Starting Treatment for Cystic Fibrosis: 9 Things to Know

Cystic fibrosis is a complex disease that involves multiple organs and body systems. Because of this, it requires a team approach to therapy. In addition to your doctor, a respiratory therapist, dietitian, physical therapist, nurse, and psychologist may be involved in managing your care.

The sticky mucus in your lungs is the perfect breeding ground for bacteria. Lung infections can worsen your existing lung problems and possibly land you in the hospital. Oral or inhaled antibiotics will likely be part of your daily treatment regimen to avoid infections.

It’s hard to breathe with so much sticky mucus plugging up your lungs. Medications like hypertonic saline and dornase alfa (Pulmozyme) are mucus thinners. As their name suggests, they make your mucus thinner and less sticky, so you can more easily cough it up.

Your doctor might also recommend that you perform airway clearance therapy (ACT) to rid your lungs of mucus. You can do this in one of a few ways:

• huffing — breathing in, holding the breath, and letting it out — and then coughing
• clapping on your chest, or percussion
• wearing a VEST jacket to shake up mucus
• using a flutter device to make mucus vibrate in your lungs

People with cystic fibrosis have mutations to the cystic fibrosis transmembrane conductance regulator (CFTR) gene.

This gene provides the instructions for a protein to make healthy, thin mucus that flows easily through the airways. Mutations in the CFTR gene lead to the production of a defective protein, which results in abnormally sticky mucus.

A new group of drugs called CFTR modulators fix the protein made by some – but not all – CFTR gene mutations. These drugs include:

• ivacaftor (Kalydeco)
• lumacaftor/ivacaftor (Orkambi)
• tezacaftor/ivacaftor (Symdecko)

A gene test can determine which mutation you have and whether you’re a good candidate for one of these medications. Taking one of these drugs could help you maintain, or even improve, your lung function.

The pancreas normally releases enzymes needed to digest food and absorb nutrients from it. In people with cystic fibrosis, thick mucus prevents the pancreas from releasing these enzymes. Most people with the disease need to take enzymes right before they eat to help their body absorb nutrients.

You’ll use a nebulizer for breathing in medications that help keep your airways open. If you don’t clean this device correctly, germs can build up inside it. If those germs find their way into your lungs, you could get an infection.

Each time you use your nebulizer, clean and disinfect it.

You can:

• boil it
• put it in the microwave or dishwasher
• soak it in 70 percent isopropyl alcohol or 3 percent hydrogen peroxide

Your doctor can give you specific instructions on how to clean it.

When you have cystic fibrosis, you definitely don’t want to cut calories. In fact, you’ll need extra calories each day just to maintain your weight. Because you lack pancreatic enzymes, your body can’t get all the energy it needs from the foods you eat.

Plus, your body is burning off extra calories from always coughing and having to ward off infections. As a result, women need 2,500 to 3,000 calories daily, while men need 3,000 to 3,700 calories.

Get the extra calories from high-energy, nutrient-dense foods like peanut butter, eggs, and nutritional shakes. Supplement your three main meals with a variety of snacks throughout the day.

Managing a disease like cystic fibrosis requires a lot of follow-up care. Expect to see your doctor every few weeks right after you’re diagnosed. As your condition gradually becomes more manageable, you may be able to stretch your visits to once every three months and eventually to once a year.

During these visits, expect your doctor to:

• perform a physical exam
• review your medications
• measure your height and weight
• counsel you about nutrition, exercise, and infection control
• ask about your emotional well-being and discuss whether you may need counseling

Despite great advances in medical research, researchers still haven’t discovered a cure for cystic fibrosis. Yet, new treatments can:

• slow down your disease
• help you feel better
• protect your lungs

Sticking to the therapies your doctor prescribes will give you the best medical advantages available to help you enjoy a longer, healthier life.

Embarking on treatment for any disease can feel a little overwhelming. In time, you’ll get into the routine of taking your medications and performing techniques to clear the mucus from your lungs.

Use your doctor and the other members of your treatment team as resources. Whenever you have questions or think you might need to change one of your treatments, talk with them. Never make changes to your regimen without your doctor’s OK.