Review of the dietetic and MDT management of Cystic Fibrosis
DOI:
https://doi.org/10.33178/SMJ.2024.1.5Keywords:
Cystic Fibrosis, dietetic management, CFTR gene, physical activity in cystic fibrosis, prevalence of cystic fibrosis in IrelandAbstract
INTRODUCTION: Cystic Fibrosis (CF) is an autosomal recessive disorder due to mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to abnormality of chloride channels in mucus and sweat producing cells. The respiratory
system (lungs) and digestive system (GIT) are primarily impacted, leading to life threatening complications (Rafeeq and Murad, 2017). Ireland has the highest incidence of CF in the world. Approximately 1 in 19 Irish people are said to 'carry' one copy of the altered gene that causes CF (Cystic Fibrosis Ireland, 2023). More than 1900 mutations of CF have been identified (Rafeeq and Murad, 2017).
METHODS: Articles for review were sourced from the academic database PubMed. Results were screened using PICOS criteria, focusing on dietetic management of CF. Papers dating back as far as the 1980’s were included in the review due to their continuing relevance in CF treatment today.
RESULTS: Initial database searches identified 61 results, which were then screened for relevance to the objectives of this review. Treatment of CF requires a multi-disciplinary team approach, for which Nutrition and Dietetic management is integral. Lifelong management of CF includes pharmaceutical treatment to manage symptoms, case specific diet and lifestyle
therapy, management of complications and co-morbidities, and novel therapies such as CFTR modulators.
CONCLUSION: The identification of the faulty CFTR gene that causes CF was an important step in managing the disorder, yet has not led to a cure for the condition. Life expectancy for patients with CF has steadily improved during the last three decades, with medical
management of symptoms and advances in CF therapies. Complications associated with the condition are treated on a case-by-case basis due to complexity of symptoms and individuality of the condition. Dietetic management includes a high calorie, high salt, and high protein diet and routine monitoring for changes in symptoms and nutritional deficiencies.
References
Burnett D.M., Barry A.N. and Mermis J.D. (2020), Physical Activity Level and Perception of Exercise in Cystic Fibrosis. Respiratory Care. 65 (4), pp. 500-506.
Colombo C. (2007), Liver disease in Cystic Fibrosis. Current Opinion in Pulmonary Medicine, 13 (6) pp. 529-536.
Conway S.P., Brownlee K., Perkham D. and Lee T. (2008), Cystic Fibroris in children and adults: The Leeds method of management for the treatment and management of cystic fibrosis. St. James and Seacroft university hospitals, Leeds Teaching hospitals Trust, UK, revised edition, Number 7, 2008.
Cox N.S., Alison J.A., Button B.M., Wilson J.W., Morton J.M. and Holland A.E. (2016), Physical activity participation by adults with cystic fibrosis: an observational study. Respirology. 21, pp. 511-518.
Cystic Fibrosis Foundation. (2016) About CF. http:// www.cff.org/about_cf/what_is_cf
Cystic Fibrosis Ireland (2023), https://www. cfireland.ie
Dodge J.A., Lewis P.A., Stanton M. and Wilsher J. (2007), Cystic Fibrosis mortality and survival in the UK: 1947-2003. European Respiratory Journal, 29 (3), pp. 522-526.
Dwyer T.J., Daviskas E., Zainuldin R., Verschuer J., Eberl S., Bye P.T. and Alison, J.A. (2019), Effects of exercise and airway clearance (positive expiratory pressure) on mucus clearance in cystic fibrosis: a randomised crossover trial. European Respiratory Journal, 53 (4).
Elborn J.S. (2019), Adult Care in Cystic Fibrosis. In Seminars in respiratory and critical care medicine, Volume 40, No. 06, pp. 857-868.
Fitzgerald C., Linnane B., George S., Ni Chroinin M., Mullane D., Herzig M., Greally P., Elnazir B., Healy F., McNally P. and Javadpour S. (2020), Neonatal Screening Programme for CF: results from the Irish Comparative Outcomes Study (ICOS). Pediatric Pulmonology, 55 (9), pp. 2323-2329. 66
Garcia S.T., Sanchez M.A.G., Cejudo P., Gallego E.Q., Dapena J. and Jimenez R.G. (2011), Bone health, daily physical activity, and exercise tolerance in patients with cystic fibrosis. Chest. 2011; 140: 475-481
Granados A., Chan C.L., Ode K.L., Moheet A., Moran A. and Holl R. (2019), Cystic fibrosis related diabetes: Pathophysiology, screening and diagnosis. Journal of Cystic Fibrosis, 18. Pp. S3-S9.
