Routine Cystic Fibrosis Research Articles

Innovations in Evaluating Ambulatory Costs of Cystic Fibrosis Care: A Comparative Study Across Multidisciplinary Care Centers in Ireland and the United States

Cystic fibrosis (CF) affects more than 160,000 individuals globally and has seen improved survival rates due to multidisciplinary care models and pharmacotherapy innovations. However, the associated costs remain substantial, prompting the authors to study and evaluate the expense of CF ambulatory care to understand how care structure influences costs. People with CF (PwCF) at large pediatric CF centers in both the United States and Ireland were recruited for parallel observational, prospective studies. Based upon the process of care, the lead clinicians at both sites identified and agreed on three strata of patients (0–11 months, 1–5 years, and 6–17 years of age). Process maps were developed for each of the age cohorts at each site, and the costs of ambulatory care — with emphasis on routine CF clinic visits — were measured utilizing time-driven activity-based costing (TDABC). A dollar-per-minute capacity cost rate (CCR) was calculated for all resources used in the care cycle. The total direct cost was obtained by multiplying the CCR for each resource by the time the resource was used during the patient’s care cycle. The cost was summed across all resource types to obtain the cost over the entire care cycle for each site. Service operations were benchmarked to one site and variance analysis was performed. In total, 58 PwCF were included in the analysis (49 in the United States and 9 in Ireland); 4 were 0–11 months, 17 were 1–5 years, and 37 were 6–17 years of age. Physicians (United States) and respiratory consultants (Ireland) had the highest CCRs. Physicians and registered dietitians spent the most time with patients in the United States, compared with the clinical nurse specialists and dietitians in Ireland. The total variance in cost for clinical visits was largest in the 6- to 17-year-old group (28% variance, with 100% in the United States vs. 128% in Ireland). In the 6- to 17-year-old group, the largest drivers in total variance were quantity variance (variance in duration of time spent with patients), which was 108% greater in Ireland); the skill mix variance (variance in clinician type performing service for a given time), which was 49% greater in the United States; and the rate variance (variance in compensation levels across sites), which was 31% greater in the United States. The authors’ use of TDABC to characterize the cost of multidisciplinary care during ambulatory clinic visits for PwCF, in combination with variance analysis (the quantitative investigation of the difference between actual and expected costs), provides new and innovative ways to compare costs across similar health care service delivery sites, providing insights into the distinctive features of each. A granular understanding of cost and comparison of resource utilization between centers provides valuable, organizationally relevant insights.

Potential, Pitfalls, and Future Directions for Remote Monitoring of Chronic Respiratory Diseases: Multicenter Mixed Methods Study in Routine Cystic Fibrosis Care.

The current literature inadequately addresses the extent to which remote monitoring should be integrated into care models for chronic respiratory diseases (CRDs). This study examined a remote monitoring program (RMP) in cystic fibrosis (CF) by exploring experiences, future perspectives, and use behavior over 3 years, with the aim of developing future directions for remote monitoring in CRDs. This was a mixed methods, multicenter, observational study in 5 Dutch CF centers following a sequential explanatory design. Self-designed questionnaires using the technology acceptance model were sent out to people with CF who had a minimum of 12 months of experience with the RMP and local health care professionals (HCPs). Questionnaire outcomes were used to inform semistructured interviews with HCPs and people with CF. Qualitative findings were reported following the COREQ (Consolidated Criteria for Reporting Qualitative Research) checklist. Anonymous data on use frequency of all people with CF were analyzed. Between the second quarter of 2020 and the end of 2022, a total of 608 people with CF were enrolled in the program, and a total of 9418 lung function tests and 2631 symptom surveys were conducted. In total, 65% (24/37) of HCPs and 89% (72/81) of people with CF responded to the questionnaire, and 7 HCPs and 12 people with CF participated in semistructured interviews. Both people with CF and HCPs were positive about remote monitoring in CF care and found the RMP a good addition to daily care (people with CF: 44/72, 61%; HCPs: 21/24, 88%). Benefits ranged from supporting individual patients to reducing health care consumption. The most valued monitoring tool was home spirometry by both people with CF (66/72, 92%) and HCPs (22/24, 92%). Downsides included the potential to lose sight of patients and negative psychosocial effects, as 17% (12/72) of people with CF experienced some form of stress due to the RMP. A large majority of people with CF (59/72, 82%) and HCPs (22/24, 92%) wanted to keep using the RMP in future, with 79% (19/24) of HCPs and 75% (54/72) of people with CF looking forward to more replacement of in-person care with digital care during periods of well-being. Future perspectives for the RMP were centered on creating hybrid care models, personalizing remote care, and balancing individual benefits with monitoring burden. Remote monitoring has considerable potential in supporting people with CF and HCPs within the CF care model. We identified 4 practice-based future directions for remote monitoring in CF and CRD care. The strategies, ranging from patient driven to prediction driven, can help clinicians, researchers, and policy makers navigate the rapidly changing digital health field, integrate remote monitoring into local care models, and align remote care with patient and clinician needs.

