Pharmacological Management of Cystic Fibrosis and its Associated Nursing Implications

Pharmacological Management of Cystic Fibrosis and its Associated Nursing Implications


Cystic fibrosis is one of the most dangerous genetic disorders affecting white population. The goal of this paper is to review available pharmacological treatments and their implications for nurses. The paper includes a description of a chronic pulmonary treatment, anti-inflammatory agents, and antibiotics. The effectiveness of therapies, which is curbing gastrointestinal manifestations of cystic fibrosis, is reviewed. The paper includes a discussion of nursing implications of the pharmacological treatments for cystic fibrosis.

Keywords: cystic fibrosis, pharmacological, antibiotics, anti-inflammatory, nursing.

Cystic fibrosis remains one of the most challenging health conditions that young patients face. The disease develops as a result of a genetic dysfunction. The diagnosis is the most prevalent among white European populations, although the actual incidence and prevalence of cystic fibrosis varies considerably among the countries (O’Sullivan & Freedman, 2009). The overall purpose of treatment of cystic fibrosis is maintaining an optimal lungs’ function. Unfortunately, the contemporary medicine lacks any knowledge of how cystic fibrosis can be successfully cured. The current state of pharmacological treatment for cystic fibrosis has profound implications for nursing care: cystic fibrosis specialist nurses must consider the multifaceted nature of this disease, while being ready to adopt emerging pharmacological strategies and ensure the patients’ adherence to the prescribed treatment regimens.

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Cystic Fibrosis: A Brief Insight

In order to understand how nurses should approach some pharmacological treatment strategies for cystic fibrosis, the very nature of the disease needs to be reviewed. According to O’Sullivan and Freedman (2009), cystic fibrosis is the most prevalent lethal genetic disorder among white population. Over the past years, life expectancy for patients with cystic fibrosis has grown from 31 to 37 years, but it is still too early to speak about realistic cures (O’Sullivan & Freedman, 2009). The pathophysiological roots of the disease can be found in the mutation of a gene “that encodes fibrosis transmembrane conductance regulator (CFTR) protein, which is expressed in many epithelial cells and blood cells” (O’Sullivan & Freedman, 2009, p. 1891). The most problematic aspect of cystic fibrosis in relation to pharmacology is that its symptoms and manifestations can vary considerably between patients (O’Sullivan & Freedman, 2009). Consequently, no pharmacological strategy in cystic fibrosis can be regarded as universally effective. At the same time, nurses should not hope to cure cystic fibrosis: even the most aggressive pharmacological treatment is aimed at achieving and maintaining the optimal lung function in patients over their lifespan.

Pharmacological Management of Cystic Fibrosis

Chronic Pulmonary Treatment

A chronic pulmonary treatment remains the foundational element of the pharmacological strategies used to deal with cystic fibrosis. The purpose of these pharmacological approaches is to reduce the risks of pulmonary exacerbations while improving the patients’ wellbeing and quality of life in a long-term perspective. In this sense, aerosolized antibiotics remain the most common pharmacological solution for cystic fibrosis. The importance of aerosolized antibiotics and their efficacy are justified by the emerging evidence that they can curb an initial infection and suppress a chronic one and related complications (Flume et al., 2007). Tobramycin remains the most common aerosolized antibiotic recommended for patients with cystic fibrosis (Flume et al., 2007; O’Sullivan & Freedman, 2009); it is particularly useful for patients who are older than 6 with mild to severe forms of the disease (Flume et al., 2007). Certainly, nurses can also recommend other inhaled antibiotics, such as colistin. However, the clinical evidence to support its positive effects on the lung function is quite scarce (Flume et al., 2007). Pharmacological strategies to curb cystic fibrosis can also incorporate the use of recombinant human deoxyribonuclease, inhaled hypertonic saline, ibuprofen, and chronic azithromycin as well as inhaled beta-agonists (O’Sullivan & Freedman, 2009).

A special attention should be paid to the use of recombinant DNase in patients with cystic fibrosis. It can be used by nurses to “improve the viscoelastic properties of airway secretions and promote airway clearance” (Flume et al., 2007, p. 960). Positive effects of DNase on the patients’ lung function in the short and long runs have been widely established (Flume et al., 2007). Recommendations regarding the dosage and length of administration should be developed basing on the severity of the disease. Hypertonic saline can be used as a supplementary mechanism to facilitate airway hydration, leading to improved mucociliary clearance (Flume et al., 2007). In fact, it is believed to be effective not only in relation to symptoms but also a genetic defect that underlies cystic fibrosis (O’Sullivan & Freedman, 2009).

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Anti-Inflammatory Agents and Corticosteroids

The use of inhaled corticosteroids remains one of the most controversial aspects of pharmacological management in cystic fibrosis. Flume et al. suggest that the use of corticosteroids can be justified by the fact that cystic fibrosis is a disease of inflammatory nature. Excessive inflammatory responses among the patients’ airways are not uncommon. Chronic inflammation is claimed to result in the destruction of airways, leading to the development of an obstructive airway disease (Flume et al., 2007). However, the evidence to confirm the efficacy and safety of corticosteroids against inflammation in cystic fibrosis is far from being sufficient (Flume et al., 2007; O’Sullivan & Freedman, 2009). Nurses should know that the Cystic Fibrosis Foundation recommends against the use of oral corticosteroids in children and adults (Flume et al., 2007). In this context, nonsteroidal anti-inflammatory drugs, such as inhaled ibuprofen, can be used since they increase the patients’ chances to preserve the optimal lung function (Flume et al., 2007). These pharmacological solutions are relatively inexpensive, have few side effects, and are readily available to patients (O’Sullivan & Freedman, 2009). Nurses should also pay the particular attention to the benefits of macrolide antibiotics, which have proved to be effective in patients with cystic fibrosis. Macrolide antibiotics can be administered to reduce the risks and frequency of pulmonary exacerbations (O’Sullivan & Freedman, 2009). They are known for their effects on the cytokine production, altering polymorph nuclear cell function and turning into a relevant anti-inflammatory agent (O’Sullivan & Freedman, 2009).

