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Background: Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator triple combination therapy (TCT) is available to approximately 85% of the U.S. CF population. Clinical trials of TCT demonstrate numerous improvements in physical health and healthrelated quality of life (HRQoL), but fewstudies have examined the effects of TCTon mental health and psychosocial outcomes, and little is known about whether gains in HRQoL are sustained over time.We aimed to describe the HRQoL and psychosocial outcomes of people with CF (PwCF) initiating TCT and explored changes in these outcomes up to 1 year after starting TCT. Method(s): This longitudinal study enrolled PwCF aged 14 and older who were followed at a large, combined pediatric and adult CF center. Questionnaires were administered within 6 months of initiating TCT (baseline) and 3, 6, and 12 months later. Study self-report measures evaluated were HRQoL (Cystic Fibrosis Questionnaire-Revised;CFQ-R), optimism, self-efficacy, medication-related beliefs (Medication Beliefs Questionnaire;MBQ), perceived social stigma of illness, and body image. Data were also collected from medical charts on measures of health and mental health screening. Four open-ended questionswere included at each timepoint to elicit qualitative data on experiences starting TCT. Longitudinal data were analyzed using linear mixed-effects models for repeated measures. Result(s): Sixty-three adults and adolescents with CF completed the full set of surveys at baseline. Mean participant age was 30.0 +/- 14.2. Fifty-four percent identified as female, 43% as male, and 2% as nonbinary. Seventyfour percent had private insurance. Mean percentage predicted forced expiratory volume in 1 second (FEV1pp) at baseline was 76.0 +/- 24.1%, and mean body mass index (BMI) was 22.9 +/- 3.1 kg/m2. At 12 months, mean FEV1pp was 80.8 +/- 21.9%, and mean BMI was 24.5 +/- 4.1 kg/m2. On standard measures used in CF mental health screening, mean baseline Patient Health Questionnaire (PHQ-9) score was 3.4 +/- 3.5, and mean General Anxiety Disorder score was 3.4 +/- 3.7. Mean PHQ-9 (3.5 +/- 4.0) and GAD-7 (3.4 +/- 3.7) scores at 12 months were similar to baseline. We found no statistically significant differences between the survey time points in participants' physical, respiratory, or emotional functioning on the CFQ-R, but there was a significant change in social functioning ( p < 0.001). There was no statistically significant change over time in optimism or selfefficacy, but there was a significant difference in CF medication beliefs between the four survey time points ( p = 0.008 for MBQ Importance subscale), with a decrease in perceived importance from baseline to 12 months. Conclusion(s): Whereas lung function and BMI increased in our sample by 12 months, similar improvementswere not seen in standard mental health outcomes. There was no change over time in physical, respiratory, or emotional functioning, optimism, or self-efficacy. Only CFQ-R social functioning had changed by 12 months, perhaps reflecting decreased COVID-related social isolation. There was also a change in medicationrelated beliefs, with a decrease in perceived importance of taking CF medications at 12 months. Future directions include conducting qualitative analyses of open-ended questions and further examining data on social stigma, motivation to take medications, and body image, as well as examining relationships between outcome variables and baseline FEV1 and BMICopyright © 2022, European Cystic Fibrosis Society. All rights reserved
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Background: With FDA approval in October 2019 of elexacaftor/ tezacaftor/ivacaftor, a CFTR modulator triple combination therapy (TCT), approximately 85% of the CF population was eligible to initiate this treatment. Clinical trial data indicates numerous improvements in physical health and health-related quality of life (HRQoL). Real-world studies of treatment initiation recommend including mental health as an outcome meaure, not reported in clinical trials. Objective: To describe the QoL and mental health outcomes of people with CF (pwCF) initiating TCT in a real-world setting. Methods: This longitudinal study enrolled pwCF 14 years and older who are followed at a large, combined pediatric and adult CF center. Data will be obtained at the following timepoints: within 6 months of initiating TCT (baseline), and then at 3, 6, and 12 months after baseline. Study self-report measures evaluate: HRQoL (CFQ-R), optimism, perceived social stigma of illness, self-efficacy, medication-related beliefs, and body image. Four open-ended questions were included to elicit qualitative data on experiences starting TCT. Data from the baseline survey are reported here. Results: Sixty-five adults and adolescents with CF completed the full set of surveys at baseline. Mean participant age was 30.2 years (SD= 