195 Impact of elexacaftor/tezacaftor/ivacaftor on serum markers of fat-soluble vitamin levels in people with cystic fibrosis

Hergenroeder, G.; Faino, A.; Bridges, G.; Bartlett, L.; McNamara, S.; Strub, B.; Nichols, D.; Ramos, K.

doi:10.1016/s1569-1993(22)00885-2

Cited by 5 publications

(18 citation statements)

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“…The most consistent finding across studies is an increase in vitamin A levels. 16,22,23 The changes in vitamin D and vitamin E were variable between modulators and between the studied cohorts (Table 4). Sommerburg and colleagues noted an increase in vitamin A and a decrease in vitamin E following Lum/Iva therapy for a follow-up duration of up to 2 years after its initiation.…”

Section: Discussionmentioning

confidence: 99%

Impact of elexacaftor/tezacaftor/ivacaftor on lipid and fat‐soluble vitamin levels and association with body mass index

Patel,

McBennett,

Sankararaman

et al. 2024

Pediatric Pulmonology

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IntroductionCystic fibrosis transmembrane conductance regulator (CFTR) modulators improve gastrointestinal absorption of nutrients and may result in changes in body mass index (BMI), serum lipids, and fat‐soluble vitamin levels. We hypothesized that serum lipids and vitamin levels would increase with CFTR modulator therapy and that greater increase in lipids and vitamin levels would be related to greater increase in BMI.MethodsA retrospective study was performed to evaluate the impact of elexacaftor/tezacaftor/ivacaftor (ETI) on nutritional parameters, serum lipids, and fat‐soluble vitamin levels. Pre‐ETI values (<2 years prior) and post‐ETI values (>1 month after) were compared. Linear regression was used to evaluate whether change in BMI is associated with the change in lipid and/or vitamin levels and whether modulator duration is associated with the degree of rise in lipid and/or vitamin levels.ResultsAdults and adolescents with CF (n = 137) were evaluated before and 31–300 days after starting ETI. Median BMI (adults 21.9 vs. 23.5 kg/m2; adolescents 48 vs. 63 percentile) increased after initiation of ETI. Total cholesterol (126 vs. 154 mg/dL), low‐density lipoprotein cholesterol (63 vs. 78 mg/dL), non‐high‐density lipoprotein cholesterol (84 vs. 102 mg/dL), and high density lipoprotein cholesterol (43 vs. 49 mg/dL) increased after ETI, while triglycerides and very low density lipoprotein did not change. Median values for vitamin D (34.5 vs. 38.0 ng/mL) and vitamin A (40.1 vs. 47.9 µg/dL) increased, while vitamin E did not change significantly. There was no significant correlation between BMI change or duration of modulator therapy with vitamin levels or lipid changes.ConclusionAfter initiation of ETI therapy, serum lipids increased in our population, but most values remained within the normal range. Vitamins A and D levels increased post‐ETI and no changes were noted in vitamin E. No significant correlation between the degree of BMI change and the magnitude of increase in lipids or vitamin levels was found.

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Section: Discussionmentioning

confidence: 99%

Impact of elexacaftor/tezacaftor/ivacaftor on lipid and fat‐soluble vitamin levels and association with body mass index

Patel,

McBennett,

Sankararaman

et al. 2024

Pediatric Pulmonology

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“…Three children exhibited high retinol levels, with one experiencing this increase as early as 24 days after starting ETI. There were no differences in levels of tocopherol and 25‐hydroxyvitamin D before and after ETI treatment 41 . In other studies examining the impact of ETI treatment on vitamin status, Wright et al 42 observed a modest increase in vitamin D levels in 76 adults after 1 year of treatment whereas Francalanci et al 43 showed significant increases in vitamin A, E and D levels in 29 adults who had been using modulators for at least 1 year.…”

