Adherence type impacts completion rates of frequent mobile cognitive assessments among older adults with and without cognitive impairment.

Background: Prior to a diagnosis of Alzheimer's disease, many individuals experience cognitive and behavioral fluctuations that are not detected during a single session of traditional neuropsychological assessment. Mobile applications now enable high-frequency cognitive data to be collected remotely, introducing new opportunities and challenges. Emerging evidence suggests cognitively impaired older adults are capable of completing mobile assessments frequently, but no study has observed whether completion rates vary by assessment frequency or adherence type. Methods: Thirty-three older adults were recruited from the Boston University Alzheimer's Disease Research Center (mean age = 73.5 years; 27.3% cognitively impaired; 57.6% female; 81.8% White, 18.2% Black). Participants remotely downloaded and completed the DANA Brain Vital application on their own mobile devices throughout the study. The study schedule included seventeen assessments to be completed over the course of a year. Specific periods during which assessments were expected to be completed were defined as subsegments, while segments consisted of multiple subsegments. The first segment included three subsegments to be completed within one week, the second segment included weekly subsegments and spanned three weeks, and the third and fourth segments included monthly subsegments spanning five and six months, respectively. Three distinct adherence types - subsegment adherence, segment adherence, and cumulative adherence - were examined to determine how completion rates varied depending on assessment frequency and adherence type. Results: Adherence type significantly impacted whether the completion rates declined. When utilizing subsegment adherence, the completion rate significantly declined (p = 0.05) during the fourth segment. However, when considering completion rates from the perspective of segment adherence, a decline in completion rate was not observed. Overall adherence rates increased as adherence parameters were broadened from subsegment adherence (60.6%) to segment adherence (78.8%), to cumulative adherence (90.9%). Conclusions: Older adults, including those with cognitive impairment, are able to complete remote cognitive assessments at a high-frequency, but may not necessarily adhere to prescribed schedules.

Functional activity of the caudate mediates the relation between early childhood microstructural variations and elevated metabolic syndrome scores.

BACKGROUND: Metabolic syndrome score in children assesses the risk of developing cardiovascular disease in future. We aim to probe the role of the caudate in relation to the metabolic syndrome score. Furthermore, using both functional and structural neuroimaging, we aim to examine the interplay between functional and structural measures. METHODS: A longitudinal birth cohort study with functional and structural neuroimaging data obtained at 4.5, 6.0 and 7.5 years and metabolic syndrome scores at 8.0 years was used. Pearson correlation and linear regression was used to test for correlation fractional anisotropy (FA) and fractional amplitude of low frequency fluctuations (fALFF) of the caudate with metabolic syndrome scores. Mediation analysis was used to test if later brain measures mediated the relation between earlier brain measures and metabolic syndrome scores. Inhibitory control was also tested as a mediator of the relation between caudate brain measures and metabolic syndrome scores. RESULTS: FA at 4.5 years and fALFF at 7.5 years of the left caudate was significantly correlated with metabolic syndrome scores. Post-hoc mediation analysis showed that fALFF at 7.5 years fully mediated the relation between FA at 4.5 years and metabolic syndrome scores. Inhibitory control was significantly correlated with fALFF at 7.5 years, but did not mediate the relation between fALFF at 7.5 years and metabolic syndrome scores. CONCLUSIONS: We found that variations in caudate microstructure at 4.5 years predict later variation in functional activity at 7.5 years. This later variation in functional activity fully mediates the relation between microstructural changes in early childhood and metabolic syndrome scores at 8.0 years.

Muslim perspectives on palliative care in perinatal and neonatal patients: a mini-review.

Muslims comprise nearly a quarter of the worldwide population, with significant populations in the United States, Canada, and Europe. As clinicians, it is important to be familiar with Islamic religious and cultural perspectives on medical treatment, life-prolonging measures and comfort and palliative care, but historically, this has been a gap in the literature. Recently, there have been multiple papers discussing Islamic bioethics, particularly in regards to end of life care in adults; however, there has been a lack of literature discussing the Islamic perspective on issues related to neonatal and perinatal end of life care. This paper uses clinical scenarios to review key relevant principles of Islamic law, discussing the primary and secondary sources used in formulating fatawa, including the Quran, hadith, qiyas, and 'urf, and the importance of preservation of life and upholding of human dignity (karamah). Neonatal and perinatal scenarios are used to specifically explore the Islamic perspective on withholding and withdrawal of life-sustaining measures and determining what constitutes an acceptable quality of life. In some Islamic cultures the expertise of the patient's physician is given significant weight in making these judgments, and as such, families may appreciate frank assessment of the situation by the clinical team. Because of the various factors involved in issuing religious ruling, or fatwa, there is a wide spectrum of opinions on these rulings, and physicians should be aware of these differences, seek counsel and guidance from local Islamic leaders, and support families in their decision-making process.

