Describe in detail the pathophysiological process of cystic fibrosis.

It is an inherited disease that can affect the digestive and respiratory systems. The disease is caused by a mutation in the CFTR gene, which codes for a protein called cystic fibrosis Transmembrane Conductance Regulator (CFTR). A defective gene causes the accumulation of mucus, which is thick and sticky. This can cause inflammation in different organs including lungs.

The abnormal mucus not only blocks the airways, but also accumulates within the lung. It can lead to difficulty breathing. In addition, because of the reduced ciliary movement associated with Cystic Fibrosis sputum can remain within pulmonary and alveoli causing infection.

This condition causes inadequate digestion of food and absorption nutrients due to the viscosity increase of glands that line all affected areas, including the pancreas. Malnutrition and dehydration are the result of this condition, as fluids cannot be absorbed correctly.

Moreover , malfunctioning CFTR proteins lead to impairment of salt balance regulation throughout body tissues thus allowing ions such sodium chloride) to accumulate on the surface epithelial cells present on all organs involved. The activation of certain immune pathways can worsen existing symptoms and further disturb normal functioning.

Altogether , it’s evident that when a person inherits two copies mutations of CFTR gene severe consequences ensue eventually leading potentially fatal complications if left untreated . Early diagnosis and aggressive treatment strategies that aim to improve patient’s quality of life will be essential for any successful treatment.