Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

Student No. N00180321

Pediatric Health: Cystic Fibrosis, Consequences of Interventions Not Implemented

Student Name: Lara Pabandero-de Lasan
Student No.: N00180321
Date Submitted: November 19, 2015
NURS209 (360): Practical Nursing Theory 3
Professor:
Humber College ITAL

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

Student No. N00180321

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

Student No. N00180321

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

Student No. N00180321

Pediatric Health: Cystic Fibrosis, consequences of interventions not implemented

Cystic Fibrosis (CF) is the most common genetic disease affecting Canadian children
and young adults with the ratio of 1 in 3600 born each year (Cystic Fibrosis Canada (CFC),
2015). Defined as an autosomal-recessive disorder of the exocrine glands (Mosby, 2013,
p.477). The cause of CF is the defective mutated gene in chromosome seven and the protein
cystic fibrosis transmembrane regulator (CFTR) inherited by the affected child from both
parents; and the primary factor causing many clinical manifestations is the mechanical
obstruction by increased viscosity of mucoprotein and secretions coagulating to form
concretions in glands and ducts (Perry et al., 2013, p.1220). As of yet, there is no known cure
for CF, however treatments are available to manage manifestations and early fetal screening
(Ratjen, 2009, p.595).
According to Perry et al (2013), manifestation of CF can vary from meconium ileus in the
newborn, the small intestine is blocked with thick, puttylike, tenacious mucilaginous
meconium; prolapsed rectum affects infants to childhood; pancreatic fibrosis is the impairment
of digestion and absorption of nutrients; and the most serious threat to life is pulmonary
complications, present in almost all CF patients (p.1220). The purpose of this essay is to present
a critical review of literature and data sources pertaining to a priority nursing intervention for
pediatric cystic fibrosis which is eradication of the obstruction of mucous and secretion build up
to improve gas exchange and airway clearance, emphasizing the factors of consequences if this
intervention is not addressed. Collaborative care providers and community resources will also be
discussed.

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

Student No. N00180321

Priority nursing intervention: eradication of mucous and secretions

In any circumstance, it is vital to have a clear and patent airway without obstruction to
keep the heart oxygenated and the body functioning at equilibrium (Canadian Red Cross, 2011,
p.56). Airway clearance is imperative in CF care, therefore routine airway clearance therapies
(ACT) facilitate prevention in build-up of mucus in the lungs and allow them to work at their
highest capacity, and in turn may also reduce chest infections and enhance lung function (CFC,
2015). Flume et al (2009) examined ACTs efficacy, seven ACTs tested and none proved to be
better than the other, however it was proved that ACTs whether independent or combined will
prolong the life expectancy and quality of life (QOL) of a patient with CF, the different ACTs are
more on patient preference and individualized care (p.524).
An example of ACT is percussion and postural drainage (P&PD) also known as chest
physical therapy (CPT), Borowitz et al. (2009) recommends the use of pharmacotherapy in
conjunction, prior to CPT (p. S76). For infants P&PD is used, teaching ACT to parents early
and encouraging airway clearance to be part of the childs daily routine are believed to have
positive benefits (p. S85). Positive expiratory pressure (PEP) with devices such as the Flutter,
cause pressure and vibrations in the lungs to help loosen and move mucus out of the airway
(Mahesh, McDougal, & Haluszka, 1996, p.165). Nurses are to encourage CF patients to
exercise. Grossman and Grossman (2005) agree that ACTs with exercise for patients who were
mobile, was effective and imperative for sputum clearance(p.50). Prasad declares that physical
exercise is fundamental in CF management, with favourable effects on fitness levels, muscles,
bone structure and posture, as well as airway clearance because it aids in maintaining the chest
clear (Prasad, 2013, p.10). Thus, airway clearance is a nursing priority if not addressed, will

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

Student No. N00180321

impact the respiratory tract leading to respiratory distress and complications like lung collapse,
other organ failures when oxygen is inadequate in the body, and ultimately premature demise.
Collaborative healthcare providers
Pediatric CF is a complex multisystem disease and a multidisciplinary healthcare team is
involved in providing care which consists of specialist physicians, nurse specialists,
physiotherapist, psychotherapist, and others (Proesmans, Vermeulen & Deboeck, 2008, p.843).
Physiotherapists (PT) or physical therapists work in partnership with individuals of all ages to
break down the barriers to physical function such as chronic diseases, injury, motor vehicle
accidents, strokes, and respiratory conditions (Ontario Physiotherapy Association, 2015). PTs
treat with physical agents and methods such as massage, manipulation, therapeutic exercises,
cold, heat, hydrotherapy, electrical stimulation and light to assist in habilitating or rehabilitating
clients (Mosby, 2013, p.1392). CF patients and their family must be seen regularly by their CF
team, including the physiotherapist, to aid in providing and educating the proper way of airway
clearance through CPT, and explaining how to use devices like the Flutter (Prasad, 2013, p.4).
Psychotherapists are individuals who practice psychotherapy like psychiatrists, licensed
psychologists, psychiatric nurses, psychiatric social workers and individuals trained in
counseling (Mosby, 2013, p.1485). The CF patient and family can go to therapy sessions in a
controlled and therapeutic setting with the therapist. Duff (2001) states, parental response to
dietary treatment result in the inadvertent reinforcement of negative or non-feeding behaviours,
as they try to bribe, entice, beg or even force their child to eat, behaviour therapy is highly
effective in modifying actions such as bribing or begging (p.354). Cognitive behaviour therapy
(CBT) is now the treatment of choice for depression and anxiety, both of which are anticipated in
young people with CF where the patient and therapist have an established treatment goal, and use

