Running Head: CYSTIC FIBROSIS

Cystic Fibrosis: Priority Nursing Interventions with Collaborative Care and Community
Resources for Managing Ineffective Airway Clearance
Sadiq Adisa Bello
Humber College
Practical Nursing Theory 3 (NURS 209)
Submitted to: Janet Jeffery
19th November, 2015

Cystic fibrosis a genetic disease caused by mutation in a gene that

encodes cystic fibrosis transmembrane conductance regulator (CFTR) leading

Running Head: CYSTIC FIBROSIS

to abnormal production of mucus (Lewis et al, 2014 p.755). This mucus

occludes the airway, leads to infertility (endocrine function) and indigestion
of nutrient especially fatty foods. The disease progression cause the disrupts
organ function which makes the individual to be placed on long term
medications and frequent medical check as this disease cannot be cured, it
can only be managed through life term course of treatment involving
collaboration with various health care professionals (O Sullivan & Freedman,
2009 p.1900). The availability of support and preventive measures helps to
reduce the disease complication. Generally people with cystic fibrosis have
trouble clearing their airway due to thick mucus plug which if not expelled
are grounds for bacteria and virus to developed which leads to pulmonary
infection. Symptoms of cystic fibrosis presents itself as wheezing, coughing,
thick mucus sputum, asthma, shortness of breath, crackles in lungs and
cyanosis which needs immediate attention and remediation to prevent
further complication. The priority nursing intervention for a client
experiencing ineffective airway clearance is positioning the client in semi
fowler, administering of oxygen by nasal prongs and autogenic coughing to
clear mucus from the airway.
A client experiencing ineffective airway clearance presents with
shortness of breath, wheezing, crackles in lungs and often cyanosis due to
ineffective tissue perfusion. Assessment of such a patient will reveal low
oxygen saturation, presence of use of accessory muscle while breathing and
a high respiratory rate. Positioning the client in a semi or high fowler position

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allows for the airway to be opened which promotes continuous breathing

because lack of oxygen to the heart (ischemia) or brain could lead to
syncope the most effective position for patients with cardiopulmonary
diseases is the 45-degeree semi Fowlers position, using gravity to assist in
lung expansion and reduce pressure from the abdomen on the diaphragm
(Potter & Perry, 2014, p.920).
Also, administering 4L of oxygen by nasal prongs helps to supplement
oxygen deficiency being experienced by the patient as evident by cyanosis
and low oxygen blood saturation. Putting the client in semi fowler positions
affords for this second intervention as the position allows for continuous and
unobstructed air flow into the lungs thereby raising the level of oxygen in the
lungs, heart and peripheral vascular tissues and organs. If this nursing
intervention is not done a client could faint due to low oxygen to the brain,
the lungs could collapse due to mucus accumulation and the need for
emergent attention. Keeping the airway patent is always the first line of
action as breathing is among the physiological needs according to the
Maslow hierarchy of needs. Positioning the client in semi fowler and
administering oxygen by nasal prongs if not done or left unattended to could
lead to cardiac dysrhythmias and death (Potter & Perry, 2014, p.926).
Autogenic coughing to move mucus up the airway so that it can be
expelled out of the lungs is recommended for a patient experiencing
ineffective airway clearance. The process involves postural compressions and

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positions in moving up mucus out of the airway to facilitate breathing. Non

clearance of airway with oxygen therapy often reduce the level of oxygen
being administered and could compromise the health of the individual if
mucus plugs the airway.
The collaborative care for a patient with cystic fibrosis involves a
multidisciplinary team including nurses, physician, respiratory therapist,
physiotherapist, dietitians, pharmacist and social worker (Lewis et al, 2014
p.755). The important collaborative care for a patient experiencing
ineffective airway clearance involves a respiratory therapist, physiotherapist
and dietician. The job of a respiratory therapist is to educate and help and
the client achieve optimal breathing through practice of purse lip breathing,
incentive spirometry and techniques for postural drainage of mucus from the
lungs to prevent further complications of the disease such as pneumonia
The core responsibilities of RTs typically include: administering and teaching
about the proper use of inhaled medications; teaching how to maintain and
disinfect nebulizers and compressors; teaching airway-clearance techniques;
assessing adherence to the airway-clearance regimen and inhaled
medications; and promoting exercise (Marshall et al, 2009, p.794). The duty
of a physiotherapist to the pediatric client is to promote exercise and
activities that will increase lung capacity and aeration of the alveoli. Chest
physiotherapist helps the client with postural drainage of mucus by gravity
through using percussion and vibration to loosen up secretions in the airways
(Lewis et al, 2015, p.755). The responsibility of a dietician in this case is to

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educate and provide parent of client with food rich in antioxidants, adequate
fluid aeration and eating regular meals to maintain body mass index. The
dietician monitors the nutritional needs and supplements the clients may
need at a particular at interval after airway is cleared.
The community resources necessary to help with the nursing
intervention stipulated are Cystic fibrosis Canada, sick kids Canada and
Holland Bloorview kids rehabilitation hospital. All these organizations
provide families of cystic fibrosis patient with information about care and
research on curative measures on how to manage the disease effectively
without many complications Parents found routines difficult to establish,
used trial and error, encountered barriers, and found support helpful to
manage care demands (Grossoehme , Filigno & Bishop, 2014, p.125-135).
They also provide family with financial support in managing the disease,
financial aid include oxygen therapy and free enzymatic drugs to aid food
digestion. They provide learning modules on chest physiotherapy and how to
effectively use nebulizers and other inhalants medications. Families with
cystic fibrosis client can benefit from a whole lot by going to this organization
because they provide emotional support and hold support groups for
enlightenment to share other family views on how they have been coping
well with the disease The most challenging aspect of providing care for the
family of a child or adolescent with CF is meeting the emotional needs of the
child and family. The diagnosis, treatment, and prognosis for CF are often

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associated with many problems and frustrations (Perry, Lowdermilk &

Hockenberry , 2014, p.1366).
In conclusion, cystic fibrosis patient often have to deal with repetitive
course of treatment which can be a pain in the neck. Adhering to the course
of treatment without resentment is challenging and requires strict dedication
and emotional support from family and various healthcare providers. Families
have to seek support from foundations such as the Cystic Fibrosis Canada
and others to seek assistance and share their experience of living the
disease. Nursing interventions are aimed at reducing the disease
complication through collaboration with other health professionals such as
physiotherapist, respiratory therapist, dietitians, and pharmacist. The goal of
treatment is to promote compliance and encourage better coping
mechanism of dealing with the disease manifestations.

References
Grossoehme, D. H., Filigno, S. S., & Bishop, M. (2014). Parent routines
for managing
cystic fibrosis in children.Journal of Clinical
Psychology in Medical Settings, 21(2),
125-135.
doi:10.1007/s10880-014-9396-1
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Marshall, B. C., Penland, C. M., Hazle, L., Ashlock, M., Wetmore, D.,
Campbell, Preston,
W., III, & Beall, R. J. (2009). Cystic fibrosis
foundation: achieving the mission. Respiratory Care, 54(6), 788+.

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