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BACKGROUND: Anxiety and depressive symptoms are common among individuals with cystic fibrosis (CF) and are associated with decreased lung function [3], quality of life [4], and treatment adherence [2]. However, CF-specific targeted psychotherapeutic interventions are lacking. This study examined whether Acceptance and Commitment Therapy (ACT) [7], delivered via telehealth, would address this need and improve clinical symptoms. Telehealth is ideal for CF patients, given exposure precautions and frequent hospitalization. ACT emphasizes acceptance, thereby reducing avoidance of anxiety and depressive symptoms associated with CF. It was hypothesized that our ACT with CF protocol [11] would also improve lung function among people with CF. METHODS: Participants were 28 adults with CF and elevated clinical symptoms who completed 6 ACT with CF sessions. They completed measures of depression, anxiety, and cognitive fusion at baseline, post-intervention, and at a 3-month follow-up. Lung function was calculated 3 months pre- and 3 months post-treatment. RESULTS: The majority of participants selected treatment via telehealth (n = 22; 79%). 96% of participants (n=27) completed all 6 sessions, with 93% (n=26) voicing a strong desire to continue treatment with ACT. 79% of the sample (n=22) indicated, after just 1 session of ACT with CF, that treatment seemed logical and feeling confident that it would reduce symptoms of anxiety and depression. ACT with CF was associated with a statistically significant reduction in a composite score of psychological distress from pre to post treatment, corresponding to a large standardized effect size, that was not sustained at 3 months. Telehealth-delivered ACT with CF was as effective as in-person. Reductions in cognitive fusion were strongly related to improvements in psychosocial functioning. This is particularly promising as it reflects the proposed mechanism of action of ACT. ACT with CF was also associated with increased FEV1/FVC ratio at post-treatment follow-up. CONCLUSIONS: ACT with CF delivered via telehealth or in-person is a feasible and potentially effective treatment for improving anxiety and depressive symptoms, and increasing psychological flexibility via reductions in cognitive fusion. Due to the effect size associated with reduction in psychosocial distress, we are cautiously optimistic that ACT with CF will prove an effective treatment. Larger randomized controlled trials are needed to confirm the observed findings and further delineate the potential effects of ACT with CF on clinical outcomes among individuals with CF.

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BACKGROUND: The usage and attitudes towards medical marijuana in Cystic Fibrosis (CF) patients is unknown. Through the use of a survey we aim to clarify rates and reasons for use. METHODS: An anonymous survey was sent out to six centers in the Mid-Atlantic region of the United States. Use of and reason for medical marijuana was assessed, along with attitudes of the perceived utility of medical marijuana. RESULTS: A total of 637 surveys were sent out, and 193 surveys were returned (30.3% return rate). Three did not give consent, and one was empty, for a total of 189 completed surveys. 31 subjects (16.5%) reported having used marijuana for medical purposes in their lifetime, with 29 (15.4%) of these in the past year. The most used forms were edible and vaporized. The most common indications for usage were pain and stress. 28 out of 31 found marijuana to be a great deal effective for their symptoms. 21 of the 31 rated marijuana very important or important to their health. There were two reported side effects, both mild. Of 156 subjects who responded to the question if they would be interested in medical marijuana if available, 72 (46.2%) replied yes. CONCLUSION: The use of marijuana for medical reasons was 15.4% in the past year in this sample CF population, although more expressed interest if it was available through prescription. Side effects were rare. CF physicians are going to have to familiarize themselves with advantages and disadvantages of medical marijuana as there is a great deal of interest within the community, and legalization becomes more common.

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CASE PRESENTATION: A 49-year-old man was sent by his primary care physician to the rheumatology clinic with complaints of several months of bilateral lower extremity swelling. The swelling migrated from both ankles up to his knees. Presenting symptoms consisted of bilateral knee pain as well as bilateral wrist and hand pain with swelling. Pulmonary symptoms consisted of a nagging productive cough of several months. He also complained of significant weight loss: 50 pounds over 12 months. He was a never smoker. The examination was notable for bilateral knee effusions. Radiographs of his wrists, hands, and knee were obtained, along with a chest radiograph. He was then referred to a pulmonologist for further workup.

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To help answer the question of length of intravenous antibiotics during an acute exacerbation of cystic fibrosis (CF), we had subjects to follow daily home spirometry while on intravenous antibiotics. CF patients, 18 and older, with an acute exacerbation requiring intravenous antibiotics had a daily FEV1. The average time to a 10% increase over their initial sick FEV1 was calculated, as well as the time to a new baseline. A total of 25 subjects completed the study. Ten of the 25 subjects did not have a sustainable 10% increase in FEV1. Of the 15 subjects with a sustainable 10% increase in FEV1, it took 5.2 days (±4.5) after day 1, while a new baseline was achieved on average at 6.6 days (±4.8) after day 1. Given the wide range of time to a 10% improvement and new baseline, it is recommended there should be flexibility in length of intravenous antibiotics in CF, not by a preset number.

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RATIONALE AND OBJECTIVES: The use of hyperpolarized (3)He magnetic resonance imaging as a quantitative lung imaging tool has progressed rapidly in the past decade, mostly in the assessment of the airway diseases chronic obstructive pulmonary disease and asthma. This technique has shown potential to assess both structural and functional information in healthy and diseased lungs. In this study, the regional measurements of structure and function were applied to a bleomycin rat model of interstitial lung disease. MATERIALS AND METHODS: Male Sprague-Dawley rats (weight, 300-350 g) were administered intratracheal bleomycin. After 3 weeks, apparent diffusion coefficient and fractional ventilation were measured by (3)He magnetic resonance imaging and pulmonary function testing using a rodent-specific plethysmography chamber. Sensitized and healthy animals were then compared using threshold analysis to assess the potential sensitivity of these techniques to pulmonary abnormalities. RESULTS: No significant changes were observed in total lung volume and compliance between the two groups. Airway resistance elevated and forced expiratory volume significantly declined in the 3-week bleomycin rats, and fractional ventilation was significantly decreased compared to control animals (P < .0004). The apparent diffusion coefficient of (3)He showed a smaller change but still a significant decrease in 3-week bleomycin animals (P < .05). CONCLUSIONS: Preliminary results suggest that quantitative (3)He magnetic resonance imaging can be a sensitive and noninvasive tool to assess changes in an animal interstitial lung disease model. This technique may be useful for longitudinal animal studies and also in the investigation of human interstitial lung diseases.

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Neonatal and adult cardiomyocytes were isolated from rat hearts. Some of the adult myocytes were cultured to allow for cell dedifferentiation, a phenomenon thought to mimic cell changes that occur in stressed myocardium, with myocytes regressing to a fetal pattern of metabolism and stellate neonatal shape.Using fluorescence deconvolution microscopy, cells were probed with fluorescent markers and scanned for a number of proteins associated with ion control, calcium movements and cardiac function. Image analysis of deconvoluted image stacks and sequential real-time image recordings of calcium transients of cells were made.All three myocyte groups were predominantly comprised of binucleate cells. Clustering of proteins to a single nucleus was a common observation, suggesting that one nucleus is active in protein synthesis pathways, while the other nucleus assumes a 'dormant' or different role and that cardiomyocytes might be mitotically active even in late development, or specific protein syntheses could be targeted and regulated for reintroduction into the cell cycle.Such possibilities would extend cardiac disease associated stem cell research and therapy options, while producing valuable insights into developmental and death pathways of binucleate cardiomyocytes (word count 183).