Cystic fibrosis-related diabetes: what the pediatrician should know

Cystic fibrosis-related diabetes: what the pediatrician should know | Image Credit: © KaterynaNovikova - © KaterynaNovikova - stock.adobe.com.

In a recent article, the effects and management of cystic fibrosis were discussed.

Cystic fibrosis, caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, increases the risks of respiratory disease, exocrine pancreatic insufficiency, and liver impairment. This occurs because of impairments in the sodium chloride cotransport channel.

Delta F508, defined as, “a deletion of phenylalanine at residue 508 of the CFTR gene” by authors of the article, is the most common cystic fibrosis mutation. As pancreatic fibrotic damage progresses, endocrine function of the pancreas may also be impaired, causing derangement of glucose metabolism.

Cystic fibrosis-related diabetes (CFRD) has a complex and multifactorial pathophysiology, and there are multiple types of glucose metabolism alterations which can occur in cystic fibrosis. A review was performed to evaluate diagnosis and therapy procedures of CFRD.

Authors highlighted the diagnostic criteria used to diagnose CFRD. Diagnosis is often accomplished through an oral glucose tolerance test (OGTT). Once yearly screening with OGTT is recommended in children aged at least 10 years with cystic fibrosis.

The 6 classes of glucose metabolism alterations in cystic fibrosisidentified in pediatric patients include impaired fasting glucose, abnormal glucose tolerance, impaired glucose tolerance, CFRD without fasting hyperglycemia, CFRD with fasting hyperglycemia, and intermediate hyperglycemia. These classes should be identified to reduce morbidity and mortality.

Therapy methods for different types of glucose arrangement are associated with improved metabolic outcomes, pulmonary function, growth, nutritional status, and respiratory infection recurrence. 

One method of therapy is nutritional treatment, but it is not recommended above a pharmacological approach. Children and adolescents with CFRD are recommended to have a hypercaloric and hyperproteic diet without restricting salt, fats, and carbohydrates.

A recent survey indicated pediatric practitioners are more likely to treat CFRD with insulin while adult practitioners are more likely to treat CFRD with repaglinide. However, data has indicated safety and efficacy from repaglinide equal to that of insulin in patients aged 10 years and older.

Insulin remains the recommended method of therapy in children and adolescents with CFRD. Insulin therapy should be given with extreme personalization to ensure anindividual’s therapy needs are met, with factors such as the degree of hyperglycemia and the time of day when hyperglycemic events commonly occur needing to be considered.

An insulin regimen can vary from exclusive basal insulin to classic basal-bolus insulin based on glycemic phenotype. In a classic basal-bolus insulin regimen, prandial insulin is added to the basal insulin at each meal.

Insulin sensitivity should be monitored in children who are often more insulin-sensitive than adults. Insulin pumps may be used as an alternative method to intensive multiinjection insulin therapy.

New CFTR-modulating therapies such as Elexacaftor, Tezacaftor and Ivacaftor have increased the life expectancy of patients with cystic fibrosis. Investigators concluded early detection and treatment is vital to improve patient quality of life.

Reference

Schiaffini R & Pampanini A. Diabetes and prediabetes in children with cystic fibrosis. Current Opinion. 2023;35(4):481–485. doi:10.1097/MOP.0000000000001259