14.0). Among this group, 57% identified as female, 42% as male, and 2% as nonbinary. With respect to education, 20.8% completed high school or less, 23.6% completed some college, and 45.9% completed college or above. As compared to a large 2010 US sample (Quittner, et al), participants reported higher Physical functioning (t=3.0;p<0.01), lower Emotional functioning (t=-6.7;p<0.001), and lower Social functioning (t=-2.1;p<0.05) on the CFQ-R measure of HRQoL. In terms of mental health, participants reported a mean score of 15.6 (SD= 5.7) on the LOT-R Optimism scale, falling in the Moderate optimism range. Participants had a mean score of 32.4 (SD= 4.3) on the General Self-Efficacy Scale measure, representing a t-score of 56 (73rd percentile). Open-ended questions revealed that patients' expectations regarding initiating TCT ranged from skepticism, to cautious optimism, to high expectations for life-changing results. Common hopes for TCT included reduction in treatment burden and increased quality of life, while collective fears included ineffectiveness and negative side effects. Many patients identified a change in future planning in response to starting TCT, namely increased hope and ambition. Conclusion: On average, pwCF in our sample who were starting TCT reported feeling moderate optimism and self-efficacy. They reported better physical functioning, but worse emotional and social functioning, than a 2010 sample. Whether these differences in HRQoL are due to TCT, COVID-19, or other factors requires further study. Open-ended questions elicited a mixture of positive and negative feelings related to starting TCT. Future analyses for this study include evaluation of key outcomes from the 3-month follow-up timepoint, including data on the mental health impact of COVID-19. Future directions include longitudinal analyses of the impact of TCT on HRQoL and mental health.
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Background: The 2019 novel coronavirus pandemic (COVID-19) necessitated a rapid shift from conducting multidisciplinary cystic fibrosis (CF) care from in-person to telehealth-based visits. Given infrequent use of telehealth in the CF chronic care model prior to this time, experience of care in this new paradigm for patients is unknown. Objective: To determine experience of telehealth in the care model of patients in adult CF centers and assess barriers to future visits. Methods: Adults across 3 CF centers were issued online surveys via email and text link within one week of the patient's first telehealth visit in fashion under IRB approval. In the survey, patients identified their CF care site and their age range. The survey focused on three themes to assess telehealth experience of care: convenience of the visit, level of concern with absence of usual in-person assessments (pulmonary function testing (PFT), weight, sputum culture, vital signs and physical exam), and the role of telehealth in their future care. Using a 5-point Likert scale of concern, we defined 1 as not concerning and 5 as very concerning. Low level of concern was defined as a score of 2 or less. Results: Between March and June 2020, 90 adults with CF were surveyed at 3 CF centers following telehealth visits. Participants' ages were consistent with usual range of adult CF patients. The majority (71%) had not participated in a telehealth visit before March 2020. During the multidisciplinary visits, MD/DOs were seen most often (91% of visits), followed by advanced practice providers (32%), registered dietitians (31%), registered nurses (13%), and social workers (12%). Nearly all respondents (93%) were satisfied with the overall treatment experience and found utilizing telehealth services convenient. Almost all (96%) felt that their issues and concerns were addressed during the visit. The concern for lack of usual in-person assessments varied: 68% indicated low concern with lack of vital signs and physical exam, 80% indicated low concern with lack of weight measurement, 52% for lack of throat/sputum culture, while only 41% indicated low concern with lack of PFTs. When asked how many future care visits would be preferred to be telehealth, 7% indicated all visits, 35% indicated most visits, 50% indicated some visits, and 8% indicated none. Nearly all respondents (97%) were interested in home lung function assessment. Conclusions: These data show that telehealth is well-received in the adult CF population, despite lacking hallmarks of routine care, such as PFTs and sputum culture. This study supports the notion that multidisciplinary telehealth care should be a key component in the chronic CF care model, even after the COVID-19 pandemic, but provisions for home assessments are needed. Additional survey and longitudinal data for ongoing patient attitude is being analyzed to gain a larger understanding of the necessary components for the role of longitudinal telehealth in the CF chronic care model.
