CYSTIC FIBROSIS

Cystic Fibrosis
Student: Janna Enikeeva
Student No: N01026810
Date Submitted: Nov. 19, 2015
Course Code: NURS209
Humber College

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Boyle defines Cystic fibrosis as an autosomal recessive, multisystem

disease characterized by altered function of the exocrine glands involving
primarily the lungs, pancreas, and the sweat glands (as cited in Lewis,
Dirksen, Heitkemper, Bucher and Camera, 2014, p.753). It is one of the most
common life-shortening genetic diseases out there, with an incidence rate of
1 in 3500 in whites and 1in 2717 in Native Americans (Allen, Vessey &
Shapiro, 2010, p. 405). Unfortunately, there is no cure for cystic fibrosis at
this time. As a result, several nursing interventions need to be implemented
in order to keep patients healthy. The specific intervention to be discussed in
this paper revolves around digestion and absorption issues. In order to
combat this, supplements, vitamins and an adjustment in diet are the first
steps that need to be considered. The consequences of not following through
with this intervention can be as dire as biliary cirrhosis or even colon cancer
(France & Bell, 2014, Gastrointestinal Cancers in Cystic Fibrosis, para. 5-8).
An example of the kind of impact vitamins and supplements have on an
individual with cystic fibrosis can be seen in a study done at the University
Hospital Central of Asturias. The study was meant to demonstrate the
difference in overall health between compliant and non-compliant patients
regarding different therapeutic options. Table 5 illustrates this by showing
that compliant patients saw a significant increase in Vitamin A, K, E and
albumin values, as well as an increased percentage in nutritional index and
forced expiratory flow (Llorente, Garcia & Martin, 2008, Treatment

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Compliance in Children and Adults with Cystic Fibrosis, Section 3.5, Table 5).
The deficiencies shown by the non-compliant patients can prove to cause a
lot of complications down the road when transplants and surgeries are the
only options left.
Since there is no cure for cystic fibrosis, treating the disease becomes
all about proper management of day to day life, including something as
mundane as diet. A nutritionist is someone who understands the nutritional
values that foods have. Their primary role in this intervention is education.
They can teach families about what foods/supplements the patients should
be taking. The reason why this is so important to someone with cystic fibrosis
is because of the implications it has on body parts that we dont typically
associate with food. Hansen (2013) discusses a study done in Netherlands
regarding patients with cystic fibrosis awaiting a lung transplant and how
their nutritional status affected their survival rate, both while still on the
waiting list and after the transplant itself. Needless to say, treating
malnourishment proved to cause more favourable outcomes for these
particular patients (Effects of Nutritional Status and Dietetic Interventions on
Survival in Cystic Fibrosis Patients Before and After Lung Transplantation,
para. 2-4).
A dietician is very similar to a nutritionist in many ways, with the only
thing separating them being certain courses of study and the specific field
they chose to pursue. What a dietician can add to the situation is their

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expertise in crafting a proper dietary plan for the patient to follow. An

example of how dietary habits can affect children with cystic fibrosis can be
found in a study done by Groleau et al. In this study, Groleau and her team
organized a 12 month trial for children between the ages of 5-17, where they
were either given a lipid matrix called LXS or a placebo supplement with the
same nutritional value. The purpose of the trial was to determine how dietary
interventions affected growth, body composition and resting energy
expenditure (REE). Groleau et al. (2014) concluded with the following:
Over a 12 month nutrition intervention with either LXS or placebo, the
growth status, muscle stores and REE improved. Sustained increased
energy intake improved energy metabolism, growth and nutritional
status in school age children with CF, PI and mild lung disease (Effect
of a Dietary Intervention on Growth and Energy Expenditure in Children
With Cystic Fibrosis, Conclusions section, para. 1)
Partnered with the example discussed by Hansen, we can begin to see and
understand why proper dietary habits are so important to people dealing
with these disease.
Dealing with cystic fibrosis is an enormous emotion and financial
burden. The cost of supplements, equipment and antibiotics places a
financial hardship on the entire family involved and financial support varies
considerably amongst the provinces (Lewis et al., 2014, p. 758). This is
where a social worker can prove to be a significant boon to families

