries. The lung is subdivided into several alveoli, which is the
primary organ for gas exchange. Restrictive lung disease like CF restricts lung expansion and result in decreased
lung volumes (Haddad & Sharma, 2019).
The homeostasis of respiratory system is maintained by gas exchange and regulation of the pH of
blood. The Lungs is involved in gas exchange function by eliminating carbon dioxide and inhaling
oxygen. ATP produced by cellular respiration plays a role in providing energy to the body. A
complex system of local immune pathways also maintains homeostasis in the lungs. The figure 1
below shows the homeostasis of the lungs and the factors involved in it (Lloyd & Marsland, 2017).
However, in patients diagnosed with CCF, year to year change in forced expiratory volume in one
second is seen. This results in dehydrated mucous which contributes to airway obstruction,
inflammatory response, chronic bacterial infections and eventually respiratory failure (Collawn &
Matalon,2014)..
Figure 1: Homeostatis of the lungs. Source: (Lloyd & Marsland, 2017).
blood. The Lungs is involved in gas exchange function by eliminating carbon dioxide and inhaling
oxygen. ATP produced by cellular respiration plays a role in providing energy to the body. A
complex system of local immune pathways also maintains homeostasis in the lungs. The figure 1
below shows the homeostasis of the lungs and the factors involved in it (Lloyd & Marsland, 2017).
However, in patients diagnosed with CCF, year to year change in forced expiratory volume in one
second is seen. This results in dehydrated mucous which contributes to airway obstruction,
inflammatory response, chronic bacterial infections and eventually respiratory failure (Collawn &
Matalon,2014)..
Figure 1: Homeostatis of the lungs. Source: (Lloyd & Marsland, 2017).
Pathophysiology of the disease
CF is a disease that is caused by mutation in the CF gene which causes dysfunction of the
transmembrane conductance regular (CFTR) protein. A child must inherit two copies of defective genes
from each parent for the disease to occur. If there is only one copy of the defective gene, then the person is said
to be the carrier of the disease. This mutated genes lead to the building of thickened layers of mucous in the
lungs, liver and pancreas. CFTR has a role in regulating the movement of sodium and chrolide ions in
epithelial cells. When mutation occurs in one or both copies of the gene, the ion transport system is
disrupted. This change leads to respiratory insufficiency and systemic abnormalities resulting in the
build up of mucus and other secretory granules. Along with altered ion transport, decrease in
mucociliary clearance takes place too. This change has impact on the cells of many organs. It leads to
respiratory insufficiency as well as many other systemic obstructions in the human body.
Overwhelming inflammatory response is also activated after muco-ciliary clearance leading to
colonization of bacteria in the respiratory tract. Some examples of these bacteria include
Staphylococcus aureus, Pseudomonas and Haemophilus influenza. This form of repetitive
inflammatory response leads to airway destruction in the affected individuals. Till date, more than six
classes of CF mutation has been identified (Haack, Aragão & Novaes, 2013; Brown et al., 2017).
Figure 1: Chest radiograph showing mucous in the lungs of patient with CF Source: (Brown et al.,
2017)
CF is a disease that is caused by mutation in the CF gene which causes dysfunction of the
transmembrane conductance regular (CFTR) protein. A child must inherit two copies of defective genes
from each parent for the disease to occur. If there is only one copy of the defective gene, then the person is said
to be the carrier of the disease. This mutated genes lead to the building of thickened layers of mucous in the
lungs, liver and pancreas. CFTR has a role in regulating the movement of sodium and chrolide ions in
epithelial cells. When mutation occurs in one or both copies of the gene, the ion transport system is
disrupted. This change leads to respiratory insufficiency and systemic abnormalities resulting in the
build up of mucus and other secretory granules. Along with altered ion transport, decrease in
mucociliary clearance takes place too. This change has impact on the cells of many organs. It leads to
respiratory insufficiency as well as many other systemic obstructions in the human body.
Overwhelming inflammatory response is also activated after muco-ciliary clearance leading to
colonization of bacteria in the respiratory tract. Some examples of these bacteria include
Staphylococcus aureus, Pseudomonas and Haemophilus influenza. This form of repetitive
inflammatory response leads to airway destruction in the affected individuals. Till date, more than six
classes of CF mutation has been identified (Haack, Aragão & Novaes, 2013; Brown et al., 2017).
Figure 1: Chest radiograph showing mucous in the lungs of patient with CF Source: (Brown et al.,
2017)
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