Goetz D.M. and Savant A.P. (2021), Review of CFTR modulators 2020. Pediatric Pulmonology, 56 (12), pp. 3595-3606.
Holliday K.E., Allen J.R., Waters D.L., Gruca M.A., Thompson S.M. and Gaskin K.J. (1991). Growth of human milk-fed and formula-fed infants with cystic Fibrosis. Journal of Pediatrics, 118 (1), pp. 77-79.
Houwen R.H., van der Doef H.P., Sermet I., Munck A., Hauser B., Walkowiak J., Robberecht E., Colombo C., Sinaasappel M., Wilschanski M. (2010), ESPGHAN Cystic Fibrosis Working Group: Defining DIOS and Constipation in Cystic Fibrosis with a Multicentre Study on the Incidence, Characteristics, and Treatment of DIOS. Journal of Pediatric Gastroenterology Nutrition, 50 (1), pp. 38-42.
Kalnins D. and Wilschanski M. (2012), Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies. Drug Design, Development and Therapy, 6, pp. 151-161.
Konrad J., Eber E. and Stadlbauer V., (2022), Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators. Paediatric Respiratory Reviews, 42, pp. 9-16.
Laughlin J.J., Brady M.S. and Howard E. (1981), Changing Feeding Trends as a Cause of Electrolyte Depletion in Infants with Cystic Fibrosis. Pediatrics, 68 (2), pp. 203-207.
Lederer D. J., Wilt J. S., D'Ovidio F., Bacchetta M. D., Shah L., Ravichandran S., Lenoir J., Klein B., Sonett J. R. and Arcasoy, S. M. (2009), Obesity and underweight are associated with an increased risk of death after lung transplantation. American Journal of Respiratory and Critical Care Medicine, 180 (9), pp. 887-895.
MacDonald A. (1996), Nutritional management of cystic fibrosis. Archives of Disease in Childhood, 74 (1), pp. 81-87.
McDonald C.M., Alvarez J.A., Bailey J., Bowser E.K., Farnham K., Mangus M., Padula L., Porco K. and Rozga M. (2021), Academy of nutrition and dietetics: 2020 cystic fi- brosis evidence analysis center evidence-based nutrition practice guideline. Journal of the Academy of Nutrition and Dietetics, 121 (8), pp.1591-1636.
Murphy J.L., Wootton S.A., Bond S.A. and Jackson A.A. (1991), Energy content of stools in normal healthy controls and patients with cystic fibrosis. Archives of Disease in Childhood, 6 (4), pp. 495-500.
Rafeeq M. M. and Murad H.A.S. (2017), Cystic Fibrosis: current therapeutic targets and future approaches. Journal of Translational Medicine, 15 (1), pp.1-9.
Schneiderman J.E., Wilkes D.L., Atenafu E.G., Nguyen T., Wells G.D., Alarie N., Tullis E.,Lands L.C., Coates A.L., Corey M. and Ratjen F. (2014), Longitudinal relationship between physical activity and lung health in patients with cystic fibrosis. European Respiratory Journal, 43 (3), pp. 817-823.
Sinaasappel M., Stern M., Littlewood J., Wolfe S., Steinkamp G., Heijerman H.G.M., Robberecht E. and Döring G. (2022), Nutrition in patients with Cystic Fibrosis: a European Consensus. Journal of Cystic Fibrosis, 1 (2), pp. 51-75.
Turck D., Braegger C.P., Colombo C., Declercq D., Morton A., Pancheva R., Robberecht E., Stern M., Strandvik B., Wolfe S., Schneider S.M. and Wilschanski M. (2016), ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clinical Nutrition, 35 (3), 557-577.
Voynow J. A. and Shinbashi M. (2021), Neutrophil Elastase and Chronic Lung Disease.Biomolecules 2021, 11 (1065), 15 pages.
Watson H., Bilton D. and Truby, H. (2008), A Randomized Controlled Trial of a New Behavioral Home-Based Nutrition Education Program, “Eat Well with CF,” in Adults with Cystic Fibrosis. Journal of the American Dietetics Association. 108 (5), pp. 847- 852.
Yeung J.C., Machuca T.N., Chaparro C., Cypel M., Stephenson A.L., Solomon M., Saito T., Binnie M., Chow C.W., Grasemann H., Pierre A.F., Yasufuku K., de Perrot M., Donahoe L.L., Tikkanen J., Martinu T., Waddell T.K., Tullis E., Singer L.G. and Keshavjee S. (2020), Lung transplantation for cystic fibrosis. Journal of Heart Lung Transplant, 39 (6), pp. 553-560
Downloads
Published
License
Copyright (c) 2024 Aoife Twomey
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
