An Informative Article on Cystic Fibrosis and Bronchodilators

This report provides a comprehensive overview of cystic fibrosis, a genetic disease characterized by excessive mucus production in the respiratory tract, resulting from mutations in the CFTR gene. It explains the pathophysiology, detailing how defective CFTR protein leads to impaired ion transport, mucus buildup, and respiratory distress. The report focuses on pharmacological interventions, particularly bronchodilator drugs like salbutamol, which widen airways by relaxing smooth muscle cells. It discusses the mechanism of action, indications, contraindications, and potential side effects of salbutamol. Additionally, the report highlights alternative therapies such as exercise and proper nutrition. The target audience for this article is patients with cystic fibrosis and their families, and healthcare professionals. The report emphasizes the importance of understanding the disease and its management, and its relevance to nursing practice. The report concludes by summarizing the key points, including the genetic basis of cystic fibrosis, the role of bronchodilators, and the importance of lifestyle interventions for improving the quality of life for affected individuals.

Running head: CYSTIC FIBROSIS AND BRONCHODILATORS
CYSTIC FIBROSIS AND BRONCHODILATORS
Name of the Student:
Name of the University:
Author Note:

1CYSTIC FIBROSIS AND BRONCHODILATORS
Abstract:
This lay article aims forproviding a brief understanding of the cystic fibrosis disease
condition and the use of bronchodilator drugs to the patients and their families. The cystic
fibrosis is a disease condition that involves excessive mucus production in the respiratory
tract. The disease results from a genetic mutation in both copies of CFTR genes in an
individual.The CFTR gene encodes the CF Transmembrane Conductance Regulator (CFTR)
protein in the human chromosome, which is involved in the ion transport event across the
epithelial membranes. A mutation in both copies of CFTR genes results in a defect in the
CFTR protein activity. As a result, the ion transport event across the epithelial cell membrane
also becomes defective, which in turn promotes excess mucus production and mucus
retention. The thick sticky mucus creates a blockage in the respiratory airways, and that is the
reason for the cystic fibrosis individuals to experience the symptoms of respiratory distress.
There is no absolute cure for this disease condition. The pharmacological interventions for
the treatment of this disease condition are addressed at widening the airways, lowering the
inflammation and lowering the mucus production. The bronchodilator drugs, such as
salbutamol widen the airways by promoting the relaxation of the smooth muscle cells in the
respiratory wall.Apart from the pharmacological interventions, few other interventions can
also be followed by the patients of cystic fibrosis to promote their health and well-being,
which can be included following a proper diet chart and an exercising routine.

2CYSTIC FIBROSIS AND BRONCHODILATORS
Brief Overview:
This paper aims to provide a brief understanding of the cystic fibrosis disease
condition and the use of bronchodilator drugs to the patients and their families.Cystic fibrosis
is a disease condition that arises from a genetic default. The presence of a mutation in both of
the CFTR gene in the children is considered to be the reason for the disease (Cutting, 2015).
The children are mainly born with this condition to the parents who are the carriers of the
mutated CFTR gene. The condition can be diagnosed in the children at the early stage of their
lives. The possible symptoms in the children include a frequent phenomenon of encountering
infections in the lungs, episodes of persistent coughing with thick mucus, lower growth rate,
persistent feeling of breathlessness, frequent event of the sinus infections and various others
(Brill, Patel, Singh, Mackay, Brown & Hurst, 2015).This disease condition is associated
withexcessive production of mucous. The mucus is also very thick and sticky. The condition
is associated with a blockage in the respiratory system and continuous damage to the lungs.
That is why the symptom of respiratory distress is so common in this disease condition
(Goodfellow,Hawwa, Reid, Horne, Shields &McElnay, 2015). There is no permanent cure
for this condition. However, airway clearance techniques (ACT) are common treatment
methods for this condition. The use of bronchodilator drugs can be considered as one of those
ACT therapies.
Pathophysiology:
The CFTR gene encodes the CF Transmembrane Conductance Regulator (CFTR)
protein in the human chromosome. The protein is involved in the ion transport event across
the epithelial membranes (Haq, Gray, Garnett, Ward&Brodlie, 2016). The chloride ions and
the sodium ions are mainly transported through this mechanism. A mutation in a single CFTR
gene cannot display a severe effect since another normal gene can supplement the effect.

