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-Cystic Fibrosis  

Cystic fibrosis is a hereditary, non-contagious disease that affects mainly the lungs and digestive system. In Brazil alone there are an estimated 3,000 children diagnosed as having cystic fibrosis and a further 6 to 7 thousand as yet undiagnosed.

Cystic fibrosis is a chronic illness that causes an increase in the viscosity of the mucus produced by various systems within the body. This increased viscosity causes mucus build-up, which can obstruct various organs, especially the lungs and pancreas.

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When the bronchi are blocked, the patient finds it difficult to breathe and an increase of bacteria within the lungs will cause inflammation and frequent infections, causing progressive pulmonary damage.

When mucous build up blocks the pancreatic folds, the pancreatic enzymes are prevented from reaching the intestine. These enzymes are essential to digestion and nutrient absorption. Cystic fibrosis sufferers frequently need to replace these enzymes in order to achieve proper nutrition.

The reproductive organs in both sexes can also present problems. Women may experience difficulty in becoming pregnant while most male sufferers are sterile, despite having otherwise normal sexual functions.

The main symptoms of CF


CF sufferers may present some of the following symptoms:

  • Salty-tasting sweat;
  • A persistent cough;
  • Frequent lung infections;
  • Respiratory difficulties;
  • Reduced growth and weight-gain, despite having a good appetite;
  • Fatty feces, in large volumes, or difficulty with bowel movements.

Diagnosis

The most common methods are:

  • Foot testing: a post-natal triage exam that is an important indicator for continued diagnostic examination, despite occasionally yielding false positive results.
  • Sweat testing: the most common diagnostic test for cystic fibrosis. CF sufferers present higher chlorine levels.
  • Genetic testing: the genetic test is used when sweat testing proves inconclusive.

Treatment

The treatment of cystic fibrosis has improved a lot since the disease was first discovered in the 1930s. Today, a CF patient has a far longer life–expectancy and much better quality of life.

The best treatment for cystic fibrosis will vary from patient to patient. Cystic Fibrosis Treatment Centers generally tailor their treatment to suit individual cases.

As CF affects the lungs in the majority of patients, an important aspect of treatment concerns eliminating mucous build-up in the lungs by draining and unblocking the airways.

There are various medications designed to treat the pulmonary complications caused by CF and these help the patient to breathe more easily. Chief amongst these are:

  • Mucolithics;
  • Antibiotics, especially inhalants;
  • Anti-inflammatory drugs;
  • Bronchodilators.

Another important aspect is nutrition. CF sufferers need a large calorie intake in order to achieve adequate physical growth and reduce the chance of pulmonary infection. A good diet, rich in calories and unrestricted in terms of fatty foods, is fundamental for these patients. Most CF sufferers need pancreatic enzyme replacement and vitamins in order to successfully achieve this aim.

United Medical and Cystic Fibrosis Treatment

United Medical provides doctors with innovative products for the treatment of cystic fibrosis, developed at the world’s most advanced research laboratories. The high costs of this development would normally mean that it could take years for these drugs to come available on the Brazilian market. But we know that sufferers of chronic illnesses have a whole life ahead of them and cannot afford to wait. That’s why we unite behind a common purpose: to equip health professionals who save and prolong lives with the most advanced therapies science has to offer.

 

Fibrose Cística News - www.fibrosecisticanews.com



Links


Associação Brasileira de Assistência à Mucoviscidose  - www.abram.org.br
Drauzio Varella – Fibrose Cística - drauziovarella.ig.com.br/arquivo/arquivo.asp?doe_id=113
Fibrose Cística - www.ufv.br/dbg/trab2002/DHG/DHG017.htm
Cystic Fibrosis Fundation: www.cff.org




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