Implementation and outcomes of a quality improvement project to systematically assess the gross motor skills in school-aged children with cystic fibrosis.

Limited data exist on the gross motor abilities of children with cystic fibrosis (CF). The objective of this research project was to implement a systematic gross motor assessment in children with CF ages 4-12 years. Secondarily, we aimed to assess demographic characteristics associated with gross motor delays. Physical therapists aimed to evaluate at least 50% of eligible children (4-12 years) at our CF Center over 1 year using the Bruininks-Oseretsky Test of motor Proficiency, second edition (BOT-2). Delays are defined by scores less than 18th percentile. Demographic and clinical data included body mass index, hospitalizations, genotype, and comorbidities. Basic descriptive statistics summarized patient information. Parametric and nonparametric methods compared groups of interest. Linear regression assessed associations between BOT-2 measures and clinical characteristics. The BOT-2 evaluation was successfully implemented with 69% of eligible patients being evaluated. Forty-five (62.5%) scored below average. Impaired strength (22.2%) was most common, followed by impaired balance (16.7%), running speed and agility (15.3%), and bilateral coordination (8.3%). 15.5% scored below average on their total motor composite score (TMC). Increased age, comorbidities and hospitalizations were associated with a lower TMC. The BOT-2 was successfully implemented as part of routine CF care to screen for gross motor delays in children. Results suggest that a high percentage of children with CF, especially older children with comorbid conditions or a history of hospitalization, have impaired gross motor function. These findings support the need for routine gross motor evaluations and physical therapy interventions within pediatric CF clinics.

Glycemic Variability and the Thickness of Retinal Layers in Cystic Fibrosis Patients with and without Cystic Fibrosis Related Diabetes

Purpose Patients with cystic fibrosis (CF) are at risk to develop CF related diabetes (CFRD) and subsequently even diabetic neuro- and/or vasculopathy. We sought to determine if there are typical signs of diabetes-related retinal alterations present in CF patients with preserved and impaired glycemic control. Methods During routine annual examination CF patients were offered an additional 7-day period of real time continuous glucose monitoring (rtCGM) and an ophthalmological examination including retinal optical coherence tomography (OCT). Patients were categorized according to the glycemic control, i.e. the results of an oral glucose tolerance test (OGTT) and rtCGM were taken into consideration. OCT data was analyzed by our previously published visual analysis software generating dedicated and spatially resolved deviation maps for visualization and quantification of differences in total retinal thickness and thickness of retinal nerve fiber layer (RNFL) as well as ganglion cell layer (GCL) in comparison to age-matched healthy controls and patients with either type 1 or type 2 diabetes mellitus. Results Results of the rtCGM and/or OGTT enabled discrimination between patients with normal glycemic control (CFNG; n = 6), with abnormal glycemic control (CFAG; n = 6) and overt CFRD (n = 4). OCT data indicates gradually increasing retinal thinning in all 3 groups, depending on the degree of glucose metabolism disorder compared to healthy controls. At the foveal region total retinal thickness and GCL thickness were significantly thinner in CFRD patients compared to CFNG patients (total retinal thickness: 260.4 µm (239.3–270.8) vs. 275.4 µm (254.3–289.5); GCL: 11.82 µm (11.16–15.25) vs. 17.30 µm (13.95–19.82); each p < 0.05). Conclusion Although we investigated a rather small number of patients, we obtained evidence that intraretinal neurodegenerative changes occur in each of our subgroups (CFNG, CFAG, CFRD). Beyond this, our results favor the detrimental role of additional diabetes, as the deviations from healthy controls were most pronounced in the CFRD group and are similar to those seen in patients suffering from type 1 or type 2 diabetes.

