Cystic fibrosis-related diabetes (CFRD) is a common extrapulmonary manifestation of cystic fibrosis (CF) affecting up to 50% of adults. Cystic fibrosis-related diabetes is associated with poorer lung function, lower nutrition, and increased mortality. Abnormal blood glucoses, especially post-prandial hyperglycemia, can precede a diagnosis of CFRD by many years, and even the prediabetic state has been associated with poorer health outcomes in people with CF (pwCF). With the advent of cystic fibrosis transconductance regulator (CFTR) modulator therapies, the clinical landscape of CF is changing. With increasing longevity, the prevalence of CFRD is anticipated to rise. Continuous glucose monitoring (CGM) technology has been applied in research and clinical settings for insights into CFRD pathophysiology, and its use for early dysglycemia detection in the CF population is increasing. However, guidance around management of these early glucose abnormalities is limited. This article aims to review and summarize the current literature on use of CGM in the prediabetic state in pwCF and to highlight ongoing areas of research need.
GuoJGarrattAHillA.Worldwide rates of diagnosis and effective treatment for cystic fibrosis. J Cyst Fibros. 2022;21(3):456-462.
2.
Cystic Fibrosis Foundation Patient Registry.2023 Patient Registry Annual Data Report. Bethesda, MD: Cystic Fibrosis Foundation; 2024.
3.
OngTRamseyBW.Cystic fibrosis: a review. JAMA. 2023;329(21):1859-1871.
4.
MiddletonPGMallMADřevínekP, et al. Elexacaftor-Tezacaftor-Ivacaftor for cystic fibrosis with a single Phe508del allele. N Engl J Med. 2019;381(19):1809-1819.
5.
GuoJKingIHillA.International disparities in diagnosis and treatment access for cystic fibrosis. Pediatr Pulmonol. 2024;59(6):1622-1630.
6.
ZampoliMMorrowBMPaulG.Real-world disparities and ethical considerations with access to CFTR modulator drugs: mind the gap!Front Pharmacol. 2023;14:1163391.
7.
KohlerCLVigersTPyleL, et al. Trends in cystic fibrosis-related diabetes epidemiology between 2003 and 2018 from the U.S. cystic fibrosis foundation patient registry. Diabetes Care. 2025;48(7):1251-1259.
8.
LewisCBlackmanSMNelsonA, et al. Diabetes-related mortality in adults with cystic fibrosis. Role of genotype and sex. Am J Respir Crit Care Med. 2015;191(2):194-200.
9.
NorrisAWOdeKLMerjanehL, et al. Survival in a bad neighborhood: pancreatic islets in cystic fibrosis. J Endocrinol. 2019;241(1):R35-R50.
10.
MalikSSPadmanabhanDHull-MeichleRL.Pancreas and islet morphology in cystic fibrosis: clues to the etiology of cystic fibrosis-related diabetes. Front Endocrinol. 2023;14:1269139.
11.
MoheetAMoranA.New concepts in the pathogenesis of cystic fibrosis-related diabetes. J Clin Endocrinol Metab. 2022;107(6):1503-1509.
12.
AksitMAPaceRGVecchio-PagánB, et al. Genetic modifiers of cystic fibrosis-related diabetes have extensive overlap with type 2 diabetes and related traits. J Clin Endocrinol Metab. 2020;105(5):1401-1415.
13.
LanngSThorsteinssonBNerupJKochC.Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. Eur J Pediatr. 1992;151(9):684-687.
14.
AraratESonawallaABerlinskiATasE.Nutritional status between 5-10 years is associated with cystic fibrosis-related diabetes in adolescence. Pediatr Pulmonol. 2021;56(10):3217-3222.
15.
OdeKLMoranA.New insights into cystic fibrosis-related diabetes in children. Lancet Diabetes Endocrinol. 2013;1(1):52-58.
16.
American Diabetes Association Professional Practice Committee. 2. Diagnosis and classification of diabetes: standards of care in diabetes-2024. Diabetes Care. 2024;47(suppl 1):S20-S42.
17.
OdeKLBallmanMBattezzatiA, et al. ISPAD clinical practice consensus guidelines 2022: management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes. 2022;23(8):1212-1228.
