Understanding Cystic Fibrosis
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Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system, and other organs in the body.
Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in people with CF, a defective gene causes the secretions to become sticky and thick. Instead of acting as lubricants, the secretions plug up tubes, ducts, and passageways, especially in the lungs and pancreas.
Although cystic fibrosis is progressive and requires daily care, people with CF are usually able to attend school and work. They often have a better quality of life than people with CF had in previous decades. Improvements in screening and treatments mean that people with CF now may live into their mid-to late 30s or 40s, and some are living into their 50s.
Symptoms
In the U.S., because of newborn screening, cystic fibrosis can be diagnosed within the first month of life, before symptoms develop. But people born before newborn screening became available may not be diagnosed until the signs and symptoms of CF show up.
Cystic fibrosis signs and symptoms vary, depending on the severity of the disease. Even in the same person, symptoms may worsen or improve as time passes. Some people may not experience symptoms until their teenage years or adulthood. People who are not diagnosed until adulthood usually have milder disease and are more likely to have atypical symptoms, such as recurring bouts of an inflamed pancreas (pancreatitis), infertility, and recurring pneumonia.
People with cystic fibrosis have a higher than normal level of salt in their sweat. Parents often can taste the salt when they kiss their children. Most of the other signs and symptoms of CF affect the respiratory system and digestive system.
Respiratory signs and symptoms
The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause signs and symptoms such as:
A persistent cough that produces thick mucus (sputum)
Wheezing
Exercise intolerance
Repeated lung infections
Inflamed nasal passages or a stuffy nose
Recurrent sinusitis
Treatments & Therapies
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Although every treatment plan is unique, comprehensive CF treatment plans usually include regular visits to your physician and the following components: airway clearance techniques; oral, inhaled, and nebulized medications; nutrition therapies; and a fitness plan.
If you or your child has symptoms of Cystic Fibrosis - or if someone in your family has CF - contact the team at CCMG Pulmonary & Sleep Medicine. 307-688-3615.
Article Sources: Mayo Clinic- Cystic Fibrosis
