Managing Cystic Fibrosis-Related Diabetes (CFRD): An Instruction Guide for Patients & Families

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CF patients not known to have diabetes who are undergoing any transplantation procedure should be screened preoperatively by OGTT if they have not had CFRD screening in the last 6 months.

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Plasma glucose levels should be monitored closely in the perioperative critical care period and until hospital discharge. Screening guidelines for patients who do not meet diagnostic criteria for CFRD at the time of hospital discharge are the same as for other CF patients. Testing should be done on 2 separate days to rule out laboratory error unless there are unequivocal symptoms of hyperglycemia polyuria and polydipsia ; a positive FPG or A1C can be used as a confirmatory test, but if it is normal the OGTT should be performed or repeated.

If the diagnosis of diabetes is not confirmed, the patient resumes routine annual testing. Diagnosis of gestational diabetes mellitus should be made based on the recommendations of the IADPSG 45 where diabetes is diagnosed based on 0-, 1-, and 2-h glucose levels with a g OGTT if any one of the following is present:. The onset of CFRD should be defined as the date a person with CF first meets diagnostic criteria, even if hyperglycemia subsequently abates. Patients with CFRD should ideally be seen quarterly by a specialized multidisciplinary team with expertise in diabetes and CF.

Oral diabetes agents are not as effective as insulin in improving nutritional and metabolic outcomes in CFRD and are not recommended outside the context of clinical research trials. Patients with CFRD should strive to attain plasma glucose goals as per the ADA recommendations for all people with diabetes, bearing in mind that higher or lower goals may be indicated for some patients and that individualization is important. Patients with CFRD should be advised to do moderate aerobic exercise for at least min per week.

Education about the symptoms, prevention, and treatment of hypoglycemia, including the use of glucagon, is recommended for patients with CFRD and their care partners.

CF Foundation - Living with CFRD

Annual monitoring for microvascular complications of diabetes is recommended using ADA guidelines, beginning 5 years after the diagnosis of CFRD or, if the exact time of diagnosis is not known, at the time that FH is first diagnosed. Patients with CFRD diagnosed with hypertension or microvascular complications should receive treatment as recommended by ADA for all people with diabetes, except that there is no restriction of sodium and, in general, no protein restriction. An annual lipid profile is recommended for patients with CFRD and pancreatic exocrine sufficiency or if any of the following risk factors are present: obesity, family history of coronary artery disease, or immunosuppressive therapy following transplantation.


CFRD is often clinically silent. In other populations, the primary consequences of unrecognized diabetes are macrovascular and microvascular disease. In CF, the nutritional and pulmonary consequences of diabetes are of greater concern.

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CFRD is associated with weight loss, protein catabolism, lung function decline, and increased mortality 2 , 3 , 7 — 17 , and thus regular screening is warranted. Although hemoglobin A1C A1C may become the standard screening test for type 2 diabetes 5 , the committee concluded that it is not sufficiently sensitive for diagnosis of CFRD and thus should not be used as a screening test.

Eight studies were identified that assessed A1C as a screening test in this population 7 , 18 — The authors of one prospective cohort study of 62 participants with CF and healthy control subjects reported that A1C levels were higher in the CF group than among the control subjects, leading them to suggest that the use of A1C was appropriate However, six studies including two prospective cohort studies [ 7 , 21 ], two cross-sectional studies [ 19 , 20 ], one case-control study [ 23 ], and one case series [ 22 [ with a total of participants demonstrated low degrees of correlation between A1C and glucose tolerance status 7 , 19 — Additionally, a cross-sectional study of participants with CF demonstrated a low positive predictive value of the A1C test Fructosamine, urine glucose, and random glucose levels have low sensitivity in the CF population 20 , 23 , Continuous glucose monitoring is not recommended as a screening tool because intermittent hyperglycemia detected in this fashion is not diagnostic for diabetes and there are no outcome data to determine its clinical significance.

Fasting plasma glucose FPG identifies patients with CFRD with but not those without fasting hyperglycemia FH , and thus this test will miss the diagnosis of diabetes in approximately half of CF patients 1. Although it is an imperfect test due to the inherent variability of the test and the variability observed in individual CF patients over time, longitudinal studies demonstrate that a diabetes diagnosis by OGTT correlates with clinically important CF outcomes including the rate of lung function decline over the next 4 years 12 , the risk of microvascular complications 27 , and the risk of early death 1 , 2.

In a multicenter, multinational study, the OGTT identified patients who benefited from insulin therapy The OGTT should be performed in the morning during a period of stable baseline health at least 6 weeks since an acute exacerbation using the World Health Organization protocol 5. Patients fast for at least 8 h water is permitted and should consume a minimum of g kcal of carbohydrate per day for the preceding 3 days generally not an issue because CF patients have high-calorie diets.