377 Use of telemedicine to provide routine cystic fibrosis care does not compromise health and can save patients time and money

377 Use of telemedicine to provide routine cystic fibrosis care does not compromise health and can save patients time and money

“I think most people feel like healthcare professionals tell them to take their treatments and judge them for not taking them”: reflexive thematic analysis of the views of adults with cystic fibrosis on how treatment adherence is discussed in healthcare

Objective Previous research exploring patient-practitioner communication in relation to adherence in cystic fibrosis (CF) is limited. This UK study explored the views of adults with CF on how treatment adherence (related to all CF treatments) is discussed in routine CF care. Methods 12 White British adults (ten females; aged 20–37 years; mean 30.1 years) with CF participated in semi-structured interviews. Results Three overarching themes were developed through reflexive thematic analysis: (1) ‘The power of language’; (2) ‘Healthcare professionals do not recognise the importance of context’; and (3) ‘“Admitting” non-adherence is difficult’. The way in which adherence is discussed in adult CF care is viewed as paternalistic and infantilising. Participants reported that healthcare professionals do not always consider the desire to balance treatment-taking with living a normal life. Unwelcome responses from healthcare professionals, and the inability to accurately self-report the amount of treatment taken made it difficult to ‘admit’ non-adherence. Conclusions A culture change is needed in CF care such that people who struggle to take their treatments are not labelled as disobedient, wilfully disobeying orders from healthcare professionals in positions of authority. Instead, an open, honest, non-judgemental approach, as recommended by healthcare agencies for over a decade, should be adopted.

Telehealth use in Australian cystic fibrosis centers: Clinician experiences.

Telehealth has been rapidly adopted by cystic fibrosis (CF) centers and ongoing use in routine CF care is endorsed by CF consumers. However, data describing CF clinician perceptions regarding telehealth are scarce. We aimed to describe clinician experiences and attitudes towards telehealth in CF care among health professionals across Australia. CF multidisciplinary health professionals from all CF clinics in Australia were sent an anonymous electronic survey. Eighty-five responses were received representing 15 of 23 (65%) centers. Most clinicians reported using telehealth for routine clinic visits, and a range of other clinical encounters (69.9%). Telehealth was widely perceived as acceptable (91.8%), and clinicians were comfortable/very comfortable (81.2%) integrating telehealth into future CF care. Despite this, 64.1% of respondents considered telehealth clinics to be much worse than face-to-face clinics and 57.5% reported quality of care was somewhat/much worse using telehealth. Home spirometry was available in 73.7% of centers, however, only 26.7% of clinics could provide spirometers for >75% eligible patients. Growth and microbiology assessments were often missed in telehealth clinics and 75.7% reported a technical issue had prevented a telehealth consultation from occurring. Telehealth for CF in Australia is considered feasible and acceptable by CF clinicians, although use of telehealth varies widely between centers. Concerns exist around the impact of telehealth on health outcomes, especially given core assessments are frequently omitted. Guidelines may help ensure the benefits of telehealth are realized for people with CF without compromising the standard of care.

The experience of living with and managing cystic fibrosis related diabetes: a qualitative review.

