Table 2

Summary of recommendations for the clinical care of CFRD

Screening recommendations
  1. The use of A1C as a screening test for CFRD is not recommended. (ADA-B; USPSTF-D)

  2. Screening for CFRD should be performed using a 2-h 75-g OGTT. (ADA-E; Consensus)

  3. Annual screening for CFRD should begin by age 10 years in all CF patient s who do not have CFRD. (ADA B; USPSTF-B)

  4. CF patients with acute pulmonary exacerbation requiring intravenous antibiotics and/or systemic glucocorticoids should be screened for CFRD by monitoring fasting and 2-h postprandial plasma glucose levels for the first 48 h. If elevated blood glucose levels are found by SMBG, the results must be confirmed by a certified laboratory. (ADA-E; Consensus)

  5. 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 feeding initiation and then monthly by SMBG. Elevated glucose levels detected by SMBG must be confirmed by a certified laboratory. (ADA-E; Consensus)

  6. Women with CF who are planning a pregnancy or confirmed pregnant should be screened for preexisting CFRD with a 2-h 75-g fasting OGTT if they have not had a normal CFRD screen in the last 6 months. (ADA-E; Consensus)

  7. Screening for gestational diabetes mellitus is recommended at both 12–16 weeks' and 24–28 weeks' gestation in pregnant women with CF not known to have CFRD, using a 2-h 75-g OGTT with blood glucose measures at 0, 1, and 2 h. (ADA-E; Consensus)

  8. Screening for CFRD using a 2-h 75-g fasting OGTT is recommended 6–12 weeks after the end of the pregnancy in women with gestational diabetes mellitus (diabetes first diagnosed during pregnancy). (ADA-E; Consensus)

  9. 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. 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. (ADA-E; Consensus)

Diagnosis recommendations
  1. During a period of stable baseline health the diagnosis of CFRD can be made in CF patients according to standard ADA criteria. 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. (ADA-E; Consensus)

    • 2-h OGTT plasma glucose ≥200 mg/dl (11.1 mmol/l)

    • FPG ≥126 mg/dl (7.0 mmol/l)

    • A1C ≥ 6.5% (A1C <6.5% does not rule out CFRD because this value is often spuriously low in CF.)

    • Classical symptoms of diabetes (polyuria and polydipsia) in the presence of a casual glucose level ≥200 mg/dl (11.1 mmol/l)

  2. The diagnosis of CFRD can be made in CF patients with acute illness (intravenous antibiotics in the hospital or at home, systemic glucocorticoid therapy) when FPG levels ≥126 mg/dl (7.0 mmol/l) or 2-h postprandial plasma glucose levels ≥200 mg/dl (11.1 mmol/l) persist for more than 48 h. (ADA-E; Consensus)

  3. The diagnosis of CFRD can be made in CF patients on enteral continuous drip feedings when mid- or postfeeding plasma glucose levels exceed 200 mg/dl (11.1 mmol/l) on 2 separate days. (ADA-E; Consensus)

  4. 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 75-g OGTT if any one of the following is present:

    • FPG ≥92 mg/dl (5.1 mmol/l)

    • 1-h plasma glucose ≥180 mg/dl (10.0 mmol/l)

    • 2-h plasma glucose ≥153 mg/dl (8.5 mmol/l) (ADA-E; Consensus)

    CF patients with gestational diabetes mellitus are not considered to have CFRD, but require CFRD screening 6–12 weeks after the end of the pregnancy. (ADA-E; Consensus)

  5. Distinguishing between CFRD with and without FH is not necessary. (ADA-B, USPSTF-D)

  6. The onset of CFRD should be defined as the date a person with CF first meets diagnostic criteria, even if hyperglycemia subsequently abates. (ADA-E; Consensus)

Management recommendations
  1. Patients with CFRD should ideally be seen quarterly by a specialized multidisciplinary team with expertise in diabetes and CF. (ADA-E; Consensus)

  2. Patients with CFRD should receive ongoing diabetes self-management education from diabetes education programs that meet national standards for DSME. (ADA-E; Consensus)

  3. Patients with CFRD should be treated with insulin therapy. (ADA-A; USPSTF-B)

  4. 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. (ADA-A; USPSTF-D)

  5. Patients with CFRD who are on insulin should perform SMBG at least three times a day. (ADA-E; Consensus)

  6. 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. (ADA-E; Consensus)

  7. A1C measurement is recommended quarterly for patients with CFRD. (ADA-E; Consensus)

  8. For many patients with CFRD, A1C treatment goal is <7%, bearing in mind that higher or lower goals may be indicated for some patients and that individualization is important. (ADA-B; USPSTF-B)

  9. CFF evidence-based guidelines for nutritional management are recommended for patients with CFRD. (ADA-E; Consensus)

  10. Patients with CFRD should be advised to do moderate aerobic exercise for at least 150 min per week. (ADA-E; Consensus)

Diabetes complications recommendations
  1. Education about the symptoms, prevention, and treatment of hypoglycemia, including the use of glucagon, is recommended for patients with CFRD and their care partners. (ADA-E; Consensus)

  2. Patients with CFRD should have their blood pressure measured at every routine diabetes visit as per ADA guidelines. Patients found to have systolic blood pressure ≥130 mmHg or diastolic blood pressure ≥80 mmHg or >90th percentile for age and sex for pediatric patients should have repeat measurement on a separate day to confirm a diagnosis of hypertension. (ADA-E; Consensus)

  3. 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. (ADA-E; Consensus)

  4. 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. (ADA-E; Consensus)

  5. 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. (ADA-E; Consensus)