3CYSTIC FIBROSIS AND BRONCHODILATORS
However, if both of the CFTR genes are defective in an individual, the CFTR protein activity
becomes defective as well. There is a defect in the ion transport event across the epithelial
cell membrane. As a result, there is a depletion in the airway surface liquid, which plays an
important role to support the ciliary function and stability. The ciliary function includes the
management of mucus in the respiratory tract and thus, a ciliary collapse result in a prolonged
mucosal retention event. The event increases the rate of infection and inflammation, which
again is associated with increased mucus production in an individual (Bergeron &Cantin,
2019). The thick sticky mucus produced by this vicious cycle creates a blockage in the
respiratory airways, and that is the reason for the cystic fibrosis individuals to experience the
symptoms of respiratory distress (Refer to Figure 1).
Figure 1: Pathophysiology of Cystic Fibrosis
Source: (Firth et al., 2015)

4CYSTIC FIBROSIS AND BRONCHODILATORS
Pharmacology related to the treatment:
There is no absolute cure for this disease condition (Bosch & De Boeck, 2016).
However, there are many treatment strategies for improving the quality of life in patients. The
treatment approaches are mostly focused on lowering the mucus production and mucus
retention in the respiratory airways, the reduction of the inflammatory response, widening the
airways by using pharmacological interventions (Proesmans, 2017). The use of
bronchodilator drugs can be considered to be one of those most common pharmacological
interventions. The mechanism of action of this drug involves widening the respiratory
airways by relaxing the smooth muscle cells of the respiratory wall. There is a swelling event
result from the inflammatory reactions. As a result, there the smooth muscle cells are unable
to relax, and there is additional pressure on the respiratory airways from the swelled
respiratory wall. The bronchodilator drugs bind to a receptor present in the smooth muscle
cells, named beta-adrenergic receptors (Almadhoun& Sharma, 2019). The binding results in
an increased level of cAMP level, which is a secondary messenger that creates a signalling
chain promoting the relaxation of the smooth muscle cells (Refer to Figure 2).
Figure 2: Mechanism of Action of the Bronchodilator Drugs
Source: (Dr Matt & Dr. Mike., 2018)

5CYSTIC FIBROSIS AND BRONCHODILATORS
An example of such bronchodilator drug, which is commonly used in cystic fibrosis is
salbutamol drug. That is the generic name of the drug, and it is available under many brand
names such as Proventil and Ventolin in the market. The IUPAC name of the drug is 4-[2-
(tert-butylamino)-1-hydroxyethyl]-2-(hydroxymethyl)phenol
(Pubchem.ncbi.nlm.nih.gov.,2020).
The mechanism of action of this drug is similar to any other bronchodilators, as
mentioned above. There are many routes of administrations used for this drug. It can be taken
orally in the form of a tablet; it can be inhaled in the form of liquid, and it can also be
administered intravenously by injection. The indication for this drug use is to lower the
hyperkalemia condition (or high potassium level) and to provide relief to the patients with
bronchospasm conditions, such as in cystic fibrosis (Ullmann, Caggiano&Cutrera, 2015). The
contraindications for this drug use is the patients who have hypokalemia condition (which is
the medical term for indicating a low potassium level in the patient) or are allergic to this
drug (Cock, 2015).
There are also a few side effects of this drug use that the patients should be aware of.
The patients should be monitored for displaying tremors, a higher heart rate (tachycardia),
hypokalemia and other symptoms. These are the indicators for the side effects of the
salbutamol drug (Hostrup, Kalsen, Auchenberg, Bangsbo& Backer, 2016).
The salbutamol drug also interacts with a few other drugs. Thus the patients must
inform their physicians or healthcare staffs if they are already taking the drugs like
corticosteroids, diuretics, theophylline or other beta-blocker drugs. There is a higher risk of
the patient developing hypokalemia condition if a high dose of corticosteroid or diuretics or
theophylline is being taken with the salbutamol drug (Dopour, Hosseini, Kanki,

