Comprehensive Approach to Managing Cystic Fibrosis: Integrating Clinical Nutrition, Nursing, Radiology, Family Medicine, General Dentistry, Pharmacy, Psychology, and Respiratory Therapy

Authors

  • Mashael Saleh Alotaibi
  • Fatma Mahdi Nahari
  • Fatimah Abdulhadi Ali Alsaeedi
  • Sultan Abubakr Hakami
  • Muayad Youssof Al Awwas
  • Reema Abdulkader Azzeem
  • Ali Salman Al-khalaf
  • Mohamed Mohamed Basha
  • Hawraa Abdulmohsen Aljasim
  • Tariq Mishal Alharbi
  • Ahmed Khalid Bamousa

DOI:

https://doi.org/10.22399/ijcesen.5494

Keywords:

Cystic Fibrosis, Comprehensive Approach, Multidisciplinary Care, Clinical Nutritio

Abstract

Managing cystic fibrosis (CF) requires a comprehensive and multidisciplinary approach due to the complexity of the disease, which affects multiple organ systems, primarily the lungs and digestive tract. Key healthcare professionals, including nurses, respiratory therapists, and dietitians, collaborate to formulate personalized treatment plans that address the unique needs of each patient. Clinical nutrition plays a pivotal role, as individuals with CF often struggle with malabsorption and require tailored diets rich in calories and nutrients. Concurrently, regular monitoring through radiological imaging helps assess lung function and detect complications early, allowing for timely interventions. Family medicine physicians coordinate overall care, considering the mental and emotional health of patients and their families, which is crucial in managing a lifelong condition. Integrating various specialties, such as general dentistry and pharmacy, enhances the comprehensive care for CF patients. Dental professionals ensure oral health is maintained, as patients may face increased risks of dental issues due to medications and dietary choices. Pharmacists are essential in managing complex medication regimens by optimizing dosing, minimizing interactions, and ensuring adherence to therapies like mucolytics or antibiotics. Additionally, psychology plays an integral role in providing emotional support and coping strategies for both patients and their families, addressing issues such as anxiety and depression that can arise from chronic illness. By fostering a team-oriented approach that includes these diverse fields, healthcare providers can significantly improve the quality of life and health outcomes for individuals living with cystic fibrosis.

References

1. De Boeck K, Weren M, Proesmans M, Kerem E. Pancreatitis among patients with cystic fibrosis: correlation with pancreatic status and genotype. Pediatrics. 2005 Apr;115(4):e463-9. DOI: https://doi.org/10.1542/peds.2004-1764

2. Rajapaksha IG, Angus PW, Herath CB. Current therapies and novel approaches for biliary diseases. World J Gastrointest Pathophysiol. 2019 Jan 05;10(1):1-10. DOI: https://doi.org/10.4291/wjgp.v10.i1.1

3. Sellers ZM, Assis DN, Paranjape SM, Sathe M, Bodewes F, Bowen M, Cipolli M, Debray D, Green N, Hughan KS, Hunt WR, Leey J, Ling SC, Morelli G, Peckham D, Pettit RS, Philbrick A, Stoll J, Vavrina K, Allen S, Goodwin T, Hempstead SE, Narkewicz MR. Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations. Hepatology. 2024 May 01;79(5):1220-1238. DOI: https://doi.org/10.1097/HEP.0000000000000646

4. Awatade NT, Wong SL, Hewson CK, Fawcett LK, Kicic A, Jaffe A, Waters SA. Human Primary Epithelial Cell Models: Promising Tools in the Era of Cystic Fibrosis Personalized Medicine. Front Pharmacol. 2018;9:1429. DOI: https://doi.org/10.3389/fphar.2018.01429

5. von Drygalski A, Biller J. Anemia in cystic fibrosis: incidence, mechanisms, and association with pulmonary function and vitamin deficiency. Nutr Clin Pract. 2008 Oct-Nov;23(5):557-63. DOI: https://doi.org/10.1177/0884533608323426

6. Poncin W, Lebecque P. [Lung clearance index in cystic fibrosis]. Rev Mal Respir. 2019 Mar;36(3):377-395. DOI: https://doi.org/10.1016/j.rmr.2018.03.007

7. Goss CH. Acute Pulmonary Exacerbations in Cystic Fibrosis. Semin Respir Crit Care Med. 2019 Dec;40(6):792-803. DOI: https://doi.org/10.1055/s-0039-1697975

8. Lyamin AV, Ismatullin DD, Zhestkov AV, Kondratenko OV. [The laboratory diagnostic in patients with mucoviscidosis: A review.]. Klin Lab Diagn. 2018;63(5):315-320.

