(201) Caregiver perceptions of a lifestyle education tool and subsequent behavior changes with Elexacaftor-Tezacaftor-Ivacaftor initiation

Background: Elexacaftor/tezacaftor/ivacaftor (ETI) is a CFTR modulator, currently approved for persons with cystic fibrosis (CF) 6 years of age and older with at least one copy of the F508del mutation or another eligible mutation. ETI should be taken every 12 hours with fat-containing (10-20 grams) foods and pancreatic enzyme therapy for optimal absorption. In clinical trials, ETI increased body mass index (BMI) by 1kg/m2. Some adolescents started on ETI at our center had rapid weight gain leading to overweight/obese status. We developed a proactive educational tool to increase caregiver knowledge about nutrition, physical and mental well-being, and anticipated outcomes with ETI use in children 6-11 years, with the goal of providing education and avoiding rapid weight gain.

Methods: As part of a quality improvement project, an educational tool (Figure 1) was created by multidisciplinary clinical care team members and family partners to educate patients and families on the impact of ETI therapy on BMI and potential lifestyle changes. Several months after the ETI educational session, a survey was sent to caregivers of children started on ETI between 6 and 11 years via the patient portal in our hospital’s electronic medical record. This survey assessed caregiver perceptions on the educational tool and subsequent behavioral changes at home following ETI initiation.

Results: We received 37 survey responses. Twenty-eight (76%) remembered receiving the educational tool and found it helpful while 2 (5%) did not find it helpful and 7 (19%) did not remember receiving it. Two-thirds requested future educational materials be provided electronically. Caregivers reported potential side effects (26/37) as the biggest concern with ETI initiation, while some were concerned about insurance coverage (13/37) or lack of improvement with treatment (11/37). Nutritional and lifestyle changes that were made following ETI initiation included increasing fat at breakfast (14/37), switching ETI dose from bedtime snack to dinner (13/37) and increasing activity levels (8/37). Most respondents (57%) did not endorse making any dietary changes to reduce caloric intake, but the most common reported changes were choosing healthier snacks (11%), stopping oral supplements (9%) and switching to lower fat milk (9%). Caregivers identified multiple improvements with ETI use including eating more food at meals (18/37), less stomach aches (11/37), more actively playing (11/37), eating bigger snacks (10/37) and sleeping better (8/37). Follow up BMI measurements will be performed as the next step of this project (data will be available prior to NACFC).

Conclusions: sing a standardized education tool, we hoped to improve CF caregivers’ knowledge prior to ETI initiation. This educational tool was well received by families in our CF clinic and aided our medical team when initiating the discussion of starting ETI. The majority of caregivers reported dietary or lifestyle changes after beginning ETI therapy as well as some improvements in eating, activity and sleeping. As FDA approval of ETI expands to lower age ranges, appropriate nutritional and lifestyle education will be required to ensure normal growth in young children treated with CFTR modulators.

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