Cystic fibrosis is a genetic disorder affecting various body systems, notably the respiratory system, leading to thick mucus blockages and increased infection risks. Diagnosis involves a sweat test, and treatment focuses on clearing mucus, preventing infections, and addressing nutritional deficiencies.

Cystic fibrosis is a genetic disorder affecting the exocrine glands, leading to thick mucus that blocks airways, causing respiratory issues, infertility, skin problems, and digestive complications. The disease is diagnosed through a sweat test and is most common in Caucasians and males, with a median life expectancy of 37 years.

The CFTR gene, responsible for regulating sodium and chloride channels, is crucial in maintaining the thin and slippery mucus in the body. Mutations in CFTR lead to thick and sticky mucus, causing respiratory issues like obstructive pulmonary disease and pulmonary hypertension in cystic fibrosis patients.

The chapter discusses how cystic fibrosis affects the GI system, focusing on the role of the pancreas in producing enzymes for digestion and the consequences of enzyme blockage. It also highlights the impact on the liver, bile ducts, intestines, and the development of complications like meconium ileus and distal intestinal obstruction syndrome.

Patients with cystic fibrosis (CF) face challenges in conceiving children due to issues related to thick and sticky mucus affecting fertility in both men and women. Women with CF have difficulty conceiving because their cervical mucus, essential for sperm travel, is not sperm-friendly, while men may have issues with the vas deferens duct being blocked by mucus. Additionally, CF patients experience electrolyte imbalances and dehydration due to excessively salty sweat and tears, requiring them to take extra salt supplements during episodes of heavy sweating or strenuous activities.

Cystic fibrosis patients should wear masks, practice strict hand-washing, stay up to date on vaccines, and avoid sick individuals to prevent infections. Nutritionally, they require a high caloric, high protein, high fat diet, fat-soluble vitamin supplements, and pancreatic enzymes for proper digestion.