Cystic Fibrosis Signs and Symptoms
What You Should Know About Cystic Fibrosis
Cystic fibrosis, referred to as CF, is a genetic disease affecting approximately 30,000 people in the United States. The disease causes a buildup of thick mucus in the pancreas, lungs, and other organs. Persons with cystic fibrosis are prone to potentially life-threatening lung infections, difficulty breathing, and malnutrition.
Individuals with CF have received a copy of the CF gene from both parents. Each parent has to have a copy of the CF gene for the disease to be passed on to the child. Any time two people with one copy of the CF gene have a child, there is a 25 percent chance the child will have CF and a 50 percent chance they will be a carrier themselves.
Symptoms of Cystic Fibrosis
The most common symptoms of CF include the following:
- Frequent lung infections such as bronchitis and pneumonia
- A persistent, phlegmy cough
- Skin that tastes salty
- Frequent shortness of breath and wheezing
- Greasy, bulky, and very foul smelling stool
- Poor weight gain and growth even with a good appetite
Treatment and Management of Cystic Fibrosis
The severity and specific symptoms of CF can vary drastically from person to person. Individuals with the disease should work closely with specialists to create a treatment plan to fit their needs. In most cases, people with CF will use some or all of the following therapies:
- Airway Clearance: CF patients must loosen the thick mucus that can block the airway or lead to infections. A family member may use a technique called postural drainage and percussion where the caregiver claps in various positions on the patient's chest. Specialized vests are also available to help with airway clearance. The vests vibrate at a high rate which helps loosen the mucus.
- Medication: CF patients frequently use medicine to thin mucus and open the airway. The medications are typically inhaled through a nebulizer and often contain antibiotics to ward off infections.
- Dietary Supplements: The mucus prevents the pancreas from releasing enzymes necessary for absorbing nutrients. As a result, CF patients often have slowed growth and poor weight gain. Individuals with CF usually take multivitamins or other supplements to ensure they receive vital nutrients.
These modern treatments have added decades to the lives of CF patients. In the 1950s, children with CF usually died before they reached school age. Today, the average life expectancy of a CF patient is 41 years.