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Objective: To use a multidisciplinary quality improvement (QI) approach for telehealth visits to facilitate the care of people with cystic fibrosis (CF) in our pediatric program in the midst of a pandemic. Background/Significance: Care guidelines recommend that people with CF are seen quarterly with multidisciplinary (MDR) visits to monitor health and well-being. In 2020, the COVID-19 pandemic posed a threat to this care model as routine ambulatory clinics were closed and in-person visits were quickly converted to telehealth visits. Our CF program utilized Zoom technology to facilitate our MDR clinical care approach with telehealth. Methods: Our MDR team consisted of physicians, nurses, nurse practitioners, social workers, dietitians, physical therapists, and administrators. We collaborated with our parent QI advisors, the parents of two young teens with CF. We created a process map for telehealth visits and identified barriers. We adapted a pre-existing, pre-visit planning (PVP) tool to telehealth visits. The modified PVP form included: information for the next visit;SARS-CoV-2 exposure and COVID-19 risk;review of equipment;sick plan;and details for communications with the CF center. Our weekly pediatric MDR meetings were used to discuss patients scheduled for the telehealth program and identify potential communication barriers to parent and patient use of the system. Appropriate staff members were identified to join the Zoom visit. The CF nursing coordinator (1) arranged Zoom visits with identified administrative staff;(2) provided education during the visit;(3) arranged for out-patient testing and followed the results;(3) coordinated CF consults with endocrine, diabetic nurse educators, and the gastroenterologist;(4) provided sick plans for families, and (5) communicated with homecare companies for respiratory equipment. The team sent post-visit surveys to all families who had telehealth visits. Results/Findings: We began offering telehealth visits 3/22/20 through a hospital-wide platform and added Zoom visits 3/29/20 which better suited the multidisciplinary care model where different members of the MDR team could join. Physical therapy was unable to participate in telehealth visits. From 3/29/2020 to 5/29/2020 we provided 144 virtual visits, 73 of these virtual visits were Zoom visits (51%) with social work participating in 67%, nutrition 73%, and nursing 100%. Conclusion/Next Steps: We rapidly established a telehealth program using Zoom visits to assess and provide CF care during the COVID-19 crisis. Patients, families and staff were satisfied with their telehealth visits. The next phase of the telehealth program will include both telehealth and in-person visits. We will improve our program by addressing the barriers we have identified, such as (1) the need to develop web-based learning tools for patients and parents, and (2) the need to ensure a nursing presence on the telehealth visit. A nursing presence will help alleviate fears about telehealth (particularly during the pandemic) and address frequent patient needs: (1) checking respiratory equipment such as nebulizers, compressors, and vest, (2) creating a sick plan, (3) following up on lab work, (4) checking prescription refills, and (5) coordinating future care.
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Background: People with cystic fibrosis (CF) are 2-3 times more likely to experience depression, anxiety, or both. Untreated depression and anxiety can affect both physical and emotional health. Left untreated, people with CF are less likely to manage their treatment plans effectively, have worse lung function, have a lower BMI, and experience more hospitalizations. At Boston Children's Hospital/Brigham and Women's Hospital combined pediatric and adult CF center, we have 598 patients total (274 in the pediatric program and 324 in the adult program). 79% of our patients are over 12 and eligible for depression and anxiety screening. Our pediatric care team consists of 15 MDs/NPs and 2 social workers. Our adult care team consists of 7 MDs/NPs and 1 social worker. There is 1 psychologist that sees both pediatric and adult patients. Objectives: (1) To streamline mental health screening process, (2) improve mental health screening rates for our CF pediatric and adult populations, and (3) identify patients who need further mental health services. Methods: To ensure we address mental health, we developed a new process for distributing the PHQ-9 assessment tool for depression and GAD-7 assessment tool for anxiety. We implemented a screening note template into our electronic medical record for better documentation of completed screens and began including mental health screens as a part of the weekly pre-visit planning amongst clinicians, nurses, and social workers. We refined the process to allow for our clinic administrators to hand out the assessments to patients at check-in and nursing assisted in collecting the screens and responding appropriately depending on the score. Results: Our average mental health screening rate in 2018 was 46% for the pediatric program and 71% for the adult program. After implementing our new process in 2019, our screening rates increased to 71% for the pediatric program and 83% for the adult program. With increased rates of screening, we did not observe a significant difference in the percentage of patients screening at-risk. A majority of patients scored in the minimal-mild range on both the PHQ-9 and GAD-7. On the PHQ-9, 8% scored in the moderate range (vs 7% in 2018) and 2% scored in the severe range (vs 2% in 2018). On the GAD-7, 10% scored in the moderate range (vs 9% in 2018) and 3% scored in the severe range (vs 6% in 2018). On question #9, 98% indicated “not at all” (vs 96% in 2018). Conclusion: Our mental health screening process has become an embedded part of our CF clinic flow. Our process has led to sustained increases in mental health screening rates. Future work will focus on follow-up on mental health treatment referrals. In addition, with the rapid shift to telehealth due to COVID, we are adapting our process to account for telehealth visits.