Improved survival rates for people with cystic fibrosis have led to increased rates of co-morbidity, of which diabetes is the most common. Cystic fibrosis related diabetes affects 19% of adolescents and up to 50% of adults, although little is known about their experiences of this co-morbidity. To investigate the experiences of living with and managing cystic fibrosis related diabetes among adolescents and adults. Systematic review and thematic analysis of qualitative evidence. Six studies, rated good quality, were included in the review and four main themes emerged from the data: knowledge and understanding; emotional and social impact; balancing both conditions; acceptance and adjustment. Although the main themes reflect adolescent and adult experiences, there were subtle variations in their sub-themes. Participants' overriding story was of journeying towards acceptance and integration of cystic fibrosis related diabetes into their lives. This included their unpreparedness for the likely onset of cystic fibrosis related diabetes and their struggles to balance the competing demands of living with and managing cystic fibrosis and diabetes. The diagnosis of cystic fibrosis related diabetes and its incorporation into daily life is challenging for many people with cystic fibrosis. Review findings indicate opportunities for cystic fibrosis related diabetes interventions pre-diagnosis, at diagnosis, and during ongoing management, which need integrating into routine cystic fibrosis care.

Patient engagement in the development of CF-CBT: A cystic fibrosis-specific cognitive-behavioral intervention for adults.

Individuals with cystic fibrosis (CF) are at high risk for depression and anxiety, with negative consequences for health and quality of life. Cystic Fibrosis Foundation/European Cystic Fibrosis Society guidelines recommend routine screening, treatment, and preventative efforts. Cognitive-behavioral therapy (CBT) has a large evidence-base for depression/anxiety prevention and treatment. However, traditional CBT protocols require adaptation to address the emotional challenges of coping with CF, stressors related to disease management, and barriers to access to care. The goal of this study was to partner with the CF community to develop an innovative CBT-based intervention for the prevention and treatment of depression and anxiety tailored to CF-specific needs. In-depth feedback was collected via audio-recorded telephone interviews with 16 adults with CF from 3 U.S. CF centers, with purposive sampling across gender, age, ethnicity, and disease severity. A semi-structured interview guide elicited discussion of patient experiences of coping with CF, and perspectives on the acceptability of the content, structure, and delivery model of the proposed intervention. Qualitative analysis utilized a content analytic approach. Participants ranged from 21 to 53 years (M = 35); eight were female; three were Hispanic. Patient-reported most recent FEV1, a measure of lung function based on forced expiratory volume in in one second, ranged from 25 to 113% predicted (M = 72). One participant was post-double lung transplant. Qualitative interviews were analyzed thematically revealing core themes related to the experience of coping with CF. The most frequently cited CF-related stressors were Treatment Burden, Illness Uncertainty, and Financial/Insurance Stress. Participants talked about the interaction of physical symptoms and emotional distress in their daily lives, a topic not typically discussed in routine CF care. Resilience was also a major theme with participants describing strategies they use to cope with CF and hospitalizations. Description of patients’ experiences was incorporated into the program’s intervention manual and patient workbook. Participants also provided direct feedback on the proposed program. Feedback was largely positive regarding program content and structure, suggesting the acceptability of a CF-specific CBT-based intervention for adults with CF. Features to increase accessibility of care including telehealth, inpatient delivery, and team-based care were perceived as advantageous, and participants emphasized the value of a CF-specific mental health intervention. Qualitative findings directly informed the development of CF-CBT, a cognitive-behavioral skills-based program to promote emotional well-being for adults with CF.

The feasibility of collecting the physiotherapy outcomes airway clearance, physical activity and fitness for the Australian Cystic Fibrosis Data Registry

