Respiratory Cystic Fibrosis: Understanding the Condition and Treatment

This presentation provides a comprehensive overview of respiratory cystic fibrosis (CF), an inherited disorder affecting the body's water, salt, and mucus-producing cells. It delves into the anatomy of CF, focusing on how the CFTR gene mutation leads to thick mucus buildup in organs like the lungs, small intestine, and reproductive systems. The presentation further explains the physiology of CF, detailing how defects in the CFTR gene disrupt chloride transport and lead to infection and inflammation. Treatment methodologies, including medications like antibiotics and mucus-thinning agents, surgical procedures such as bowel surgery and lung transplants, and home care strategies, are discussed. Finally, it addresses the needs of patients and families affected by CF, emphasizing the importance of comprehensive care and support. This presentation is intended for educational purposes and provides a solid foundation for understanding respiratory cystic fibrosis.

Respiratory cystic fibrosis

Overview
Cystic fibrosis us the inherited disorder that is marked by the abnormality
in the human body's water, salt, and mucus-producing cells. The respiratory
CF is although not curable and gets worse with time (University of
Rochester medical centre2019). The quality treatment can help people to
live longer with the disorder most of this disorder live with this disorder
lives into their late 30s and majority into their 50s.

Anatomy of cystic fibrosis
CF causes thick mucus to develop and clog certain parts
of the human body such as lungs. This build-up is
produced by an atypical gene named the CFTR or cystic
fibrosis Trans-membrane controller. This CFTR regulates
the transportation of salt and H2O inside and outside of
the human body's cells.

Cont…
These are the little growth of tissue from the lining of the nose.
Nearly 30 000 kids and youngsters in the US and 70 000 persons
worldwide suffer from CF (Grief, 2008).
Some of the major body parts and systems specifically affected
by CF are;
Bronchioles
Small intestine
The male reproductive organs
cervical canal
The musculoskeletal system

Bronchioles; blockage
Small intestine; obstruction
The pancreatic ducts and bile ducts;
obstruction
female reproductive organs; cervical canal
obstruction
male reproductive organs; vas deference
obstruction
musculoskeletal system; weakness, atrophy

Physiology of CF
CF is generally triggered by the flaws in the gene of cystic
fibrosis, which normally function for the protein trans-
membrane conductance controller that works as the
chloride network and is controlled by the cyclic adenosine
monophosphate. Any type of mutation in the CFTR gene
may lead to defects of cAMP-monitored chloride carriage
through epithelial cells on the mucosal surfaces
(Lubamba, Dhooghe, Noel, & Leal, 2012).

Cont..
After that, the infection converts established with the
characteristic bacterial flora. The repeated cycle of the
contamination and neutrophilic swelling develops (Moran,
Becker, Casella, Gottlieb, Kirkman, Marshall, & CFRD
Consensus Conference Committee. (2010).

Treatment methodologies
Medication
Antibiotics might be prescribed by the doctor or
physicians to get rid of the lung infection in CF and o stop
another infection from occurring in the coming future. The
medicines are given in the form of tablets, liquids, or
capsules. In additional severe cases, the injection of
antibiotics is given intravenously or via veins.

Cont…
The mucus-thinning medications change the mucus thinner and
less sticky (Quittner, Modi, Wainwright, Otto, Kirihara, &
Montgomery, 2009). They can also help the patient to cough up the
mucus so theta it leaves the lungs and improves the functioning of
the lungs. The non-steroidal anti- provocative medicines or
NSAIDs like ibuprofen drug and indomethacin medicine can help
to decrease the aching and temperature related to the cystic
fibrosis. The nebulizers relax the respiratory muscles nearby the
ducts that transfer the oxygen to the lungs; this aids the increase in
the airflow (Mogayzel Jr, Naureckas, Robinson, Mueller,
Hadjiliadis, Hoag, J& Pulmonary Clinical Practice Guidelines
Committee, 2013).

Surgical procedures
Bowel surgical treatment is the emergency operation that
includes the exclusion of the part of the bowel.
Chest pain therapy
It benefits loosen the dense mucus inside the lungs, helping it
in the stress-free to cough it up. The common procedure
includes retaining the head over the corner of a bed and
clapping with the cupped hands alongside the sides of the
ribcage. A lung transplant includes the removal of the injured
lung and substituting it with a well one, commonly from a
departed person or donor. The surgical procedure might be
essential when somebody with cystic fibrosis has serious
breathing issues (Flume, Mogayzel Jr, Robinson, Rosenblatt,
Quittell, & Marshall, (2010).

Home care is another way treat the health condition by
consuming a healthy, avoiding smoking, drinking plenty of
water or fluids, doing exercise daily, and getting influenza
and pneumonia vaccinations regularly (Flume, Robinson,
O'Sullivan, Finder, Vender, Willey-Courtland, & Clinical
Practice Guidelines for Pulmonary Therapies Committee,
2009).
Needs of the patient/family
Cystic fibrosis is the deadly disease that increases over
time if not treated well on time. The patient might be
feeling exotic and depressed while experiencing this
health condition, therefore the requires a complete
treatment strategy.

References
Flume, P. A., & Van Devanter, D. R. (2012). State of progress in treating cystic fibrosis respiratory disease. BMC Medicine, 10(1), 88.
Flume, P. A., Mogayzel Jr, P. J., Robinson, K. A., Rosenblatt, R. L., Quittell, L., & Marshall, B. C. (2010). Cystic fibrosis pulmonary guidelines:
pulmonary complications: hemoptysis and pneumothorax. American journal of respiratory and critical care medicine, 182(3), 298-306.
Flume, P. A., Robinson, K. A., O'Sullivan, B. P., Finder, J. D., Vender, R. L., Willey-Courant, D. B., ... & Clinical Practice Guidelines for Pulmonary
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Grief, J. (2008). Medical imaging in patients with Cystic Fibrosis. Retrieved on April 11, 2009. Available
at: http://www.eradimaging.com/site/article.cfm?ID=327
Lubamba, B., Dhooghe, B., Noel, S., & Leal, T. (2012). Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy. Clinical
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Quittner, A. L., Modi, A. C., Wainwright, C., Otto, K., Kirihara, J., & Montgomery, A. B. (2009). Determination of the minimal clinically important
difference scores for the Cystic Fibrosis Questionnaire-Revised respiratory symptom scale in two populations of patients with cystic fibrosis and
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Ramsey, B. W., Davies, J., McElvaney, N. G., Tullis, E., Bell, S. C., Dřevínek, P., ... & Moss, R. (2011). A CFTR potentiator in patients with cystic
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Smyth, A. R., Bell, S. C., Bojcin, S., Bryon, M., Duff, A., Flume, P., ... & Sermet-Gaudelus, I. (2014). European cystic fibrosis society standards of
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University of Rochester medical center (2019). Cystic fibrosis and the respiratory system. Retrieved from:
https://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=90&contentid=P02936