Feature Article

Cystic fibrosis-related diabetes: early diagnosis and management

Feature Article

Cystic fibrosis-related diabetes: early diagnosis and management

Shihab Hameed, Bernadette J. Prentice, Charles F. Verge

Figures

© fizkes/istockphoto.com model used for illustrative purposes only
© fizkes/istockphoto.com model used for illustrative purposes only

Abstract

Cystic fibrosis-related diabetes (CFRD) can present slowly  and may significantly impair patient health and survival. Regular screening of glucose levels and insulin therapy are cornerstones of management. Patient education is important to help informed decision-making. GPs can play an important role in the early identification and management of CFRD as part of a multidisciplinary team.

Key Points

  • As a part of the broader multidisciplinary cystic fibrosis (CF) team, GPs should be aware of the symptoms and signs of emerging CF-related diabetes (CFRD).
  • Initially these may include gradual unexplained weight and/or lung function decline, more frequent respiratory exacerbations and infections and, later, the classic symptoms of diabetes – polyuria and polydipsia. Ketoacidosis may occur but is rare.
  • Insulin therapy to correct insulin deficiency is the mainstay of treatment. The high-calorie CF diet is continued, and is guided by a dietitian with expertise inCF.
  • Self-monitoring of blood glucose levels using finger-prick testing is needed. Continuous glucose monitoring is also increasingly used and provides real-time interstitial glucose levels to help the patient make informed decisions on dosing, activity and diet. Appropriate education is needed.
  • Current diagnostic thresholds for CFRD are taken from type 2 diabetes, and patients with CFRD often show a decline in weight and lung function before diagnosis.
  • CF modulatory therapies may delay the timing and expression of insulin deficiency in patients with CF, but more research is needed in this area.