BackgroundPhysiotherapy-related data, such as airway clearance techniques (ACTS), physical activity and aerobic fitness are not consistently included in international cystic fibrosis (CF) data registries. This study aimed to pilot the collection of ACTS, physical activity and fitness in a hospital CF clinic, as a step towards informing future national implementation.MethodsThis study was undertaken in a CF clinic within a major tertiary hospital. Patients and families were invited to participate. Participants completed self-report questionnaires on ACT use and those aged ≥ 10 years completed a physical activity questionnaire (Core Indicators and Measures of Youth Health Survey) and aerobic fitness test (the A-STEP test). Participants also completed a survey to explore the tolerance and acceptability of the fitness test, and the perceived accuracy of the self-reported data collection.ResultsForty patients agreed to participate in the study (mean age = 9.8, SD = 4.1 years old; 52.5% female). All patients and/or families that were approached agreed to participate and completion rate for the ACTs and physical activity surveys was 98% and 100% (respectively). Completion rate for the fitness test was 55%, due to time constraints. Most participants agreed (≥ 90%) they could accurately provide ACT and physical activity data, and the assessments were tolerable and acceptable.ConclusionsPatients with CF and their families are able to and can acceptably provide physiotherapy-related data, and collecting self-report ACTs and physical activity data is highly feasibly during routine CF clinic visits. However, aerobic fitness testing using the A-STEP test may be less feasible in clinic environments, due to time constraints.

Developing an ecological approach to physical activity promotion in adults with Cystic fibrosis.

BackgroundThere are few examples of interventions designed to promote physical activity (PA) in adults with Cystic fibrosis (CF). Increasing levels of habitual PA may be more feasible and result in greater compliance than conventional exercise training inventions which give little or no attention to long-term PA behaviour. Despite this there is limited research exploring perceptions of PA among adults with CF. The study aimed to understand the ecological correlates of PA in adults with CF and to involve individuals with CF, their families (where applicable) and clinicians in a formative process to inform the development of an ecological approach to PA promotion in this population.MethodsAn iterative approach was utilised, whereby findings from earlier phases of the research informed subsequent phases. Semi-structured interviews were conducted to explore patients’ perceptions of PA, devised using the PRECEDE component of the PRECEDE-PROCEED model. Followed by, focus groups to discuss the perceived barriers, facilitators and opportunities for PA participation and how this information could inform the development and delivery of a PA intervention. Separate focus groups were conducted with individuals with CF (n = 11) and their families and CF MDT members. Thematic analysis was used to construct themes.ResultsPhysical and mental wellbeing manifested as both barriers and facilitators of PA. CF is characterised by a progressive decline in physical function, which presents as a number of challenging symptoms and set-backs for an individual with CF. PA represents an opportunity for participants to slow the rate of this decline and manage the symptoms associated with the condition. Enjoyment was an important facilitator of PA. Exercise professionals and family reinforce PA behaviour, particularly during adolescence.ConclusionsPA promotion should form part of routine CF care with additional exercise professional support during adolescence.

What Do We Know about the Microbiome in Cystic Fibrosis? Is There a Role for Probiotics and Prebiotics?

Cystic fibrosis (CF) is a life-shortening genetic disorder that affects the cystic fibrosis transmembrane conductance regulator (CFTR) protein. In the gastrointestinal (GI) tract, CFTR dysfunction results in low intestinal pH, thick and inspissated mucus, a lack of endogenous pancreatic enzymes, and reduced motility. These mechanisms, combined with antibiotic therapies, drive GI inflammation and significant alteration of the GI microbiota (dysbiosis). Dysbiosis and inflammation are key factors in systemic inflammation and GI complications including malignancy. The following review examines the potential for probiotic and prebiotic therapies to provide clinical benefits through modulation of the microbiome. Evidence from randomised control trials suggest probiotics are likely to improve GI inflammation and reduce the incidence of CF pulmonary exacerbations. However, the highly variable, low-quality data is a barrier to the implementation of probiotics into routine CF care. Epidemiological studies and clinical trials support the potential of dietary fibre and prebiotic supplements to beneficially modulate the microbiome in gastrointestinal conditions. To date, limited evidence is available on their safety and efficacy in CF. Variable responses to probiotics and prebiotics highlight the need for personalised approaches that consider an individual’s underlying microbiota, diet, and existing medications against the backdrop of the complex nutritional needs in CF.

