What Is Atypical Cystic Fibrosis?

Atypical cystic fibrosis is a mild form of cystic fibrosis. People with this type of cystic fibrosis can experience symptoms that come and go or that are less severe than those people with classic cystic fibrosis experience.

Atypical cystic fibrosis is a form of cystic fibrosis that causes mild symptoms or symptoms that come and go. People with this form of cystic fibrosis still experience classic cystic fibrosis symptoms, such as wheezing and chronic cough, but symptoms are typically not as severe.

Treatments for atypical cystic fibrosis and classic cystic fibrosis are the same and include options such as chest physical therapy and medications.

Learn more about cystic fibrosis.

What is atypical cystic fibrosis?

Atypical cystic fibrosis is a mild form of cystic fibrosis. People with this form might have symptoms that come and go. It sometimes only affects one lung. Typically, adults or older children receive a diagnosis of this form of cystic fibrosis.

What are the symptoms of atypical cystic fibrosis?

Atypical cystic fibrosis causes many of the same symptoms as classic cystic fibrosis. However, they might be mild or occur less often. These symptoms may include:

• chronic coughing
• trouble breathing
• wheezing
• frequent lung infections
• slow growth
• dehydration
• low appetite
• unintentional weight loss
• chronic sinusitis
• nasal polyps
• pancreatitis
• diarrhea

What causes atypical cystic fibrosis?

Cystic fibrosis is caused by changes to a gene called the CFTR gene. This gene makes a protein that controls chloride ions in the body. The chloride ions move water through cells and affect mucus throughout the body. People with changes to the CFTR gene have mucus that is stickier and thicker than people without these changes. This causes cystic fibrosis.

To have cystic fibrosis, and individual must have two copies of the mutated CFTR gene – one from thier mother and one from thier father. In individuals with typical cystic fibrosis, both copies of the CFTR gene have severe mutations. Individuals with atypcial cystic fibrosis generally have one CFTR gene with the more severe mutation and one CFTR gene with a rarer, milder form of the mutation.

How is atypical cystic fibrosis diagnosed?

Typically, newborns are screened for cystic fibrosis. A healthcare professional takes a few drops of blood from a baby’s heel and tests it in a lab.

However, atypical cystic fibrosis is often diagnosed later. Testing can include:

• Sweat test: People with cystic fibrosis typically have higher chloride levels in their bodies, which a sweat test can measure. However, atypical cystic fibrosis doesn’t always raise chloride levels.
• Pulmonary function tests: These tests measure how well the lungs are working.
• Sputum culture: Doctors use sputum cultures to test mucus you cough up, looking for bacteria that are more common in people with cystic fibrosis.
• Genetic testing: Blood testing helps doctors look for the gene changes that cause cystic fibrosis.
• Pancreatic biopsy. A pancreatic biopsy helps healthcare professionals look for damage to the pancreas.
• Imaging tests: Imaging helps doctors look at the lungs and airways.
• Nasal potential difference (NPD): This test helps doctors look at the ion movement in the lining of your nose. People with cystic fibrosis often have less ion movement.
• Intestinal current measurement (ICM): This test measures how much chloride you secrete in waste material. It involves taking a sample of rectal tissue.

What’s the treatment for atypical cystic fibrosis?

The treatments for atypical cystic fibrosis are similar to those for classic cystic fibrosis. The exact treatment plan can depend on your symptoms and your response to treatment.

Common treatment options include:

• chest physical therapy
• breathing and airway clearance exercises
• a cystic fibrosis diet
• antibiotic therapy
• pancreatic enzyme replacement therapy
• CFTR modulator medications

What’s the outlook for people with atypical cystic fibrosis?

Recent treatment advances have improved the outlook for people with all types of cystic fibrosis. As a rule, people with atypical cystic fibrosis have a longer life span than people with classic cystic fibrosis. Currently, the predicted life expectancy for people with cystic fibrosis born within the past few years is 50 years.

If you have classic cystic fibrosis, your individual outlook can be affected by a variety of factors, including genetics, response to treatment, and overall health.

Frequently asked questions

What is the life expectancy for cystic fibrosis?

The life expectancy for someone born in recent years who has a diagnosis of cystic fibrosis is 50. People with atypical cystic fibrosis generally have a longer life expectancy.

What is the 6-foot rule for cystic fibrosis?

When a person coughs or sneezes, germs can spread about 6 feet. They can then land on nearby surfaces and enter another person’s eyes, nose, and mouth.

People with cystic fibrosis are at a greater risk of serious infection. Cystic fibrosis can make infections harder to treat, and infections can worsen symptoms of cystic fibrosis. If you have cystic fibrosis, stay at least 6 feet away from people with cold and flu symptoms.

Can you have mild cystic fibrosis and not know it?

It’s possible to have mild cystic fibroids without knowing it. Sometimes the symptoms of atypical cystic fibrosis may lead to a healthcare professional diagnosing a different respiratory condition. It can be a good idea for people who experience symptoms such as chronic sinus and lung infections, chronic cough with sticky mucus, and difficulty maintaining a moderate body weight to talk with a healthcare professional.

Takeaway

Atypical cystic fibrosis is a mild form of cystic fibrosis. It causes many of the same symptoms as classic cystic fibrosis, including chronic cough and recurrent infections.

However, people with atypical cystic fibrosis might experience milder versions of these symptoms or symptoms that come and go. Treatment can help manage atypical cystic fibrosis.