Cystic Fibrosis

Cystic Fibrosis is an inherited chronic disease that directly affects the cells that produce mucus, digestive juice and sweat. In Cystic Fibrosis however, the defective gene makes these secretions thick and sticky clogging up the organ tubes, ducts and passageways of the lungs and pancreas when compared to having these fluids be innately thin and slippery.

The clogging of the lungs may lead to life threatening lung infections, while those clogging in the pancreas may lead to pancreatic inefficiency where natural enzymes are no longer produced to break down food in digestion and help absorb them in the gut.

Cystic Fibrosis Symptoms

Cystic Fibrosis symptoms may be broken into two main categories, as follows.

Lung Symptoms in Cystic Fibrosis include:

  • Persistent coughing with thick sticky sputum
  • Wheezing
  • Breathlessness
  • Recurrent lung infections
  • Stuffy nose

Digestive Symptoms of Cystic Fibrosis include:

  • Foul smell greasy stools
  •  Poor weight gain
  •  Intestinal obstruction
  •  Frequent constipation

Newborn screening in the US may include an Immuno-Reactive Tripsinogen (IRT) test to detect Cystic Fibrosis as early infancy. A confirmatory test like the Sweat Test and Genetic Testing may be needed to confirm the diagnosis of Cystic Fibrosis.

Treatment Options

Treatment modalities for Cystic Fibrosis may only limited in the active prevention of lung infections, removal of lung mucus, treatment of intestinal obstructions, and the provision of adequate nutrition. The following treatment options may be available for Cystic Fibrosis.

Medications – Antibiotics are given to prevent infections, while mucolytics and bronchodilators may also be given to prevent lung clogging. Exogenous pancreatic enzymes may be given to aid digestion in affected individuals.

Chest Therapy and Rehabilitation – Physical Chest therapy using pulmonary exercises and devices may aid individuals with the disease to expectorate mucus effectively. Pulmonary rehabilitation may be part of the ongoing management plan to improve the overall well-being of affected individuals.

Surgical Procedures – Nasal polypectomy may be recommended by your physician. Endoscopy and lavage may relieve an obstructive gastro-intestinal tract and airways. Bowel surgery may be afforded for intestinal obstruction.

It is important to recognize that medications and medical procedures are associated with benefits and risks that should be discussed with your physician. It is important to recognize that all information contained on this website cannot be considered to be specific medical diagnosis, medical treatment, or medical advice. As always, you should consult with a physician regarding any medical condition. Your Health Access disclaims any liability for the decisions you make based on this information.