May is Cystic Fibrosis Awareness Month: Make Every Breath Count!

Cystic Fibrosis Awareness Month

A Day in the Life of Someone with Cystic Fibrosis

Cystic fibrosis is a progressive, genetic disease that affects the respiratory, digestive, and reproductive systems, and limits the ability to breathe over time.

In people with CF, mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to become dysfunctional. When the protein is not working correctly, it is unable to help move chloride — a component of salt — to the cell surface. Without the chloride to attract water to the cell surface, the mucus in various organs becomes very thick and sticky.

To be honest I did not really know much about CF until my son’s diagnosis and still did not really understand it right away. At his 2-week appointment the doctor sat down next to us and asked if we were contacted by the North Dakota Department of Health regarding his newborn screen and that he could potentially have Cystic Fibrosis. He would be referring us to Sanford Health’s Cystic Fibrosis Specialists. As new parents that news was devastating. A week later we were at Sanford having a sweat chloride test (this measures the salt in your body). After multiple tests we finally received the definitive diagnosis that he indeed has CF, and we would start our CF journey. Dr. Kunkel was very honest, caring, and extremely empathetic when delivering the news to us. The Coordinated Treatment Center at Sanford has been our lifesaver and our team is top-notch. Our team consists of our CF specialist, infectious disease doc, respiratory therapist, dietician, and social worker.

The type and severity of CF symptoms can differ widely from person to person because of everyone’s specific genotype. Therefore, although treatment plans can contain many of the same elements, they are tailored to everyone’s unique circumstances.

Social distancing quickly became our new norm before, “social distancing” was a thing. This is especially crucial during cold and flu season, pandemics, and when people are not feeling well. A common cold/ respiratory infection to us can be life-threatening to someone with cystic fibrosis.

Did you know people with Cystic Fibrosis are not supposed to be in close contact with each other? If needed they are supposed to be outdoors and at least six feet apart.

A normal day for us starts at 5 am with his vesting and nebulizer treatments which are done twice a day and usually last about 1 hour.

He also takes specific medication for his genotype (R711C) called Kalydeco in the morning and evenings. His most important medication is SALT. Yes, regular table salt.

Silas looks and acts like a healthy one-year-old, but remember not all Cystic Fibrosis patients are the same. We are very fortunate that his type is very rare and seems to be on the path that is not as extreme as some. For example, some are on oxygen, have feeding tubes, and are hospitalized for periods of time.

I remind myself often that we are only given as much as we can handle. And at times we may not understand why, but it could be so much worse.

We are incredibly lucky and fortunate to have such loving friends, family, and coworkers who have supported us from day one.

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