Cystic Fibrosis Awareness Month
May is National Cystic Fibrosis Awareness Month in the USA, and across the country, people are raising money and awareness to find a cure for the disease. At Cambridge Mask Company, we provide our products to people with cystic fibrosis (also known as CF) on an almost daily basis.
We have been incredibly touched to know that what started out as a simple concept of a pollution mask, has evolved into a fashionable piece of medical wear that can enable people living with this condition to go out in public without fearing the worst for their health. So we add our voice to the many others out there and urge you to spend a little time or money this month supporting the cause by donating to the Cystic Fibrosis Foundation, registering as an organ donor and sharing our article or one like it so that one of the most common lethal inherited diseases in the world can finally be cured.
What is Cystic Fibrosis?
CF is a genetic disease that can cause severe and constant lung infections and irreparable lung damage over time. People living with cystic fibrosis have a defective gene which causes excessive sticky, this mucus build-up in the lungs, pancreas and other organs. The mucus in the lungs clogs up airways and traps pollutants and bacteria, which can lead to infections, lung damage and ultimately respiratory failure. Mucus in the pancreas prevents digestive enzymes from being released which our bodies need to break down food and absorb vital nutrients.
Why is Cystic Fibrosis so common?
CF is most common among Caucasians of North-European descent, although no ethnic group is exempt from the disease. Approximately 30 000 Americans have cystic fibrosis and around 1 000 new cases are documented each year. While millions and millions of people carry the mutated gene, to be affected by the disease a person must inherit two of the genes. In the USA, studies suggest that around one in 29 White Americans carry the cf gene.
It is thought to have developed around 52 000 years ago during the Stone Age in Europe, spreading via hunters and gatherers that moved nomadically across the steppes. Scientists believe that there must be a benefit to having the gene otherwise it would have been selected out long ago. Researchers from the University of North Carolina in Chapel Hill found that mice affected by the cystic fibrosis gene were not as severely affected by cholera, which is an infectious and often fatal bacterial disease of the small intestine, typically contracted from infected water supplies. Since such bacterial pandemics were life-threatening to inhabitants of Western Europe up until Pasteur developed a vaccine in the late 1800’s, having the defective cystic fibrosis gene may have been a substantial selective advantage.
What are the symptoms of Cystic Fibrosis?
Symptoms of Cystic Fibrosis include:
- Very salty-tasting skin
- Persistent coughing, at times with phlegm
- Frequent lung infections including pneumonia or bronchitis
- Wheezing or shortness of breath
- Poor growth or weight gain in spite of a good appetite
- Frequent greasy, bulky stools or difficulty with bowel movements
- Male infertility
What is it like to live with Cystic Fibrosis?
Chief Medical Scientist of Respiratory and Sleep Medicine, Dr D. Parsons, illuminates:
“One way to gauge how CF lung disease might feel is to breathe for a while through a short length of garden hose. After a short time, you will feel an increasing urgency to breathe more than you are able to – you will probably make increasing efforts to move more air in and out of the hose, but your lungs cannot do that, no matter how much effort is put into it.
Now imagine this feeling of “not enough breath“, starting off imperceptibly, steadily increasing, never halting or reversing as you grow up through your teenage years. Expecting to enjoy life as it develops you are faced with an increasing amount of mucus and fluid trapping in your lungs that you relentlessly attempt to cough out to be able to breathe and survive. The risk of getting a serious CF-specific lung infection from friends with Cystic Fibrosis means that you can not even meet in person to gain much needed support.
Your condition progressively affects your ability take part in any activities or even to breathe enough to satisfy your most primal biological instinct of humans – to satisfy the hunger for air.”
Cystic Fibrosis can be an alienating condition, especially before the advent of the internet, as sufferers can never meet in person. Cross-contamination can happen with extreme ease between two people with CF, as they grow bacteria in their lungs that is mostly harmless to people without the condition but can be transmitted easily between two affected people. Meetings and conferences are places where cross-infection could occur, so even at cystic fibrosis-related events, there should only be one person with CF in attendance at a time.
According to Cystic Fibrosis News Today, studies like “The psychosocial and psychiatric side of cystic fibrosis in adolescents and adults” demonstrate that the psychological and psychosocial functioning of people with cystic fibrosis is similar to that of people without the condition until the disease becomes severe.
What is the average life expectancy of someone diagnosed with Cystic Fibrosis?
In living memory, children affected by cystic fibrosis were unlikely to reach school age. However, considerable advantages in modern medicine mean that many young adults can live increasingly full lives, finish college, find jobs and reach an average age of 37. Decreased lung function is the most common cause of death in those living with Cystic Fibrosis and patients must spend hours each day undergoing chest physiotherapy to clear their lungs of the mucus build up. Cleanliness is vital for anyone dealing with the disease, as even a common cold be life-threatening.
How do doctors know if someone has Cystic Fibrosis?
Newborn screening programs are now catching roughly 75% of cases in children under the age of 2. If there is evidence that a baby may have the condition, a doctor will perform a sweat test which measures the level of salt found in sweat. Blood tests can also help in identifying the CF gene.
How can I support people with Cystic Fibrosis?
The Cystic Fibrosis Foundation has all the advice you need to get involved and support their cause. If you’re living in the UK, you don’t need to wait for June’s cystic fibrosis awareness week, but can donate at any time, but do remember to put on your sunniest outfit for Wear Yellow Day on Friday, 23rd June.
And in the meantime, sharing is caring – so put yourself out there share your time, share this information with others, share your resources this May to support Cystic Fibrosis Awareness and inspire others to do the same.