18.
GilmourJASykesJEtchellsETullisE.Cystic fibrosis-related diabetes screening in adults: a gap analysis and evaluation of accuracy of glycated hemoglobin levels. Can J Diabetes. 2019;43(1):13-18.
19.
LiouTGJensenJLAllenSE, et al. Improving performance in the detection and management of cystic fibrosis-related diabetes in the Mountain West Cystic Fibrosis Consortium. BMJ Open Diabetes Res Care. 2016;4(1):e000183.
20.
HicksROdeKLVigersTChanCL.A provider survey of cystic fibrosis related diabetes screening and management practices at North American CF centers. Front Endocrinol. 2023;14:1183288.
21.
DobsonLSheldonCDHattersleyAT.Validation of interstitial fluid continuous glucose monitoring in cystic fibrosis. Diabetes Care. 2003;26(6):1940-1941.
22.
O’RiordanSMHindmarshPHillNR, et al. Validation of continuous glucose monitoring in children and adolescents with cystic fibrosis: a prospective cohort study. Diabetes Care. 2009;32(6):1020-1022.
ZorronMMarsonFALMorcilloAM, et al. Can continuous glucose monitoring predict cystic fibrosis-related diabetes and worse clinical outcome?J Bras Pneumol. 2022;48(2):e20210307.
25.
ChanCLVigersTPyleLZeitlerPSSagelSDNadeauKJ.Continuous glucose monitoring abnormalities in cystic fibrosis youth correlate with pulmonary function decline. J Cyst Fibros. 2018;17(6):783-790.
26.
MoreauFWeillerMARosnerV, et al. Continuous glucose monitoring in cystic fibrosis patients according to the glucose tolerance. Horm Metab Res. 2008;40(7):502-506.
27.
Clemente LeónMBilbao GassóLMoreno-GaldóA, et al. Oral glucose tolerance test and continuous glucose monitoring to assess diabetes development in cystic fibrosis patients. Endocrinol Diabetes Nutr. 2018;65(1):45-51.
28.
RakotoarisoaLWeissLLefebvreF, et al. Comparison of continuous glucose monitoring in cystic fibrosis patients with or without pancreatic exocrine insufficiency. Horm Metab Res. 2022;54(6):407-412.
29.
ShahVNDuBoseSNLiZ, et al. Continuous glucose monitoring profiles in healthy nondiabetic participants: a multicenter prospective study. J Clin Endocrinol Metab. 2019;104(10):4356-4364.
30.
CichoszSLKronborgTLaugesenE, et al. From stability to variability: classification of healthy individuals, prediabetes, and type 2 diabetes using glycemic variability indices from continuous glucose monitoring data. Diabetes Technol Ther. 2025;27(1):34-44.
31.
ElidottirHDiemerSEklundEHansenCR.Abnormal glucose tolerance and lung function in children with cystic fibrosis. Comparing oral glucose tolerance test and continuous glucose monitoring. J Cyst Fibros. 2021;20(5):779-784.
32.
PrenticeBJChelliahAOoiCY, et al. Peak OGTT glucose is associated with lower lung function in young children with cystic fibrosis. J Cyst Fibros. 2020;19(2):305-309.
33.
American Diabetes Association. Standards of medical care in diabetes—2010. Diabetes Care. 2010;33(suppl 1):S11-S61.
34.
BrennanALGyiKMWoodDMHodsonMEGeddesDMBakerEH.Relationship between glycosylated haemoglobin and mean plasma glucose concentration in cystic fibrosis. J Cyst Fibros. 2006;5(1):27-31.
35.
ChanCLHopeEThurstonJ, et al. Hemoglobin A1c accurately predicts continuous glucose monitoring–derived average glucose in youth and young adults with cystic fibrosis. Diabetes Care. 2018;41(7):1406-1413.
36.
ChanCLSteckAKSevernCPyleLRewersMZeitlerPS.Lessons from continuous glucose monitoring in youth with pre–type 1 diabetes, obesity, and cystic fibrosis. Diabetes Care. 2020;43(3):e35-e37.
37.
