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Sunabh answered on
Sep 06 2021
Running Head: CYSTIC FIBRIOSIS 1
CYSTIC FIBRIOSIS 2
CYSTIC FIBRIOSIS
Table of Contents
Introduction 3
Discussion about Cystic Fi
osis (CF) 3
Clinical Manifestations 3
Pathophysiology 4
Contributing Factors 5
Preventative Measures 6
Chronic Disease Management Plan 7
Conclusion 8
References 9
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Introduction
Medical issues can affect every individual differently and may present different symptoms under every case. Medical world have been threatened with the spread of cystic fi
osis from a very longer duration because it is a life-threatening condition and results into shorter than usual life span. This disease has been known to effect the lungs and digestive systems of the individuals and it can also result into fatal diseases such as liver failure and diabetes. It is a genetic disease therefore patient may receive it from their parents through a phenomenon known as ‘hereditary’.
The defective gene responsible for this disease leads to secretion and creation of thicker and stickier fluids, which sticks to the lungs causing severe lung infection and cough. One of the major drawbacks that medical industry has regarding this disease is the inability to develop any potential treatment. There is not potential cure for this disease til now however, medications may help to reduce the severity of symptoms yet there is a requirement for the development of a proper cure.
Likewise, CF can also occur as a result of certain mutation within the genes that may further lead to over production of mucus and ultimately lead to CF or symptoms identical to this disease. This paper will further discuss the pathophysiology of this disease along with the disease management plan as well as potential preventive measures that could be taken. Along with this efforts will be made in order identify some of the potential treatment for this incurable disease.
Discussion about Cystic Fi
osis (CF)
Clinical Manifestations
Cystic fi
osis (CF) is a genetic disease and therefore, it may reflect different and dynamic manifestation in every patient. Since during this disease, there is an excessive production of fluid also known as mucus; therefore, excessive cough deposition could be a major symptom (Fa
ell et al., 2017).
Further, within lower airway severe infection could occur especially with Staphylococcus aureus, Haemophilus influenza and Pseudomonas aeruginosa. VanDevanter, Kahle, O’Sullivan, Sikirica and Hodgkins (2016) suggested that this could lead to atypical mycobacterial infection, haemoptysis,
onchitis, pneumothorax, and other
onchiolar or pulmonary allergies. The infection may also spread to the upper as well as middle airway, which further may result into series of infections.
Infection in liver may result into Biliary ci
hosis, gallstones, bile duct stricture and portal hypertension (Lahiri et al., 2016). Other than this, some of the common clinical manifestations related to CF may include persistent coughing, shortness of
eath in cases of lung infection, wheezing, greasy and bulky stools due to cough, salty skin, poor weight gain even if the appetite is good and small fleshy growth found in nose also known as nasal polyps (Cogen et al., 2017).
Stoltz, Meyerholz and Welsh (2015) infe
ed that lung obstruction due to CF may also result into pneumonia and
onchitis, while obstruction of pancreas may lead to poor growth and malnutrition (Sathe & Freeman, 2016). Further, CF has also be associated with an increased risk of osteoporosis and diabetes (Cutting, 2015).
Pathophysiology
Pathophysiology of this disease initially is linked with any microorganism or infection caused by them. Instead, the individual may receive this heritable disease from their parents. CF Transmem
ane Conductance Regulator (CFTR) gene is responsible for the production of CFTR protein (Brown, White & Tobin, 2017). This protein is regulates the movement of sodium and chloride ions across epithelia cell mem
anes.
Mutations in this gene may result into defective ion transport because of which thick mucus starts to develop throughout the body. This further may be responsible for the respiratory insufficiency and other systemic abnormalities and obstructions. Cutting, Engelhardt and Zeitlin (2019) suggested that this decreased mucocilliary clearance and disrupted ion transport has been considered as the potential reason behind bacterial colonization of respiratory tract mostly due to Haemophilus influenza, Staphylococcus aureus and...