Cystic Fibrosis-Related Diabetes Clinical Care Guidelines

As early cystic fibrosis-related diabetes (CFRD) may be clinically silent, these guidelines highlight the importance of regular screening. Management recommendations focus on insulin therapy and ongoing care by a team with knowledge of CF and diabetes.

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Summary
  • Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity in cystic fibrosis and occurs in up to 20 percent of adolescents and 50 percent of adults with CF.
  • Management recommendations focus on insulin therapy and ongoing care provided by a multidisciplinary team with knowledge of CF and diabetes.

Clinical Care Guidelines for Cystic Fibrosis-Related Diabetes: Executive Summary

Clinical Care Guidelines for Cystic Fibrosis-Related Diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010;33(12):2697-2708. 

Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity in cystic fibrosis and occurs in up to 20 percent of adolescents and 50 percent of adults with CF. A diagnosis of CFRD has a negative impact on lung function, nutrition, and survival. As early CFRD may be clinically silent, these guidelines highlight the importance of regular screening in various clinical contexts. A diagnosis of CFRD is made based on standard American Diabetes Association criteria, but may also be made after detection of intermittent hyperglycemia during illness or gastrostomy feedings. Management recommendations focus on insulin therapy and ongoing care provided by a multidisciplinary team with knowledge of CF and diabetes.

Methodology

The latest Cystic Fibrosis-Related Diabetes (CFRD) Clinical Care Guidelines from 2010 summarize screening, diagnosis and management recommendations resulting from a joint collaboration of the Cystic Fibrosis Foundation, American Diabetes Association (ADA) and the Pediatric Endocrine Society. The report references the ADA Standards of Medical Care, published annually in Diabetes Care for all people with diabetes, but focuses on aspects unique to the care of CFRD. The process for developing these guidelines included an: expert committee, specific questions, systematic literature review, recommendations drafted, committee vote, final recommendations, and grade (US Preventative Task Force [USPSTF] and ADA Schemes). Find additional information about the USPSTF grading definitions.

Recommendations

Screening Patients Without a Confirmed CFRD Diagnosis

Recommendations Evaluation of the Evidence

1. Recommended test is a 2-hour 75g-Oral Glucose Tolerance Test (OGTT).

  1. Annual screening should begin by age ≥ 10 yrs.
  2. If planning a pregnancy or confirmed pregnant, obtain OGTT if prior results were abnormal or not done in last 6 months.
  3. If undergoing any organ transplantation and not screened in last 6 months, obtain OGTT preoperatively.

ADA - E
Consensus

ADA - B
USPSTF - B

ADA - E
Consensus

ADA - E
Consensus

2. HbA1c as a screening test for CFRD is not recommended.

ADA - B
USPSTF - D

3. Self-monitoring blood glucose (SMBG) -- if elevated, confirm by central laboratory glucose measurements.

  1. Pulmonary exacerbations: Monitor fasting and 2h-postprandial glucoses for 48 hours with SMBG when admitted for exacerbations, IV antibiotics, and/or systemic glucocorticoids.
  2. Gastrostomy feedings: Monitoring mid- and post feeding is recommended for those on continuous enteral feeds, at time of gastrostomy feeding initiation, then monthly.

 

 

ADA - E
Consensus

 

ADA - E
Consensus

4. Gestational diabetes screening is recommended at both 12-16 weeks and 24-48 weeks gestation with a 2h 75g-OGTT and 0, 1, and 2h glucose measures.

  1. If GDM is diagnosed, repeat OGTT is recommended 6-12 weeks post-partum.

ADA - E
Consensus

 

ADA - E
Consensus

Diagnosis

Recommendations Evaluation of the Evidence
5. During a period of stable baseline health, the diagnosis of CFRD can be made according to standard ADA criteria (below). Testing should be done on 2 separate days to confirm, unless there are unequivocal symptoms of hyperglycemia (polyuria, polydipsia) or symptoms and the presence of random glucoses ≥ 200 mg/dl.
  1. 2-hour OGTT plasma glucose ≥ 200 mg/dl (11.1 mmol/L)
  2. Fasting plasma glucose (FPG) ≥ 126 mg/dl (7.0 mmol/L)
  3. Hemoglobin A1c ≥ 6.5% (note: HbA1c is often low in CF and a value < 6.5% does not rule out CFRD)
ADA - E
Consensus
6. A diagnosis of CFRD can be made in patients with acute illness (hospitalized on IV antibiotics or systemic glucocorticoids) when FPG ≥ 126 mg/dl or 2h post-prandial glucoses are ≥ 200 mg/dl and persist for more than 48 hours. ADA - E
Consensus
7. A diagnosis of CFRD can be made in patients on enteral continuous drip feedings when mid- or post-feeding glucose levels ≥ 200 mg/dl on 2 separate days. ADA - E
Consensus
8. A diagnosis of gestational diabetes should be made based on International Association of the Diabetes and Pregnancy Study Groups recommendations where diabetes is diagnosed based on 0-, 1-, and 2-hr glucose levels with a 75-g OGTT if any one of the following is present:
  1. FPG ≥ 92 mg/dl (5.1 mmol/L)
  2. 1h plasma glucose ≥ 1 80 mg/dl (10.0 mmol/L)
  3. 2h plasma glucose ≥ 153 mg/dl (8.5 mmol/L)
ADA - E
Consensus
9. The onset of CFRD should be defined as the date a person first meets diagnostic criteria, even if hyperglycemia subsequently abates. ADA - E
Consensus