6CYSTIC FIBROSIS AND BRONCHODILATORS
Davoodpour,Srinivas&Shekar, 2016). The use of other beta-blocker drugs may simply inhibit
the effect of the salbutamol drug (Rodríguez-Álvarez, Rodil, Quintana &Cela, 2015).
Alternative Therapies:
Apart from the pharmacological interventions, there area few other interventions that
can be followed by the patients of cystic fibrosis to promote their health and well-being. It
has been found from the studies that the intervention of exercising daily can be an effective
measure for the patients with cystic fibrosis (Hebestreit, Kriemler&Radtke, 2015). Exercising
improves the endurance of the respiratory muscles and the tolerance of the muscle. The
aerobic exercises are considered to be sufficiently effective in improving the functions of the
lungs. Thus maintenance of a regular exercising routine can be considered to have a
considerable clinical benefit for improving the quality of life in the patients with cystic
fibrosis(Hebestreit, Kriemler&Radtke, 2015).
The nutrition or the eating habit also has a significant role in promoting the well-being
of cystic fibrosis patients. The cystic fibrosis patients are considered to be in requirement of a
higher calorie from their food, since they require more energy for breathing, inhibiting the
infection in the respiratory tract (Hollander, de Roos&Heijerman, 2017). They also require
vitamin supplement and enzyme supplement for better disease management in those patients.
Maintenance of the proper diet is also necessary for the cystic fibrosis condition since it is
often associated with malnutrition conditions.
Relevance to the nursing practice and the Target Audience:
It is very important for the nurses and the healthcare workers to have sufficient
knowledge of the disease condition and the relevant pharmacological and non-
pharmacological interventions associated with the disease condition. A disease condition
such as the cystic fibrosis presents many challenges to the health of the patients. The

7CYSTIC FIBROSIS AND BRONCHODILATORS
challenges include the successful management of the direct symptoms of the condition andthe
associated health risks to the patients, such as the possibility of lung infections and
malnutrition.Hence the nursing staffs should possess proper knowledge on the matter to
develop an appropriate care plan for the patient, which will include many interventions
addressing every aspect of the disease condition(Gravelle, Paone, Davidson &Chilvers,
2015).
The target audience of this article is the patients with cystic fibrosis disease condition
and their families. The knowledge is important for the patients to improve their efficacy in
the self-management of the disease condition (Sawicki, Heller,Demars& Robinson, 2015).
The family of the patients are often the primary caregivers to the patients. Thus it is also
necessary for them to understand the pathophysiology of the disease and the possible
interventions for successful disease management in the patients.
Conclusion:
Hence, it can be concluded from the above discussion that the cystic fibrosis is a
disease condition that involves excessive mucus production in the respiratory tract. The
disease results from a genetic mutation in both copies of CFTR genes in an individual. A
child born to the parents, both of whom are the carriers of CF, has a high chance of
developing the disease. The pharmacological interventions for the treatment of this disease
condition are addressed at widening the airways, lowering the inflammation and lowering the
mucus production. The bronchodilator drugs, such as salbutamol widen the airways by
promoting the relaxation of the smooth muscle cells in the respiratory wall. The other
interventions for improving the quality of life in cystic fibrosis patients include following a
proper diet chart and an exercising routine.

8CYSTIC FIBROSIS AND BRONCHODILATORS
References:
Almadhoun, K., & Sharma, S. (2019). Bronchodilators. In StatPearls [Internet]. StatPearls
Publishing.https://www.ncbi.nlm.nih.gov/books/NBK519028/
Bergeron, C., &Cantin, A. M. (2019, October). Cystic Fibrosis: Pathophysiology of Lung
Disease. In Seminars in respiratory and critical care medicine. Thieme Medical
Publishers.DOI: 10.1055/s-0039-1694021
Bosch, B., & De Boeck, K. (2016). Searching for a cure for cystic fibrosis. A 25-year quest in
a nutshell. European journal of pediatrics, 175(1), 1-
8.https://link.springer.com/article/10.1007/s00431-015-2664-8
Brill, S. E., Patel, A. R., Singh, R., Mackay, A. J., Brown, J. S., & Hurst, J. R. (2015). Lung
function, symptoms and inflammation during exacerbations of non-cystic fibrosis
bronchiectasis: a prospective observational cohort study. Respiratory research, 16(1),
16.https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-015-0167-
9
Cock, I. E. (2015). The safe usage of herbal medicines: counter-indications, cross-reactivity
and toxicity. Pharmacognosy
Communications, 5(1).https://core.ac.uk/download/pdf/143900189.pdf
Cutting, G. R. (2015). Cystic fibrosis genetics: from molecular understanding to clinical
application. Nature Reviews Genetics, 16(1), 45-
56.https://www.nature.com/articles/nrg3849
Dopour, A., Hosseini, F., Kanki, K., Davoodpour, S., Srinivas, C., &Shekar, H. (2016).
Prospective study on identification and assessment of potential drug-drug interactions
in emergency medicine department at KIMS hospital and research

