Cystic Fibrosis-Related Diabetes

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Cystic fibrosis-related diabetes (CFRD) is a unique kind of diabetes that affects people with cystic fibrosis. The pediatric endocrinologists with Norton Children’s Endocrinology, affiliated with the UofL School of Medicine, and the Wendy Novak Diabetes Institute work alongside Norton Children’s pulmonologists to provide individualized treatment plans to help children with cystic fibrosis (CF) manage their CFRD.

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CFRD shares some characteristics of both Type 1 and Type 2 diabetes. Children with CF experience a thick and sticky mucus that clogs up tubes, ducts and passageways in different organs. This mucus can cause the pancreas to scar, preventing it from producing insulin as it should. This causes children with CF to become insulin deficient like children with Type 1 diabetes.

The pancreas may still make insulin, but not at healthy levels. Children with CFRD may be insulin resistant like people with Type 2 diabetes. This means that they do to respond to insulin the way people without diabetes do, which causes high levels of glucose (sugar) in the blood. While CFRD has similarities to Type 1 and Type 2 diabetes, the treatment and management of CFRD is different.

CFRD Diagnosis

Children with CF who are age 8 and older should be screened for CFRD every year through an oral glucose tolerance test (OGTT). An OGTT is a two-hour test that checks blood sugar levels before and after drinking a sweet drink. The OGTT is the best way of diagnosing CFRD because the traditional hemoglobin A1c (HbA1c) test often used to diagnose other types of diabetes often is normal in children with CF.

CFRD Treatment

The board-certified, fellowship-trained endocrinologists with Norton Children’s and UofL work to provide an individualized plan for your child to manage CFRD.

The Wendy Novak Diabetes Institute team works alongside other Norton Children’s specialists, including pulmonologists, to help you and your child manage CF and CFRD by:

  • Providing nutritional guidance to help your child gain and maintain a healthy weight. Children with CFRD need a diet that addresses both their CF and their CFRD, and our registered dietitians and diabetes educators can create a unique plan for your child.
  • Monitoring blood sugar and adjusting insulin therapy to keep blood sugar levels stable to avoid complications

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