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throughout Canada. A social worker can not only provide monetary

assistance by directing these families to specific programs for people in their
situation, but they can also help them find mental health professionals who
specialize in these matters.
There are several resources available to Torontonians who are living with cystic fibrosis.
The most important part is finding a hospital that has a department dedicated to this particular
disease. Luckily, St. Michaels hospital (adult) and The Hospital for Sick Kids (children) both
have them. At these departments, patients and families can receive a vast amount of help from
different health care professionals regarding medication, diet, equipment and any other questions
and concerns that may arise.
Next is a matter of procuring the necessary vitamins and supplements for a reasonable
cost. There is a conveniently placed Specialty Foods shop at the Hospital for Sick Kids that sells
the kinds of dietary supplements (some even for free) that are important for maintaining a
healthy diet.
Finally, Toronto Employment and Social Services centres are located all across the city.
At these centres, families can receive social assistance or even job opportunities to help cover the
costs of medications and equipment. They can also be referred to psychiatrists to help deal with
some of the emotional burden theyre feeling.
In conclusion, cystic fibrosis is a terrible disease that requires constant maintenance of
daily life. One of the more important aspects of this procedure is facilitating a healthy diet to
keep complications from occurring. Nutritionists, dieticians and social workers can be a great

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asset in dealing with this. Fortunately for Torontonians, there are plenty of resources throughout
the city that can help families find what they need.

References
Allen, P. J., Vessey, A. J., & Schapiro, N. M. (2010). Primary care of the: Child with a chronic
condition (5th ed.). St. Louis, MO: Mosby, Inc.
France, M. W., & Bell, S. C. (2014). Gastrointestinal cancers in cystic fibrosis. Current Medical
Literature, 4(1), 1-14. Retrieved from
http://search.proquest.com.ezproxy.uwindsor.ca/docview/1540935989/fulltext?accountid=14789

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Groleau, V., Schall, J. I., Dougherty, K. A., Latham, N. E., Maqbool, A.,
Mascarenhas, M. R., & Stallings, V. A. (2014). Effect of a dietary intervention
on growth and energy expenditure in children with cystic fibrosis. Journal of
Cystic Fibrosis, 13(5), 572 -578. http://dx.doi.org/10.1016/j.jcf.2014.01.009
Hansen, C. R. (2013). Effects of nutritional status and dietetic interventions on survival in cystic
fibrosis patients before and after lung transplantation. Current Medical Literature 3(4), 105-106.
Retrieved from
http://search.proquest.com.ezproxy.uwindsor.ca/docview/1540935296/91268D2E98E74EBEPQ/
2?accountid=14789
Lewis, S. L., Dirksen, S. R., Heitkemper, M. M., Bucher, L., & Camera, I. M. (2014). Medicalsurgical nursing in Canada: Assessment and management of clinical problems (3rd Canadian
ed.). M. A. Barry, S. Goldsworthy & D. Goodridge (Eds.). Toronto, ON: Elsevier Canada
Llorente, R. P. A., Gargia, C. B., & Martin, J. J. D. (2008). Treatment compliance in children and
adults with Cystic Fibrosis. Journal of Cystic Fibrosis, 7(5), 359-367.
http://dx.doi.org/10.1016/j.jcf.2008.01.003

Community Resources
Toronto Adult Cystic Fibrosis Centre (St. Michaels Hospital)
http://www.torontoadultcf.com/
Cystic Fibrosis Centre (Hospital for Sick Kids)

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http://www.sickkids.ca/cysticfibrosiscentre/index.html
Specialty Food Shop (Hospital for Sick Kids)
https://www.specialtyfoodshop.ca/default.aspx
Toronto Employment and Social Services Centre
http://www1.toronto.ca/wps/portal/contentonly?
vgnextoid=0ebe83cf89870410VgnVCM10000071d60f89RCRD