NURS 209 Scholarly Assignment

ID: 823-435-053

Cystic fibrosis: Minimizing pulmonary exacerbations, a nursing intervention

Student: Kristina Milaniak
Student ID: 823-425-053
Course: NURS 209 Theory 3
Professor: Dianna David
Due Date: November 17th, 2015
Humber College ITAL

NURS 209 Scholarly Assignment

ID: 823-435-053

Cystic fibrosis: Minimizing pulmonary exacerbations, a nursing intervention

Cystic Fibrosis (CF) is the most common genetic autosomal recessive disease amongst Caucasians,
and affects approximately 1 in 2000 Canadian births (Perry, Hockenberry, Lowdermilk, Wilson, & Sams,
2013). With no cure, health care professionals, such as nurses, have created medical interventions that
provide the pediatric patient the ability to manage the disease and ascertain a higher quality of life. There
are many bodily system complications and clinical manifestations apparent with the progression of CF.
However, the first symptom that is most common amongst this patient population is a frequent productive
cough (Perry et al., 2013). Over time the cough causes pulmonary exacerbations from ineffective airway
clearance related to abundant, thick bronchial mucous secretions, which if left untreated, can lead to
debilitating respiratory infections such as bronchiolitis, bronchitis, pneumonia, pneumothorax and
ultimately premature death.
Nurses being at the forefront of care with this pediatric patient population must make interventions
for these mucous secretions and decrease respiratory complications. The multidisciplinary health care team
collaborates with nurses to prevent these pulmonary exacerbations related to excessive mucosal secretions.
With medical support from multiple clinical providers, the CF patient along with their family is able to
manage this debilitating disease. Resources are readily available in the community for patients suffering
from CF, which should also be included in the patients care plan. Over the past few decades, the research
for cystic fibrosis has been increasing and has promoted evidence-based practice for patients with CF (Perry
et al., 2013). The following discussion will illustrate the importance of respiratory nursing interventions that
help with mucous secretions. These interventions reduce the consequences for the pediatric patient related
to the disease. Prioritizing these pulmonary interventions within the nursing process allows the patient to
cope with this genetic disease and increase the longevity of their life.
Studies have shown that 85% of CF patients die due to the pulmonary exacerbations related to
thickened mucous secretions described above (Geller & Robin, 2009). The management of pulmonary
problems is directed toward treatment and prevention of pulmonary infection and exacerbations. With
problems starting as young as three years of age, it is imperative that nurses make interventions in
preventing and treating pulmonary exacerbations, a top priority in all CF patients (Geller et al., 2009).
There are numerous studies that demonstrate the consequences of not reducing pulmonary exacerbations
related to mucopurulent secretions, including recurring and chronic lung infections, chronic fatigue from
increased work of breathing, increased hospital visits and longer hospital stays (Flume, OSullivan,

NURS 209 Scholarly Assignment

ID: 823-435-053

Robinson, Goss, Mogayzel, Willey-Courand, Beth, Bujan, Finder, Lester, Quittell, Rosenblatt, Vender,
Hazle, Sabadosa, & Marshall, 2007). Nonetheless, the most consequential impact of not minimizing
pulmonary exacerbations related to thick mucous secretions is death as a result of respiratory failure.
Nurses, therefore, play a vital role and are an integral component to the pediatric patients plan of care and
overall health.
The complications that come from ineffective removal of mucopurulent secretions trapped in the
respiratory airway are caused by the pooling of these secretions, making the lung field a reservoir for
bacteria (Perry et al., 2013). A nurse must be able to recognize significant findings within the respiratory
assessment and monitor lab values and blood gases to detect pulmonary infection. Geller et al. (2009)
states that CF patients are absent of symptoms that are typical to pulmonary infection, such as fever,
tachypnea, and chest pain. Therefore, nurses being care providers should inform the practitioner if there is
presence of weight loss, anorexia, and fatigue. Evidence shows that informed nurses who are able to detect
these characteristics of pulmonary infections in the CF patient appropriately early on, decrease the amount
of lung destruction occurred due to infection, and ultimately reduce the number of hospital visits in their
lifetime (Geller et al., 2009).
Dehydrated, thickened secretions cause increased work of breathing resulting in the CF patient to
have chronic fatigue (Flume, Mogayzel, Robinson, Goss, Rosenblatt, & Kuhn, 2009). The use of an
incentive spirometer is recommended for the CF patient to promote optimal chest expansion, increase
inspiration and expiration capacity, decrease work of breathing, and help promote the patient to
expectorate mucosal secretions effectively (Lewis, Dirsken, Heitkemper, Bucher, Camera, Barry,
Goldsworthy, & Goodridge, 2014). Supportive studies have shown that patients who are able to maintain
the ability to expectorate secretions and manage their breathing capacity through incentive spirometry
interventions have less hospital visits and decreased respiratory complications that are associated with the
disease. Encouraging patient ambulation and correct coughing techniques also decreases pulmonary
infection related to mucous secretions. Nurses reduce pulmonary exacerbations in their patients by
including these respiratory interventions in their nursing process.
As stated, individuals with CF have difficulty clearing pathogens from the lung field and experience
chronic pulmonary infections and inflammation (Flume et al, 2007). Pharmacotherapy is at the forefront of
treatment to reduce mucosal secretions and respiratory infections in CF patients. Therapies, such as
bronchodilators, antibiotics and corticosteroids, have resulted in great improvements in length and quality

