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What is Cystic Fibrosis?

What-is-Cystic-Fibrosis--Blog-Image

Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body.

The most commonly affected organs include the:

  • lungs
  • pancreas
  • liver
  • intestines

Cystic fibrosis affects the cells that produce sweat, mucus, and digestive enzymes. Normally, these secreted fluids are thin and smooth like olive oil. They lubricate various organs and tissues, preventing them from getting too dry or infected.

Symptoms of cystic fibrosis

The symptoms of cystic fibrosis can vary depending on the person and the severity of the condition. The age at which symptoms develop can also differ.

Symptoms may appear at infancy, but for other children, symptoms may not begin until after puberty or even later in life. As time passes, the symptoms associated with the disease may get better or worse.

One of the first signs of cystic fibrosis is a strong salty taste to the skin. Parents of children with cystic fibrosis have mentioned tasting this saltiness when kissing their children.

Other symptoms of cystic fibrosis result from complications that affect:

  • the lungs
  • the pancreas
  • the liver
  • other glandular organs

Causes of cystic fibrosis

A defect in the CFTR gene causes cystic fibrosis (CF). This gene makes a protein that controls the movement of salt and water in and out of your body’s cells. In people who have CF, the gene makes a protein that doesn’t work well. This causes thick, sticky mucus and very salty sweat.

Research suggests that the CFTR protein also affects the body in other ways. This may help explain other symptoms and complications of CF.

More than a thousand known defects can affect the CFTR gene. The type of defect you or your child has may affect the severity of CF. Other genes also may play a role in the severity of the disease.

When to see a doctor

If you or your child has symptoms of cystic fibrosis — or if someone in your family has CF — talk with your doctor about testing for the disease. Consult a physician who is knowledgeable about CF.

Cystic fibrosis requires consistent, regular follow-up with your doctor, at least every three months. Contact you doctor if you experience new or worsening symptoms, such as more mucus than usual or a change in the mucus color, lack of energy, weight loss, or severe constipation.

Seek immediate medical care if you’re coughing up blood, have chest pain or difficulty breathing, or have severe stomach pain and distention.

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