Paediatric Cystic Fibrosis Clinic Research Articles

635 Discontinuing unnecessary proton-pump inhibitors in a pediatric cystic fibrosis clinic: a quality improvement initiative

635 Discontinuing unnecessary proton-pump inhibitors in a pediatric cystic fibrosis clinic: a quality improvement initiative

675 Increasing physical therapy encounters in a pediatric cystic fibrosis clinic

675 Increasing physical therapy encounters in a pediatric cystic fibrosis clinic

Implementation of a comprehensive pharmacy-driven immunization care process model in a pediatric cystic fibrosis clinic.

Members of an integrated pharmacy team (pharmacists and pharmacy technicians) have roles that have been identified in the literature as part of the multi-disciplinary cystic fibrosis (CF) care team. One role that has not specifically addressed is the administration of routine and recommended immunizations to people with CF (PwCF). According to care guidelines, PwCF of all ages should be provided all age-appropriate and recommended immunizations. Pharmacists and pharmacy technicians can administer immunizations per state laws. The Primary Children's CF Center decided to implement a comprehensive pharmacy-driven immunization care process model to impact immunization rates. A 24-month retrospective analysis was conducted with pediatric (≤18 years) PwCF at the Primary Children's CF Center. The primary outcome measures were the percentage (%) of PwCF who received PPSV23, and/or HPV, and/or meningococcal conjugate vaccine (MCV) immunizations 1-year post-care process model implementation (October 1, 2021, to September 30, 2022) as compared to baseline values. The secondary outcome measures are the total number of immunizations, the number of each immunization provided, and the financial impact of pharmacy-driven immunization care process model 1-year post-implementation. During the 1-year post-care process model implementation (October 1, 2021, to September 30, 2022), a total of 523 immunizations were provided to 243 pediatric PwCF. The most frequent immunizations provided were PPSV23 (160/523, 31%) and Coronavirus Disease 2019 (COVID-19) (154/523, 29%). The baseline percentages of eligible PwCF of PPSV23, HPV, and MCV were 27% (58/217), 43% (32/74), and 24% (8/34), respectively. The 1-year post-implementation percentages of PPSV23, HPV, and MCV were 99% (217/218, p < 0.00001), 91% (67/74, p < 0.00001), and 97% (33/34, p < 0.00001), respectively. For COVID-19 immunizations, 56% of eligible PwCF (181/321) have received their first dose. Of these 181 PwCF, 70% (126/181) have received at least one dose of their primary series or booster during the 1-year post-implementation period. The rate of those PwCF who have received at least one dose of a COVID-19 immunization from the age of 6 months to 4 years, 5-11 years, and 12-18 years, was 37% (30/82), 60% (78/129), and 66% (73/110), respectively. For the financial impact generated during the 1-year immunization care process model post-implementation period, 404 non-VFC immunizations were given for an estimated profit of $11,930. The implementation of a pharmacy-driven immunization care process model is a way for integrated pharmacy teams to evolve with the CF center care model and have a role expansion in the care provided to PwCF.

99 Improving screening for cystic fibrosis–related diabetes in a pediatric cystic fibrosis clinic

99 Improving screening for cystic fibrosis–related diabetes in a pediatric cystic fibrosis clinic

262 Implementation of a comprehensive pharmacy-driven immunization program in a pediatric cystic fibrosis clinic

262 Implementation of a comprehensive pharmacy-driven immunization program in a pediatric cystic fibrosis clinic

187 Use of sweat chloride testing to assess adherence to and efficacy of elexacaftor/tezacaftor/ivacaftor treatment in a pediatric cystic fibrosis clinic

187 Use of sweat chloride testing to assess adherence to and efficacy of elexacaftor/tezacaftor/ivacaftor treatment in a pediatric cystic fibrosis clinic

85 Quality improvement project: Human milk feeding and lactation support in a large pediatric cystic fibrosis clinic

85 Quality improvement project: Human milk feeding and lactation support in a large pediatric cystic fibrosis clinic

Development and Evaluation of a Pharmacist-Driven Vitamin D Protocol for a Cystic Fibrosis Clinic.

Vitamin D deficiency is commonly found in patients with cystic fibrosis (CF) and can have a negative effect on patients who are not at target goal according to Cystic Fibrosis Foundation's Vitamin D Deficiency Clinical Care Guidelines. The objective of this study is to determine the effectiveness of a pharmacist-driven vitamin D protocol (PDVDP) in improving, achieving, and maintaining 25-hydroxyvitamin D levels of patients in a pediatric CF clinic. A retrospective chart review was conducted for pediatric patients with CF from August 2018 to March 2020 to determine the percent of patients with improvement in 25-hydroxyvitamin D levels to target goal (≥ 30 ng/mL). Patients' 25-hydroxyvitamin D levels at 6, 12, and 18 months after automatic enrollment into the PDVDP were compared to determine if improvement occurred, and to calculate relative percent increase of 25-hydroxyvitamin D levels for these patients. The mean 25-hydroxyvitamin D levels of the patients at baseline before the protocol and 6, 12, and 18 months after enrollment in the protocol were 23.2, 33.3, 32.7, and 34.6 ng/mL, respectively. These results demonstrate mean 25-hydroxyvitamin D levels at all follow-up time points were significantly greater than baseline (p < 0.001). At 6 months, 50% (n = 20) of pediatric patients reached the target 25-hydroxyvitamin D levels. The PDVDP was effective in increasing the number of patients able to reach target 25-hydroxyvitamin D levels. Our PDVDP process may also be used at other CF clinics to improve vitamin D outcomes collaboratively with the interprofessional CF team.

