Question of the Week

June 3, 2008


Unless people have friends or family members who have been personally touched by cystic fibrosis, they may not know what it is.

"'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. ... 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."

A lot has changed since 1965. At that time, parents who were told that their children had CF didn't expect them to live long enough to go to elementary school. In fact, young Richard Weiss--at age four--had almost reached his life expectancy.

"Fortunately, the prognosis for children with CF is far more hopeful than it has been in the past. In the 1960s life expectancy for an infant diagnosed with CF was only about five years. For children living with CF today, the average life expectancy has increased to 32 years. For newborn babies the odds are even greater, with a life expectancy of 45 years and beyond. While a cure remains elusive, treatment for CF has obviously improved."

While those diagnosed with CF have hope for a future that earlier generations of CF patients did not have, most are still are forced to learn to live with a chronic illness from a very young age. As one teenager described it:

"Leesa, aged 15
'People can tell there's something wrong with me because I cough a lot. I used to say I had asthma, but now I say it's complicated; asthma and something else. My closest friends know I've got CF (cystic fibrosis), but others don't. Most just assume it's asthma. I read that Cameron, another CF patient who died, always kept it a secret, so I'm not the only one who does. I'd like everyone to know I've got CF but it's so hard to sit down and explain.'"

" ... it's so hard to sit down and explain."

"Cystic fibrosis (CF) is an inherited disease that causes the body to produce mucus that's extremely thick and sticky. The mucus in people with CF is thicker than normal because CF affects cells in the epithelium (pronounced: eh-puh-thee-lee-um), the layer of cells that lines the passages in the body's organs. In a person who does not have CF, the epithelial cells produce a thin, watery mucus that acts like a lubricant and helps protect the body's tissues. In a person with CF, however, the thicker mucus doesn't move as easily. This thick, sticky mucus clogs passages in many of the body's organs and infection sets in. The two organs that are most affected are the lungs and pancreas, where the thick mucus causes breathing and digestive problems. The thicker mucus has trouble moving out of the lungs, so bacteria can remain and cause infections. The thick mucus can also be found in the pancreas -- an organ that produces proteins called enzymes that flow into the intestine to support the body's digestion process. Because the mucus can block the path between the pancreas and the intestines, people with CF have trouble digesting food and getting the vitamins and nutrients they need from it. CF can also affect the liver, the sweat glands, and the reproductive organs."

Most people with CF have never known a life without lifesaving treatment for the breathing and digestive problems that are caused by the disease.

"About one in five people with cystic fibrosis are diagnosed at birth, when their gut becomes blocked by extra thick meconium (the black tar-like bowel contents that all babies pass soon after birth). This bowel blockage may need surgery. Just over half of people with CF are diagnosed as babies. This is because they are not growing or putting on weight as they should due to the digestive system not breaking down the fat content in food."

While "Just over half of people with CF are diagnosed as babies," those with milder forms of the disease may not be diagnosed until later in life.

"The symptoms and severity of CF vary from person to person. Some people with CF have serious lung and digestive problems. Other people have more mild disease that doesn't show up until they are adolescents or young adults. Respiratory failure is the most common cause of death in people with CF. Until the 1980s, most deaths from CF occurred in children and teenagers."

With the vast majority of cases diagnosed in young children, those who develop symptoms later in life can find that they have a more difficult time finding a doctor who initially identifies their symptoms for what they truly are.

"Cystic fibrosis is usually diagnosed before three years of age. A late diagnosis--one in adolescence or adulthood--may indicate that the person has a mild form of the disease or their symptoms are atypical (does not follow the norm). The genetic defect that leads to CF in adult cases is often a less common variety. Since CF is usually considered a pediatric disease, many physicians may not consider it when they see older patients who may have CF symptoms. Even so, approximately 6% of the diagnoses of CF are made in adults and the number of late diagnoses is increasing."

In fact, while some doctors may not initially identify the symptoms accurately because they don't think of CF as a disease that presents in teens and adults, others may not even believe older patients who complain of CF symptoms.

"The incidence and prevalence of late diagnosis of cystic fibrosis (CF) are increasing, and patients given a CF diagnosis as adults differ from those who received the diagnosis as children. ... Over half the adults with CF were men, but women were significantly more likely to receive a late diagnosis and more likely to have milder disease. While respiratory symptoms most frequently led to the diagnosis of CF for all adults, there was more diversity in conditions leading to diagnosis for men. ... 'As we suggest in the article, women's complaints may not be taken seriously by physicians, so that women are diagnosed later. For instance, in an earlier study, two women told me their physicians diagnosed them as anorexic and wouldn't believe they were eating. Others told me they were accused of seeking attention via their symptoms.' ... In their study, Dr. Widerman and her coauthors reminded physicians to be aware that 'a diagnosis of CF is possible in an adult; that patients who are part of racial and ethnic minorities can have CF; and that adults with CF can present with a range of symptoms, conditions, and/or complications, some of which are gender-related.'"

Whether the diagnosis is made at birth or in early adulthood, those with a CF diagnosis then need to begin treatment that will last for the rest of their lives--or until a cure is discovered.

"There is a lot of research under way to try to find a cure for cystic fibrosis lung disease through gene therapy. Current treatments aim to control the symptoms and have two main aims:

Treatments consist of a combination of methods that can help control symptoms and improve not only the quality of life for a patient with CF but allow the opportunity for a longer life than those who were born with CF just a few decades ago:

  • "Physiotherapy: If you have cystic fibrosis you will need daily chest physiotherapy, which involves vigorous massage to help loosen the sticky mucus. Parents of a child with cystic fibrosis are taught by hospital staff how to do this. Older children and adults with cystic fibrosis can be taught to do this for themselves.
  • Enzyme therapy: With each meal or snack, most people with cystic fibrosis need to take replacement enzymes such as pancreatin (eg Pancrex). These supply the missing pancreatic enzymes and allow proper digestion. People with cystic fibrosis normally need vitamin and mineral supplements too.
  • Other cystic fibrosis-related therapy: There is a range of other possible treatments, according to each person's condition. ... People with cystic fibrosis may also need help to overcome fertility problems as well as counselling to help cope with the psychological aspects of the illness."

Whatever the age of diagnosis, CF can be a difficult disease to live with as a teen. Having friends and classmates who are understanding and supportive can make all the difference.

"Teenagers, especially, may feel embarrassed about their cough, their slenderness and their medications. Most of all, they may worry that their illness will keep them from having a normal social life. And in fact, all the turbulence and difficulty of adolescence is compounded for teenagers with cystic fibrosis. Yet many teens find that being frank and open about cystic fibrosis can ease some of their concerns. Still, adolescents understandably may want to pretend they don't have cystic fibrosis. They may rebel against dietary restrictions, treatments and medications. And like other teenagers they may start to smoke. But normal rebelliousness can be life-threatening for young people with cystic fibrosis."

Questions of the Week:

  • If you know of a friend, classmate, or relative who has cystic fibrosis (CF), what do you and your peers need to know about the disease?
  • What can you do to help people you come into contact with who are living CF to feel more comfortable sharing about their disease?
  • Even if you do not think that you currently know anyone with CF, what information should all people have?
  • Why is it important for those who are currently unaffected by CF to know about it?

Please email me with any ideas or suggestions.
Note: Due to increasing amounts of SPAM sent to this account, please include "QOW" in the subject line when sending me email.

I look forward to reading what you have to say.

Health Community Coordinator
Access Excellence @ the National Health Museum

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