Treatments for cystic fibrosis (CF) are constantly evolving, making a huge difference for those who live with the disease. According to the Cystic Fibrosis Trust, these are five of the most important discoveries for cystic fibrosis patients:
Creon has come a long way in recent years; the important enzyme is necessary to help those with CF absorb nutrients from their food but it hasn’t always been available in the capsule form it is today. Many older people with CF will remember having to sprinkle Creon powder directly onto their food, which both tasted and smelled terrible. The first Creon capsules led to many people experiencing painful burning as it went down the esophagus. Over the years, it’s been refined so that fewer capsules are needed and an improved coating ensures that it stays intact until it reaches the stomach.
It was first discovered that the drug tobramycin was able to treat pseudomonas during a 1976 study at the Royal Hospital for Sick Children in Edinburgh, Scotland. By the 1980s, the drug was being administered in IV form and then via nebulizers. Over the years, scientists have come up with formulas that reduce the number of times the drug needs to be taken, thus reducing the risk of some of the side effects associated with the treatment such as loss of hearing.
Originally developed to treat problems such as ear infections or UTIs, clinical trials in 1986 found that Ciprofloxacin could also help manage some cystic fibrosis symptoms, in particular, pseudomonas and staphylococcal infections. When used in conjunction with Colomycin, it was 80 percent effective in delaying or reducing pseudomonas infection.
Airway Clearance Techniques
In the 1950s, doctors advised postural drainage to help CF patients clear excess mucus from their lungs. This was quickly followed by the “percussion technique” which is still in use today. However, newer devices such as pep masks, flutter, and acapella overtook the need for postural drainage which researchers discovered caused more problems than it solved, with many patients experiencing gastrointestinal reflux. Today, people living with CF can use portable vests which do their airway clearance while they get on with other things.
Kalydeco is the only treatment available that works on the cause of the disease rather than managing the symptoms. It works by gating some of the gene mutations that occur in around 5 percent of CF patients.
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