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The battle for a better life with cystic fibrosis and a future without
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What is Cystic Fibrosis?
Cystic Fibrosis (CF) is a life-threatening disease that causes mucus to build up and clog some of the organs in the body, particularly in the lungs and pancreas.
Sometimes inaccurately described as a “lung” or "digestive” disease it is actually not either of these individually but rather an inherited GENTIC disease which affects the cells that produce mucus, sweat, saliva and digestive juices. The basic problem can be defined as the body’s abnormal exchange of water and salt (or more specifically, sodium and chloride). Normally, the body’s secretions are thin and slippery, but in cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways. When mucus clogs the lungs, it can make breathing very difficult and allows bacteria (or germs) to get stuck in the airways causing inflammation (or swelling) and infections that lead to lung damage. The mucus in the digestive tract and pancreas prevents digestive enzymes from getting to the intestines where the body needs them to break down food to get the nutrients needed and therefore results in improper/poor digestion and absorption. Since salt absorption in the sweat ducts is also impaired, patients produce extremely salty sweat resulting in a salty taste to the skin, a trademark of CF. Due to the fact that cystic fibrosis is genetic we must receive the CF gene from both parents to actually have the disease. Carries of one gene are said to be symptom-less and it is estimated that there are currently 10 million Americans (1 in 31 people) who carry the gene. There are over 30,000 individuals in the US (70,000 worldwide) who fight this disease every day. Some ethnicities are known to have higher incidences of the disease, most commonly among the Caucasian race with approximately 1 in every 3,000 births whereas the Asian community shows the least frequent occurrence at 1 in every 32,000 births. The severity of cystic fibrosis symptoms is different from person to person. The most common symptoms are:
Sometimes inaccurately described as a “lung” or "digestive” disease it is actually not either of these individually but rather an inherited GENTIC disease which affects the cells that produce mucus, sweat, saliva and digestive juices. The basic problem can be defined as the body’s abnormal exchange of water and salt (or more specifically, sodium and chloride). Normally, the body’s secretions are thin and slippery, but in cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways. When mucus clogs the lungs, it can make breathing very difficult and allows bacteria (or germs) to get stuck in the airways causing inflammation (or swelling) and infections that lead to lung damage. The mucus in the digestive tract and pancreas prevents digestive enzymes from getting to the intestines where the body needs them to break down food to get the nutrients needed and therefore results in improper/poor digestion and absorption. Since salt absorption in the sweat ducts is also impaired, patients produce extremely salty sweat resulting in a salty taste to the skin, a trademark of CF. Due to the fact that cystic fibrosis is genetic we must receive the CF gene from both parents to actually have the disease. Carries of one gene are said to be symptom-less and it is estimated that there are currently 10 million Americans (1 in 31 people) who carry the gene. There are over 30,000 individuals in the US (70,000 worldwide) who fight this disease every day. Some ethnicities are known to have higher incidences of the disease, most commonly among the Caucasian race with approximately 1 in every 3,000 births whereas the Asian community shows the least frequent occurrence at 1 in every 32,000 births. The severity of cystic fibrosis symptoms is different from person to person. The most common symptoms are:
- Very salty-tasting skin
- Persistent coughing, at times with phlegm
- Frequent lung infections, like pneumonia or bronchitis
- Wheezing or shortness of breath
- Poor growth/weight gain in spite of a good appetite
- Frequent greasy, bulky stools or difficulty in bowel movements
- Small, fleshy growths in the nose called nasal polyps
Sometimes people are mistreated for asthma or chronic bronchitis when they really have cystic fibrosis. New research shows that the severity of CF symptoms is partly based on the types of CF gene mutations (defects). Scientists have found more than 1,500 different mutations of the CF gene. Fortunately CF has developed from just a “childhood” disease to a chronic illness with 40% of the population now reaching adulthood. Currently there is still NO CURE but our average life expectancy has increased and recently reached 36 years of age.
Transplant:
It is important that I specify the following applies to any organ transplanted (not just the lungs) in a person affected by cf as I, myself had liver failure which lead to transplant. However it is most commonly lungs that are the transplanted organ due to cf damage.
A (lung) transplant will not cure CF because the defective genes that cause the disease are in all of the cells in the body, not just in the lungs. At this time, scientists are not sure how to “fix” genes permanently. While a transplant does give a person with CF a new set of lungs, the rest of the cells in the body still have CF and may already be damaged by the disease. Further, organ rejection drugs can cause other health problems.
Hopefully this information was helpful and able to eliminate some of the mystery from “just another disease.” All statistics or otherwise verifiable information has been complied from websites such as www.cff.org and www.transplantliving.org in addition to some personal experience. For more specific or detailed information please check the links and resources page or feel free to contact me with any questions.
The Sixty Five Roses Story
"65 Roses" is what some children with cystic fibrosis (CF) call their disease because the words are much easier for them to pronounce.
"65 Roses" is what some children with cystic fibrosis (CF) call their disease because the words are much easier for them to pronounce.
Mary G. Weiss became a volunteer for the Cystic Fibrosis Foundation in 1965 after learning that her three little boys had CF. Her duty was to call every civic club, social and service organization seeking financial support for CF research. Mary's 4-year-old son, Richard, listened closely to his mother as she made each call.
After several calls, Richard came into the room and told his Mom, "I know what you are working for." Mary was dumbstruck because Richard did not know what she was doing, nor did he know that he had cystic fibrosis. With some trepidation, Mary asked, "What am I working for, Richard?" He answered, "You are working for 65 Roses." Mary was speechless.
He could not see the tears running down Mary's cheeks as she stammered, "Yes Richard, I'm working for 65 Roses."
Since 1965, the term "65 Roses" has been used by children of all ages to describe their disease. But, making it easier to say does not make CF any easier to live with. The "65 Roses" story has captured the hearts and emotions of all who have heard it. The rose, appropriately the ancient symbol of love, has become a symbol of the Cystic Fibrosis Foundation.
65 Roses® is a registered trademark of the Cystic Fibrosis Foundation.
The Cystic Fibrosis Foundation is a donor-supported national non-profit organization established over 50 years ago, at a time when children with CF rarely lived to be school age. CFF works very hard to allocate research funds towards the future of better medications & an eventual cure for CF. We are thrilled to announce that median age of survival for a person with CF has increased to 36.8 years, up from 32.9 in 2003. This milestone was achieved in part due to the efforts & innovative approaches of the CF Foundation. We will not be satisfied until we stop losing precious lives to this disease, but there is gratification in the realizations that we are impacting both length & quality of life for all people with CF. Together, let’s help make CF stand for Cure Found !!!
betterbattle.org has been created for the purpose of educating, raising awareness and funds for Cystic Fibrosis and is a proud supporter of the Cystic Fibrosis Foundation www.cff.org
To find out more information about the CF Foundation or to join our volunteer team, please contact:
Cystic Fibrosis Foundation, Sacramento Chapter
4600 Roseville Rd., Suite 140, North Highlands, CA 95660
916-349-1243, fax: 916-349-1244
