Marina Litvin, MD was recently featured on Healio.com about her presentation at the North American Cystic Fibrosis Conference 2021. Dr. Litvin discussed the “changing parameters” in nutritional status of patients with cystic fibrosis and the emergence of overnutrition and obesity, as well as the effects of highly effective modulator therapy on cardiovascular parameters.
“In addition to increase in overnutrition, and likely increase in incidence of hyperlipidemia and hypertension, our patients are living longer, and that fact within itself may place them at higher risk for cardiometabolic conditions, as well as coronary artery disease and stroke,” Litvin said.
Highly effective modulator therapy with elexacaftor/tezacaftor/ivacaftor was approved by the FDA for treatment of cystic fibrosis in 2019. The triple therapy improves the processing and trafficking of the CFTR protein and was shown in clinical trials that, in addition to improving lung function, there was a decrease in sweat chloride and an increase in weight, Litvin said, which was also seen with other medications that target CFTR such as ivacaftor and lumacaftor.
This medication should be taken in conjunction with high fat foods, which may cause an increase in caloric intake. In addition, this therapy may cause decreases in energy expenditure and can improve a patient’s sense of taste, smell, absorption and appetite, which can all result in higher caloric intake and overall weight gain, according to Litvin.
Dr. Litvin’s recommendations for clinicians caring for patients with cystic fibrosis who are on the highly effective modulator therapy are:
- Assess weight, BMI and BP at each visit.
- Provide individualized counseling for each patient on caloric goals, fitness goals and nutritional status to aim for normal BMI.
- If a patient has abnormal BP, have the patient monitor BP at home in case this becomes a pattern wherein medical therapy may be considered.
- Continue annual oral glucose tolerance testing.
- Screen patients for hyperlipidemia development once a year.
“Patients would benefit from nutritional support services to identify and treat patients whose weight is not in a reasonable range,” Litvin said.