The month of May is Cystic Fibrosis (CF) Awareness month. It is time again to take notice of those around you who may have this genetic illness and notice the impact of this illness and see the realities of their life. The CF community also comes together to help others learn about the disease, share personal stories, and unite in the mission of finding a cure.
Cystic Fibrosis is a progressive, genetic illness that causes repeated and persistent lung infections. Over time these infections cause permanent lung damage and makes breathing difficult and shortens the usual lifespan.
There are over 1800 different CF causing genetic mutations. A combination of two of these gene mutations cause CF. A malfunctioning protein in the body called the cystic fibrosis transmembrane conductance regulator (CFTR) protein causes the mucus on the cell to become dry, thick, and sticky. This offers poor lubrication to areas that require slippery mucus for protection against germs and improve nutrition.
The mucus congests in the airways and traps germs causing infections, inflammation and complications like respiratory failure. The sinuses retain mucus and become plugged leading to poor ability to breath through the nose. Thickened mucus also occurs in the gut and does not allow for key nutrients to be absorbed through the sticky mucus. Liver disease and infertility happen when thickened mucus causes changes in the normal mucus flow in the body.
Individuals with CF are very familiar with the art of avoiding germs. They know the importance of self-isolation, a clean environment and proper nutrition to find a healthy balance and improve the body’s ability to fight against germs.
They struggle throughout their life with cough and mucus production, breathing problems, infections, poor growth or weight gain and maintaining proper vitamin levels in the blood. There are difficulties in balancing home life, work life and medical treatment needs such as hospitalizations.
The Cystic Fibrosis Foundation is committed to finding new treatments and a cure for this illness. You can track their research and medication development on their website at CFF.org. There is a wave of hope across the CF community as each new medication targeting a way to improve the protein malfunction is approved by the FDA. Some day we hope to see that CF stands for Cure Found.
There are about 30,000 individuals with cystic fibrosis in America and about 70,000 worldwide. The Cystic Fibrosis Foundation (CFF.org) has started a hashtag movement for individuals with CF to tell their story on social media using the hashtag #CFawareness. You may soon see these courageous stories in your social media feed and have a chance to see true champions among us.
(Deb Fueller is a nurse practitioner at CHI St. Alexius Health Heart & Lung Clinic and is the coordinator for the cystic fibrosis adult clinic. CHI St. Alexius Health Heart & Lung Clinic’s Cystic Fibrosis Center is an accredited Cystic Fibrosis Center and follows the care model developed by the Cystic Fibrosis Foundation. The CF care center delivers effective and efficient health care for pediatric and adult individuals with CF. Patients receive collaborative care from an interdisciplinary team of specialists.)