Management of CFRD

Recommendations Evaluation of the Evidence
10. Patients should ideally be seen quarterly by a specialized multidisciplinary team with expertise in diabetes and CF. ADA - E
Consensus
11. Patients should receive ongoing diabetes self-management education from programs that meet national standards. ADA - E
Consensus
12. Patients should be treated with insulin therapy. ADA - A
USPSTF - B
13. 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
14. Patients on insulin should perform SMBG at least 3x daily. ADA - E
Consensus
15. Patients should strive to attain plasma glucose goals as per ADA recommendations for all people with diabetes. ADA - E
Consensus
16. HbA1c measurements are recommended quarterly. ADA - E
Consensus
17. HbA1c treatment goal is < 7%. ADA - E
Consensus
18. CF Foundation evidence-based guidelines for nutritional management are recommended. ADA - E
Consensus
19. Moderate aerobic exercise for at least 150 minutes per week is advised.  

20. Monitoring for complications:

  1. Hypoglycemia education, including glucagon use, is recommended.
  2. Blood pressure should be measured at routine diabetes visits per ADA guidelines. Those with elevated blood pressure measurements should have their measurements repeated on a separate day to confirm a diagnosis of hypertension.
  3. Annual monitoring for microvascular complications is recommended beginning 5 years after CFRD diagnosis.
  4. Patients with hypertension or microvascular complications should receive treatment as recommended by ADA for all people with diabetes, with the exception of no sodium restriction and, in general, no protein restriction.
  5. An annual lipid profile is recommended for patients with any of the following risk factors: obesity, family history of coronary artery disease, or immunosuppressive therapy following transplantation.

ADA - E
Consensus
ADA - E
Consensus
ADA - E
Consensus

 

 

ADA - E
Consensus
 

ADA - E
Consensus

 

 

ADA - E
Consensus

New Issues

Since 2010, additional data have been published reporting higher rates of glucose abnormalities in children with CF aged younger than 10 years than previously reported. More recent insights into CFRD pathophysiology suggest a potential role of CFTR mutations in the beta cell, and these defects may contribute to early impairments in insulin secretion detectable in young children. Therefore, screening of high-risk children aged younger than 10 years may also be warranted.

Unanswered Questions

  • Should the age of CFRD screening be lowered to include patients under age 10?
  • What is the role of alternate CFRD screening modalities (e.g., glucose challenge test, continuous glucose monitoring)?
  • How will CFTR modulators impact the natural history of CFRD development and progression?
  • Is there a role for newer anti-diabetic agents in CFRD (e.g., GLP1 agonists, DPP4 inhibitors, SGLT2 inhibitors)?
  • What is the role for insulin in CF-prediabetes?
  • What is the impact of the burden of therapy on self-management?

Further Reading

Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.

  1. Ode KL, Moran A. New insights into CFRD in children. Lancet Diabetes Endocrinol. 2013;1(1):52-58.
  2. A review of updates on CFRD pathophysiology including findings from animal studies and research in young children.
  3. Lewis C, Blackman SM, Nelson A, 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.
  4. An update on the latest mortality trends in adults with CF and CFRD.
  5. Bellin MD, Laguna T, Leschyshyn J, et al. Insulin secretion improves in cystic fibrosis following ivacaftor correction of CFTR: a small pilot study. Pediatr Diabetes. 2013;14(6):417-421.
  6. A small pilot study finding improved insulin secretion in CF with CFTR modulators.

This executive summary was prepared by:
Christine L. Chan, MD (University of Colorado) and Antoinette Moran, MD (University of Minnesota)

The guidelines were published in December 2010, they were reviewed in April 2019 and it was determined that no update is needed at this time.

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