Introducing a MAP for adherence care in the paediatric cystic fibrosis clinic: a multiple methods implementation study

BackgroundPeople with cystic fibrosis are required to adhere to a burdensome daily treatment regimen. Comprehensive adherence protocols can support more consistent use of adherence interventions and improve treatment adherence rates. This study aimed to explore the feasibility, acceptability, and appropriateness of implementing an adherence protocol into the outpatient cystic fibrosis clinic of a tertiary, paediatric hospital.MethodsThis implementation study employed a pre-post observation design, using multiple methods. Focus groups and semi-structured interviews were conducted pre-implementation to understand clinician and consumer perspectives on adherence care. A multicomponent adherence protocol (including multidisciplinary written treatment plans, digital mental health screening and customised communication tools) was then implemented as standard care for a three-month implementation phase. Quantitative data was collected throughout using purpose-designed audit tools and surveys. The Replicating Effective Practice (REP) Framework guided the implementation process. Analysis was informed by The Consolidated Framework for Implementation Research (CFIR) to identify factors that support or challenge the integration of adherence protocols into standard care.ResultsThirteen clinicians, eight parents and two adolescents participated in focus groups or interviews that informed development of the tailored multicomponent adherence protocol for implementation. Medical chart audits demonstrated that the protocol was used with 44–57% of eligible consumers three months after introduction. Eighteen clinicians and five consumers participated in post-implementation phase questionnaires. The protocol was considered acceptable and appropriate to clinicians and consumers. Changes in clinicians’ practice behaviour were short-lived peaks in response to targeted intervention strategies throughout the implementation phase, such as audit and feedback.ConclusionsAn adherence protocol is not an “off the shelf” solution to the adherence challenge in a hospital outpatient setting. Despite the tailored adherence protocol being considered appropriate and acceptable to clinicians and consumers, low fidelity indicates limited feasibility in the outpatient clinic setting, where multi-disciplinary members are all considered responsible for adherence care interventions. Key implementation factors and strategies to consider prior to introducing an adherence protocol are described.Trial registrationAustralian New Zealand Clinical Trials Registry ACTRN12619001730190 (Retrospectively registered).

Quality improvement and rapid PDSA cycles to maintain routine surveillance of pulmonary pathogens during the COVID-19 pandemic in a pediatric cystic fibrosis clinic

BackgroundThe COVID-19 pandemic necessitated immediate transition from in person to telehealth encounters; novel nursing practices were needed to ensure that children with cystic fibrosis (CF) receive care that approximates evidence-based guidelines. Local problemThe aim was to ensure that as many children as possible received routine surveillance of pulmonary pathogens by a CF culture sputum culture during a pandemic. MethodsMultiple Plan-Do-Study-Act (PDSA) cycles were utilized to implement practice change over four months. InterventionsCultures were obtained via curbside appointment with a registered nurse (RN) or at the patients' home with mailed equipment. Results133 cultures obtained: 50.37% (67) by RN collection curbside and 49.62% (66) by self/caregiver at home. 120 culture swabs or sterile cups were mailed; 55% (66) were returned. Cost of mailing equipment was $760.16. ConclusionNursing utilization of PDSA cycles developed novel processes that ensured guideline-based care during the initial months of the pandemic.

137: Improving FEV1 in patients aged 6–12 years in a pediatric cystic fibrosis clinic

137: Improving FEV1 in patients aged 6–12 years in a pediatric cystic fibrosis clinic

71: Collection of expectorated sputum or oral pharyngeal cultures during initial period of the COVID-19 pandemic in a pediatric cystic fibrosis clinic

71: Collection of expectorated sputum or oral pharyngeal cultures during initial period of the COVID-19 pandemic in a pediatric cystic fibrosis clinic

92: Improving pneumococcal polysaccharide vaccination rates in a pediatric cystic fibrosis clinic

92: Improving pneumococcal polysaccharide vaccination rates in a pediatric cystic fibrosis clinic

78: Using pre-visit planning and electronic health record messaging to improve liver function and annual lab monitoring in the pediatric cystic fibrosis clinic

78: Using pre-visit planning and electronic health record messaging to improve liver function and annual lab monitoring in the pediatric cystic fibrosis clinic

Impact of a pharmacy technician and pharmacist on time to inhaled tobramycin therapy in a pediatric cystic fibrosis clinic.