ScullyKJSherwoodJSMartinK, et al. Continuous glucose monitoring and HbA1c in cystic fibrosis: clinical correlations and implications for CFRD diagnosis. J Clin Endocrinol Metab. 2021;107(4):e1444-54.
38.
ChanCLPyleLVigersTZeitlerPSNadeauKJ.The relationship between continuous glucose monitoring and OGTT in youth and young adults with cystic fibrosis. J Clin Endocrinol Metab. 2021;107(2):e548-e560.
39.
SchiaffiniRBrufaniCRussoB, et al. Abnormal glucose tolerance in children with cystic fibrosis: the predictive role of continuous glucose monitoring system. Eur J Endocrinol. 2010;162(4):705-710.
40.
Taylor-CousarJLJanssenJSWilsonA, et al. Glucose >200 mg/dL during continuous glucose monitoring identifies adult patients at risk for development of cystic fibrosis related diabetes. J Diabetes Res. 2016;2016:1527932.
41.
IzsákVDSoósASzakácsZ, et al. Screening methods for diagnosing cystic fibrosis-related diabetes: a network meta-analysis of diagnostic accuracy studies. Biomolecules. 2021;11(4):520.
42.
BrughaRWrightMNolanSBridgesNCarrSB.Quantifying fluctuation in glucose levels to identify early changes in glucose homeostasis in cystic fibrosis. J Cyst Fibros. 2018;17(6):791-797.
43.
ScullyKJBrennerLMartinK, et al. Continuous glucose monitoring and advanced glycation endproducts for prediction of clinical outcomes and development of cystic fibrosis-related diabetes in adults with CF. Front Endocrinol. 2024;15:1293709.
44.
MontaserEFarhyLSRichSS.Enhancing type 1 diabetes immunological risk prediction with continuous glucose monitoring and genetic profiling. Diabetes Technol Ther. 2025;27(4):292-300.
45.
LeonardAJudwareIRVanscoyLL, et al. Feasibility of using continuous glucose monitoring to detect glycemic abnormalities in children with cystic fibrosis [published online ahead of print November 27, 2024]. Horm Res Paediatr. doi:10.1159/000542786.
46.
NICE Guideline. Cystic Fibrosis: Diagnosis and Management [Internet]. London: National Institute for Health and Care Excellence; 2017. https://www.nice.org.uk/guidance/ng78. Accessed January 28, 2026.
47.
AidooEAlexanderSAlshafiKAl-YaghchiC. Clinical Guidelines: Care of Children With Cystic Fibrosis. 9th ed.London: Royal Brompton Hospital; 2023.
48.
The UK Cystic Fibrosis Trust Diabetes Working Group. Management of Cystic Fibrosis Diabetes.2nd ed.London: Cystic Fibrosis Trust; 2022.
49.
HameedSMortonJRJafféA, et al. Early glucose abnormalities in cystic fibrosis are preceded by poor weight gain. Diabetes Care. 2009;33(2):221-226.
50.
WeissLRonsinOReynaudQ, et al. Clinical practice versus guidelines for the screening of cystic fibrosis-related diabetes: a French survey from the 47 centers. J Clin Transl Endocrinol. 2022;28:100298.
51.
YungBLandersAMathaloneBGyiKMHodsonME.Diabetic retinopathy in adult patients with cystic fibrosis-related diabetes. Respir Med. 1998;92(6):871-872.
52.
AndersenHULanngSPresslerTLaugesenCSMathiesenER.Cystic fibrosis-related diabetes: the presence of microvascular diabetes complications. Diabetes Care. 2006;29(12):2660-2663.
53.
SchwarzenbergSJThomasWOlsenTW, et al. Microvascular complications in cystic fibrosis-related diabetes. Diabetes Care. 2007;30(5):1056-1061.
54.
van den BergJMMortonAMKokSWPijlHConwaySPHeijermanHG.Microvascular complications in patients with cystic fibrosis-related diabetes (CFRD). J Cyst Fibros. 2008;7(6):515-519.
55.
PerrinFMSerinoW.Ischaemic heart disease—a new issue in cystic fibrosis. J R Soc Med. 2010;103 suppl 1(suppl 1):S44-S48.
56.
