Cystic fibrosis (CF) is a genetic disease that causes thick and sticky mucus to build up in your organs, particularly your pancreas and lungs. It takes the regular mucus that lines your lungs and nose and makes it thick instead of slippery and watery as it should be. This results in the mucus blocking ducts inside your pancreas and causing digestive problems.
CF is a chronic condition that gets worse over time. It can also impact your sinus, intestines, liver, and sex organs.
Another form of the disease, called atypical cystic fibrosis, is milder than CF and generally only impacts one organ. Atypical CF usually starts later in life whereas typical CF is usually detected within the first few years of a child’s life.
Cause of Cystic Fibrosis
Cystic fibrosis is a genetic condition. You must inherit the gene from both parents to get cystic fibrosis. It is considered a recessive condition, so your parents can carry the gene without having cystic fibrosis. Your family doesn’t need to have a history of cystic fibrosis for you to inherit it.
Symptoms of Cystic Fibrosis
The symptoms of cystic fibrosis depend on whether you have classic or atypical cystic fibrosis.
Classic Cystic Fibrosis Symptoms
A nagging cough
Frequent lung infections, such as pneumonia or bronchitis
Reoccuring sinus infections
Failure to thrive, such as minimal weight gain
These are all symptoms that occur in children with cystic fibrosis.
Atypical Cystic Fibrosis
Atypical cystic fibrosis tends to emerge in adulthood. The symptoms of atypical cystic fibrosis include:
Chronic Obstructive Pulmonary Disease (COPD)
Unintended weight loss
Diagnosing Cystic Fibrosis
Cystic fibrosis is typically diagnosed during childhood. However, atypical cystic fibrosis is diagnosed during adulthood. Common diagnostic tests include:
Lung function tests
Nasal Potential Difference (NPD)
Intestinal Current Measurement (ICM)
Treating Cystic Fibrosis
Cystic fibrosis is a complicated condition that requires you to work with a healthcare team to keep your airways clear. There are three different levels of treatment with cystic fibrosis:
Keeping the airways clear
Medicating for cystic fibrosis
Surgery for cystic fibrosis
Keeping the Airways Clear
With cystic fibrosis, keeping your airways clear is a top priority. This can be achieved through different methods, such as learning specific breathing and coughing techniques, using devices to loosen mucus, and chest physical therapy.
Medication for Cystic Fibrosis
Medication can help with certain conditions and situations that arise with cystic fibrosis. Each medication is used for a different purpose:
Antibiotics are used for treating and preventing lung infections
Inhaled medicine is used to thin mucus, helping to expel it more easily
Stool softeners assist with constipation
Pancreatic enzymes aid in the digestion process
Inhaled bronchodilators assist with relaxing the airway and make breathing easier
Anti-inflammatory drugs prevent inflammation
Surgery for Cystic Fibrosis
Sometimes, surgery is required both for cystic fibrosis and for the complications of cystic fibrosis.
Bowel surgery for blockages
Nose and sinus surgery to help with breathing
Transplant surgery – either liver transplant or a double lung transplant
Treatment for Cystic Fibrosis in Portland, Oregon
Cystic fibrosis requires a comprehensive treatment team. Our team at West Hills Allergy & Asthma Associates can help diagnose and treat your cystic fibrosis.
Strictly Necessary Cookies
Strictly Necessary Cookie should be enabled at all times so that we can save your preferences for cookie settings.
If you disable this cookie, we will not be able to save your preferences. This means that every time you visit this website you will need to enable or disable cookies again.