My Name is Dan Pfaff,
I was born with Cystic Fibrosis– Cystic Fibrosis (also known as CF or mucoviscidosis) is an autosomal recessive genetic disorder that affects most critically the lungs, and the pancreas, liver, and intestine. It is characterized by abnormal transport of chloride and sodium across an epithelium, leading to thick, viscous secretions.
CF is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR). This protein is required to regulate the components of sweat, digestive fluids, and mucus. CFTR regulates the movement of chloride and sodium ions across epithelial membranes, such as the alveolar epithelia located in the lungs. Although most people without CF have two working copies of the CFTR gene, only one is needed to prevent cystic fibrosis due to the disorder’s recessive nature. CF develops when neither gene works normally (as a result of mutation) and therefore has autosomal recessive inheritance.
Cystic Fibrosis is a life-shortening disease. But thanks to research that has produced new knowledge about CF and more effective treatments, life expectancy is increasing. In 1938, when CF was first recognized as a disease, most babies with CF died before their first birthday. By the 1950’s people with CF were living a bit longer but rarely made it past early childhood. Things started to improve more in the 1980’s when people with CF could expect to live into their mid to late twenties. Since the discovery of the CFTR gene in 1989, life expectancy has continued to increase. The average life expectancy for people living with CF today is 37 year, but some people live much longer, that number is continuing to rise. because of the advances in treatment and technology, some researchers predict that babies born in the 2000’s will live into their fifties.
My life has had a large impact from having CF resulting in two double lung transplants. To this day I am grateful to have received, I have never let either issue interfere with my life. I enjoy every day as if any other person would, I have a great sense of humor and tend to pass it along wherever I go.
The purpose of this Blog is to share my life experiences so people can become more aware of CF and transplants.