Pulmonary Exacerbations and Infections

One of the biggest problems that nurses face in relation to cystic fibrosis is that it is a multifaceted disease that has many symptoms and signs. As a result, nurses often have to focus on more than one symptom, each requiring a different pharmacological approach and a treatment strategy. Pulmonary infections and exacerbations require the development of effective pharmacological models. Cystic fibrosis is normally associated with a unique range of pathogens, which are acquired at different stages of the disease (Gibson, Burns & Ramsey, 2003). The pharmacological management of cystic fibrosis necessarily includes the elements of appropriate antibiotic therapies targeting bacterial pathogens in a patients’ respiratory tract (Gibson et al., 2003). Antipseudomonal antibiotics can help prevent the risks of Pseudomonas aeruginosa infections in patients with cystic fibrosis (Gibson et al., 2003). Antistaphylococcal antibiotics and their effects on patients with cystic fibrosis raise a number of questions. Therefore, nurses should consider the use of alternative non cell active antibiotics with a critical eye (Gibson et al., 2003).

One of the chief concerns about the chronic use of antibiotics in the pharmacological management of cystic fibrosis is the gradual emergence of antibiotic resistance. Unfortunately, today, researchers are not aware of the real risks of antibiotic resistance in cystic fibrosis and the best possible ways to avoid those (Gibson et al., 2007). George, Jones, and Middleton (2009) speak about some alternative combinations of antibiotic and non-antibiotic agents. Unfortunately, the present-day pharmacological industry focuses predominantly on the development of new antibiotics from the existing drug classes rather than the development of the entirely new drug modes. The existing antibiotics cannot help nurses and physicians to overcome the risks of methicillin-resistant bacterial infections in patients with cystic fibrosis (George et al., 2009). In certain conditions, monotherapy with antibiotics may become a good option for nurses and their patients, but they should not expect that these therapies will do anything beyond the alleviation of symptoms.

Gastrointestinal Manifestations and Emerging Therapies

Some scientists state that “cystic fibrosis is a complex disease that requires more than a single approach to alleviate the causes and symptoms and the complications that accompany them” (George et al., 2009). Therefore, nurses should be prepared to deal with the existing and emerging manifestations of the disease among different patients. For instance, Brodzicki, Trawinska, and Korzon offer some recommendations to manage gastroesophageal reflux in children with cystic fibrosis. The frequency and severity of gastoesophageal reflux suggests that it can readily aggravate a physical and emotional state of children with cystic fibrosis (Brodzicki et al., 2002). In this case, cisapride or a combination of cisapride and ranitidine can be used to reduce the frequency of reflux episodes and their duration in children (Brodzicki et al., 2002). The proposed pharmacological treatments have proved to improve the patients’ endoscopic picture (Brodzicki et al., 2002).

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Certainly, not all pharmacological treatments are equally effective. Due to the complexity of the disease, nurses should be aware of the emerging methodologies as well as the factors which can potentially impact the effectiveness of their management propositions. Jones and Helm describe a number of medications that can be used in the treatment of cystic fibrosis but currently undergo pharmacological tests. These include some dry-powder versions of tobramycin, colistin, and ciprofloxacin as well as the new formulations of amikacin, ciprofloxacin, azteonam, and fosfomycin (Jones & Helm, 2009). In addition, it is not enough to develop a promising treatment strategy. Much more important is to monitor the patients’ compliance with the prescribed regimens. Nurses should remember that cystic fibrosis is a genetic disorder diagnosed early in life. Many children with cystic fibrosis experience difficulties with the adherence to treatment (Modi & Quittner, 2006). The complexity of the disease and its pharmacological implications place nurses into the center of disease management and health maintenance. Nurses should be ready to make fast and grounded decisions that will help patients to achieve and maintain an optimal level of health.

Implications for Nurses

Pharmacological management of cystic fibrosis has profound implications for nurses. As mentioned earlier, due to the complexity of the disease, nurses should be ready to deal with its multiple signs and manifestations. Kerem, Conway, Elborn, and Heijerman (2005) specify the general standards of care for nurses working with patients diagnosed with cystic fibrosis. According to Kerem et al. (2005), specialist nurses must advocate for the interests of every patient, be aware of the most recent treatment strategies, develop and maintain the patient’s and family’s liaisons, and provide support and advice to patients. Obviously, most treatments of cystic fibrosis are aimed at alleviating the symptoms rather than treating the underlying genetic defect (Flume et al., 2007). However, nurses must ground their pharmacological decisions on the most relevant and valid clinical evidence. More importantly, they should monitor the emergence of new pharmacological treatments and, at the same time, monitor patients’ compliance with the prescribed treatment regimens. Given the ambiguity surrounding many pharmacological treatments, nurses must make a final decision based on the best interests of each patient.


The use of pharmacological interventions against cystic fibrosis has far-reaching implications for nursing care. Nurses are expected to play one of the central roles in providing the quality pharmacological treatment to patients with cystic fibrosis. Apart from developing unique pharmacological approaches, nurses must ground their treatment decisions on the clinical and empirical evidence. It should be noted that the pharmacological treatment of cystic fibrosis cannot be simple. Due to the complexity of the disease, nurses often have to focus on more than one symptom of the disease, each requiring a different pharmacological approach. However, in all situations, nurses must understand that the proposed strategies will simply alleviate the symptoms of the disease. The final decision will have to be made basing on the best interests of every patient.

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