Cystic fibrosis related diabetes: clinical cases in terms of principles of pediatric palliative care

Abstract

Cystic fibrosis related diabetes (CFRD)- is the most common comorbidity in people with cystic fibrosis , occurring in 20% of adolescents and 40–50% of adults. According to the Guidetothe Development of Children's Palliative Care Services life-limiting conditions may be delineated at four broad groups. Cystic fibrosis is a problem from group 2 - conditions where there may be long periods of intensive treatment aimed at prolonging life and allowing participation in normal childhood activities, but premature death is still possible. Basic aspects of CFRD were discussed, such as cause of the disease, genetic defects, differential diagnosis and principles of treatment. Two clinical cases of pediatric CFRD patients resented to highlight problems of management in terms of potential palliative care. According to our observations, patients with the CFRD, unfortunately, are perceived as patients with cystic fibrosis with no clear attention to diabetes. Both of them were presented with pure diabetes control due to unclear understanding CFRD with concomitant diet incompliance, irregular records, missed insulin injections, exercising limitations. We concluded that CFRD patients must be assessed by members of multidisciplinary team at CF centers, working by the principle of palliative care. There is necessary since the moment of CF diagnosing to involve endocrinologist and dietician due to of endocrine problems. Psychologist, social worker and well-trained educators are necessary to increase awareness of the CF comorbidities problem.