Use of estrogen supplementation is associated with higher quality of life scores in women with cystic fibrosis

The association of estrogen supplementation use and quality of life in women with cystic fibrosis (CF) is not well characterized. In this cross-sectional study, women with CF completed quality of life questionnaires during a routine CF clinic visit. The use of estrogen supplementation was associated with higher quality of life scores in all domains of the CF questionnaire-revised (CFQ-R) and was significant in the role limitations and respiratory domains. Most participants who were not currently using estrogen supplementation had previously used estrogen supplementation. Most participants had used estrogen to regulate menses, prevent pregnancy and control symptoms around menses. Use of estrogen supplementation was not associated with differences in life-space mobility or screening for sexual dysfunction. This is the largest study to date investigating the association of estrogen supplementation and quality of life in women with CF. Prospective randomized studies are needed to clarify the association of estrogen supplementation and quality of life in women with CF.

Transitioning to telehealth during the coronavirus disease 2019 pandemic: Perspectives from partners of women with cystic fibrosis and healthcare providers.

Cystic fibrosis is a chronic, genetic disease that primarily affects the respiratory system. The coronavirus disease 2019 pandemic has altered how people with cystic fibrosis receive healthcare. This paper explores the perceptions of cystic fibrosis healthcare providers and partners of women with cystic fibrosis regarding the use of telehealth in routine cystic fibrosis healthcare in the US. As part of a larger study examining fertility counseling for women with cystic fibrosis, we conducted qualitative, semi-structured interviews. Participants included partners of women with cystic fibrosis (n = 20) and cystic fibrosis healthcare providers (n = 20). We completed the interviews before and during the global coronavirus disease 2019 pandemic. We analyzed the data using thematic analysis. When in-person healthcare could not be achieved safely, partners and healthcare providers found new value in telehealth. Three themes emerged: (1) increased connection between healthcare team and family, (2) increased efficiency of healthcare appointments, and (3) improved interdisciplinary collaboration. Partners found that telehealth allowed for more engagement in their significant others' healthcare. Providers reported enhanced efficiency and opportunities for collaboration across specialties that improved healthcare delivery and care coordination. Results from this study highlighted the positive value of telehealth. Telehealth presents as a potential alternative to delivering outpatient care for people with chronic illnesses beyond the pandemic.

81: Maintaining routine cystic fibrosis sputum surveillance during the pandemic

81: Maintaining routine cystic fibrosis sputum surveillance during the pandemic

76: Sustaining routine cystic fibrosis care in a large pediatric CF program during COVID-19

76: Sustaining routine cystic fibrosis care in a large pediatric CF program during COVID-19

IMPACT OF PORTABLE SPIROMETERS IN DELIVERY OF CYSTIC FIBROSIS CARE VIA TELEHEALTH

TOPIC: Education, Research, and Quality Improvement TYPE: Original Investigations PURPOSE: Cystic fibrosis (CF) is a chronic, multi-system disorder with significant morbidity and mortality. Consequently, long-term maintenance of this disease is heavily dependent on regular monitoring and treatments. The delivery of telehealth has become a topic of increasing importance since the onset of the COVID-19 pandemic. As such, portable spirometers have gained popularity in at-home care for CF patients. Forced expiratory volume (FEV1) is important clinical data used to assess the status of a patient’s disease and to guide their treatment plans. The purpose of this study was to examine the reliability of portable FEV1 values in comparison to hospital FEV1 values in order to explore novel approaches for remote CF monitoring during the SARS CoV-2 pandemic. METHODS: Funding from the Cystic Fibrosis Foundation and VCU Adult CF Center was used to provide patients with a ZEPHYRx home spirometry kit. Patients were provided instructional materials and were coached on how to use and maintain the device by dedicated respiratory therapists (RT). FEV1 values were reported through the ZEPHYRx Breathe Easy mobile app and these values were tracked from May 2020 to April 2021. The number of times these values fell at or above 90% of the patients’ best hospital FEV1 in the last 12 months was calculated as a percentage. The ZEYPHRx home spirometry values were deemed reliable if the percentage was ≥ 50%. RESULTS: The ZEPHYRx home spirometry kit provided reliable FEV1 values for 81% of the 48 patients who used the device. Compliance rate of the total 64 patients who received the home spirometry kit was 75%. The non-compliant patients were omitted from the final calculation. CONCLUSIONS: In conclusion, the study was successful in determining the reliability of utilizing ZEPHYRx home spirometry kits. The results of this study will expand the scope and efficacy of telehealth for the care of CF patients. CLINICAL IMPLICATIONS: Future studies can analyze the use of the kits in a longitudinal fashion to normalize their use in CF patient care. Including home spirometry kits as part of routine CF care will minimize patients’ visits to the clinic and therefore exposure to pathogens. DISCLOSURES: No relevant relationships by Madihah Alam, source=Web Response No relevant relationships by Sarah Barrett, source=Web Response Advisory Committee Member relationship with PARI Please note: 2019 Added 04/14/2021 by Nauman Chaudary, source=Web Response, value=Honoraria Moderator relationship with PARI Please note: 2021 Added 04/14/2021 by Nauman Chaudary, source=Web Response, value=Consulting fee Advisory Committee Member relationship with DYMEDSO Please note: 2021 Added 04/14/2021 by Nauman Chaudary, source=Web Response, value=Consulting fee No relevant relationships by Karen Lo, source=Web Response

