Cystic fibrosis is a severe and feared condition affecting how your body produces and manages mucus and other fluids. In a healthy person, mucus is thin and slippery and helps your body perform many crucial functions. However, the mucus becomes thick and sticky in someone with cystic fibrosis. As a result, mucus often gets stuck in places where it shouldn’t, including the lungs, pancreas, and other vital organs.
In addition to mucus, cystic fibrosis also affects the production of sweat and digestive juices. On top of making your mucus sticky, CF causes excess production of sweat and thickens your digestive juices. This often leads to lung and pancreas infections and blocked airways.
Cystic fibrosis is both chronic and terminal. There’s no cure for this condition, and it will usually claim the life of the affected person by the time they’re in their thirties. CF is a genetic condition that people are born with when both of their parents are carriers of the CF gene. The only way to prevent CF is for people who are carriers of the gene not to have children with others who are also carriers.
Because cystic fibrosis is a serious condition, it is important to get it diagnosed as early as possible. In most cases, doctors will screen for CF as soon as a child is born. However, when screening isn’t performed but you suspect you or someone you love has cystic fibrosis, here are signs and symptoms to watch out for.
Symptoms will vary according to how severe your condition is. If you suspect you have undiagnosed cystic fibrosis, it’s imperative to seek a diagnosis and treatment immediately.
Cystic fibrosis is a genetic condition caused by a defect or mutation in the CFTR gene. This gene is responsible for regulating the flow of salt throughout the cells in your body. The inability to control salt flow leads to sticky mucus, excessively salty sweat, and sticky digestive juices.
Since CF is a genetic condition you have from birth, it isn’t something you can develop later in life. However, depending on the severity of your condition, you may not display symptoms until later in life.
The key to living a functional life with cystic fibrosis is diagnosing your condition as early as possible. Depending on where you were born, your doctor screened for CF immediately when you were born. Whether your doctor did early screening or tests for CF later in life, here’s how they form a diagnosis.
This will measure the amount of immunoreactive trypsinogen in your blood. High levels indicate possible cystic fibrosis.
This will determine if you have abnormally high amounts of salt in your sweat, which indicates CF.
This will determine whether or not you and your parents have the CFTR gene.
Unfortunately, cystic fibrosis is a terminal condition for which there is no cure. However, with early diagnosis, medical treatment, and careful management, people with CF can live fairly normal lives, including going to school and having a job. Here’s what treatment and management typically consist of.
Doctors will prescribe various antibiotics, steroids, anti-inflammatories, and other medications to manage the symptoms of CF.
The most significant risk and leading causes of death for patients with CF are lung infections and airway blockages. Therefore, doctors often use oscillating devices and chest therapy to keep airways clear of mucus and digestive juices.
Certain breathing exercises and manipulations will help you maintain clear airways. A physical therapist can also teach you special coughing techniques to eliminate gunk and mucus.