By: Fontana Smith on August 10th, 2016
Have you ever met someone diagnosed with Cystic
Fibrosis? They are often times mistaken for someone with Asthma. More commonly
than not their condition is often described as just ‘a lung disease.’ It is
much more than that. “Cystic fibrosis is a rare genetic disease found in about
30,000 people in the United States and 70,000 worldwide. Most children are now
screened for CF at birth through newborn screening and the majority are
diagnosed by age 2” (Cystic Fibrosis Foundation). Genetic diseases are cause by
mutations in our own DNA. Diseases like this are not easily remedied. The
Cystic Fibrosis Foundation goes on to describe the mutation in greater detail
saying, “Cystic fibrosis is caused by mutations in the gene that produces the
cystic fibrosis transmembrane conductance regulator (CFTR) protein. This
protein is responsible for regulating the flow of salt and fluids in and out of
the cells in different parts of the body” (Cystic Fibrosis Foundation). With
more knowledge and research being done every day, it is not surprising that the
life expectancy of patients with CF has continued to raise. But it is rising at
a high cost to patients with increasingly invasive procedures. The true
question is, has their quality of life decrease as a result?
Cystic Fibrosis can affect multiple organs and systems
in the body. These can include but are not limited to: the gastrointestinal
(GI) tract, pancreas, reproductive system, and most publicly known, the respiratory
system. Patients often have to allot an hour or more each day to clearing their
airways of the thick sticky mucus formed by Cystic Fibrosis. People living with
Cystic Fibrosis are more susceptible to viral and bacterial infections related
to having that mucus, especially those that are airborne. On top of this, each
person as a different and extensive list of daily medications needed for day to
day survival. “In 1962, the predicted median survival for CF patients was about
10 years, with few surviving into their teenage years” (NIH-National Institute
of Health via Cystic Fibrosis News). Today, the median age of survival is
estimated around 40 years old. That is a very drastic change in 40 years.
“Despite significant progress treating CF, infections remain a serious problem and
can lead to worsening lung disease or death.” (Cystic Fibrosis Foundation). In
order to give a person with Cystic Fibrosis more time, invasive procedures like
the lung transplant have been implemented. But what does this mean for each
person living with Cystic Fibrosis as far as their quality of life?
The older a person with CF (Cystic Fibrosis) becomes,
the higher the likelihood of them needing a single or double lung transplant in
their lifetime. “Today, antibodies exist to fight infections; other medications
slow the progression of lung disease; and new mechanical chest physical therapy
devices loosen the mucus and make it easier for patients to clear the lungs.
Lung transplant has also become an alternative for the treatment of CF patients
with severe lung damage” (Cystic Fibrosis News). Receiving a lung transplant
for Cystic Fibrosis is not a cure, it buys that person a little more time here
with us. Lung transplants have become a common procedure today to replace a CF
patients damaged lungs when lung function dips below a certain percent. Some
never make it off the transplant list. If they somehow manage to survive the
procedure, the road to recovery is long and rough one.
Dr. Stephenson takes an optimistic viewpoint saying, “People
think when you have cystic fibrosis and need a transplant it's a death
sentence," and continues to say, "But our paper shows that one-,
three-, and five-year survival rates following lung transplantation are really
quite high" (St. Michael's Hospital ) . This makes
receiving a lung transplant seem like a positive experience. In reality, this
doesn’t tell you that the rate of survival just on year after the surgery is
80% and your chances of survival dwindle 10% or more with each additional year,
(WebMD). It also does not explain the continuous time consuming physical
rehabilitation, and complicated list of new medications added in addition a
normal CF medications and treatments regime. WebMD claims of those receiving a
lung transplant including CF patients that “Among people surviving five years
or more, up to 40% continue to work at least part time,” (WebMD). With all of
this increased therapy, recovery time, new medications, and frequent doctors’
visits, what time is left if any for someone with Cystic Fibrosis to focus on
themselves.