NURS 209 Scholarly Assignment

ID: 823-435-053

of life (Flume et al., 2007). Appropriate medication administration/ intervention by the nurse reduces
pulmonary exacerbations related to mucopurelent secretions within the lungs. Flume et al. (2007) states
that interventions such as pharmacotherapy have proven to increase the median expected survival age to
48.1, as opposed to the 24-year statistic back in the 1980s.
Along with respiratory dysfunction, CF is a multi-organ disease impairing the gastrointestinal,
genitourinary, neurological, and reproductive systems (Perry et al., 2013). There are different clinicians that
provide care for these problems along with nurses, such as CF specialist physicians, respiratory therapists,
chest physiotherapists, dieticians and social support workers. The CF pediatric patient is known to have this
multidisciplinary health care team throughout their entire life and is dependent on this collaborative care.
This collaborative care is exhibited as well to enhance the effectiveness of respiratory interventions made
by the nurse.
Pulmonary exacerbations and infections are prevented also with the help of a chest physiotherapist
(CPT). The CPT is involved in the routine plan of care and is integral to maintaining pulmonary hygiene of
the patient (Perry et al., 2013). Therapy is done twice daily, and although it is time consuming for both
child and family, patients have been found to regress faster when conventional this therapy is discontinued.
The CPT, being the corner stone of CF treatment, helps to decrease pulmonary exacerbations related to
secretions by using Flutter mucus clearance devices, handheld percussors, and high-frquency chest
compression (Perry et al., 2013). Another important collaborative health care team member that is required
to assist with pulmonary deficits is a Registered Dietician. Children with CF require a well-balanced, highprotein, high-caloric diet (Perry et al., 2013). In the patient with minimal pulmonary disease, energy
requirements (calories) up to 5 to 10% above the recommended daily allowances are necessary and for
patients with severe pulmonary disease/complications, requirements may be between 20 to 50% or more
(Flume et al., 2009). Adequate diet is imperative and children with CF experience frequent anorexia due to
the copious amounts of mucous produced and expectorated, causing dieticians to implement gastrointestinal
feeds to supplement their growth (Perry et al., 2013). Lastly, another important care provider that assists
nurses with pulmonary exacerbations is the Respiratory Therapist (RT). RTs play a key role in the care of
CF patients in the outpatient clinics, home care, and hospitals. They provide interventions including simple
oxygen therapy, noninvasive ventilation, aerosol delivery administration and airway clearance while also
providing education to patients and families (Newton, 2009). It is evident that along with nursing