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

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CBT to change negative thoughts not as facts but as hypotheses to be accepted or rejected
(Duff, 2001, p.355).
Registered dieticians are trained in nutrition, foods, and management of diets (Mosby,
2013, p.1535). Dietitians of Canada (2015) provide assistance in education and counselling
regarding nutrition, and support clients to make changes in their eating habits to promote health
and prevent chronic illnesses. According to Kelly and Slattery (2007), a dedicated CF dietician
hugely benefits CF patient care (p. S53). Adequate nutritional incorporated with improved lung
function offers parents and patients with CF more motivation to adhere to prescribed nutritional
therapies, such as enzymes, vitamins, and a balanced, high energy dietary intake (Kalnins &
Wilchanski, 2012, p.51). McIntosh (2014) says that nutritional status is closely associated with
survival, the dietitian constructs specialized diets, and works with patients and families to
optimize nutritional (p.9).
Community Resources
Firstly, the Toronto & District Chapter of Cystic Fibrosis Canada is a national non-profit
organization that funds research towards a cure and control for CF. They provide for CF clinics
in Canada by holding charities such as Walk for CF, Ride for the breath of life, and depend on
generous donations and volunteers. Secondly, located in downtown Toronto, Sick Kids Hospital
Cystic Fibrosis Centers goal is to improve QOL for CF patients and their families by their six
primary goals. 1) Therapy discovery 2) Bringing discoveries to the clinic 3) Therapy delivery
and evaluation 4) Early detection, intervention and prevention 5) Training and education of CF
professionals and families and 6)Manage and explore the CF mutation database. These goals are
achieved by vigorous research in clinical trials, clinical care and education in drug discovery,
preclinical drug and severity in newborns assessments. Thirdly, St Micheals Hospital CF

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

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program is the largest in North America, more individuals are living longer with CF with the
help of clinical research. Kalydeco, is a drug that was brought about by this program that treats a
rare form of CF in Canada, which has been shown to improve lung function, digestive issues and
quality of life of patients (DiConstanzo, 2015).
Conclusion
It is evident that airway clearance is vital in managing CF, maintaining QOL, and
therefore patients having a longer life expectancy. If the above presented interventions of airway
clearance are not implemented, consequences will be the result and ultimately premature death.
With the help of the collaborative healthcare team and resources provided, management of CF is
enhanced and a less difficult for the patient and family.

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

Student No. N00180321

References
Borowitz, D., Robinson, K. A., Rosenfeld, M., Davis, S. D., Sabadosa, K. A., Spear, S. L.,
Accurso, F. J. (2009). Cystic fibrosis foundation evidence-based guidelines for
management of infants with cystic fibrosis. The Journal of Pediatrics, 155(6 Suppl.4),
S73-S93. doi:10.1016/j.jpeds.2009.09.001. Retrieved October 1, 2015 from Science
Direct Database http://www.sciencedirect.com/science/article/pii/S0022347609008816
Canadian Red Cross. (2011). Canadian red cross: First aid and CPR manual A practical
resource
for work and home. Guelph, ON: The Canadian Red Cross Society.
Cystic Fibrosis Canada (CFC). (2015, Oct 1). What is cystic fibrosis. Retrieved October 1, 2015
from http://www.cysticfibrosis.ca/about-cf/what-is-cystic-fibrosis/
Di Conztanzo, M. (2015, Oct 1). Dr. Elizabeth Tullis of St. Michaels Hospital appointed to
cystic
fibrosis Canada chair in adult cystic fibrosis research. St. Micheal's Hospital. Retrieved
October 1, 2015 from http://www.stmichaelshospital.com/media/detail.php?
source=hospital_news/2015/20150507_hn
Dietitians of Canada. (2015, Oct 10). What does a dietitian do? Retrieved October 10, 2015
from http://www.dietitians.ca/Your-Health/Find-A-Dietitian/What-does-a-Dietitian-d
o.aspx
Duff, A. J. A. (2001). Psychological interventions in cystic fibrosis and asthma. Paediatric
Respiratory Reviews, 2(4), 350-357. doi:10.1053/prrv.2001.0171. Retrieved October 1,
2015 from Science Direct Database
http://www.sciencedirect.com.eztest.ocls.ca/science/article/pii/S152605420190171X

