What is Cystic Fibrosis – Health Hint!

Oct 5, 2022

Federated Health Charities’ mission is to improve the health and quality of life of all Ontarians by supporting 21 different health charities providing critical services to those experiencing, or affected by, illness. We believe education and prevention are key parts of supporting the health of our communities, so our weekly Health Hint series strives to provide tangible and easy to implement hints and tips on how to maintain your health, prevent disease, and enjoy increased quality of life. Check out our latest Health Hint that breaks down what cystic fibrosis is. We hope you find it helpful. If you would like to join our efforts to support the health of Ontario, please consider a donation to Federated Health Charities.

What is Cystic Fibrosis?

Cystic Fibrosis, or CF for short, is a life-threatening, inherited disease that impacts many of the body’s organs, including the lungs and digestive system. More specifically, CF causes changes to something called the electrolyte transport system. This causes the transport system to absorb too high of levels of water and sodium. Additionally, CF impacts the cells that are responsible for production of sweat, mucus and digestive fluids. In people with CF these fluids become too thick and rather than acting as a lubricant, as they should, they end up clogging passageways of the lungs and pancreas.

Causes of Cystic Fibrosis:

Since CF is a genetic disease, that means it is inherited. It is not something once can catch or cause. The disease is the result of a gene mutation (gene is called the cystic fibrosis conductance transmembrane, or CFTR). These mutations lead to changes in the body’s electrolyte transport system, as mentioned above.

For an infant to be born with the mutation they must inherit a gene mutation from both their mother and their father (so two mutated CF genes must be present). This may make it seem like a child inheriting CF would not come as a surprise, since both of their parents already had it, but this is often not the case. Some people can carry the CF gene without knowing about it due to a lack of symptoms. So, it is quite possible that either the mother or father, or both, were unaware that they were carriers of the gene.

Diagnosis of Cystic Fibrosis:

CF is usually diagnosed very early on (usually within the first month of life), during newborn screening. It does not require waiting for symptoms to appear to be diagnosed. Some of the tests that may be done to issue a diagnosis of CF include a sweat test (infants with CF have a higher-than-normal level of sodium chloride), chest x-rays, CT scans, ultrasounds, blood tests, lung function tests, and stool tests.

Prognosis for those with Cystic Fibrosis:

Due to advancements in CF screening, treatments, and care, those living with CF have a longer life expectancy than they once did. The average lifespan of a person living with CF is mid to late 30’s, although some live in to their 40’s or even 50’s.

CF is progressive and required ongoing, daily care, but with the right supports in place those living with CF can participate in daily life such as school, work, and social activities.

Symptoms of Cystic Fibrosis:

Symptoms of CF being in childhood. The symptoms experienced by those living with CF can vary greatly, depending on how severe the disease is. The symptoms and impacts of CF are progressive and worsen as time goes on.

There are two primary areas of symptoms, respiratory and digestive. Respiratory symptoms include a persistent cough, repeated lung infections, wheezing and challenged breathing, inability to exercise, ongoing stuffed nose or clogged nasal passages, and recurring sinus infections. Digestive symptoms include intestinal blockages, chronic or sever constipation, suboptimal weight gain or growth and fragrant, greasy stools.

Complications of Cystic Fibrosis:

CF can lead to serious, life-threatening complications. These complications are progressive and increase with frequency and severity over time. Complications can include cirrhosis or liver disease, vitamin deficiencies, diabetes, lung infections, pancreatitis, stunted growth, gastroesophageal reflux disease, and need for an organ transplant.

Treatment for Cystic Fibrosis:

There is, currently, no cure for CF. Treatment involves management of symptoms to ease suffering and to slow down, or prevent, development of complications. Treatment tends to focus on the two areas of symptoms, lung, and digestive problems. Treatment for lung issues includes physical therapy, exercise to loosen mucus and medications, while treatments for digestive issues includes diet, enzymes, vitamins, etc.

Support for Those Living with Cystic Fibrosis:

If you have CF, you will have a specialized care team focusing on your medical needs, but often those living with CF, or their loved ones, need further support. This is the focus of Cystic Fibrosis Canada, one of the Federated Health Charities member charities.

“Cystic Fibrosis Canada is a leading organization with a central role engaging people living with cystic fibrosis, parents and caregivers, volunteers, researchers and healthcare professionals, government, and donors. We work together to change lives through treatments, research, information, and support.

Cystic Fibrosis Canada is determined to see brighter futures ahead for all Canadians living with cystic fibrosis. We are investing in research to ensure there are promising treatments for all Canadians impacted by cystic fibrosis. As well, we are ensuring that clinical trials for new treatments reach Canada so that no one is left behind in our pursuit of longer and healthier lives for everyone living with cystic fibrosis.”