Cystic fibrosis affects 70,000 people worldwide and 30,000 individuals in the U.S. It is found to be a genetic disease, which means that there has to be one cystic fibrosis gene inherited by both parents. Even if the disease is not common in a family history, a child can still inherit it. Cystic fibrosis is an inherited genetic disease that has some serious symptoms, terrifying statistics, and helpful therapies that have been found through medical advances.

The statistics that are shown for cystic fibrosis can be scary, but at the same time, eye opening. Sadly, about 1,000 new cases of CF are diagnosed every year. More than 70% of the cystic fibrosis patients are diagnosed by age two. Over 45% of the CF population is 18 or older. In 1969, in the United States, the median age of survival was 14 years. Now, the median age of survival in 2005 was about 37 years. Unfortuantely, according to the CF Foundation there were 419 deaths in 2000. Cystic Fibrosis occurs in 1 in every 3,200 Caucasian births. Looking at some other parts of the world, Ireland has the highest incidence of cystic fibrosis in the world. CF is Ireland’s most common life-threatening genetically inherited disease affecting 1 in every 1,600 births. In Ireland there are more than 1,100 CF patients, 517 are adults (45%), 626 patients are children (55%). In the United Kingdom, cystic fibrosis affects 8,000 people. The life expectancy for UK CF patients is 31 years. Also, looking at some of our neighboring countries statistics, Canada has a population of 30 million people. There are 3,098 people that are recorded for going to cystic fibrosis clinics to receive treatment. In Mexico, the life expectancy for a person with CF is 9 years.

There are many symptoms that a CF patient has. Cystic fibrosis is a genetic disease that affects the way in which salt and water move in and out of the body’s cells. In a CF patient, thick mucus causes blockages of small tubes and ducts in their body. The mucus blockages mostly affect the lungs and digestive system. The digestive system is affected, because those who suffer from CF have a lack of digestive enzymes in their intestines. This keeps the body from digesting food properly. Chloride (part of what makes up salt) cannot pass through cells normally. Through the cells, more sodium is pumped into the lungs than usual. This may cause lung mucus and fluid to be thicker and stickier, and sweat to be more salty. People with cystic fibrosis have airway secretions that are drier, stickier, and harder to clear than normal secretions. This thick mucus can block the bronchial tubes in the lungs and can lead to gradual blockage, infection, and lung damage. Those that have cystic fibrosis suffer from the following symptoms:
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having very salty-tasting skin
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continual persistent coughing, at times with phlegm
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catching frequent lung infections
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struggling with wheezing or shortness of breath
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having poor growth/weight gain in spite of a good appetite
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suffering from frequent greasy, bulky stools or difficulty in bowel movement
Over the years, there have been advances in medicine that has allowed people who have cystic fibrosis to live longer and receive better treatment for their disease. 60 years ago, few children with cystic fibrosis lived to attend elementary school. Today, medical advances and treatments allow CF patients to live into their 30s, 40s, and beyond. To help patients that are fighting cystic fibrosis, there are drugs to help clear the thick mucus from the airways, reduce inflammation and aerosolize antibiotics for CF. Clearing mucus helps to reduce the severity of lung infections and improve lung function. One of the techniques that can be used is called “postural drainage and percussion.” This includes the CF patients sitting, standing, or lying in a position that will help free up mucus as their chest and back are pounded or clapped. Sometimes, a patient will use a mechanical “vest,” or blow into a devise that shakes the mucus loose, to help clear their airways.
Inhaled medications are used commonly, because they reach the lungs quickly and easily. Inhaled medicines can be given by aerosol—a mist made from liquid medicines. Using a cup (nebulizer) attached to a compressor, the compressor blows air through the cup and makes a mist. The CF patient will breathe in the mist using a mouthpiece or a mask for several minutes. Some of the inhaled medications include mucolytics, which thins the mucus so people can cough it up. Also, hypertonic saline can be used to draw more water into the airways and make it easier to cough out the mucus.

Some antibiotics include TOBI, cayston, and ibuprofen. TOBI (tobramycin solution for inhalation) and cayston are effective against the most common source of chronic lung infection, bacteria called Pseudomonas aeruginas. Ibuprofen helps to slow the rate that lung function usually declines for some people with CF. However, if using ibuprofen, it needs to be closely monitored by the patient’s physician.
Another alternative therapy is implanted devices. Ports and PICCs are implanted devices that allow repeated and long-term access to the bloodstream for frequent or regular administrations of drugs as an alternative to shots.

A crucial factor to consider for a cystic fibrosis patient is their diet. Everyone with CF, no matter their age, needs good nutrition to stay strong and fight against lung infections and other challenges.

I have been a member in the Lakeside 4-H club for over ten years. Each year, our club finds a way to give money to the cystic fibrosis cause, either through fundraisers, or just sending money. In the last few years, I have gained more knowledge on what cystic fibrosis is. A couple of years ago, our club were selling glow sticks at the Amery fireworks display, to raise money for cystic fibrosis. There was a woman who was a retired cystic fibrosis nurse from Alaska that came up to our club and gave us a donation. At the time she talked to us, her daughter was also a nurse who was currently working with CF patients in Anchorag, Alaska. She encouraged us to keep sending money, because it really did make a difference, and progress was being made to find a cure.

A year after that, our 4-H leader sent home a DVD for us to watch about cystic fibrosis. I thought it would be really dull, and only tell us about the medical aspect of CF, so I didn’t watch the first part of the movie. Half way through the film, I realized that the movie was based on a true story about a little girl named Alex (which was also the title of the movie) who died from cystic fibrosis at a very young age. Between these two experiences, I wanted to learn more about the disease. Doing the writing workshop in English class gave me the opportunity I needed.

Once a person has cystic fibrosis, they have it for life. Currently, there isn’t a cure for this disease, but with medical discoveries being made every day, hopefully there will be soon. Anyone could have cystic fibrosis show up in our their family down the line. It is good to know the causes, symptoms, statistics, and treatments, so that as a country that makes up almost half of the world’s cystic fibrosis patient population, we may know how to fight it.