IMPACT OF MAIL-IN SPUTUM CULTURES IN DELIVERY OF CYSTIC FIBROSIS CARE VIA TELEHEALTH

TOPIC: Education, Research, and Quality Improvement TYPE: Original Investigations PURPOSE: Cystic fibrosis (CF) is a multi-organ disease due to a mutation in the cystic fibrosis transmembrane conductance regulator gene. The mutation causes viscous mucus to accumulate in vital organs including the lungs. The thick mucus layers serve as sites of infection which are a significant cause of morbidity and mortality in CF patients. Sputum samples assess the types of pathogens that colonize CF patients’ lungs and can be used to guide treatment. Prior to the COVID-19 pandemic, sputum cultures were almost exclusively collected in the clinical setting. The purpose of this study was to examine the effectiveness of at-home collection of sputum samples. Specifically, we aimed to see if mail-in sputum samples can replace samples collected in clinic, thereby increasing accessibility of remote CF care. METHODS: Adult and pediatric patients were provided a mail-in sputum kit from the clinic along with instructions on collecting and shipping samples. Kits were completed by 7 adult and 32 pediatric patients from March 2020 to April 2021. The samples were shipped via FedEx to the microbiology lab for processing. The results were then compared with those of the patients’ previous hospital cultures from 2017-2020 for pediatrics and 2019-2020 for adults for reliability. The mail-in cultures were considered reliable if the patients’ previous in-clinic sputum samples had grown at least 50% of the same pathogens grown from the mail-in sample. RESULTS: Mail-in samples were reliable for 85.7% of the adult patients and 92.9% of the pediatric patients. Four pediatric patients were excluded from the study because they had not completed an in-hospital sputum sample. CONCLUSIONS: Given that the pathogens between adult and pediatric cultures were reliable 85.7% and 92.9% of the time respectively, mail-in sputum cultures appear to be an effective form of remote maintenance care in CF patients. Implementing mail-in culture protocols can expand the efficacy of telehealth in routine CF patient care. CLINICAL IMPLICATIONS: Future studies should look at optimizing patient education on mail-in sputum collection to facilitate ease of access. The usage of mail-in cultures can minimize the need for CF patients to come into the hospital for testing and in-clinic visits. DISCLOSURES: No relevant relationships by Madihah Alam, source=Web Response No relevant relationships by Sarah Barrett, source=Web Response Advisory Committee Member relationship with PARI Please note: 2019 Added 04/14/2021 by Nauman Chaudary, source=Web Response, value=Honoraria Moderator relationship with PARI Please note: 2021 Added 04/14/2021 by Nauman Chaudary, source=Web Response, value=Consulting fee Advisory Committee Member relationship with DYMEDSO Please note: 2021 Added 04/14/2021 by Nauman Chaudary, source=Web Response, value=Consulting fee No relevant relationships by Karen Lo, source=Web Response

Clinical Considerations for Routine Auditory and Vestibular Monitoring in Patients With Cystic Fibrosis.

