PAGE CONTENTS

Surgery

Cystic fibrosis (CF) is a genetic disease that affects multiple organs. Mutations in the CF transmembrane conductance regulator (CFTR) gene lead to the dysfunction of the CFTR protein, which in turn results in the accumulation of thick and sticky mucus in several areas including the airways.^1,2 Over time, CF leads to chronic lung disease, and it can potentiate malnutrition and digestive complications.¹

Managing CF may involve surgical procedures such as the removal of nasal polyps or the placement of a feeding tube to increase caloric intake. Major surgeries such as lung transplant and bowel surgery may be also required.³

Lung Transplant in Cystic Fibrosis

Advanced CF lung disease (ACFLD) represents the most common cause of death in patients with CF. Patients with ACFLD have a decreased quality of life, with increased exacerbations and progressive worsening of symptoms.⁴ Clinical outcomes after lung transplants in patients with ACFLD have significantly improved, and lung transplants have been shown to provide a better quality of life for these patients.^5-7The benefits of lung transplantation may be more pronounced in patients experiencing worse symptoms.⁴Discussing this surgery, as well as other potential treatments, while managing CF is recommended when the patient presents with normal lung function or mildly reduced forced expiratory volume in 1 second (FEV₁).⁸Although not all patients will be candidates for a lung transplant, this approach may contribute to better management of the fear and anxiety that patients with CF experience regarding this procedure.^1,8A focused discussion on lung transplantation led by the CF care team is recommended when the patient presents with a predicted FEV₁ <50%.⁸

Patients with CF have a median survival of 9.9 years after a lung transplant, and about 30% of patients survive for 20 years after transplantation. Additionally, the post-transplant survival in patients with CF is higher than that of other transplanted diagnoses.⁹ Patients undergoing a lung transplant should be physically and emotionally prepared, and they will benefit from social support throughout the process and rehabilitation.¹

Gastrointestinal Surgical Management in Cystic Fibrosis

Different gastrointestinal issues in patients with CF may require surgical intervention. These include meconium ileus in infants, distal intestinal obstructive syndrome (DIOS) in children and adults, and, rarely, rectal prolapse.^10-12

Meconium ileus affects about 20% of infants with CF.¹³ This intestinal disease may be simple or complex.¹⁴Simple meconium ileus involves an intraluminal distal bowel obstruction due to abnormal viscid meconium. This condition may be characterized by complications such as meconium peritonitis, pseudocyst formation, or perforation, and it requires surgery.^14,15 The goal of the surgical procedure is to decompress the bowel and remove the obstruction by clearing the abnormal meconium.¹⁵

DIOS affects about 15% of CF patients,¹¹ and it can present as a partial or complete obstruction.¹⁵ These obstructions result from the accumulation of viscid fecal material in the bowel and its combination with the sticky mucus in the intestine.¹⁶ Surgical decompression of DIOS is only performed when medical management is not successful, and it is associated with elevated morbidity and mortality.^12,16

Nasal and Sinus Surgery in Cystic Fibrosis

The changes observed in the visco-elastic properties of the abnormal mucus formed in patients with CF contribute to the mechanical obstruction of the nasal and paranasal sinuses. Chronic rhinosinusitis, which may be associated with nasal polyposis, develops in about half of patients with CF. Symptoms of this condition include nasal obstruction, sneezing, and thick nasal discharge.¹⁷ Endoscopic sinus surgery (ESS) can reduce inflammation and improve breathing in these patients.^3,17

A recent study reported that ESS can lead to better lung function in patients with moderate to severe CF, as well as in some patients with mild disease. The same study showed that the observed improvement in lung function was maintained 12 months after surgery.¹⁸ A different report also revealed that ESS interventions performed early after lung transplantation can improve the patient’s pulmonary function and decrease exacerbations, while longer waiting times may result in worsening lung function and increased antibiotic use.¹⁹

References

1. About cystic fibrosis. Cystic Fibrosis Foundation. Accessed January 25, 2022.

2. Cystic fibrosis. National Organization for Rare Disorders (NORD). Accessed January 25, 2022.

3. Cystic fibrosis: diagnosis and treatment. Mayo Clinic. Accessed January 25, 2022.

4. Kapnadak SG, Dimango E, Hadjiliadis D, et al. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros. 2020;19(3):344-354. doi:10.1016/j.jcf.2020.02.015