SandoukZNachawiNSimonR, et al. Coronary artery disease in patients with cystic fibrosis—a case series and review of the literature. J Clin Transl Endocrinol. 2022;30:100308.
57.
ThambuluruSRKyazimzadeSDespotesKAKirkDGoralskiJL.Acute ST-elevation myocardial infarction in two young women with cystic fibrosis and cystic fibrosis-related diabetes. J Cyst Fibros. 2022;21(1):e44-e47.
Escudero GarcíaJMartín RivadaÁUribe PosadaASanz SantiagoVArgenteJMartos-MorenoGÁ. Carbohydrate metabolism impairment in children and adolescents with cystic fibrosis. Endocrinol Diabetes Nutr (Engl Ed). 2022;69(8):576-583.
60.
LeclercqAGauthierBRosnerV, et al. Early assessment of glucose abnormalities during continuous glucose monitoring associated with lung function impairment in cystic fibrosis patients. J Cyst Fibros. 2014;13(4):478-484.
61.
PrenticeBJOoiCYStrachanRE, et al. Early glucose abnormalities are associated with pulmonary inflammation in young children with cystic fibrosis. J Cyst Fibros. 2019;18(6):869-873.
62.
BrennanALGyiKMWoodDM, et al. Airway glucose concentrations and effect on growth of respiratory pathogens in cystic fibrosis. J Cyst Fibros. 2007;6(2):101-109.
63.
PallinMKumarSDaleyC, et al. Continuous glucose monitoring indices predict poor FEV1 recovery following cystic fibrosis pulmonary exacerbations. J Cyst Fibros. 2021;20(5):785-791.
64.
MoranAPekowPGroverP, et al. Insulin therapy to improve BMI in cystic fibrosis-related diabetes without fasting hyperglycemia: results of the cystic fibrosis related diabetes therapy trial. Diabetes Care. 2009;32(10):1783-1788.
65.
RakotoarisoaLWeissLLefebvreF, et al. Early glucose abnormalities revealed by continuous glucose monitoring associate with lung function decline in cystic fibrosis: a five-year prospective study. J Diabetes Complications. 2024;38(4):108703.
66.
DeclercqDHelleputteSMarchandS, et al. Glycemic indices at night measured by CGM are predictive for a lower pulmonary function in adults but not in children with cystic fibrosis. J Cyst Fibros. 2023;22(1):59-65.
67.
MerjanehLSidhayeARVuPT, et al. Role of hyperglycemia in cystic fibrosis pulmonary exacerbations. J Cyst Fibros. 2023;22(5):868-874.
HameedSMortonJRFieldPI, et al. Once daily insulin detemir in cystic fibrosis with insulin deficiency. Arch Dis Child. 2012;97(5):464-467.
70.
MozzilloEFranzeseAValerioG, et al. One-year glargine treatment can improve the course of lung disease in children and adolescents with cystic fibrosis and early glucose derangements. Pediatr Diabetes. 2009;10(3):162-167.
71.
HameedSBarnesEHBriodyJ, et al. Insulin for early glycaemic abnormality in children with cystic fibrosis without cystic fibrosis-related diabetes (CF-IDEA): a randomised controlled trial. Lancet Child Adolesc Health. 2025;9(6):371-382.
72.
SchiavonMCobelliCNairKS, et al. Exogenous insulin does not reduce protein catabolism in pre-diabetic cystic fibrosis patients: a randomized clinical trial. J Cyst Fibros. 2025;24(1):57-65.
73.
AhmedAAnkireddypalliAHarindhanavudhiTMoranAMoheetA.Glucagon-like peptide1 receptor agonist treatment of cystic fibrosis-related diabetes complicated by obesity: a cases series and literature review. J Clin Transl Endocrinol. 2024;38:100375.
74.
BogdaniMBlackmanSMRidauraCBellocqJPPowersACAguilar-BryanL.Structural abnormalities in islets from very young children with cystic fibrosis may contribute to cystic fibrosis-related diabetes. Sci Rep. 2017;7(1):17231.
75.
HalilogluBGokdemirYAtayZ, et al. Hypoglycemia is common in children with cystic fibrosis and seen predominantly in females. Pediatr Diabetes. 2017;18(7):607-613.