Role of palliative care in fetal neurological consultations: Guiding through uncertainty and hope.

Fetal neurology is a rapidly evolving and expanding field. Discussions about diagnosis, prognosis, treatment options, and goals of care often begin in the antenatal period. However, there are inherent challenges to fetal counseling of neurological diagnoses due to limitations of fetal imaging, prognostic uncertainty, and variability in neurodevelopmental outcomes. In the midst of uncertainty, families are challenged with preparing a care plan for their baby while simultaneously experiencing profound grief. The paradigms of perinatal palliative care can assist with the grieving process and help frame diagnostic testing and complex decision-making within the context of a family's spiritual, cultural, and social belief system. This ultimately leads to a shared decision-making process and value driven medical care. While perinatal palliative care programs have expanded, many families faced with such diagnoses never meet with a palliative care team prior to delivery. Moreover, there is significant variability in the availability of palliative care services throughout the country. Using an illustrative vignette of a patient with a prenatally diagnosed encephalocele, this review aims to provide a basic framework of perinatal palliative care for fetal neurology diagnoses that emphasizes 1) importance of clear, consistent, and transparent communication among all subspecialists and families, 2) creation of a palliative care birth plan, 3) importance of consistent care providers and longitudinal points of contact prenatally and post-delivery, 4) close communication between the prenatal and post-natal providers to allow for optimal continuity of care, and 5) recognize that information, care plans, and goals of care often evolve over time.

Single-cell landscape analysis unravels molecular programming of the human B cell compartment in chronic GVHD.

Alloreactivity can drive autoimmune syndromes. After allogeneic hematopoietic stem cell transplantation (allo-HCT), chronic graft-versus-host disease (cGVHD), a B cell-associated autoimmune-like syndrome, commonly occurs. Because donor-derived B cells continually develop under selective pressure from host alloantigens, aberrant B cell receptor (BCR) activation and IgG production can emerge and contribute to cGVHD pathobiology. To better understand molecular programing of B cells in allo-HCT, we performed scRNA-Seq analysis on high numbers of purified B cells from patients. An unsupervised analysis revealed 10 clusters, distinguishable by signature genes for maturation, activation, and memory. Within the memory B cell compartment, we found striking transcriptional differences in allo-HCT patients compared with healthy or infected individuals, including potentially pathogenic atypical B cells (ABCs) that were expanded in active cGVHD. To identify intrinsic alterations in potentially pathological B cells, we interrogated all clusters for differentially expressed genes (DEGs) in active cGVHD versus patients who never had signs of immune tolerance loss (no cGVHD). Active cGVHD DEGs occurred in both naive and BCR-activated B cell clusters. Remarkably, some DEGs occurred across most clusters, suggesting common molecular programs that may promote B cell plasticity. Our study of human allo-HCT and cGVHD provides understanding of altered B cell memory during chronic alloantigen stimulation.

Ten steps to becoming a musculoskeletal simulation expert: A half-century of progress and outlook for the future.

Over the past half-century, musculoskeletal simulations have deepened our knowledge of human and animal movement. This article outlines ten steps to becoming a musculoskeletal simulation expert so you can contribute to the next half-century of technical innovation and scientific discovery. We advocate looking to the past, present, and future to harness the power of simulations that seek to understand and improve mobility. Instead of presenting a comprehensive literature review, we articulate a set of ideas intended to help researchers use simulations effectively and responsibly by understanding the work on which today's musculoskeletal simulations are built, following established modeling and simulation principles, and branching out in new directions.

Shared grey matter correlates of reading and attention.