Purpose Specific classes of antibiotics, such as aminoglycosides, have well-established adverse events producing permanent hearing loss, tinnitus, and balance and/or vestibular problems (i.e., ototoxicity). Although these antibiotics are frequently used to treat pseudomonas and other bacterial infections in patients with cystic fibrosis (CF), there are no formalized recommendations describing approaches to implementation of guideline adherent ototoxicity monitoring as part of CF clinical care. Method This consensus statement was developed by the International Ototoxicity Management Working Group (IOMG) Ad Hoc Committee on Aminoglycoside Antibiotics to address the clinical need for ototoxicity management in CF patients treated with known ototoxic medications. These clinical protocol considerations were created using consensus opinion from a community of international experts and available evidence specific to patients with CF, as well as published national and international guidelines on ototoxicity monitoring. Results The IOMG advocates four clinical recommendations for implementing routine and guideline adherent ototoxicity management in patients with CF. These are (a) including questions about hearing, tinnitus, and balance/vestibular problems as part of the routine CF case history for all patients; (b) utilizing timely point-of-care measures; (c) establishing a baseline and conducting posttreatment evaluations for each course of intravenous ototoxic drug treatment; and (d) repeating annual hearing and vestibular evaluations for all patients with a history of ototoxic antibiotic exposure. Conclusion Increased efforts for implementation of an ototoxicity management program in the CF care team model will improve identification of ototoxicity signs and symptoms, allow for timely therapeutic follow-up, and provide the clinician and patient an opportunity to make an informed decision about potential treatment modifications to minimize adverse events. Supplemental Material https://doi.org/10.23641/asha.16624366.

Estrogen Supplementation Is Associated With Higher Quality of Life Scores in Women With Cystic Fibrosis

Abstract Purpose: With rapid advancements in therapeutic options for patients with cystic fibrosis (CF), the median predicted survival has increased to 47 years along with the prevalence of non-pulmonary complications for patients with CF. Women with CF suffer irregular menses, sexual dysfunction and low bone mineral density. With increasing pregnancies among women with CF, they may consider contraception. Estrogen supplementation may modulate these outcomes and others. The purpose of this study was to explore the effects of supplemental estrogen use on quality of life (QOL) in CF. Methods: Women with CF ages 16-50 years were administered a validated CF-specific QOL survey (CFQ-R) during a routine CF clinic visit through an IRB-approved cross-sectional study. The QOL domain scores of subjects taking and not taking estrogen were compared pairwise by Kruskal Wallis tests and overall by Wilcoxon signed rank test. Results: The estrogen exposed and estrogen unexposed subjects with CF had similar age, BMI, FEV1, race, CF mutation, pancreatic insufficiency, diabetes and relationship status. The estrogen exposed subjects were taking 20-30 mcg of ethinyl estradiol in oral contraceptive pills. The estrogen exposed subjects had consistently higher QOL scores than the estrogen unexposed subjects (p=0.001). The estrogen exposed subjects had significantly higher scores in 7 of the 12 CFQ-R categories: physical, vitality, treatment burden and role domains and weight, respiratory and digestion symptom scales (p&amp;lt;0.05). When correcting for multiple comparisons, the estrogen-supplemented women had higher role domain scores than women not taking estrogen supplement (p=0.03). Conclusions: Estrogen supplementation was associated with improved quality of life in women with CF. This cross-sectional study highlights the need for further investigation into the potential benefits of estrogen supplementation. The dose, route, formulation and timing of estrogen therapy may modulate the beneficial effects for women with CF. Support: This work was supported by the National Center for Advancing Translational Sciences of the National Institutes of Health under award TL1TR002382 and UL1TR002378, and Cystic Fibrosis Foundation award WU20D0. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH or CFF.