9CYSTIC FIBROSIS AND BRONCHODILATORS
centre. International Journal of Research in Pharmacy and Science, 6(1), 36-
39.http://www.ijrpsonline.com/pdf/6008.pdf
Dr Matt & Dr. Mike. (2018). Salbutamol (Ventolin): Mechanism of Action. youtube.com.
Retrieved 17 April 2020, from https://www.youtube.com/watch?v=VKatFB1SEtI.
Firth, A. L., Menon, T., Parker, G. S., Qualls, S. J., Lewis, B. M., Ke, E., ... & Verma, I. M.
(2015). Functional gene correction for cystic fibrosis in lung epithelial cells generated
from patient iPSCs. Cell reports, 12(9), 1385-1390.
https://doi.org/10.1016/j.celrep.2015.07.062
Goodfellow, N. A., Hawwa, A. F., Reid, A. J., Horne, R., Shields, M. D., &McElnay, J. C.
(2015). Adherence to treatment in children and adolescents with cystic fibrosis: a
cross-sectional, multi-method study investigating the influence of beliefs about
treatment and parental depressive symptoms. BMC pulmonary medicine, 15(1),
43.https://link.springer.com/article/10.1186/s12890-015-0038-7
Gravelle, A. M., Paone, M., Davidson, A. G. F., &Chilvers, M. A. (2015). Evaluation of a
multidimensional cystic fibrosis transition program: a quality improvement
initiative. Journal of Pediatric Nursing, 30(1), 236-
243.https://doi.org/10.1016/j.pedn.2014.06.011
Haq, I. J., Gray, M. A., Garnett, J. P., Ward, C., &Brodlie, M. (2016). Airway surface liquid
homeostasis in cystic fibrosis: pathophysiology and therapeutic targets. Thorax, 71(3),
284-287.http://dx.doi.org/10.1136/thoraxjnl-2015-207588
Hebestreit, H., Kriemler, S., &Radtke, T. (2015). Exercise for all cystic fibrosis patients: is
the evidence strengthening?. Current opinion in pulmonary medicine, 21(6), 591-
595.doi: 10.1097/MCP.0000000000000214

10CYSTIC FIBROSIS AND BRONCHODILATORS
Hollander, F. M., de Roos, N. M., &Heijerman, H. G. (2017). The optimal approach to
nutrition and cystic fibrosis: latest evidence and recommendations. Current opinion in
pulmonary medicine, 23(6), 556-
561.https://doi.org/10.1097/MCP.0000000000000430
Hostrup, M., Kalsen, A., Auchenberg, M., Bangsbo, J., & Backer, V. (2016). Effects of acute
and 2‐week administration of oral salbutamol on exercise performance and muscle
strength in athletes. Scandinavian journal of medicine & science in sports, 26(1), 8-
16.https://doi.org/10.1111/sms.12298
Proesmans, M. (2017). Best practices in the treatment of early cystic fibrosis lung
disease. Therapeutic advances in respiratory disease, 11(2), 97-
104.https://dx.doi.org/10.1177%2F1753465816680573
Pubchem.ncbi.nlm.nih.gov. (2020). Salbutamol. Pubchem.ncbi.nlm.nih.gov. Retrieved 8
April 2020, from https://pubchem.ncbi.nlm.nih.gov/compound/Salbutamol.
Rodríguez-Álvarez, T., Rodil, R., Quintana, J. B., &Cela, R. (2015). Reactivity of β-
blockers/agonists with aqueous permanganate. Kinetics and transformation products
of salbutamol. Water research, 79, 48-56.https://doi.org/10.1016/j.watres.2015.04.016
Sawicki, G. S., Heller, K. S., Demars, N., & Robinson, W. M. (2015). Motivating adherence
among adolescents with cystic fibrosis: youth and parent perspectives. Pediatric
Pulmonology, 50(2), 127-136.https://doi.org/10.1002/ppul.23017
Ullmann, N., Caggiano, S., &Cutrera, R. (2015, December). Salbutamol and around.
In Italian journal of pediatrics (Vol. 41, No. 2, p. A74). BioMed
Central.https://ijponline.biomedcentral.com/articles/10.1186/1824-7288-41-S2-A74