Cystic Fibrosis: Diagnosis and Treatment

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Cystic Fibrosis Pathophysiology and Etiology

Cystic Fibrosis (CF) is a genetic disorder that causes various digestion and breathing issues. Precisely, CF occurs due to a metamorphosis in the CF transmembrane conductance regulator (CFTR) gene. The gene produces the CFTR protein, which regulates the sodium and chloride ions movement across the epithelial cell membranes (Bowden & Greenberg, 2014). The mutations in a single or both replicas of the genetic factor make the ion conveyance unreliable and form a buildup of dense mucus in the whole body. The deformities serve as the basis of respiratory deficiency and other systemic anomalies and obstructions (Brown et al., 2017). Hence, people with CF have thick and sticky mucus that clocks their airways and causes lung damage, traps germs, increases the prevalence of infections, and prevents the entry of vital proteins into the intestines.

CF Clinical Manifestations

  1. Decreased appetite
  2. Fatigue
  3. Increased cough
  4. Reduced spirometry values
  5. Poor response to outpatient treatment measures
  6. Hemoptysis (Bowden & Greenberg, 2014).
  7. Recurrent wheezing
  8. Infertility
  9. Biliary cirrhosis
  10. Abdominal distension (Brown et al., 2017).

Interdisciplinary Approach to CF Treatment

The care of CF patients requires the coordination of different treatment measures and the integration of psychosocial counseling and management in medical treatment. Bowden and Greenberg (2014) describe CF as a complex disease that requires specialized care and treatment. Hence, although pediatric pulmonologists remain the prominent directors of CF centers, the institutions need other specialists like endocrinologists, specialists of infectious diseases, gastroenterologists, and geneticists (Bowden & Greenberg, 2014). Besides, additional team members like medical social workers, physical therapists, advanced practice nurses, dieticians, genetic counselors, child life specialists, and physical therapists. Integration of interdisciplinary interventions is a sure way to treat CF effectively.

Nursing Care Responsibilities in Cystic Fibrosis Treatment

Nurses mainly assume major roles of stressing the importance of adequate dietary and fluid intake, maintaining adequate oxygenation, encouraging measures to remove pulmonary secretions, preventing complications, and ensuring good nutrition. Besides, the nurses also engage in the monitoring of essential changes in respiratory status and heart rate. According to Bowden and Greenberg (2014), nurses help offer follow-up services, emotional care, and response coordination to therapies. These services intend to support the CF patients to manage the disease effectively.

Response to First Post

The student does an incredible job addressing all the relevant questions. I agree with the post’s points regarding the CF etiology, physiology, clinical manifestations, and the need for interdisciplinary care. However, the student should improve their presentation strategy to avoid giving unclear and unsatisfactory answers. Overall, the post makes it easy for any reader to understand the basic knowledge about CF and the most preferred treatment methods.

Response to Second Post

The author does an incredible job in expressing their ideas in the essay. The writer addressed the questions systemically and concretely, making it easier for the reader to read and understand basic concepts about CF. I agree with the authors’ perceptions on the etiology and pathophysiology of the disease and the practitioner’s role in treating the disease. Besides, the way the author explained the basics of CF and its use in different ways is commendable. The post will go a long way in educating people on the nature of CF and how it affects children.

References

Bowden, V. R., & Greenberg, C. S. (2014). Children and their families: The continuum of care. (3rd ed.). Wolters Kluwer. Lippincott Williams & Wilkins.

Brown, S. D., White, R., & Tobin, P. (2017). Journal of the American Academy of PAs, 30(5), 23-27. Web.

Rao, S., Ziniel, S. I., Khan, I., & Dempsey, A. (2019). Vaccine. Web.

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