Cystic Fibrosis

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.

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. 

The symptoms of cystic fibrosis depend on whether you have classic or atypical cystic fibrosis.

• Trouble breathing
• A nagging cough
• Reoccuring wheezing
• Frequent lung infections, such as pneumonia or bronchitis
• Reoccuring sinus infections
• Failure to thrive, such as minimal weight gain
• Slow growth

These are all symptoms that occur in children with cystic fibrosis. 

Atypical cystic fibrosis tends to emerge in adulthood. The symptoms of atypical cystic fibrosis include: 

• Breathing problems:
  • Asthma
  • Chronic Obstructive Pulmonary Disease (COPD)
• Frequent pneumonia
• Nasal polyps
• Diarrhea
• Pancreatitis
• Dehydration
• Unintended weight loss

Cystic fibrosis is typically diagnosed during childhood. However, atypical cystic fibrosis is diagnosed during adulthood. Common diagnostic tests include: 

• Newborn screening
• Sweat test
• Genetic tests
• Chest x-rays
• Sinus x-rays
• Lung function tests
• Sputum culture
• Nasal Potential Difference (NPD)
• Intestinal Current Measurement (ICM)

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

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 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

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

Cystic fibrosis requires a comprehensive treatment team. Our team at West Hills Allergy & Asthma Associates can help diagnose and treat your cystic fibrosis.

For a consultation with Dr. Rene Anderson-Cowell, you can call us at (503) 297-4779 or request an appointment online.