BSPED2021 Oral Communications Oral Communications 8 (9 abstracts)
Cystic fibrosis related diabetes (CFRD): could Cystic Fibrosis Transport Receptor (CFTR) modulators be the answer?
Julie Park 1 , Anna Walsh 2 , Sue Kerr 1 , Clare Woodland 1 , Suzanne Southward 3 , Mark Deakin 1 , Rebecca Thursfield 1 & Senthil Senniappan 1
1Alder Hey Children’s NHS Foundation Trust, Liverpool, United Kingdom; 2University of Liverpool, Liverpool, United Kingdom; 3Leighton Hospital, Crewe, United Kingdom
Background: Cystic Fibrosis related diabetes affects 40-50% of adults with cystic fibrosis (CF). This can significantly affect pulmonary function and life expectancy. Kaftrio (Ivakaftor, tezacaftor and elexacaftor) has recently been licensed for use in CF. Previous data highlight that glucose regulation may be altered on commencing this treatment.
Methods: Eight children and young people (CYP), aged 14 (12-15) years, who were diagnosed with CFRD aged 12.8 (8.7-13.2) years, commenced Kaftrio. Freestyle Libre monitoring was commenced 2-5 days prior to starting treatment and continued for 7 days after. This was repeated at six monthly intervals, or earlier if clinically indicated. Insulin doses were reduced or stopped on commencing Kaftrio. Data were available for six CYP.
Results: Of the two children with no Libre data available, one stopped insulin and the other remains on long and short acting insulin at significant doses.
| Pre-kaftrio (median, [range]) | Immediately post Kaftrio (median, [range]) | 5-13 months post Kaftrio (median, [range]) | |
| Time period monitored (days) | 6.0 [2.0-7.0] | 7.0 [7.0-7.0] | 10.5 [7.0-14.0] |
| Time sensor active (%) | 69.5 [27.0-98.0] | 90.5 [62.0-100.0] | 54.0 [6.0-73.0] |
| Average glucose ( mmol/l) | 6.3 [5.9-7.5] | 6.0 [5.7-7.4] | 6.5 [5.3-7.0] |
| Glucose variability | 27.7 [21.0-39.0] | 25.1 [18.1-39.9] | 26.3 [18.6-37.1] |
| % time glucose within target range (%) | 95.0 [73.0-98.0] | 94.5 [70.0-99.0] | 93.5 [88.0-99.00] |
| % time glucose >10 mmol/l (%) | 5.0 [0.0-21.0] | 2.0 [0.0-23.0] | 3.0 [0-16.0] |
| % time glucose <3 mmol/l (%) | 1.5 [0.0-6.0] | 6.0 [0.0-7.0] | 2.0 [0.0-12.0] |
| Number of hypoglycaemic episodes recorded | 1.0 [0.0-1.0] | 0.5 [1.0-6.0] | 1.5 [0.0-17.0] |
| Dose of insulin (units/day) | 7.0 (2.0-25.0) | 0.0 (0.0-0.0) | 0.0 (0.0-1.0) |
Discussion: Our data show that glucose regulation is affected in children with CFRD when they are commenced on Kaftrio. Insulin may no longer be required. Alternatively, insulin doses may reduce significantly. Monitoring for hypoglycaemia and education are required, even for those no longer requiring insulin. CYP may need to re-introduce insulin at smaller doses over the following year. Further data on long term effects need to be assessed.
Volume 78
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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