5. Vock DM, Durheim MT, Tsuang WM, et al. Survival benefit of lung transplantation in the modern era of lung allocation. Ann Am Thorac Soc. 2017;14(2):172-181. doi:10.1513/AnnalsATS.201606-507OC

6. Singer LG, Chowdhury NA, Faughnan ME, et al. Effects of recipient age and diagnosis on health-related quality-of-life benefit of lung transplantation. Am J Respir Crit Care Med. 2015;192(8):965-973. doi:10.1164/rccm.201501-0126OC

7. Singer JP, Katz PP, Soong A, et al. Effect of lung transplantation on health-related quality of life in the era of the Lung Allocation Score: a U.S. prospective cohort study. Am J Transplant. 2017;17(5):1334-1345. doi:10.1111/ajt.14081

8. Ramos KJ, Smith PJ, McKone EF, et al.; CF Lung Transplant Referral Guidelines Committee. Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines. J Cyst Fibros. 2019;18(3):321-333. doi:10.1016/j.jcf.2019.03.002

9. Chambers DC, Cherikh WS, Harhay MO, et al.; International Society for Heart and Lung Transplantation. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: thirty-sixth adult lung and heart-lung transplantation report-2019; focus theme: donor and recipient size match. J Heart Lung Transplant. 2019;38(10):1042-1055. doi:10.1016/j.healun.2019.08.001

10. Davies JC, Alton EWFW, Bush A. Cystic fibrosis. BMJ. 2007;335(7632):1255-1259. doi:10.1136/bmj.39391.713229.AD

11. Sharma A, Morton A, Peckham D, Jayne D. Gastrointestinal surgery in adult patients with cystic fibrosis. Frontline Gastroenterol. 2012;3(4):242-247. doi:10.1136/flgastro-2012-100184

12. Abraham JM, Taylor CJ. Cystic fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer. J Cyst Fibros. 2017;16(Suppl 2):S40-S49. doi:10.1016/j.jcf.2017.06.013

13. Parikh NS, Ibrahim S, Ahlawat R. Meconium ileus. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2021. Accessed January 25, 2022.

14. Shteinberg M, Haq IJ, Polineni D, Davies JC. Cystic fibrosis. Lancet. 2021;397(10290):2195-2211. doi:10.1016/S0140-6736(20)32542-3

15. Farrelly PJ, Charlesworth C, Lee S, Southern KW, Baillie CT. Gastrointestinal surgery in cystic fibrosis: a 20-year review. J Pediatr Surg. 2014;49(2):280-283. doi:10.1016/j.jpedsurg.2013.11.038

16. Gilchrist FJ, Green J, Carroll W. Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis. Cochrane Database Syst Rev. 2021;12(12):CD012798. doi:10.1002/14651858.CD012798.pub3

17. Lazio MS, Luparello P, Mannelli G, et al. Quality of life and impact of endoscopic sinus surgery in adult patients with cystic fibrosis. Am J Rhinol Allergy. 2019;33(4):413-419. doi:10.1177/1945892419839260

18. Kawai K, Dombrowski N, Sawicki GS, Adil EA. Improvement of pulmonary function in cystic fibrosis patients following endoscopic sinus surgery. Laryngoscope. 2021;131(9):1930-1938. doi:10.1002/lary.29409

19. Johnson JR, Hwang PH, Nayak JV, Patel ZM. Comparison of endoscopic sinus surgery timing in lung transplant patients with cystic fibrosis. Int Forum Allergy Rhinol. Published online December 7, 2021. doi:10.1002/alr.22935

Reviewed by Hasan Avcu, MD, on 1/26/2022.

Diana Diez Gaspar, PharmD, PhD

Diana earned her PhD and PharmD with distinction in the field of Medicinal and Pharmaceutical Chemistry at the Universidade do Porto. She is an accomplished oncology scientist with 10+ years of experience in developing and managing R&D projects and research staff directed to the development of small proteins fit for medical use.

Cystic Fibrosis (CF)