Section: From Early Life To Childhoodmentioning

confidence: 95%

“…They attributed the increased retinol levels to improved absorptive processes; however, the contrasting decline in tocopherol levels suggests other mechanisms (e.g., altered tissue distribution, increased degradation) 40 . In a retrospective study involving 54 children (aged 6–16 years) treated with ETI, Schembri et al 41 found a significant increase in serum retinol levels following ETI initiation. Three children exhibited high retinol levels, with one experiencing this increase as early as 24 days after starting ETI.…”

Section: From Early Life To Childhoodmentioning

confidence: 99%

“…In other studies examining the impact of ETI treatment on vitamin status, Wright et al 42 observed a modest increase in vitamin D levels in 76 adults after 1 year of treatment whereas Francalanci et al 43 showed significant increases in vitamin A, E and D levels in 29 adults who had been using modulators for at least 1 year. The mechanisms by which ETI affects vitamin levels remain poorly understood but may relate to improved absorption and metabolism of these vitamins combined with a decrease in the adherence to supplementation as patients start to feel better 41,42 . Nevertheless, these findings highlight the complexity of adjusting vitamin dosing regimens in patients receiving CFTR modulators and emphasise the importance of nutritional surveillance.…”

Section: From Early Life To Childhoodmentioning

confidence: 99%

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Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges

Mailhot

Denis

Beauchamp‐Parent

et al. 2023

J Human Nutrition Diet

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Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding for the ion channel cystic fibrosis transmembrane conductance regulator (CFTR). The management of CF disease has evolved in recent decades from treating downstream disease manifestations affecting the airways, the lungs and the gastrointestinal system to addressing the CFTR gene defect. The advent of CFTR modulators, which correct the functionality of the defective CFTR, contributes to reshaping the landscape of CF demographics, prognosis and therapies, including nutritional management. A spectrum of clinical manifestations is emerging within the same patient population where undernutrition and nutritional deficiencies coexist with excessive weight gain and metabolic derangements. Such contrasting presentations challenge current practices, require adjustments to traditional approaches, and involve more individualised interventions. This narrative review examines the current state of knowledge on the nutritional management of people living with cystic fibrosis from early life to adulthood in the era of CFTR modulation.

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“…Notably, improvement in various fat-soluble vitamin levels has been demonstrated in both children and adults taking ETI [13]. One pediatric focused study identified significant elevations in vitamin A alone, sometimes in excess [14]. This combination of improvement and variability in outcomes highlights a theme common to the milestones noted in the post-HEMT era - a trend to focus on the individualized needs of the person with CF.…”

Section: Current Landscapementioning

confidence: 99%

Evolving nutritional needs, obesity, and overweight status in cystic fibrosis

Szentpetery

2023

Current Opinion in Pulmonary Medicine

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Purpose of review The nutritional landscape in cystic fibrosis has shifted dramatically in the era of CFTR modulator therapy. In this review, we will critically examine the literature on overweight and obesity in CF, current nutritional care unknowns and opportunities for further investigation or adaptation in clinical care. Recent findings Results of clinical trial and real-world data reflect marked improvement in nutritional status and quality of life. Clinical outcomes including CF related diabetes and CF related liver disease appear positively impacted. Secondary impacts on cardiometabolic disease have been noted, especially in association with excessive weight gain. Summary The prior approaches to optimizing nutrition in cystic fibrosis with caloric excess can likely be safely retired for many. As modulator access expands across the lifespan, a longitudinal focus on health maintenance should be considered.

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195 Impact of elexacaftor/tezacaftor/ivacaftor on serum markers of fat-soluble vitamin levels in people with cystic fibrosis

Cited by 5 publications

References 0 publications

Impact of elexacaftor/tezacaftor/ivacaftor on lipid and fat‐soluble vitamin levels and association with body mass index

Impact of elexacaftor/tezacaftor/ivacaftor on lipid and fat‐soluble vitamin levels and association with body mass index

Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges

Evolving nutritional needs, obesity, and overweight status in cystic fibrosis

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