Disorders of reading (developmental dyslexia) and attention (ADHD) have a high rate of comorbidity (25-40%), yet little is known about the neural underpinnings of this phenomenon. The current study investigated the shared and unique neural correlates of reading and attention in 330 typically developing children ages 8-18 from the Philadelphia Neurodevelopmental Cohort. Multiple regression analyses were used to identify regions of the brain where grey matter (GM) volume was associated with reading or attention scores (p < 0.001, cluster FDR p < 0.05). Better attention scores correlated with increased GM in the precuneus and higher reading scores were associated with greater thalamic GM. An exploratory conjunction analysis (p < 0.05, k > 239) found that GM in the caudate and precuneus correlated with both reading and attention scores. These results are consistent with a recent meta-analysis which identified GM reductions in the caudate in both dyslexia and ADHD and reveal potential shared neural correlates of reading and attention.

Independently ambulatory children with spina bifida experience near-typical knee and ankle joint moments and forces during walking.

BACKGROUND: Spina bifida, a neurological defect, can result in lower-limb muscle weakness. Altered ambulation and reduced musculoskeletal loading can yield decreased bone strength in individuals with spina bifida, yet individuals who remain ambulatory can exhibit normal bone outcomes. RESEARCH QUESTION: During walking, how do lower-limb joint kinematics and moments and tibial forces in independently ambulatory children with spina bifida differ from those of children with typical development? METHODS: We retrospectively analyzed data from 16 independently ambulatory children with spina bifida and 16 children with typical development and confirmed that tibial bone strength was similar between the two groups. Plantar flexor muscle strength was measured by manual muscle testing, and 14 of the children with spina bifida wore activity monitors for an average of 5 days. We estimated tibial forces at the knee and ankle using motion capture data and musculoskeletal simulations. We used Statistical Parametric Mapping t-tests to compare lower-limb joint kinematic and kinetic waveforms between the groups with spina bifida and typical development. Within the group with spina bifida, we examined relationships between plantar flexor muscle strength and peak tibial forces by calculating Spearman correlations. RESULTS: Activity monitors from the children with spina bifida reported typical daily steps (9656 [SD 3095]). Despite slower walking speeds (p = 0.004) and altered lower-body kinematics (p < 0.001), children with spina bifida had knee and ankle joint moments and forces similar to those of children with typical development, with no detectable differences during stance. Plantar flexor muscle weakness was associated with increased compressive knee force (p = 0.002) and shear ankle force (p = 0.009). SIGNIFICANCE: High-functioning, independently ambulatory children with spina bifida exhibited near-typical tibial bone strength and near-typical step counts and tibial load magnitudes. Our results suggest that the tibial forces in this group are of sufficient magnitudes to support the development of normal tibial bone strength.

Clinically available testing options resulting in diagnosis in post-exome clinic at one medical center.

Exome sequencing (ES) became clinically available in 2011 and promised an agnostic, unbiased next-generation sequencing (NGS) platform for patients with symptoms believed to have a genetic etiology. The diagnostic yield of ES has been estimated to be between 25-40% and may be higher in specific clinical scenarios. Those who remain undiagnosed may have no molecular findings of interest on ES, variants of uncertain significance in genes that are linked to human disease, or variants of uncertain significance in candidate genes that are not definitively tied to human disease. Recent evidence suggests that a post-exome evaluation consisting of clinical re-phenotyping, functional studies of candidate variants in known genes, and variant reevaluation can lead to a diagnosis in 5-15% of additional cases. In this brief research study, we present our experience on post-exome evaluations in a cohort of patients who are believed to have a genetic etiology for their symptoms. We have reached a full or partial diagnosis in approximately 18% (6/33) of cases that have completed evaluations to date. We accomplished this by utilizing NGS-based methods that are available on a clinical basis. A sample of these cases highlights the utility of ES reanalysis with updated phenotyping allowing for the discovery of new genes, re-adjudication of known variants, incorporating updated phenotypic information, utilizing functional testing such as targeted RNA sequencing, and deploying other NGS-based testing methods such as gene panels and genome sequencing to reach a diagnosis.

Alobar holoprosencephaly: Exploring mothers' perspectives on prenatal decision-making and prognostication.

