What is Cystic Fibrosis?

Cystic Fibrosis is a genetic condition that severely damages the respiratory and digestive systems. Cystic fibrosis is often the result of a buildup of abnormally thick and sticky mucus in the organs and typically affects the lungs, pancreas, liver, and intestines.

Individuals living with cystic fibrosis may face life-threatening issues such as infections, malnutrition, and respiratory failure, making it crucial for them to seek treatment right away. Let's take a closer look at the causes, symptoms, and treatments of cystic fibrosis.

Causes of Cystic Fibrosis

A deformity in the cystic fibrosis transmembrane conductance regulator (CFTR) gene is the major cause of cystic fibrosis. The main role of the CFTR gene is to control the movement of water and salt in and out of the body cells.

When there is a change in the CFTR gene, mucus can become thicker and stickier than it's intended to be and build up in a variety of organs. In addition, a change in the CFTR gene raises the amount of salt in sweat.

While cystic fibrosis is widely seen among caucasians of northern European descent, individuals of all types of ethnicities may face this condition.

Symptoms of Cystic Fibrosis

The severity of the condition as well as the age of the individual dictate the symptoms of cystic fibrosis. A salty taste in the skin is usually the first sign of cystic fibrosis and is often noticed when parents kiss their children.

In most cases, those with cystic fibrosis experience a variety of respiratory and digestive problems. These problems may be wheezing, a persistent cough that leads to thick mucus, shortness of breath, foul-smelling stools, constipation, appetite loss, poor weight gain, and delayed growth.

Treatment of Cystic Fibrosis

Unfortunately, there is no cure for cystic fibrosis. However, there are a number of treatments that may relieve its symptoms and reduce the risk of complications. The most common treatments for cystic fibrosis include:

  • Medications: Antibiotics may eliminate a lung infection and stop another one from occurring down the road. There are also mucus-thinning medications that may thin the mucus and make it less sticky. Nonsteroidal anti-inflammatory drugs to minimize any pain or discomfort and bronchodilators to relax the muscles surrounding the tubes that carry air into the lungs may be prescribed as well.
  • Chest Physical Therapy: The goal of chest physical therapy - which is typically performed four times per day - is to loosen the thick mucus in the lungs and make it easier to cough up. A patient may place their head over the edge of a bed and clap with cupped hands along the sides of their chest.
  • Home Care: Several examples of home care measures that may help patients with cystic fibrosis include drinking plenty of water, exercising frequently, avoiding smoking, and opting for influenza and pneumonia vaccinations on a regular basis.

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