Introduction:

Cystic Fibrosis (CF) is a hereditary recessive disease that affects several organs, mainly within the respiratory and digestive systems. Cystic Fibrosis Related Diabetes (CFRD) is one of its main complications.

Objectives:

To study the specific complications of pediatric patients suffering from CFRD and how they affect a patient’s quality of life. To compare the mortality rates of pediatric patients with CF and with CFRD. To highlight the importance of nurses in the management of pediatric patients with CFRD.

Methodology:

A systematic bibliographic review was conducted. We included studies, in English and Spanish, published over the last 10 years, which analysed a population aged between 0 and 9 years old with CF and/or CFRD.

Results:

Four databases were used for the systematic search. We analysed 10 articles to address the objectives.

Conclusions:

The complications caused by CFRD and the care that patients require affect the patient’s quality of life. Progress both with diagnosis and with treatment has improved the quality of life of patients, and has contributed to reductions in the difference between the mortality rates of patients with CF and without CFRD. Nurses who care for children with CFRD are essential as an information resource for patients and their families. Nurses must convey the importance of therapeutic adherence to achieve a better quality of life.

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