OBJECTIVES: Parents receiving a prenatal diagnosis of alobar holoprosencephaly (HPE) often experience uncertainty regarding the pregnancy prognosis. There is little known about how best to counsel and support families receiving the diagnosis. This study explored parental experiences and wishes after receiving a prenatal diagnosis of alobar HPE. METHODS: This was a retrospective qualitative study. Semi-structured interviews were conducted to determine factors impacting parents' decision-making process, experiences with healthcare providers, and expectations for their child's length and quality of life. Interviews were analyzed using qualitative content analysis. RESULTS: Eight mothers who received a prenatal diagnosis of alobar HPE between 2013 and 2019 participated in the study. Parental expectations were based on information conveyed during prenatal counseling. Religious and personal beliefs, perceived suffering, and provider prognostication contributed to parent decisions and goals of care. Participants reported pressure to terminate the pregnancy. Parents were not prepared for the possibility of survival beyond the perinatal period. Most parents reported no regret in their choices. CONCLUSION: Patients receiving a prenatal diagnosis of alobar HPE desire access to balanced prenatal counseling about prognosis, morbidity, and mortality. Providers should explore values and beliefs that contribute to parents' goals of care and offer appropriate information and referrals.

Topiramate Is Safe for Refractory Neonatal Seizures: A Multicenter Retrospective Cohort Study of Necrotizing Enterocolitis Risk.

BACKGROUND: A previously published, single-institution, case series suggested an association between topiramate administration in neonates and subsequent development of necrotizing enterocolitis (NEC). This contradicted our more extensive experiences using topiramate in this population. We therefore studied safety and tolerability of topiramate for treating refractory neonatal seizures, hypothesizing that the risk of developing NEC following topiramate exposure was low and that most infants tolerate topiramate. METHODS: This multicenter retrospective cohort study included seventy-five neonates who received topiramate to treat seizures from January 2011 to October 2019 at three geographically diverse level IV neonatal intensive care units affiliated with pediatric tertiary hospitals. Data included demographics, birth history, seizure etiology, treatment response, side effects, and occurrence and details of NEC. RESULTS: Three of seventy-five infants (4%) developed NEC following topiramate exposure. These infants did not differ in gestational age, birth weight, seizure etiology, postmenstrual age, weight when topiramate was initiated, or dosing of topiramate. Topiramate was well tolerated. Only three infants (4%) discontinued due to side effects. The most common side effect (20%) was weight loss (typically <5%). Topiramate was felt to be efficacious (61%). Most infants (72%) continued topiramate when discharged. CONCLUSIONS: Our multicenter, 75-infant study demonstrated that development of NEC after treatment with topiramate was rare (4%) and refutes prior literature suggesting an association. Topiramate was felt to be efficacious and was well tolerated. Although limited by retrospective design, study data are broadly applicable and support thoughtful use of topiramate as a safe, reasonable option for treating refractory neonatal seizures.

Assessing inertial measurement unit locations for freezing of gait detection and patient preference.

BACKGROUND: Freezing of gait, a common symptom of Parkinson's disease, presents as sporadic episodes in which an individual's feet suddenly feel stuck to the ground. Inertial measurement units (IMUs) promise to enable at-home monitoring and personalization of therapy, but there is a lack of consensus on the number and location of IMUs for detecting freezing of gait. The purpose of this study was to assess IMU sets in the context of both freezing of gait detection performance and patient preference. METHODS: Sixteen people with Parkinson's disease were surveyed about sensor preferences. Raw IMU data from seven people with Parkinson's disease, wearing up to eleven sensors, were used to train convolutional neural networks to detect freezing of gait. Models trained with data from different sensor sets were assessed for technical performance; a best technical set and minimal IMU set were identified. Clinical utility was assessed by comparing model- and human-rater-determined percent time freezing and number of freezing events. RESULTS: The best technical set consisted of three IMUs (lumbar and both ankles, AUROC = 0.83), all of which were rated highly wearable. The minimal IMU set consisted of a single ankle IMU (AUROC = 0.80). Correlations between these models and human raters were good to excellent for percent time freezing (ICC = 0.93, 0.89) and number of freezing events (ICC = 0.95, 0.86) for the best technical set and minimal IMU set, respectively. CONCLUSIONS: Several IMU sets consisting of three IMUs or fewer were highly rated for both technical performance and wearability, and more IMUs did not necessarily perform better in FOG detection. We openly share our data and software to further the development and adoption of a general, open-source model that uses raw signals and a standard sensor set for at-home monitoring of freezing of gait.

Lethal Pediatric Cerebral Vasculitis Triggered by Severe Acute Respiratory Syndrome Coronavirus 2.

