Cystic Fibrosis progression

Cystic Fibrosis progression

The blockage of pancreatic ducts by thick mucus triggered by dysfunctional CFTR causes an accumulation of enzymes in the pancreas that initiate the degradation of the pancreatic tissue. The enzymatic action on pancreatic tissue damage the pancreas causing complications that manifests as cystic fibrosis. Damages to the pancreas affect insulin-producing cells resulting in diabetes (Adler & Liou, 2016).

Cystic Fibrosis progression

The progression of Cystic Fibrosis involves organ damages due to inflammatory response and infection. Lung damage is the most common complication when Cystic Fibrosis progresses. The increased production of mucus causes the plugging of the airway and the accumulation of mucus in the lungs. The accumulated mucus allows pathogenic bacteria such as Pseudomonas aeruginosa and staphylococcus aureus that triggers an inflammatory response. The immune cells, such as macrophages and neutrophils, are recruited to eradicate the infection. The triggered inflammation in the lungs causes tissue damages that reduce gaseous exchange. Lung damage by inflammation can be diagnosed using Forced Expiratory Volume in one second, which reduces as a result of the effect. The inflammatory effect by the immune system targeting pathogens in the lungs can also cause an increase in mucus production, which influences the development of further obstruction and persistence of inflammation. The progression of Cystic Fibrosis can cause a complete dysfunction of the lungs resulting in mortalities (Adler & Liou, 2016).

The disorder can progress in the intestines in children resulting in the obstruction of the intestines. The increased mucus production and water absorption by the epithelial cells contribute to the obstruction of the intestines. High rates of fluid absorption result in dehydration that causes intestinal blockage and constipation. Fecal impaction is a common issue observed in patients with intestinal involvement of the disorder (Yu & Sharma, 2020).

Clinical manifestations of Cystic Fibrosis

Pediatrics with Cystic Fibrosis present with disease systems caused by the increased thick mucus production that influence organ damage. The first symptom observed in children with the disorder is early lung infection due to lung obstruction. Infants presenting with lung infection soon after delivery should be assessed for gene mutations in the CFTR gene. Another important symptom is anemia and poor weight gain. Anemia is due to the abnormal production of red blood cells due to the limitations caused by a gene mutation. Early severe anemia in infants is a common symptom that requires appropriate assessment to rule out gene mutations (Yu & Sharma, 2020).

Cystic Fibrosis influences respiratory damages. As such, airway obstruction due to increased mucus production is an important sign of the disorder. Patients can present with breathing difficulties as a result of the damages in the lungs. Male children suffer from undescended testicles, an effect that is poorly understood. The involvement of the intestines causes distal intestinal obstructive syndrome in pediatrics. Patient diagnosis requires patient history for the presence of Cystic Fibrosis in any of the siblings. The assessment of pediatrics for low FEV, intestinal obstruction, and pancreatic enzymes can facilitate disease diagnosis (Dos Santos et al., 2018).

Disease treatment

Cystic Fibrosis is a genetic disorder caused by mutations. As such, there is no known cure for the disorder. Patient treatment involves the management of disease complications such as the provision of bronchodilators for patients presenting with airway obstruction. Antibiotics therapy is important for patients with lung damage caused by infections and multivitamins. Pancreatic enzymes are also administered to manage effects caused by damages to the pancreas (Yu & Sharma, 2020).

Conclusion

In conclusion, Cystic Fibrosis is a common genetic disorder that affects children. Mutations cause Cystic Fibrosis in the gene coding for Cystic Fibrosis Transmembrane Conductance Regulator protein. The affected protein is important for the normal functioning of cells to facilitate the movement of molecules across the plasma membrane. The progression of the condition is due to increased thick mucus production by cells that cause an obstruction in various organs. Lung damage is the main cause of mortality in Cystic Fibrosis, which requires proper assessment and management.