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Cornerstone Health Solutions is dedicated to supporting you through your cystic fibrosis treatment. We are committed to helping you with financial assistance, providing you with thorough medication education, helping keep you on track, and being fully available to you to answer questions.

What is cystic fibrosis?

Cystic fibrosis is a genetic disorder that causes the mucus fluids in your body to become very thick and sticky. This can lead to many different problems and affect several different organ systems in your body. The lungs and the pancreas are the most heavily affected organs and the majority of treatments are focused on managing these issues. Symptoms that you may experience from cystic fibrosis include:

  • Very salty skin
  • Persistent coughing (sometimes coughing up mucus)
  • Frequent lung infections
  • Wheezing or shortness of breath
  • Poor growth/weight gain despite eating normally
  • Frequent greasy stools, difficulty having bowel movements
  • Infertility

What causes cystic fibrosis?

Cystic fibrosis is a genetic disorder that is passed down to children from their parents through genes. The condition is caused by a mutation in the gene that is responsible for making the protein CFTR (cystic fibrosis transmembrane conductance regulator). A child must receive a mutated gene from each parent to develop cystic fibrosis. The most common mutation in this gene is delta F508. However, there are more than 1,700 different mutations to the CFTR gene. Different mutations may cause cystic fibrosis to be more or less severe. 

Normally, your body produces thin, watery mucus, which is used to help protect your organs from infection. However, a mutation in the CFTR gene stops your body from being able to make normal mucus. As a result, the mucus that your body does produce becomes very thick and sticky. This thick, sticky mucus is responsible for the symptoms and complications that you may experience.

Cystic Fibrosis diagram
Cystic fibrosis diagram 

For example, having this thick, sticky mucus in your lungs makes it much harder to breathe and traps bacteria, which causes frequent infections. The abnormal mucus also makes it difficult for your pancreas to release its digestive enzymes. Without these digestive enzymes your body cannot properly break down foods and you will not receive the nutrients your body needs, leading to poor growth and weight gain. Likewise, thick, sticky mucus can affect other organs like your intestines and reproductive organs.

What specialty medications do you offer to treat cystic fibrosis?

  • Kalydeco
  • Orkambi
  • Symdeko
  • Bethkis, Kitabis Pak, TOBI, TOBI Podhaler
  • Pulmozyme
  • Hypertonic Saline
  • Creon, Pancreaze, Pertzye, Zenpep

What should I expect from treatment?

Several different treatment options exist for patients with cystic fibrosis and each regimen is specific to each patient based on which symptoms they experience and how severe their disease is. Some specialty medications used to treat cystic fibrosis are oral tablets taken twice a day. These are disease-modifying therapies meaning they work to correct the abnormal functioning of the mutated CFTR proteins. The most common side effects you may experience from these medications include headache, stomach/intestinal upset, and chest discomfort.

Patient going through treatment

Other specialty medications used to treat cystic fibrosis include different inhaled medications that you will use every day. These inhaled medications work to make the mucus in your lungs thinner and less sticky (Pulmozyme and hypertonic saline) and decrease your risk of getting lung infections (Bethkis, Katabis, TOBI). Pulmozyme and hypertonic saline only make the mucus in your lungs less thick and sticky for a short amount of time so after you do your treatments you will need to do an airway clearing technique (ie. vibrating vest) to loosen as much mucus as you can. Some common side effects that you may experience from these inhaled medications include increased cough, sore throat, chest tightness, dizziness, and runny nose.  

Other commonly used medications to treat cystic fibrosis include pancreatic enzymes. As discussed above, your pancreas cannot release its digestive enzymes due to the thick, sticky mucus. Your provider may prescribe pancreatic enzymes that you can take in pill form to replace what your body cannot produce. This will better allow you to digest your food and receive the nutrients that your body needs. These medications will need to be taken before every meal. The most common side effects that you may experience from these medications include headache and abdominal pain.

What should I expect from my condition?   

Complications from recurrent lung infections and poor nutrition often require patients to be admitted to the hospital. Although breakthrough medications work to prevent these complications, they are still a reality for patients living with cystic fibrosis. It is extremely important to follow your treatment regimen exactly as your doctor prescribes in order to reduce your risk of complications and hospitalization.

Unfortunately, there is currently no cure for cystic fibrosis. However, the newest research and treatments have extended the life expectancies of patients suffering from cystic fibrosis by decades. Researchers are still working tirelessly to find new treatments to improve quality of life and hopefully find a cure.

To learn more about cystic fibrosis visit the following resources:

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