9. Wucherpfennig L, Wuennemann F, Eichinger M, Schmitt N, Seitz A, Baumann I, Stahl M, Graeber SY, Chung J, Schenk JP, Alrajab A, Kauczor HU, Mall MA, Sommerburg O, Wielpütz MO. Longitudinal Magnetic Resonance Imaging Detects Onset and Progression of Chronic Rhinosinusitis from Infancy to School Age in Cystic Fibrosis. Ann Am Thorac Soc. 2023 May;20(5):687-697. DOI: https://doi.org/10.1513/AnnalsATS.202209-763OC

10. Penketh AR, Wise A, Mearns MB, Hodson ME, Batten JC. Cystic fibrosis in adolescents and adults. Thorax. 1987 Jul;42(7):526-32. DOI: https://doi.org/10.1136/thx.42.7.526

11. Aris RM, Merkel PA, Bachrach LK, Borowitz DS, Boyle MP, Elkin SL, Guise TA, Hardin DS, Haworth CS, Holick MF, Joseph PM, O'Brien K, Tullis E, Watts NB, White TB. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab. 2005 Mar;90(3):1888-96. DOI: https://doi.org/10.1210/jc.2004-1629

12. Kelly T, Buxbaum J. Gastrointestinal Manifestations of Cystic Fibrosis. Dig Dis Sci. 2015 Jul;60(7):1903-13. DOI: https://doi.org/10.1007/s10620-015-3546-7

13. Stern M, Picard C, Roux A. [Lung transplantation]. Rev Prat. 2018 Feb;68(2):189-194.

14. Pawlaczyk-Kamieńska T, Borysewicz-Lewicka M, Śniatała R, Batura-Gabryel H, Cofta S. Dental and periodontal manifestations in patients with cystic fibrosis - A systematic review. J Cyst Fibros. 2019 Nov;18(6):762-771. DOI: https://doi.org/10.1016/j.jcf.2018.11.007

15. Blasi F, Elborn JS, Palange P. Adults with cystic fibrosis and pulmonologists: new training needed to recruit future specialists. Eur Respir J. 2019 Jan;53(1). DOI: https://doi.org/10.1183/13993003.02209-2018

16. Hamosh A, FitzSimmons SC, Macek M, Knowles MR, Rosenstein BJ, Cutting GR. Comparison of the clinical manifestations of cystic fibrosis in black and white patients. J Pediatr. 1998 Feb;132(2):255-9. DOI: https://doi.org/10.1016/S0022-3476(98)70441-X

17. Fanen P, Wohlhuter-Haddad A, Hinzpeter A. Genetics of cystic fibrosis: CFTR mutation classifications toward genotype-based CF therapies. Int J Biochem Cell Biol. 2014 Jul;52:94-102. DOI: https://doi.org/10.1016/j.biocel.2014.02.023

18. Langton Hewer SC, Smith S, Rowbotham NJ, Yule A, Smyth AR. Antibiotic strategies for eradicating Pseudomonas aeruginosa in people with cystic fibrosis. Cochrane Database Syst Rev. 2023 Jun 02;6(6):CD004197. DOI: https://doi.org/10.1002/14651858.CD004197.pub6

19. Cystic Fibrosis Foundation. Borowitz D, Robinson KA, Rosenfeld M, Davis SD, Sabadosa KA, Spear SL, Michel SH, Parad RB, White TB, Farrell PM, Marshall BC, Accurso FJ. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009 Dec;155(6 Suppl):S73-93. DOI: https://doi.org/10.1016/j.jpeds.2009.09.001

20. Lapp V, Chase SK. How Do Youth with Cystic Fibrosis Perceive Their Readiness to Transition to Adult Healthcare Compared to Their Caregivers' Views? J Pediatr Nurs. 2018 Nov-Dec;43:104-110. DOI: https://doi.org/10.1016/j.pedn.2018.09.012

21. Pallin M. Cystic fibrosis vigilance in Arab countries: The role of genetic epidemiology. Respirology. 2019 Feb;24(2):93-94. DOI: https://doi.org/10.1111/resp.13461