NURS 209 Scholarly Assignment

ID: 823-435-053

interventions, there is a multidisciplinary health care team that provides collaborative care for the CF
patient population for pulmonary exacerbation related to mucus secretions.
Furthermore, there are many community resources within the GTA readily available for patients
and families with children diagnosed with CF. The leader in pediatric health care the Hospital of Sick
Children has a cystic fibrosis outpatient clinic that is dedicated to provide multidisciplinary support to
patients from birth to 17 years, consisting of pediatric respirologists, gastroenterologists, a specialized
nurse-practitioner, clinic nurses, dietician, physiotherapist, social worker and psychologist (SickKids,
2015). CF is a disease that requires management throughout the entire lifespan, and treatment can become a
financial burden and very time consuming. Along with outpatient clinics such as the one mentioned, there
are resources to help families and patients suffering from CF, such as The Specialty Food Shop, and the
Home Oxygen Program. The Specialty Food Shop provides CF patients/ families with particular enzymes,
vitamins, and nutritional supplements that are prescribed and covered by the Special Drugs Program in
Ontario (Cystic Fibrosis Canada, 2012). While, the Home Oxygen Program provides financial assistance to
purchase compressors, percussors, nebulizers, postural drainage boards, oxygen, and equipment to
individuals with CF that get home care. Up to 75% of the vendors selling price up to a set maximum
amount is covered for the equipment for qualified patients (Cystic Fibrosis Canada, 2012).
In summary, CF is a lifelong disease that affects multi-organ systems. However, unresolved
pulmonary exacerbations related to mucus secretion build up result in pulmonary infections that can, with
poor management, lead to respiratory failure, and ultimately premature death. It is imperative that nurses
involved in care with CF pediatric patients make respiratory interventions a priority within the nursing
process. It is clear that without pulmonary intervention, consequences related to the disease may occur to
the pediatric patient causing a deceased quality of life and reduce the childs longevity of life. Individuals
with CF require collaborative care and this is imperative to provide adequate care. Within the
multidisciplinary health care team the CPT, dietician and respiratory therapist have vital roles in reducing
abundantly, thickened mucous secretions. However, as therapies develop through research, the medical
regimen becomes increasingly burdensome in time, financial, and health resources. Fortunately, there are
community resources available in the GTA that help patients and families with the troubles of the disease.
Understanding that there is no cure for cystic fibrosis, health care professionals, such as nurses, are integral
in providing holistic care that fosters client well-being and promotes optimal functioning while living with
this condition. Decreasing pulmonary exacerbations related to mucus secretions does precisely that.

NURS 209 Scholarly Assignment

ID: 823-435-053

WORKS CITED

Cystic Fibrosis Canada. (2012). The Guide: Resources for the CF Community. Retrieved November 14,
2015, fromhttp://www.cysticfibrosis.ca/wpcontent/uploads/2013/09/theguide_resources_cf_
2011_en.pdf
Flume, P. A., O'Sullivan, B. P., Robinson, K. A., Goss, C. H., Mogayzel, P. J., Jr, Willey-Courand, D. B.,
Beth, D., Bujan, J., Finder, J., Lester, M., Quittell, L., Rosenblatt, R., Vender, R., Hazle, L.,
Sabadosa, K., & Marshall, B. Cystic Fibrosis Foundation, Pulmonary Therapies Committee. (2007).
Cystic fibrosis pulmonary guidelines: Chronic medications for maintenance of lung health.
American Journal of Respiratory and Critical Care Medicine, 176(10), 957-969.
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Flume, P. A., Mogayzel, P. J., Jr, Robinson, K. A., Goss, C. H., Rosenblatt, R. L., & Kuhn, R. J. Clinical
Practice Guidelines for Pulmonary Therapies Committee. (2009). Cystic fibrosis pulmonary
guidelines: Treatment of pulmonary exacerbations. American Journal of Respiratory and Critical
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Geller, D. E., & Rubin, B. K. (2009). Respiratory care and cystic fibrosis. Respiratory Care, 54(6), 796800. doi:10.4187/002013209790983241
Lewis, S., Dirsken, S., Heitkemper, M., Bucher, L., Camera, I., Barry, M., Goldsworthy, S., Goodridge, D.
(2014). Medical-surgical nursing in Canada: Assessment and management of clinical problems.
(3rd Canadian ed.). Toronto: Mosby Elsevier Canada.
Newton, T. J. (2009). Respiratory care of the hospitalized patient with cystic fibrosis. Respiratory Care,
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Perry, S., Hockenberry, M., Lowdermilk, D., Wilson, D., Sams, C. (2013). Maternal Child Nursing Care in
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SickKids. (2015). Cystic Fibosis Clinic. Retrieved on November 15, 2015, from
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