BACKGROUND: We report the clinical, radiological, laboratory, and neuropathological findings in support of the first diagnosis of lethal, small-vessel cerebral vasculitis triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a pediatric patient. PATIENT DESCRIPTION: A previously healthy, eight-year-old Hispanic girl presented with subacute left-sided weakness two weeks after a mild febrile illness. SARS-CoV-2 nasopharyngeal swab was positive. Magnetic resonance imaging revealed an enhancing right frontal lobe lesion with significant vasogenic edema. Two brain biopsies of the lesion showed perivascular and intraluminal lymphohistiocytic inflammatory infiltrate consistent with vasculitis. Despite extensive treatment with immunomodulatory therapies targeting primary angiitis of the central nervous system, she experienced neurological decline and died 93 days after presentation. SARS-CoV-2 testing revealed positive serum IgG and positive cerebrospinal fluid IgM. Comprehensive infectious, rheumatologic, hematologic/oncologic, and genetic evaluation did not identify an alternative etiology. Postmortem brain autopsy remained consistent with vasculitis. CONCLUSION: This is the first pediatric presentation to suggest that SARS-CoV-2 can lead to a fatal, postinfectious, inflammatory small-vessel cerebral vasculitis. Our patient uniquely included supportive cerebrospinal fluid and postmortem tissue analysis. While most children recover from the neurological complications of SARS-CoV-2, we emphasize the potential mortality in a child with no risk factors for severe disease.

Percutaneous Coronary Intervention Outcomes in Patients With Prior Thoracic Radiation Therapy: A Systematic Review and Meta-Analysis.

Background: Thoracic radiation predisposes patients to accelerated coronary artery disease. There is a paucity of data in both short-term and long-term outcomes following revascularization in patients who have undergone thoracic radiation. Methods: We performed a search of the Medline, Cochrane, and Scopus databases for studies that compared outcomes in cancer patients who have undergone thoracic radiation and percutaneous coronary intervention (PCI). The primary outcome of our meta-analysis was all-cause mortality. Secondary outcomes included cardiac mortality, myocardial infarction (MI), and restenosis. Results: The analysis included four observational studies with a total of 13,941 patients for the primary outcome of all-cause mortality. There were a total of 1,322 patients analyzed for cardiac mortality, 13,103 for MI, and 10,530 for restenosis. The longest follow-up for the primary outcome was 16 years. There was statistically significant higher risk of all-cause mortality in patients who underwent thoracic radiation (risk ratio (RR): 1.29, 95% confidence interval (CI): 1.08 - 1.54, P = 0.004). There was no statistically significant difference in cardiac mortality (RR: 1.15, 95% CI: 0.83 - 1.61, P = 0.40), MI (RR: 1.01, 95% CI: 0.20 - 5.08, P = 0.99), and restenosis (RR: 1.92, 95% CI: 0.24 - 15.35, P = 0.54). Conclusion: In this meta-analysis, we found a higher risk of all-cause mortality in patients with a history of thoracic radiation undergoing PCI, likely from underlying malignancy itself.

Clinical Characteristics and Outcomes in Immune Checkpoint Inhibitor Therapy-Associated Myocarditis.

Immune checkpoint inhibitor (ICI) therapy has played an important role in the treatment of several groups of cancers. Although a life prolonging treatment, many side effects have been shown with ICI therapy. This study looked at individual level clinical characteristics and outcomes with ICI therapy in patients who developed ICI-related myocarditis. A comprehensive review of the National Library of Medicine PubMed database was performed. Inclusion criteria were all studies that were composed of case reports and case series of individual patients undergoing ICI therapy that developed myocarditis. To appreciate individual patient level data, observational studies, clinical trials, systematic reviews, and meta-analyses were excluded. Our search yielded 333 results with 71 cases reviewed of ICI therapy-related myocarditis. The findings included an average age of 68 years, higher incidence in men, and pretreatment cardiac history of hypertension. Melanoma was the most prevalent malignancy with nivolumab being the most used ICI therapy. Heart failure was the most prevalent adverse event that was co-prevalent with myocarditis. Corticosteroid therapy alone was the most utilized therapy to treat ICI-related myocarditis. Mortality was seen in nearly half of the patient population. Our study reviewed the preexisting literature of prior reported myocarditis secondary to ICI therapy. Periodic surveillance should be performed by the cardio-oncologist and internist. Due to the expanding role of ICI therapy in treating a variety of cancer patients, appreciation of its impact on the development of myocarditis is needed.

