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The Household Item That Could Have Cystic Fibrosis Patients Breathing Easy

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Stress is a common part of everyday life and at times many will find themselves succumbing to the emotional consequences of the flood of hormones released during the "fight or flight" response. Inevitably, when someone is seen exhibiting these symptoms, another person will offer a very simple yet effective piece of advice: "Just Breathe."

The suggestion is scientifically sound and offers a momentary relief from the scourge of external stimuli by focusing on the one natural function that many of us take for granted.

For people living with cystic fibrosis however, breathing itself can be a significant cause of stress.

Take Erin McCaw for example. The Ottawa copy editor was first diagnosed with CF at nine years of age and has been continually struggling with the disease ever since, all in the name of breath. "When I was young I had no awareness of what cystic fibrosis was; all I knew was that it was really hard to breathe sometimes. Then my life quickly changed to revolve around a regimen of enzymes, antibiotics, physiotherapy, nutrition and exercise that was designed to help me live as long as possible."

Today, at 42, she has more than doubled her initially expected lifespan of less than 20 years, although each day brings new challenges. "Some days those breaths come more easily than others. Other days the coughing that accompanies each breath leaves me completely exhausted."

The disease was first described in 1938, when the focus of illness was not on the lungs but on the gastrointestinal organs of children suffering from Celiac disease. Children commonly had cysts made of fibrous tissue in their pancreases leading to the name, cystic fibrosis. While the lungs were given some focus, they didn't play a prominent role in the understanding of the disease until 1949 when it became evident that children were dying of bacterial lung infections.

Research continued to find a cause and cure but most efforts were unfruitful. Then, in 1989, a group led by Canadian researcher Lap-Chee Tsui finally determined the cause of CF to be a mutation in the gene known as CFTR, which has a number of functions in the body including helping fight off infection.

The discovery set off a race to find meaningful treatment options that could help patients prevent infection and increase their ability to breathe. McCaw has been witness to these advancements and their benefit to CF patients. "I've seen better bronchodilators, mucolytics, and antibiotics become available since the CFTR discovery, and I've also seen the lifespan increase, not only for me but many others as well."

Now there may be yet another step forward. Earlier this week, a group led by Dr. Joseph Zabner at the University of Iowa published a paper in the journal Nature, highlighting how infection in CF patients may be prevented and possibly treated with a multifunctional chemical known as sodium bicarbonate.

Most people tend to call that baking soda.

Zabner's team worked with a certain kind of pig which develops the same CF that humans inherit genetically, in the hopes of learning exactly how the mutation of the CFTR gene allows infection to occur. Much like what has been seen in humans, the pig experiments found that the CFTR gene mutation changed one of the lung's defense mechanisms, known as the airway surface liquid (ASL), by lowering the pH (i.e. more acidic) and rendering it less able to kill the bacteria known to cause problems in CF: Staphylococcus aureus and Pseudomonas aeruginosa.

Taking a lesson from Chemistry 101, the team decided to try increasing the pH of the ASL by aerosolizing bicarbonate, which is a gentle alkaline known to raise pH, and allowing the pigs to breathe it in.

The result was quite promising. In cases where bacterial infection had not yet begun, the pigs stayed healthy. If bacteria were already present, the bicarbonate-treated ASL was able to fight off infection. There wasn't a total clearance of the bacteria but the results were impressive enough for the authors to suggest that this technique may be an option for CF infection management into the future.

As with any medical advancement, the Nature paper is just the beginning and there is much work to be done before a bicarbonate treatment is available for CF patients. However, for McCaw, the results offer more than just a promise; they offer hope.

"The idea that a simple ingredient that we all have in our cupboards and fridges could help me fight infection is wonderful. Anything that could help me decrease the antibiotics that I need to take, orally and intravenously would be welcome as it would be much easier on my stomach, liver and my poor, scarred up veins."

That most certainly would be a welcome breath of relief.