Gitelman Syndrome Provisionally Diagnosed During the First Presentation of Diabetic Ketoacidosis.

Gitelman syndrome (GS) is an autosomal recessive disease characterized by hypokalemia, hypomagnesemia, metabolic alkalosis, and hypocalciuria. It is caused by mutations in gene SLC12A3 (located in chromosome 16q) encoding NaCl cotransporter. GS is usually asymptomatic for several years and is diagnosed in late childhood or adulthood. The association between GS and diabetic ketoacidosis (DKA) is rare. We present a case of a 25-year-old man with newly diagnosed diabetes mellitus and DKA with profound hypokalemia and hypomagnesemia who was provisionally found to have GS.

Implementing Automated Prone Ventilation for Acute Respiratory Distress Syndrome via Simulation-Based Training.

BACKGROUND: Prone position ventilation (PPV) is recommended for patients with severe acute respiratory distress syndrome, but it remains underused. Interprofessional simulation-based training for PPV has not been described. OBJECTIVES: To evaluate the impact of a novel interprofessional simulation-based training program on providers' perception of and comfort with PPV and the program's ability to help identify unrecognized safety issues ("latent safety threats") before implementation. METHODS: A prospective observational quality improvement study was done in the medical intensive care unit of an academic medical center. Registered nurses, physicians, and respiratory therapists were trained via a didactic session, simulations, and structured debriefings during which latent safety threats were identified. Participants completed anonymous surveys before and after training. RESULTS: A total of 73 providers (37 nurses, 18 physicians, 18 respiratory therapists) underwent training and completed surveys. Before training, only 39% of nurses agreed that PPV would be beneficial to patients with severe acute respiratory distress syndrome, compared with 96% of physicians and 70% of respiratory therapists (P < .001). Less than half of both nurses and physicians felt comfortable taking care of prone patients. After training, perceived benefit increased among all providers. Comfort taking care of proned patients and managing cardiac arrest increased significantly among nurses and physicians. Twenty novel latent safety threats were identified. CONCLUSION: Interprofessional simulation-based training may improve providers' perception of and comfort with PPV and can help identify latent safety threats before implementation.

Successful Treatment of Refractory Massive Pulmonary Embolism with Repeated Administration of Systemic Thrombolysis.

BACKGROUND: We report a case series of two patients in the intensive care unit with massive pulmonary embolism and obstructive shock who had resolution of shock after repeated administration of alteplase. CASE SUMMARIES: Both patients were initially dosed 10 mg of alteplase followed by infusion of 90 mg over 2 hours, but remained in obstructive shock requiring significant inotropic and vasopressor support. Both patients were deemed poor candidates for embolectomy. The first patient received repeated doses reaching an accumulative dose of 200 mg alteplase over 15 hours. The second patient received an accumulative dose of 250 mg alteplase over 36 hours. Both patients had resolution of shock within 24 hours of repeated administration of alteplase, but also experienced significant drops in hemoglobin, which were supported with transfusions. They were transferred out of the intensive care unit after resolution of obstructive shock and hemorrhage. The first patient died one week after transfer from the intensive care unit due to invasive candidiasis and septic shock. The second patient was weaned from the ventilator and discharged home. CONCLUSION: Patients with obstructive shock secondary to massive pulmonary embolism despite a one-time dose of alteplase and poor candidacy for embolectomy may benefit from repeated doses of alteplase. Due to the short half-life, repeated administration of thrombolytic may be appropriate for younger patients without absolute contraindications to thrombolysis, but future studies are needed to identify the optimal patient population.

Reduced survival in adult cystic fibrosis despite attenuated lung function decline.

BACKGROUND: There is limited data on disease progression and survival in adult diagnosis cystic fibrosis (CF). This study evaluates change of lung function over time and rates of death/lung transplant in adult diagnosis CF. METHODS: The CF Foundation Patient Registry was reviewed for patients diagnosed 1993-2003. Rate of FEV1 decline was calculated up to 2010 for age groups 6-11, 12-17, and 18 and above. Kaplan Meier method was used for 10 and 15year survival rate calculations for patients diagnosed as adults. Cox Proportional hazards models using predictors affecting disease progression and survival without transplant were run. RESULTS: Between 1993 and 2003, 11,884 patients were diagnosed with CF, of which 2848 were ages 6 and older. Annual rate of change of FEV1% predicted over 5years differed by diagnosis age group: -1.42% per year for ages 6-11, -2.04% for ages 12-17 and -1.13% for ages 18-65 (p<0.0001). Pseudomonas aeruginosa infection was associated with faster rates of lung function decline in all age groups. Survival without transplant for CF patients diagnosed at ≥18years were 76% and 65% by 10 and 15years, respectively. Of adults with FEV1 of >70% predicted at diagnosis, 95% were alive without transplant at 10years, whereas of those with FEV1<40% predicted at diagnosis, 31% were alive without transplant at 10years. CONCLUSIONS: Lung function declines at a slower rate in adult diagnosis CF. However, particularly in those with low lung function at diagnosis, rates of death or transplant in adult diagnosis CF after 10 and 15years is not negligible.

A Novel Picture Guide to Improve Spiritual Care and Reduce Anxiety in Mechanically Ventilated Adults in the Intensive Care Unit.

RATIONALE: Hospital chaplains provide spiritual care that helps patients facing serious illness cope with their symptoms and prognosis, yet because mechanically ventilated patients cannot speak, spiritual care of these patients has been limited. OBJECTIVES: To determine the feasibility and measure the effects of chaplain-led picture-guided spiritual care for mechanically ventilated adults in the intensive care unit (ICU). METHODS: We conducted a quasi-experimental study at a tertiary care hospital between March 2014 and July 2015. Fifty mechanically ventilated adults in medical or surgical ICUs without delirium or dementia received spiritual care by a hospital chaplain using an illustrated communication card to assess their spiritual affiliations, emotions, and needs and were followed until hospital discharge. Feasibility was assessed as the proportion of participants able to identify spiritual affiliations, emotions, and needs using the card. Among the first 25 participants, we performed semistructured interviews with 8 ICU survivors to identify how spiritual care helped them. For the subsequent 25 participants, we measured anxiety (on 100-mm visual analog scales [VAS]) immediately before and after the first chaplain visit, and we performed semistructured interviews with 18 ICU survivors with added measurements of pain and stress (on ±100-mm VAS). MEASUREMENTS AND MAIN RESULTS: The mean (SD) age was 59 (±16) years, median mechanical ventilation days was 19.5 (interquartile range, 7-29 d), and 15 (30%) died in-hospital. Using the card, 50 (100%) identified a spiritual affiliation, 47 (94%) identified one or more emotions, 45 (90%) rated their spiritual pain, and 36 (72%) selected a chaplain intervention. Anxiety after the first visit decreased 31% (mean score change, -20; 95% confidence interval, -33 to -7). Among 28 ICU survivors, 26 (93%) remembered the intervention and underwent semistructured interviews, of whom 81% felt more capable of dealing with their hospitalization and 0% felt worse. The 18 ICU survivors who underwent additional VAS testing during semistructured follow-up interviews reported a 49-point reduction in stress (95% confidence interval, -72 to -24) and no significant change in physical pain that they attributed to picture-guided spiritual care. CONCLUSIONS: Chaplain-led picture-guided spiritual care is feasible among mechanically ventilated adults and shows potential for reducing anxiety during and stress after an ICU admission.