Cystic fibrosis (CF) patients who grow Pseudomonas aeruginosa on respiratory culture are commonly prescribed inhaled tobramycin (TIS) to eradicate the organism. The objective of this study was to determine the impact of a pharmacy technician/pharmacist team, in conjunction with an integrated health-system specialty pharmacy (IHSSP), on the time from positive culture to prescribing and access to TIS in a pediatric CF clinic. A retrospective study of CF patients positive for P. aeruginosa who were prescribed TIS for eradication. The study included 20 patients in the pregroup and 42 patients in the postgroup. Total median (interquartile range) days from positive culture to TIS being shipped to the patient from the pharmacy was significantly different: 15 (10.25-21) days in the pregroup and 9 (7-14) days in the post groups (p = .005). The time from positive culture to TIS prescribing was significantly different: 6 (5-12.75) days in the pregroup and 5 (3.75-6) days in the postgroup (p = .01). In the postgroup median time from prescription to the patient receiving the TIS was significantly different between the two groups 2 (2-5) days IHSSP group versus 6 (3-9) external specialty pharmacy group (p = .003). Time from prescription to prior authorization approval was the same in both groups. The addition of the pharmacy team reduced time from culture to TIS being received by the patient. Patients able to fill at the IHSSP received their medication sooner than an external specialty pharmacy. The study shows the benefit of an integrated pharmacy model in conjunction with an IHSSP.

P225 Parents’ feedback on virtual paediatric cystic fibrosis clinics during COVID-19 pandemic

Introduction: The COVID-19 pandemic led to cancellation of all face-to-face clinics in our health board in mid-March 2020. Overnight we had to develop an alternative service for our cystic fibrosis children. We set up video clinics from end of March 2020 using Webex initially and then Attend Anywhere when this became available in July 2020. We also arranged supplies of rescue antibiotics at home and arranged to collect cough swabs from patients’ doorsteps. Objectives: To obtain feedback from parents on the cystic fibrosis service provided during COVID-19 pandemic, including virtual clinics, and to get their opinion about use of virtual cystic fibrosis clinics post COVID-19. Methods: Short questionnaire was devised and e-mailed to parents in January 2021. Results: 23 of 26 questionnaires have been returned to date. 100% were very satisfied with service provided during the COVID-19 pandemic and 78%were very satisfied, with 17% satisfied with use of virtual consultations. Mean score for ease of use of Attend Anywhere was 9.4 out of 10. 74% were very satisfied and 22% satisfied that their needs were met via virtual consultations. 39% were very satisfied, 35% satisfied with alternate virtual and face-to-face clinic appointments post-COVID-19. Concerns were raised around no clinical examination and delays in replacing physiotherapy equipment with virtual appointments. Areas suggested for improvement included the ability to do lung function at home. Conclusion: Parents were supportive of the use of virtual clinics during COVID-19 but there were mixed views with continuing virtual clinics post-COVID-19.

P383 Exploring the need for enhanced mental health services in a paediatric cystic fibrosis clinic

P383 Exploring the need for enhanced mental health services in a paediatric cystic fibrosis clinic

P336 Pioneering a snack trolley for cystic fibrosis nutrition assessment and education in a paediatric cystic fibrosis clinic

P336 Pioneering a snack trolley for cystic fibrosis nutrition assessment and education in a paediatric cystic fibrosis clinic

P404 Implementing a pathway for the investigation of Cystic Fibrosis-Related Diabetes in a paediatric cystic fibrosis clinic

P404 Implementing a pathway for the investigation of Cystic Fibrosis-Related Diabetes in a paediatric cystic fibrosis clinic

Appetitive characteristics in children with cystic fibrosis: Questionnaire validation and associations with nutritional status

BackgroundAppetitive characteristics are an important factor in the nutritional status of children with cystic fibrosis (CF). We administered a brief parent-report eating behavior questionnaire, validated in healthy children, to determine the relationship between appetitive characteristics and body weight in children with CF. MethodsParents of children attending the Johns Hopkins Pediatric CF Clinic completed the Child Eating Behavior Questionnaire (CEBQ) at a routine clinic visit. Responses were correlated with anthropometric and other clinical data. ResultsParents of 64 children with CF aged 7.74 ± 3.17 years (mean ± SD) completed the CEBQ. The CEBQ subscales demonstrated good internal consistency (Cronbach's α = 0.76–0.94). Higher scores on food avoidance subscales (Slowness in Eating) were associated with lower body mass index (BMI) z-scores, and higher scores on food approach subscales (Food Responsiveness, Enjoyment of Food, Emotional Overeating) with higher BMI z-scores. Children with feeding aids (i.e. gastric tube or appetite-stimulating medications) demonstrated greater food avoidance (Slowness in Eating) and lesser food approach (Enjoyment of Food) when compared to those without feeding aids. Children with pancreatic insufficiency also demonstrated greater food avoidance (Slowness in Eating). ConclusionsThe CEBQ can be used in a clinical setting to identify children with CF with appetitive characteristics associated with difficulty gaining weight. These children could potentially benefit from earlier interventions to aid in weight gain. Characterization of appetite using the CEBQ could aid investigation of the biological etiology of low appetite, and optimization of clinical and parental approaches to achieving a healthy nutritional status.