Physically a person living with CF devotes hours or more
a day to the necessary treatments needed to maintain lung function. Once the
function decrease, it extremely difficult to improve it and the lungs never
heal after damage is done. This study done by the Department of Rehabilitation
in The Netherlands found, “A The majority of patients with cystic fibrosis (CF)
will grow into adulthood. Despite this improvement in survival, the disease is
progressive, especially with respect to the decline in pulmonary function,
which in turn may have an impact on a patient's quality of life. In this study
we evaluated the quality of life in CF adults and examined the relationship
between quality of life and pulmonary function, exercise capacity, and
dyspnea.” and “These results show that CF affects quality of life in adults
primarily due to a limitation in physical functioning” (Department of
Rehabilitation, University Hospital, The Netherlands via NCBI PubMed). This
study found that due to the physical limitations associated with Cystic
Fibrosis that increases throughout the subject’s lives, caused the research
participates to feel they had less quality of life. This study clearly states
that it found actual physical evidence that the quality of their live has
decreased, while the life expectancy has increased.
Simply put, the life expectancy is rising, but the
quality of life is not. What is a life that cannot be lived? Emotionally and
mentally, Cystic Fibrosis is a very lonely disease. People diagnosed with CF
cannot be in the same room as another person with CF due to the high chance of
cross contamination. The degree of distance is shorted outside to a 6ft radius
but no physical contact is allowed. Family of those with CF have to take like
precautions to make sure they do not bring something back to their family
member with Cystic Fibrosis. There are no support groups or hugs from others
that know what it feels like to fight for every breath like they do. That said
it is not very surprising that, “People with chronic diseases, such as CF, are
at greater risk for developing clinical depression” (Cystic Fibrosis
Foundation). A study has also been done and published in the Cystic Fibrosis
Journal with the following results, “After controlling for lung function,
patients with symptoms of anxiety reported lower on vitality, emotional
functioning, social, treatment burden, health perceptions and respiratory
symptoms. Those with depressive symptoms reported lower HRQoL scores for emotional
functioning, eating disturbances and body image” (Trudy Havermans) . Multiple studies have shown that
depression, anxiety, less self-worth and self-image options of CF patients are
still prevalent even with lung quality improving some patient’s quality of life
is not. Even after the symptoms of Cystic Fibrosis are being ‘controlled’
people living with CF cannot truly enjoy living their lives.
So much time is taken away from each one of these people
daily. Time they cannot afford to lose. They have to follow a daily routine
that only gets more time consuming and complicated as they contract new
infections and grow older. The reasons why continuing funding and support are
endless. We should strive for better, more manageable forms of treatment to
increase the quality of life with the life expectancy, instead of rising one
and denying the other. “The Cystic Fibrosis Foundation is the world's leader in
the search for a cure, funding promising research and working to provide access
to quality, specialized care and treatments for people with CF. Nearly every CF
drug available today was made possible because of the Foundation's support” (Cystic
Fibrosis Foundation). These people, these sons, daughters, mothers, fathers,
deserve better than a 67% chance at life (St. Michael's Hospital ) . They deserve to
pursue their happiness without the physical and emotional barriers limit them
in accomplishing their dreams. They deserve to live, period.
Works Cited
Cystic Fibrosis Foundation. "About Cystic
Fibrosis." March 29 2016. Cystic Fibrosis Foundation. Website
Article. 10 August 2016.
—. "Depression and CF." 29 March 2016. Cystic
Fibrosis Foundation. Website Article. 10 August 2016.
—. "Why Are Some Germs Particularly Dangerous
for People With CF?" 29 March 2016. Cystic Fibrosis Foundation.
Website Article. 10 August 2016.
Cystic Fibrosis News. "Cystic Fibrosis Life
Expectancy." 2016. Cystic Fibrosis News.
http://cysticfibrosisnewstoday.com/cystic-fibrosis-life-expectancy/. 10 August
2016.
Department of Rehabilitation, University Hospital,
The Netherlands. Quality of life in patients with cystic fibrosis. 22
Febuary 1997. http://www.ncbi.nlm.nih.gov/pubmed/9065946. 10 August 2016.
St. Michael's Hospital . "Lung transplant
survival rates good for cystic fibrosis patients." 15 June 2015. Science
Daily. https://www.sciencedaily.com/releases/2015/06/150615191516.htm. 10
August 2016.
Trudy Havermans, Kristine Colpaert, Lieven J. Dupont.
"Quality of life in patients with Cystic Fibrosis: Association with
anxiety and depression." November 2008. Cystic Fibrosis journal.
http://www.cysticfibrosisjournal.com/article/S1569-1993(08)00101-X/abstract?cc=y=.
10 August 2016.
WebMD. "Lung Transplant Surgery." 22
November 2015. WebMD.
http://www.webmd.com/lung/lung-transplant-surgery?page=3. 10 August 2016.
No comments:
Post a Comment