What is cystic fibrosis?
Cystic Fibrosis is a genetic disorder that causes the body to produce an excess of thick mucus with a consistency similar to that of peanut butter. This mucus is produced in the glands and it clogs internal organs such as the lungs, pancreas and liver.
Cystic Fibrosis (CF) is a genetic disorder that causes the body to produce an excess of thick mucus with a consistency similar to that of peanut butter. This mucus is produced in the glands and it clogs internal organs such as the lungs, pancreas and liver.
How does cystic fibrosis affect someone’s body?
The thick mucus produced by Cystic Fibrosis can infect organs and cause the body to have difficulty absorbing nutrients. It can also lead to breathing problems, blockages (e.g., bowel blockages), pneumonia and other conditions.
When the body’s natural mucus that moves through the respiratory and digestive systems is too thick, it can cause breathing problems, intestinal blockages, and other health issues. The condition is caused by a genetic mutation that makes it difficult for lungs to clear out thick mucus. This excess mucus can clog airways and limit airflow in the lungs, leading to infections like pneumonia.
Thick mucus can also lead to breathing problems like coughing or chest pain that might feel like an asthma attack. Many symptoms of cystic fibrosis are similar to other childhood respiratory illnesses, so diagnosis may involve a variety of tests and procedures. Blood tests, sweat tests (to look for high salt concentrations), chest x-rays and imaging scans are common diagnostic tools used by doctors to identify Cystic Fibrosis.
Is there a cure for cystic fibrosis?
There is no cure currently available for cystic fibrosis, but treatment can help patients live longer with better quality of life by reducing symptoms and lessening complications. Treatments include medication such as antibiotics; oxygen therapy; physical therapy; enzyme replacement therapies (e.g., enzymes that help break down food); dietary supplements (e.g., vitamins A, D and E); and lung transplantation surgery in more severe cases.
The first treatment for Cystic Fibrosis was developed about 50 years ago when physicians learned how to clear patients’ airways of mucus using chest physical therapy.
Medical researchers have made tremendous progress in developing treatments for cystic fibrosis (CF) over the past few decades.
The first treatment for CF was developed about 50 years ago, when physicians learned how to clear patients’ airways of mucus using chest physical therapy. In the 1960s and 70s, scientists discovered that a deficiency of a protein called anion transporter was causing thick mucus buildup in patients’ airways. Since then, doctors and researchers across the world have been working to develop medications that could correct this protein abnormality and treat CF more effectively.
Advances in research have led to improved treatments, longer life expectancies, and even better understanding of CF pathology itself. To date, there are over 1,800 ongoing clinical trials dedicated to finding a cure for CF! With so many trials available today, it can be overwhelming trying to decide which trial is best for you or your child.
What are current treatments like?
Today’s treatments are focused on helping patients breathe more easily, preventing infections, boosting nutrient absorption and limiting lung damage caused by CF. For example, one treatment is Kalydeco, an FDA-approved drug given orally that targets the basic defect in cystic fibrosis at the cellular level and helps thicken or thin secretions depending on where they are located in the body. Another example is Cayston, an FDA-approved inhaled antibiotic used in patients who are infected with Pseudomonas aeruginosa as part of their routine maintenance regimen.
There are a number of treatments currently available for CF, but there is no cure. Today’s treatments are focused on helping patients breathe more easily, preventing infections, boosting nutrient absorption and limiting lung damage caused by CF. For example, one treatment is Kalydeco, an FDA-approved drug given orally that targets the basic defect in cystic fibrosis at the cellular level and helps thicken or thin secretions depending on where they are located in the body. Another example is Cayston, an FDA-approved inhaled antibiotic used in patients who are infected with Pseudomonas aeruginosa as part of their routine maintenance regimen.
Today’s treatments are focused on helping patients breathe more easily, preventing infections, boosting nutrient absorption and limiting lung damage caused by CF. For example, one treatment is Kalydeco, an FDA-approved drug given orally that targets the basic defect in cystic fibrosis at the cellular level and helps thicken or thin secretions depending on where they are located in the body. Another example is Cayston, an FDA-approved inhaled antibiotic used in patients who are infected with Pseudomonas aeruginosa as part of their routine maintenance regimen.