22. Kiedrowski MR, Bomberger JM. Viral-Bacterial Co-infections in the Cystic Fibrosis Respiratory Tract. Front Immunol. 2018;9:3067. DOI: https://doi.org/10.3389/fimmu.2018.03067

23. Eschenhagen P, Schwarz C. [Patients with cystic fibrosis become adults : Treatment hopes and disappointments]. Internist (Berl). 2019 Jan;60(1):98-108. DOI: https://doi.org/10.1007/s00108-018-0536-9

24. Guo J, Garratt A, Hill A. Worldwide rates of diagnosis and effective treatment for cystic fibrosis. J Cyst Fibros. 2022 May;21(3):456-462. DOI: https://doi.org/10.1016/j.jcf.2022.01.009

25. Mandalia A, Wamsteker EJ, DiMagno MJ. Recent advances in understanding and managing acute pancreatitis. F1000Res. 2018;7. DOI: https://doi.org/10.12688/f1000research.14244.1

26. Bush A, Floto RA. Pathophysiology, causes and genetics of paediatric and adult bronchiectasis. Respirology. 2019 Nov;24(11):1053-1062. DOI: https://doi.org/10.1111/resp.13509

27. Accurso FJ, Sontag MK, Wagener JS. Complications associated with symptomatic diagnosis in infants with cystic fibrosis. J Pediatr. 2005 Sep;147(3 Suppl):S37-41. DOI: https://doi.org/10.1016/j.jpeds.2005.08.034

28. Fenker DE, McDaniel CT, Panmanee W, Panos RJ, Sorscher EJ, Sabusap C, Clancy JP, Hassett DJ. A Comparison between Two Pathophysiologically Different yet Microbiologically Similar Lung Diseases: Cystic Fibrosis and Chronic Obstructive Pulmonary Disease. Int J Respir Pulm Med. 2018;5(2). DOI: https://doi.org/10.23937/2378-3516/1410098

29. Radovanovic D, Santus P, Blasi F, Sotgiu G, D'Arcangelo F, Simonetta E, Contarini M, Franceschi E, Goeminne PC, Chalmers JD, Aliberti S. A comprehensive approach to lung function in bronchiectasis. Respir Med. 2018 Dec;145:120-129. DOI: https://doi.org/10.1016/j.rmed.2018.10.031

30. Panos RJ, Mortenson RL, Niccoli SA, King TE. Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. Am J Med. 1990 Apr;88(4):396-404. DOI: https://doi.org/10.1016/0002-9343(90)90495-Y

31. Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2006 Mar 01;173(5):475-82. DOI: https://doi.org/10.1164/rccm.200505-840OE

32. Pilewski JM, Frizzell RA. Role of CFTR in airway disease. Physiol Rev. 1999 Jan;79(1 Suppl):S215-55. DOI: https://doi.org/10.1152/physrev.1999.79.1.S215

33. Berg P, Jeppesen M, Leipziger J. Cystic fibrosis in the kidney: new lessons from impaired renal HCO3- excretion. Curr Opin Nephrol Hypertens. 2021 Jul 01;30(4):437-443. DOI: https://doi.org/10.1097/MNH.0000000000000725

34. Welsh MJ, Smith AE. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell. 1993 Jul 02;73(7):1251-4. DOI: https://doi.org/10.1016/0092-8674(93)90353-R

35. Hewer SCL, Smyth AR, Brown M, Jones AP, Hickey H, Kenna D, Ashby D, Thompson A, Williamson PR., TORPEDO-CF study group. Intravenous versus oral antibiotics for eradication of Pseudomonas aeruginosa in cystic fibrosis (TORPEDO-CF): a randomised controlled trial. Lancet Respir Med. 2020 Oct;8(10):975-986. DOI: https://doi.org/10.1016/S2213-2600(20)30331-3

Downloads

Published

2025-02-28

How to Cite

Mashael Saleh Alotaibi, Fatma Mahdi Nahari, Fatimah Abdulhadi Ali Alsaeedi, Sultan Abubakr Hakami, Muayad Youssof Al Awwas, Reema Abdulkader Azzeem, … Ahmed Khalid Bamousa. (2025). Comprehensive Approach to Managing Cystic Fibrosis: Integrating Clinical Nutrition, Nursing, Radiology, Family Medicine, General Dentistry, Pharmacy, Psychology, and Respiratory Therapy. International Journal of Computational and Experimental Science and Engineering, 11(1). https://doi.org/10.22399/ijcesen.5494

Issue

Section

Research Article

Most read articles by the same author(s)