Cystic Fibrosis is a genetic disease that is chronic and life-threatening. A large portion of patients suffering from Cystic Fibrosis are children and young adults. The condition affects the lungs, gastrointestinal system, sweat glands and male reproductive system.
Who is at Risk?
Cystic Fibrosis can affect anyone from any ethnic background, but the disease is more common in Caucasians. It is estimated that one out of every 29 Caucasians carry the defective gene that causes the disease. Carriers do not typically show symptoms of Cystic Fibrosis, but if both parents carry the defective gene, chances of children having an active form of the disease increase dramatically. The symptoms of Cystic Fibrosis appear early in life with most children being diagnosed by two years of age.
Symptoms of Cystic Fibrosis occur early in infanthood and may include:
- Slower than average growth
- Slow weight gain or no weight gain
- Salty skin
- Abdominal pain associated with constipation
- Distended abdomen
- Lack of appetite or refusal to eat
- Weight loss
- Difficulty breathing
As the disease progresses, patients often suffer from complications including male infertility, coughing up blood, shortness of breath similar to asthma and death.
Testing for Cystic Fibrosis is part of the IRT (immunoreactive trypsinogen) test performed just after childbirth. If the test shows higher than normal levels of IRT, there is an increased chance the infant has Cystic Fibrosis.
Patients may also be diagnosed after a CT scan, fecal testing, lung testing or upper/lower GI testing.
If Cystic Fibrosis is diagnosed early, treatment increases the chance of survival. Antibiotics are often given to treat lung infections and inhalants are used to increase lung function. Medications may also be administered to reduce mucus in the lungs, making breathing easier.
Patients may be placed on a diet to increase protein and calorie consumption. Due to absorption problems associated with pancreatitis, patients may be given enzymes to increase absorption of proteins and fats. Vitamin supplements are a crucial part of the treatment process.
With proper treatment and medical attention, the lifespan of a person with Cystic Fibrosis is about 37 years. If lung complications are kept to a minimum, patients may live longer. Typically, early childhood and teen years are riddled with fewer symptoms than adulthood. As the disease progresses, lung function is reduced leading to complications and death.