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Information About Cystic Fibrosis

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  Cystic Fibrosis
Cystic Fibrosis is a genetic disorder that mainly affects the lungs and digestive system. Cystic Fibrosis makes a child more vulnerable to frequent lung infections. Medical advances in drug therapy and genetics have made it so that children born with Cystic Fibrosis can look forward to longer and more comfortable lives. The inherited Cystic Fibrosis gene causes the body's Epithelial Cells to produce a defective form of a protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) found in cells that line the lungs, digestive tract, sweat glands, and genitourinary system. When the CFTR Protein is defective, Epithelial Cells can't regulate the way chloride passes across cell membranes. Defective CFTR Protein disrupts the essential balance of salt and water that is needed to maintain a normal thin coating of fluid and mucus inside the lungs, pancreas, and passageways in other organs. As a result the mucus becomes thick, sticky, and hard to move.

In a healthy person, there is a constant flow of mucus over the surfaces of the air passages in the lungs. This removes debris and bacteria. However in a person with Cystic Fibrosis, this mucus is excessively sticky, as mentioned above, and cannot perform this role properly. In fact, the sticky mucus provides an ideal environment for bacterial growth. People with Cystic Fibrosis are at risk of Bacterial Chest Infections. About half of people with Cystic Fibrosis have Repeated Chest Infections and Repeated Pneumonia. If patients are not treated early and properly, the infections are very difficult to treat. Cystic Fibrosis symptoms include the following:

  • Persistent Coughing
  • Excess Sputum (Excess Saliva and Excess Mucus)
  • wheezing
  • shortness of breath with ordinary activities
  • oily bowel movements
  • constipation
  • extreme stomach pain
  • small growths in the nose (nose polyps)
  • an enlarged liver and spleen
  • diabetes
  • infertility in men and women

The Cystic Fibrosis Gene is found on chromosome number 7 in the human DNA. A child must inherit the Cystic Fibrosis Gene from both parents in order for the child to show symptoms of Cystic Fibrosis. If a child inherits only one Cystic Fibrosis Gene then the child will become a Cystic Fibrosis Carrier, but will not experience any Cystic Fibrosis symptoms. Consider the following Cystic Fibrosis t reatments:

  • daily chest physiotherapy (vigorous chest massages)
  • chest infection treated quickly with antibiotics
  • measles, mumps and rubella shots
  • diptheria, tetanus and whooping cough shots
  • vaccinations against flu and pneumococcus
  • daily oral or inhaled antibiotics to counter lung infection
  • inhaled anti-asthma therapy
  • corticosteroid tablets
  • dietary vitamin supplements, especially A and D
  • inhalation of a medication called pulmozyme to make the sputum less sticky
  • medicines to relieve constipation or to improve the activity of the enzyme supplements
  • insulin for Cystic Fibrosis Diabetes
  • medication for Cystic Fibrosis Liver Disease
  • oxygen to help with breathing
  • in severe cases, a lung or heart and lung transplant operation
  • help to overcome fertility problems
  • counseling to help cope with the psychological aspects


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