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

10

Student No. N00180321

Flume, P. A., Robinson, K. A., O'Sullivan, B. P., Finder, J. D., Vender, R. L., Willey-Courand,
D.-B., ...the Clinical Practice Guidelines for Pulmonary Therapies Committee. (2009).
Cystic fibrosis pulmonary guidelines: Airway clearance therapies. Respiratory Care,
54(4), 522-537. Retrieved from http://go.galegroup.com/ps/i.do?id=GALE
%7CA198171922&v=2.1&u=humber&it=r&p=AONE&asid=7d62078ff595a5a2f5c2371
856ff476e
Grossman, S., & Grossman, L. C. (2005). Pathophysiology of cystic fibrosis: Implications for
critical care nurses. Critical Care Nurse, 25(4), 46-51. Retrieved October 10, 2015 from
ProQuest Database http://search.proquest.com/docview/228174058?accountid=11530
Kalnins, D., & Wilschanski, M. (2012). Maintenance of nutritional status in patients with cystic
fibrosis: New and emerging therapies. Drug Design, Development and Therapy, 6, 151.
doi:10.2147/DDDT.S9258. Retrieved October 10, 2015 from
http://go.galegroup.com.eztest.ocls.ca/ps/i.do?p=AONE&u=humber&id=GALE|
A344827831&v=2.1&it=r&sid=summon&userGroup=humber&authCount=1
Kelly, V., & Slattery, D. (2007). The benefits to patients with cystic fibrosis of a dedicated
dietician. Journal of Cystic Fibrosis, 6, S53-S53. doi:10.1016/S1569-1993(07)60199-4
Retrieved October 10, 2015 from Science Direct Database
http://www.sciencedirect.com.eztest.ocls.ca/science/article/pii/S1569199307601994
Mahesh, V. K., McDougal, J. A., & Haluszka, L. (1996). Efficacy of the flutter device for airway
mucus clearance in patients with cystic fibrosis. The Journal of Pediatrics,128(1), 165165. doi:10.1016/S0022-3476(96)70460-2. Retrieved October 1, 2015 from Science
Direct Database http://www.sciencedirect.com/science/article/pii/S0022347696704602
McIntosh, I.D. (2014). Health human resources guidelines: Minimum staffing standards for cf

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

11

Student No. N00180321

healthcare teams. Cystic Fibrosis Canada. 1-18. Retrieved October 10, 2015 from
http://www.cysticfibrosis.ca/wp-content/uploads/2014/10/Health-Human-ResourcesGuidelines.pdf
Mosby. (2013). Mosby's dictionary of medicine, nursing & health professions, 9th Ed. Mosby
Elsevier Canada. VitalBook file, Pageburst.
Ontario Physiotherapy Association. (2015, Oct 10). About physiotherapy. Retrieved October 10,
2015 from http://www.opa.on.ca/about_phys.shtml
Perry, S.E., Hockenberry, M.J, Lowdermilk, D.L., Wilson, D., Keenan-Lindsay, L., & Sams, C.
(2013). Maternal Child Nursing Care in Canada. Mosby Elsevier Canada. VitalBook file.
Pageburst.
Prasad, S.A. (2013). Cystic fibrosis our focus: Physiotherapy treatment for babies and toddlers
with cystic fibrosis factsheet. Cystic Fibrosis United Kingdom. 1-7.
https://www.cysticfibrosis.org.uk/media/127518/FS_Physiotherapy_Airway_Clearance_
Mar_13.pdf
Proesmans, M., Vermeulen, F., & De Boeck, K. (2008). What's new in cystic fibrosis? from
treating symptoms to correction of the basic defect. European Journal of
Pediatrics, 167(8), 839. doi:10.1007/s00431-008-0693-2. Retrieved October 1, 2015
from ProQuest Database
http://search.proquest.com/docview/221965132?pq-origsite=summon&accountid=11530
Ratjen, F. A. (2009). Cystic fibrosis: Pathogenesis and future treatment strategies. Respiratory
Care, 54(5), 595-605. Retrieved October 1, 2015 from http://go.galegroup.com/ps/i.do?
id=GALE

Running head: PEDIATRIC HEALTH: CYSTIC FIBROSIS

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Student No. N00180321

%7CA201032442&v=2.1&u=humber&it=r&p=AONE&asid=4d5eb775cadc378f96c7ed
b767ecda6d
Sick Kids Hospital Cystic Fibrosis Center. (2015, Oct 10). Cystic Fibrosis Center. Retrieved
October 10, 2015 from http://www.sickkids.ca/Centres/Cystic-Fibrosis-Centre/
Toronto District Chapter Cystic Fibrosis Canada. (2015, Oct 10). Chapter information. Retrieved
October 10, 2015 from http://www.cfchapters.ca/toronto-district/