Influence of landscape management practices on urban greenhouse gas budgets.

BACKGROUND: With a lack of United States federal policy to address climate change, cities, the private sector, and universities have shouldered much of the work to reduce carbon dioxide (CO2) and other greenhouse gas emissions. This study aims to determine how landcover characteristics influence the amount of carbon (C) sequestered and respired via biological processes, evaluating the role of land management on the overall C budget of an urban university. Boston University published a comprehensive Climate Action Plan in 2017 with the goal of achieving C neutrality by 2040. In this study, we digitized and discretized each of Boston University's three urban campuses into landcover types, with C sequestration and respiration rates measured and scaled to provide a University-wide estimate of biogenic C fluxes within the broader context of total University emissions. RESULTS: Each of Boston University's three highly urban campuses were net sources of biogenic C to the atmosphere. While trees were estimated to sequester 0.6 ± 0.2 kg C m-2 canopy cover year-1, mulch and lawn areas in 2018 emitted C at rates of 1.7 ± 0.4 kg C m-2 year-1 and 1.4 ± 0.4 kg C m-2 year-1, respectively. C uptake by tree canopy cover, which can spatially overlap lawn and mulched landcovers, was not large enough to offset biogenic emissions. The proportion of biogenic emissions to Scope 1 anthropogenic emissions on each campus varied from 0.5% to 2%, and depended primarily on the total anthropogenic emissions on each campus. CONCLUSIONS: Our study quantifies the role of urban landcover in local C budgets, offering insights on how landscaping management strategies-such as decreasing mulch application rates and expanding tree canopy extent-can assist universities in minimizing biogenic C emissions and even potentially creating a small biogenic C sink. Although biogenic C fluxes represent a small fraction of overall anthropogenic emissions on urban university campuses, these biogenic fluxes are under active management by the university and should be included in climate action plans.

The pink elephant in the room: Declining public interest in breast cancer and the impact of marketing efforts.

BACKGROUND: Public interest in breast cancer is associated with breast cancer awareness month. However, no study has analyzed the temporal trends in breast cancer interest or assessed how funding for breast cancer research is influenced by public interest. METHODS: We performed a cross-sectional analysis of Google Trends using the search query "Breast Cancer" between January 2004 - July 2020. We then computed bivariate correlations to assess the association between relative search interest (RSI) and the financial records of the Susan G. Komen Foundation. RESULTS: Average search interest has declined since 2004. We identified increasing breast cancer RSI beginning in October 2009, corresponding with breast cancer awareness month, with an overall peak in 2012 followed by a downtrend through 2017. These fluctuations in peak RSI corresponded with the U.S. National Football League's initiation of "Pink October" during which the players wore pink jerseys which started in 2009 and ceased in 2017. We also found a strong correlation between peak RSI and Susan G. Komen Foundation net assets as well as funding of public health screening services and public health education. CONCLUSIONS: Average interest in breast cancer has been declining since 2004 and peak interest, as well as breast cancer funding, has been declining since 2012. This suggests new marketing efforts are needed to improve breast cancer awareness and to increase fundraising. POLICY SUMMARY: Considering the decline in breast cancer interest and funding, we recommend implementing local, regional, and national policies that improve marketing campaigns and improve breast cancer awareness.

Factors influencing clinical trial participation for adult and pediatric patients with cystic fibrosis.

There remains a limited understanding of the factors influencing clinical trial participation for individuals with Cystic Fibrosis (CF). A comprehensive survey was developed to examine the interests, preferences, and barriers/facilitators to research and clinical trial participation for CF patients. A consecutive sample of 198 CF adults attending the St. Paul's Hospital CF Clinic and parents of children with CF attending the BC Children's Hospital CF Clinic from Vancouver, Canada were surveyed. Parents of pediatric patients were less comfortable with blood collection, required more safety data prior to participating, and were more concerned about potential side effects. Very few respondents (<10%) appeared able/willing to fulfill the typical requirements to participate in a phase 1 clinical trial. Overall, there were more similarities than differences between the responses of adult and parents of pediatric CF patients. The patient-centered information can be used to inform the design of future clinical trials to enhance feasibility.