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How does cystic fibrosis affect ventilation?

How does cystic fibrosis affect ventilation?

How does CF affect the respiratory system? The abnormal electrolyte transport system in CF causes the cells in the respiratory system, especially the lungs, to absorb too much sodium and water. This causes the normal thin secretions in the lungs to become very thick and hard to move.

What is the longest someone with cystic fibrosis has lived?

Thanks to advances in DNA testing, doctors are identifying more and more people with CF for the first time well into their 50s, 60s, and 70s. The oldest person diagnosed with CF for the first time in the U.S. was 82, in Ireland was 76, and in the United Kingdom was 79.

What causes death in cystic fibrosis patients?

Mucus in CF patients is very thick and accumulates in the intestines and lungs. The result is malnutrition, poor growth, frequent respiratory infections, breathing difficulties, and eventually permanent lung damage. Lung disease is the usual cause of death in most patients.

Does cystic fibrosis make it hard to breathe?

Cystic fibrosis (CF) is an inherited disease that causes thickened mucus to form in the lungs, pancreas and other organs. In the lungs, this mucus blocks the airways, creating lung damage and making it hard to breathe.

What are the outcomes of intensive care for cystic fibrosis?

Cystic fibrosis (CF) causes progressive respiratory failure and death in more than 90% of patients. Mechanical ventilation has been discouraged in CF because of poor outcomes, but improved survival and the availability of lung transplantation have increased the indications for care of CF patients in the intensive care unit (ICU).

Why do people with cystic fibrosis need ventilatory support?

Ventilatory support is appropriate for some transplant candidates. Cystic fibrosis (CF) causes abnormal ion transport in airway epithelial cells and alters the clearance of airway secretions ( 1 ). Subsequent chronic infection leads to inflammation, mucus hypersecretion, and bronchoconstriction that obstruct airways and cause bronchiectasis.

Why is mechanical ventilation discouraged in CF patients?

Mechanical ventilation has been discouraged in CF because of poor outcomes, but improved survival and the availability of lung transplantation have increased the indications for care of CF patients in the intensive care unit (ICU).

How old was patient with CF when admitted to ICU?

The case history of a patient with CF admitted to an ICU is presented and the appropriateness of intensive care management for patients with CF is discussed. Issues relevant to the ICU care of patients with CF are highlighted. A 26 year old man was admitted to the intensive care unit (ICU) on two occasions.