A Closer Look at Cystic Fibrosis

Did you know that May is Cystic Fibrosis Awareness Month? Impacting over 30,000 American adults and children, cystic fibrosis is a progressive chronic disease that, without regular monitoring and treatment, can cause severe, life-threatening damage to your lungs and other organs.

Although cystic fibrosis is a serious condition, with medical advancements and improved care, life expectancies have more than doubled. With the right treatment plan in place, you can live a full, active life and thrive with cystic fibrosis.

In honor of Cystic Fibrosis Awareness Month, chronic disease specialist Thomas Nguyen, MD, MS of Nguyen Medical Group in Boynton Beach, Florida, takes a closer look at cystic fibrosis.

What is cystic fibrosis?

Cystic fibrosis is a lifelong, chronic disorder. Cystic fibrosis impairs your body’s ability to produce sweat, digestive enzymes, and mucus, which causes these secreted fluids to thicken and become sticky, causing blockages and clogs in the various ducts and tubes in your body.

These blockages have the potential to cause significant damage to your respiratory system, digestive system, liver, and pancreas. People with untreated cystic fibrosis are at risk of respiratory failure, frequent infections, and malnourishment.

Cystic fibrosis can range in its severity, but it nearly always requires daily care management. When your cystic fibrosis is properly managed, you can expect to be able to do typical life activities like working a full-time job.

What causes cystic fibrosis?

Cystic fibrosis is a genetic disease caused when the CFTR gene, which controls your body’s ability to transmit salt around your cells, is defective. The disease is inherited and present from birth.

To develop cystic fibrosis, you must inherit one copy of the damaged CFTR gene from each of your parents. If you only receive one copy, you will be a carrier with the potential to pass on cystic fibrosis to your children, but won’t have the condition yourself.

Symptoms of cystic fibrosis

In the USA, newborn babies are regularly screened for cystic fibrosis and diagnosed when they’re infants. You can also receive a diagnosis as an older child or adult through a sweat test if you display the following symptoms:

• Chronic respiratory problems and lung infections
• Chronic digestive problems
• Poor appetite and difficulty with weight gain
• A productive, persistent chronic cough
• Chronic stuffy sinuses and nose
• Shortness of breath
• Stools that are greasy and smell foul
• A chronically swollen abdomen

Some people with cystic fibrosis also notice a salty taste to their skin.

Treating cystic fibrosis

As part of our chronic disease management program, Dr. Ngygen and our team develop a treatment plan to ensure your cystic fibrosis is managed daily. This personalized plan ensures your cystic fibrosis is well-managed and allows you the best possible quality of life.

Treating cystic fibrosis often involves a combination of medications, surgical procedures when needed, chest physical therapy, and home care. Home care includes lifestyle modifications like developing a safe exercise program that works with your cystic fibrosis, drinking sufficient fluids, and avoiding irritants that worsen your cystic fibrosis.

As part of your regular management, our team checks in with you regularly to ensure your cystic fibrosis is stable and you understand how to manage your symptoms. Our team is available for appointments via telehealth as well as at our Boynton Beach, Florida office.

With the right interventions, the long-term outlook for living with cystic fibrosis is a good one. For assistance with diagnosis or treatment management of cystic fibrosis, contact us online or by phone to request your appointment.