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
"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
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.