76.
KilbergMJSheikhSStefanovskiD, et al. Dysregulated insulin in pancreatic insufficient cystic fibrosis with post-prandial hypoglycemia. J Cyst Fibros. 2020;19(2):310-315.
77.
HicksRMarksBEOxmanRMoheetA.Spontaneous and iatrogenic hypoglycemia in cystic fibrosis. J Clin Transl Endocrinol. 2021;26:100267.
78.
FinnESevernCPyleL, et al. Hypoglycemia in children and young adults with cystic fibrosis during oral glucose tolerance testing vs continuous glucose monitoring. Pediatr Pulmonol. 2023;58(9):2495-2504.
79.
SheikhSStefanovskiDKilbergMJ, et al. Early-phase insulin secretion during mixed-meal tolerance testing predicts β-cell function and secretory capacity in cystic fibrosis. Front Endocrinol. 2024;15:1340346.
80.
ArmaghanianNBrand-MillerJCMarkovicTPSteinbeckKS.Hypoglycaemia in cystic fibrosis in the absence of diabetes: a systematic review. J Cyst Fibros. 2016;15(3):274-284.
81.
MannikLAChangKAAnnohPQK, et al. Prevalence of hypoglycemia during oral glucose tolerance testing in adults with cystic fibrosis and risk of developing cystic fibrosis-related diabetes. J Cyst Fibros. 2018;17(4):536-541.
82.
KunzSWilliBSteigertMCasaultaC.Case study: TRIKAFTA and severe symptomatic hypoglycemia: cause or coincidence?J Clin Case Rep Med Images Health Sci. 2022;1(4):001035. https://jmedcasereportsimages.org/jcrmhs-v1-1035/. Accessed July 2, 2025.
83.
YakubovichNGersteinHC.Serious cardiovascular outcomes in diabetes: the role of hypoglycemia. Circulation. 2011;123(3):342-348.
84.
ZinmanBMarsoSPChristiansenE, et al. Hypoglycemia, cardiovascular outcomes, and death: the LEADER experience. Diabetes Care. 2018;41(8):1783-1791.
85.
DesouzaCVBolliGBFonsecaV.Hypoglycemia, diabetes, and cardiovascular events. Diabetes Care. 2010;33(6):1389-1394.
86.
FrostFDycePNazarethDMaloneVWalshawMJ.Continuous glucose monitoring guided insulin therapy is associated with improved clinical outcomes in cystic fibrosis-related diabetes. J Cyst Fibros. 2018;17(6):798-803.
87.
ChanCLShirley BezerraMStefanovskiD, et al. Glycemia and insulin secretion in cystic fibrosis two years after Elexacaftor/Tezacaftor/Ivacaftor: PROMISE-ENDO. J Clin Endocrinol Metab. 2024;110:2674-2684.
88.
WalterEBassJL.The effect of Elexacaftor/Tezacaftor/Ivacaftor on hospitalizations and intravenous antibiotic use. Perm J. 2022;26(1):73-79.
89.
KeremEOrentiAAdamoliA, et al. Cystic fibrosis in Europe: improved lung function and longevity—reasons for cautious optimism, but challenges remain. Eur Respir J. 2024;63(3):2301241. https://publications.ersnet.org/content/erj/63/3/2301241. Accessed August 2, 2025.
90.
ZiaiSCoriatiASt-PierreD, et al. Glucose fluctuations are not modulated by the proportion of calories from macronutrients or spontaneous total energy expenditure in adults with cystic fibrosis. Can J Diabetes. 2016;40(5):389-392.
91.
ScullyKJTruexLBrennanA, et al. Safety and tolerability of a low glycemic load dietary intervention in adults with cystic fibrosis: a pilot study. Front Nutr. 2024;11:1441201.
92.
SimonSLVigersTCampbellK, et al. Reduced insulin sensitivity is correlated with impaired sleep in adolescents with cystic fibrosis. Pediatr Diabetes. 2018;19(7):1183-1190.
93.
MaderJKWongJCFreckmannG, et al. The use of continuous glucose monitoring to diagnose stage 2 type 1 diabetes. J Diabetes Sci Technol. 2025;19(4):1109-1127.