The patient drinks a standard beverage containing 1. Glucose levels are measured at baseline and 2 h. Three studies with a total of participants were identified that provided information about the appropriate age at which to start screening for CFRD 1 , 21 , These studies—a retrospective cohort study 1 , a prospective cohort study 21 , and a cross-sectional study 24 —reported a significantly higher prevalence and incidence of CFRD beyond the first decade of life.

Screening included both pancreatic sufficient and insufficient patients. The committee concluded that these findings suggest that annual screening for CFRD should start by age 10 years in all CF patients. Because clinical deterioration in nutritional and pulmonary status begins 6—24 months prior to a diagnosis of CFRD 29 , 30 , early detection by annual screening is warranted. CF patients experience frequent pulmonary exacerbations, some of which require treatment either in the hospital or at home with intravenous antibiotics.

Treatment at times includes systemic glucocorticoids. In clinical experience, hyperglycemia that develops during acute illness occasionally resolves after a day or two of medical therapy, but usually lasts for at least 2—6 weeks. CF patients are frequently ill, and hyperglycemia returns with each subsequent bout of illness, often several times a year. Insulin deficiency and insulin resistance generally progress over time. Long-term microvascular 27 and pulmonary 1 , 2 outcomes correlate with duration of CFRD first diagnosed during acute illness, even with intervening periods of normal or impaired glucose tolerance IGT.

If glucose levels do not meet diagnostic criteria for CFRD, testing can be discontinued after 48 h. For patients receiving therapy at home, SMBG can be performed. However, SMBG levels are not sufficiently accurate to make a diagnosis of CFRD, and hyperglycemia should be confirmed by laboratory plasma glucose measurement.

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Supplemental continuous drip feedings are commonly prescribed for malnourished CF patients. Although there are few data available specific to this situation, mid-feeding hyperglycemia may compromise efforts to gain weight. The Committee felt that glucose levels in the middle and immediately after the gastrostomy tube feeding should be measured in the hospital and at these same time points once a month at home using SMBG.

Screening for CFRD by measuring mid- and immediate postfeeding plasma glucose levels is recommended for CF patients on continuous enteral feedings, at the time of gastrostomy tube feeding initiation and then monthly at home. Pregnancy is a state of marked insulin resistance, and many women with CF cannot produce the extra insulin required to meet this demand 31 — In addition to the usual concerns about the effect of hyperglycemia on the fetus, diabetes can exacerbate the difficulties many women with CF have in achieving a positive protein balance and sufficient weight gain during pregnancy Women with CF not known to have CFRD who are contemplating pregnancy should be evaluated prior to conception to rule out preexisting CFRD or be tested immediately upon confirmation of the pregnancy if they have not had an OGTT in the previous 6 months.

Because women with CF are at high risk for development of hyperglycemia during pregnancy gestational diabetes mellitus , the 2-h g OGTT should be performed at the end of both the first and second trimesters. There is an almost universal requirement for insulin in the immediate critical care postoperative period in CF patients undergoing transplantation procedures, and many have long-term insulin requirements after transplantation 34 — A diagnosis of CFRD prior to transplantation may increase complications of surgery and has a negative impact on survival, at least in the early postoperative period when infection, bleeding, and multiorgan failure are the most common causes of death 34 , Aggressive management may have a positive impact on outcomes Many patients with normal fasting and 2-h glucose levels have elevation in the middle of the OGTT indeterminate glycemia [INDET] or when assessed randomly or by continuous glucose monitoring.

In the general population, they are considered pre-diabetic conditions, associated with a high risk of future development of diabetes They are based on the population risk of microvascular disease, and patients with CF are also at risk for these complications 27 , 41 — The committee questioned whether the diagnostic thresholds should be lower for the CF population as CFRD is known to have a negative impact on CF pulmonary status 2 , 10 , 11 , given that pulmonary disease is the chief morbidity in CFRD.

Even less severe glucose tolerance abnormalities such as IGT are associated with lung function decline 12 , However, sufficient outcome-based data are not available at present to determine whether more stringent diagnostic glucose thresholds more appropriately reflect risk for the CF population. Testing should be done on two separate days to rule out laboratory error unless there are unequivocal symptoms of hyperglycemia polyuria and polydipsia ; a positive FPG or A1C can be used as a confirmatory test, but if it is normal the OGTT should be performed or repeated.

There are special situations when a diagnosis of CFRD must be considered in patients who are not in their baseline state of health. CF patients frequently first develop hyperglycemia during stressors such as acute illness or continuous enteral nutrition. Blood glucose levels may normalize when the stress is not present. Longitudinal outcome data have shown that CF morbidity and mortality are associated with CFRD first diagnosed in the acute illness setting when hyperglycemia has persisted beyond 48 h 1 , 2 , Based on this experience, the committee developed the following recommendations.

