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Cystic Fibrosis - An Overview

Cystic fibrosis is an inherited disorder that affects the lungs and digestive system causing them serious damage. Cystic fibrosis affects the cells that produce sweat, mucus and digestive juices and make them thick and sticky resulting in the blockage of ducts, tubes and passageways.

If managed the right way, people with cystic fibrosis can lead normal day to day lives.

Symptoms of Cystic Fibrosis

Symptoms of cystic fibrosis differ, based on if it is respiratory or digestive.

Respiratory symptoms

  • Breathlessness
  • Wheezing
  • Persistent cough that produces thick mucus
  • Exercise intolerance
  • Inflamed nasal passages or a stuffy nose
  • Repeated lung infections

Digestive symptoms

  • Severe constipation
  • Poor weight gain and growth
  • Foul-smelling, greasy stools
  • Intestinal blockage, particularly in new-borns (meconium ileus)

If you or your child have any/some/all of the above symptoms, immediate medical attention is advised.

Risk factors of Cystic Fibrosis

You may be at a higher risk of developing cystic fibrosis if:

  • Have a history of cystic fibrosis in the family (since it's an inherited disorder)
  • Race is an important factor in the development of cystic fibrosis and it is most common in people with northern European ancestry.

Diagnosis of Cystic Fibrosis

Diagnosis for cystic fibrosis differs according to age.

For newborns:

Your doctor may first take a blood sample to check for higher than normal levels of a immunoreactive trypsinogen, or IRT - a chemical released by the pancreas.

A newborn's IRT could also be high because of premature birth or a stressful delivery. So, in order to rule out such a possibility and confirm cystic fibrosis, the following tests will be recommended:

    • Sweat test
    • Genetic testing

For children and adults:

Cystic fibrosis tests may be recommended for both children and adults who weren't screened at birth. The doctor may suggest genetic and sweat tests if he suspects cystic fibrosis.

Treatment of Cystic Fibrosis

There is no known cure of cystic fibrosis, but by taking the right medication and/or therapy, one can manage the condition well without it interfering in one's day to day life.

Medication

Medication for cystic fibrosis includes:

  • Antibiotics
  • Bronchodilators
  • Mucus thinning drugs
  • Oral pancreatic enzymes

Surgery

Surgical procedures for cystic fibrosis include:

  • Oxygen therapy
  • Nasal polyp removal
  • Feeding tube
  • Bowel surgery
  • Endoscopy and lavage
  • Lung transplant

Therapy

Chest physical therapy: This is done one to four times a day to help loosen the mucus in the lungs. Chest physical therapy can either be done by hand or by using a machine such as a vibrating vest.

Outpatient procedures

These may include:

  • Nutritional counselling
  • Exercise training
  • Psychological counselling and group support
  • Breathing techniques
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