Cystic Fibrosis

What is cystic fibrosis?

A severe disorder that causes damage to the digestive system and the lungs is called cystic fibrosis. It is an inherited condition which can be life-threatening. Cystic fibrosis usually affects the cells that produce the digestive juice, mucus and sweat. When affected by the condition, the fluids become thick and sticky which are normally thin and slippery. It finally results in blocking the passageways, tubes and ducts in the pancreas and the lungs.

What are the symptoms of cystic fibrosis?

Depending on the severity of the condition, the symptoms may vary. Some of the common respiratory signs and symptoms of cystic fibrosis may include:

  • Breathlessness

  • Recurrent lung infections

  • Wheezing

  • Stuffy nose

  • Persistent cough

What causes cystic fibrosis?

A defect or mutation in the gene is considered to be the main factor that paves the way for cystic fibrosis. The defect changes the protein that controls the movement of salt in and out of the cells. This change will cause the mucus in the respiratory system to be sticky and thick. The severity of the condition may vary according to the type of the gene defect.

How the condition is diagnosed?

Sweat test and genetic test is usually indicated for the diagnosis of cystic fibrosis. If the individual is found to have the condition, he/she may need to undergo regular tests to manage cystic fibrosis. Some of the tests after the diagnosis of the condition may include:

  • Imaging tests like CT, X-rays and MRI

  • Sputum culture

  • Lung function test

  • Organ function tests

How is it treated?

Unfortunately, the condition cannot be completely cured. Treatments mainly aim to reduce the discomforts and symptoms caused by the condition. It requires early and aggressive intervention along with close monitoring. Treatment options may include:

  • Medication

  • Chest physical therapy- Breathing devices, chest clapper, and inflatable vest

  • Pulmonary rehabilitation- Exercise, breathing strategies, nutritional counselling, psychological counselling and energy-conserving techniques

  • Surgical procedures- Bowel surgery, nasal polyp removal, lung transplant, endoscopy, lavage and feeding tube.

  • Oxygen therapy