In the general population, the Hyperglycemia and Adverse Pregnancy Outcome HAPO study showed a continuous risk of adverse perinatal and maternal outcomes with increasing glycemia at 24—28 weeks' gestation 45 , and a recent multicenter, randomized study has demonstrated that aggressive treatment of mild gestational diabetes mellitus improves outcomes Diagnosis of gestational diabetes mellitus should be made based on the recommendations of the International Association of the Diabetes and Pregnancy Study Groups IADPSG 45 where diabetes is diagnosed based on 0-, 1-, and 2-h glucose levels with a g OGTT if any one of the following is present:.

In addition, in a randomized controlled trial, insulin therapy reversed chronic weight loss in patients with CFRD FH- 28 , suggesting that both groups of CFRD patients should receive insulin treatment and that there is no need to distinguish them diagnostically. Defining the date of onset of CFRD is important because long-term outcomes are related to disease duration. Glucose tolerance gradually worsens with age in CF as a result of steadily declining insulin production 1 , At any point in time, however, an individual's glucose tolerance may acutely fluctuate depending on his or her general state of health.

The committee defined the onset of CFRD as the first time a patient meets diabetes diagnostic criteria. Longitudinal studies of patients whose date of diagnosis was considered to be either the first time they had a positive OGTT or the first time they had persistent hyperglycemia during acute illness have shown that duration of CFRD determined by these criteria correlates with clinically relevant outcomes including microvascular complications 27 and mortality 1 , 2.

Cystic Fibrosis–Related Diabetes in Adolescents

Hyperglycemia may resolve without treatment during periods of stable health, but insulin secretion remains insufficient to control glucose under stress, and hyperglycemia will recur. Although in the general population critically ill patients who experience stress hyperglycemia are not given a diagnosis of diabetes, our recommendation differs for CF patients who develop hyperglycemia during acute exacerbations of their chronic illness. In CF, illness-associated hyperglycemia is a reflection of insulin insufficiency as well as resistance and is a recurrent event.

Defining the disease by this criterion encourages early intervention to improve long-term outcomes. The diabetes team should be intimately familiar with CFRD, recognizing differences between this and type 1 and type 2 diabetes pathophysiology and treatment. Good communication between diabetes and CF care providers is essential. Poor team communication and inadequate or conflicting information from health care providers have been identified as significant sources of stress for patients with CFRD Although there are few CF-specific data, it has been well established in the general diabetes population that patients must be given the educational tools and support they need to assume a central role in determining their treatment goals and implementing the management plan 5.

Initial and ongoing diabetes self-management education DSME is an integral component of care. In addition to medical issues, the role of the patient-centered medical team is to encourage and support the patient and family. The treatment team should address psychosocial issues and recognize the risk of depression.

Emotional well-being is strongly correlated with diabetes outcomes, and the additional diagnosis of diabetes can be a significant burden. There may also be financial concerns associated with this diagnosis. Patients with CFRD are insulin insufficient, and based on available data, insulin is the only recommended treatment. There is evidence that CF patients on insulin therapy who achieve glycemic control demonstrate improvement in weight, protein anabolism, pulmonary function, and survival.

Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes

Ten studies with a total of participants were identified that addressed insulin therapy in CFRD, including one randomized controlled trial 28 , five before-after studies 49 — 53 , one retrospective cohort study 1 , one prospective cohort study 54 , and two case-control studies 29 , These studies reported improved outcomes associated with the use of insulin in patients with CFRD, including those without FH.

There is little evidence regarding the superiority of specific insulin regimens in CFRD, and clinical judgment should be used to choose the best regimen for each patient. CFRD patients still have endogenous insulin secretion, and, except during acute illness, treatment is often similar to that of patients with type 1 diabetes in the honeymoon period.

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During acute illness or systemic glucocorticoid treatment, insulin requirements steeply rise, two- to fourfold. Once the illness resolves, it generally takes about 4—6 weeks for insulin requirements to gradually return to baseline. Careful monitoring for hypoglycemia is required during this period.

Specific insulin treatment suggestions are presented in supplementary Table 2. At the time of the last consensus conference 44 , it was not clear whether CFRD patients without FH should receive insulin treatment. A recently completed trial demonstrated that treatment with premeal rapid-acting insulin was able to reverse chronic weight loss in this population, and thus insulin therapy is indicated Whether basal insulin therapy alone 54 or basal-bolus insulin therapy would be as beneficial as premeal insulin alone in CFRD FH- remains to be determined.

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The available data suggest that oral agents are not as effective as insulin in CFRD. Four studies with a total of participants compared oral hypoglycemic therapy with insulin therapy in CFRD 14 , 28 , 56 , These included one randomized controlled trial 28 , one randomized controlled crossover trial 57 , one prospective cohort study 56 , and one retrospective cohort study Oral hypoglycemic agents studied included sulfonylureas e. Potential CF-specific concerns associated with various noninsulin diabetes agents are presented in supplementary Table 3.

ADA has established plasma glucose goals for people with diabetes 5.