Cystic Fibrosis (CF) is a genetic disease that results in damage to organs such as the pancreas and lungs. These injuries, caused by a defective gene, produce a thick mucus buildup in the pancreas, lungs, and breathing passages. This thick mucus results in dangerous lung infections, lung damage, digestive problems, and nutritional deficiencies. Due to the thick mucus in the lungs, bacteria growth is rampant and severe infections eventually cause damage to the lungs, often necessitating an eventual lung transplant. Mucous plugs up the pancreas, inhibiting its ability to produce and secrete enzymes necessary to digest food. The thickness of the mucous also makes normal lubrication of the digestive tract, sinuses, and sex organs impossible.

Cystic Fibrosis affects different people in different ways and to varying degrees. Common symptoms of CF include:

• Frequent coughing

• Frequent sinus and lung infections

• Wheezing

• Foul-smelling greasy stool

• Poor weight-gain and growth

• Malnutrition • Pancreatitis

• Gall stones

• Nasal polyps

• Asthma

• Clubbing (enlarged fingertips)

Most patients with CF are diagnosed at an early age. All states now require routine screening for CF in newborns. CF occurs most frequently in people of northern European ancestry, occurring in about 1 out of 3,000 births. In the past, people with CF rarely lived past their teenage years. Today, with improved screening, lung transplants, early and advanced treatments, and better awareness, patients may live into their 50s.

There are many different treatments for the many different symptoms and problems associated with CF:

Antibiotics are used in CF patients to battle and prevent the frequent lung infections that accompany the disease. They can be given as injections, oral preparations (tablets and capsules), or inhalations. CF patients often require higher than usual doses of antibiotics.

Mucus-thinning drugs (mucolytics) help thin the mucus in the lungs. This helps improve lung function and decreases the likelihood of infection. Dornase alpha, an inhaled mucolytic, has been shown to reduce pulmonary exacerbations and improve lung function and is currently the only mucolytic agent with proven efficacy in CF.

Bronchodilators, usually administered by inhalers or nebulizer machines, help open the airways and improve breathing. Albuterol and ipratropium are examples of these drugs.

Oral digestive enzymes are given around meals and snacks to help digest proteins and fats since the pancreas is not working properly. Also, fat-soluble vitamins (A,D,E, and K) are supplemented daily.

Yearly flu and pneumococcal vaccines are a must since CF patients are very vulnerable.

When lung function becomes severe, a lung transplant may be necessary.

The prognosis for Cystic Fibrosis has improved dramatically over the past three decades. Before then, a diagnosis of CF meant certain childhood death, but now the average lifespan is 37 years. Most children with CF are able to participate in normal activities and attend school. The lung damage eventually worsens to the point of disablement requiring a lung transplant if available.

1. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001167/

2. http://www.aboutcysticfibrosis.com/cystic-fibrosis-prognosis.htm

3. http://cystic-fibrosis-symptom.com/treatments.htm

4. http://www.medicinenet.com/cystic_fibrosis/page7.htm#outlook

5. http://www.mayoclinic.com/health/cystic-fibrosis/DS00287/TAB=indepth