Study Could Extend the Lives of People Living with Cystic Fibrosis


Cystic fibrosis (CF) has proven an enigma for medical researchers and professionals – and patients – for years. There is still a lack of knowledge when it comes to the exact cause of the disease. Despite the missing puzzle pieces however, new medical treatments and courses for controlling the disease and enhancing the quality of life for those living with the illness come in great strides year after year while the prognosis for patients takes consistent turns for the better. Following in the trend of some trailblazing work is a study currently taking place at Aston University in the UK. The study’s researchers are referring to their work as potentially life-prolonging for people with the CF.

WHAT IS CYSTIC FIBROSIS?

Cystic fibrosis is a life threatening, more often than not fatal genetic disease that affects primarily the respiratory and digestive systems, and other bodily systems, depending upon the patient. “CFers” possess a malfunctioning gene that creates a protein which causes the body to produce an atypically thick and sticky mucous. This mucous obstructs the lungs and airways which leads to a lifetime of lung infections, and plugs up the pancreas so that natural enzymes which aid in the breaking down of food cannot be produced. Consequently, vital nutrients cannot be absorbed leading to severe malnourishment and in many cases either lifetime dependence on feeding tubes, or death.

A LOOK AT THE STUDY 

Led by Dr. Lindsay Marshall, scientists at Aston University are investigating under the premise that preventing lung infections during childhood can fend off life-threatening infections later in the lives of those with cystic fibrosis. The study entails proving the theory that bacteria pinpointed in children with the illness can disable their natural defense mechanisms, making these children more susceptible to attracting virulent bacteria that can infect airways.

One of the nasty milestones of the disease when it comes to the ravaging of a CF-infected body is the development of a deadly bacterial strain known as Pseudomonas aeruginosa (P. aeruginosa). This specific strain is notorious for being the almost-impossible to treat “superbug” that ultimately creates enough lung damage to kill a CFer. In order to determine the extent to which P. aeruginosa can be halted in its steps, Dr. Marshall and her team have created an impeccable replica of a human CF airway, made completely of human cells in order to examine the treatment of early childhood infections with a spectrum of antibiotics. The model can mimic the functions of an actual human airway and show the deadly progression of cystic fibrosis.

Observing the deterioration process will help Marshall and her crew learn information that can prove critical to developing new and revamped treatments that can actually prolong the lives of people with the illness and carry the CF community in great leaps towards a cure. Marshall claims that the project will allow for establishment of just how accurately their layered human cell prototype can be used to assess its extent for studying the body’s natural defenses and how they are affected by a whole range of inflammatory and infectious conditions – which can lead to the development and assessment of the effectiveness of new and enhanced treatments for CF and other diseases in the future.

ANOTHER DIMENSION TO THE STUDY

With financial support of the a Human Research Trust grant, Marshall’s study is able to take on another objective: that of coming up with new experimental techniques for decreasing the number of animals used to perform respiratory studies. According to an article written in the online magazine Science Daily, last year in the UK more than 115,000 animals were involved in studies for analyzing respiratory conditions like smoke-related lung diseases and asthma. However, since animals do not naturally have CF, it is not only an inaccurate avenue for testing, but quite expensive to genetically alter them for having the disease and is certainly not as effective as using a human model that can be manipulated to have actual CF. In the article, Dr. Marshall states:

“We simply cannot use animals to model the decline in lung function seen in people with CF, the infections typical of people with CF or the administration and dosage of drugs required to treat the condition. Our human CF model…is extremely representative of what happens in human airways and is both ethically and scientifically an improvement upon current animal models.”

THE GOOD NEWS

Cystic Fibrosis is amongst the most common life-threatening inherited diseases in the United States, affecting an approximate 30,000 children and adults in the country and 70,000 worldwide.  To demonstrate the benefit of the research when it comes to cystic fibrosis including the aggressive project underway at Aston University, it is important to note that in the 1950s, the disease was strictly a pediatric disease and most children did not live long enough to even attend elementary school. Today people living with the disease can look forward to living well into their 30s, 40s and beyond. Indeed, courtesy of the development of new treatments and therapies, today the average life expectancy of people with cystic fibrosis has risen to 41 years of age.

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