Cystic Fibrosis

Cystic fibrosis is a recessive genetic abnormality, which leads to complications that may put life to risks such as severe lungs, liver, intestine, pancreas, sinuses and sex organ problems. This inherited, life-threatening disorder adversely affects the cells that produce mucus, sweat and juices in the digestive system. The mucus becomes stickier and thicker, clogging up glandular secretions as well as airways then damages the pancreas and the lungs. There are about 30,000 people in the U.S. with cystic fibrosis as it is among the most common diseases of Caucasians. Previously, CF patients would not live over 20 years, but recent better checking methods and complications management have allowed patients to live until 50 years or more.

Drug nameGeneric NameCoupon
CaystonAztreonamCayston coupon
KalydecoIvacaftorKalydeco coupon
AcetylcysteineAcetylcysteine coupon
PulmozymeDornase alfaPulmozyme coupon
TobiTobramycinTobi coupon

Cystic Fibrosis Symptoms

The chronic disease cystic fibrosis has symptoms that vary in patients, which may improve or worsen over time, the most common of which are:

  • Continuous coughing that at times has phlegm
  • Salty skin
  • Frequent infections of the lungs like bronchitis and pneumonia
  • Breath shortness or wheezing
  • Fleshy or small growths in the nose called polyps
  • Poor weight gain or growth even with good appetite
  • Difficulty in bowel movement and oftentimes bulky or greasy stools
  • Bigger abdomen from constipation
  • Tenderness and swelling of the pancreas or pancreatitis, as well as respiratory symptoms

Cystic Fibrosis Causes

The inherited cystic fibrosis ailment is caused by the mutation of the Cystic Fibrosis gene, which encodes the protein called Cystic Fibrosis Transmembrane Regulator, on chromosome 7. The CFTR controls water and salt movement inside and outside of the body's cells, and its abnormality leads to salty sweat, sticky mucus and destruction of the cell's chloride channels. Cystic Fibrosis results to severe breathing problems and permanent lung damage while the sticky mucus blocks pancreas ducts causing non-absorption of proteins and fats since the digestive enzymes are not able to reach the small intestines. This leads to malnutrition and vitamin deficiency as well.

  • Gene mutation

Bacteria can grow in the sticky and thick mucus that builds up in the airways, causing infections that may cause frequent coughing and spitting of bloody mucus. Cystic Fibrosis patients likely bout with pneumonia, as well as inflammation of nasal sinus and mucous membrane.

Cystic Fibrosis Diagnosis

Cystic fibrosis may be diagnosed via blood tests, Immunoreactive Trypsinogen or IRT and sweat chloride, genetic, sputum and organ function testing.

  • IRT is a standard testing for newborns while a high level of IRT leads to possible cystic fibrosis.
  • Sweat chloride test is conducted to find out the salt levels in the sweat by an electric current triggered-chemical that makes skin sweat.
  • Genetic testing is checking DNA in the saliva and blood to find specific gene mutations.
  • Sputum test is mucous sampling to look for germs while organ function testing is done by checking on the liver or pancreas functions during the duration of treatment.
  • Chest X-Ray and CT scan will also determine respiratory blockages and assess CF.

Cystic Fibrosis Treatment

Early treatment of cystic fibrosis improves lifespan and quality of life while other treatments can be as follows:

  • Exercise to cough out mucus
  • Nutritional therapy
  • Medicines like anti-inflammatory drugs, antibiotics, mucus-thinning and bronchodilators
  • Respiratory therapy
  • Chest clapping or percussion (CPT) to loosen mucus from the lungs
  • Breathing techniques to open airways

Main Cystic Fibrosis Drugs

Azithromycin Garamycin Tobi

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