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Background Massive pulmonary emboli can cause an abrupt onset of symptoms simultaneous with large pulmonary artery occlusions. In contrast, the temporal relationship between pulmonary vascular occlusion by smaller emboli and the development of symptoms of pulmonary infarction is unknown. We describe the time interval between embolization and the onset of clinical symptoms and signs compatible with pulmonary infarction. Methods We examined the records of 56 patients with hereditary hemorrhagic telangiectasia (HHT) who underwent therapeutic balloon embolization of pulmonary arteriovenous malformation (PAVM) in a single center after noting that some of them experienced symptoms and signs compatible with pulmonary infarction. Because both the times of embolization and the onset of clinical symptoms were documented in medical records, we were able to calculate the time interval between embolic occlusion of vessels and the onset of symptoms. Results The records of 56 patients who underwent therapeutic embolization for HHT were examined. Five patients experienced a single episode of pleuritic pain postembolization, and one patient experienced episodes of pleuritic pain after each of two separate embolization procedures. Four of these pleuritic pain events evolved into a complex compatible with pulmonary infarction. The time intervals between embolization and the onset of pleuritic pain in those experiencing the infarction symptoms and signs were 24 hours, 48 ± 4 hours, 65 hours, and 67 hours, respectively. Conclusions The clinically silent time interval between embolization of a pulmonary artery and the onset of symptoms and signs compatible with lung infarction is 24 hours or greater.

Oxygen delivery and carbon dioxide removal being critical to cell survival, mammals have developed collateral vascular and ventilation systems to ensure tissue viability. Collateral ventilation, defined as ventilation of alveoli via pathways that bypass normal airways, is present in humans and many other species. The presence of collateral ventilation can be beneficial in certain disease states, whereas its relative absence can predispose to other diseases. These well defined anatomical pathways contribute little to ventilation in normal humans, but modulate ventilation perfusion imbalance in a variety of diseases, including obstructive diseases, such as asthma and emphysema. These pathways can be affected by pharmaceuticals and inhaled gas compositions. The middle lobe and lingula, constrained by their isolated, segmental anatomy, have reduced collateral ventilation, which predisposes them to disease. Recently, attempts to improve the quality of life of patients with emphysema, by performing nonsurgical lung volume reduction via use of endobronchial valves, have led to mixed results, because the role of collateral ventilation in the success or failure of the procedure was not initially appreciated. This review describes the anatomical pathways of collateral ventilation, their physiology and relationship to disease states, their modulatory effects on gas exchange, treatment considerations, and their effect on diagnostic procedures.

In emphysema airway resistance can exceed collateral airflow resistance, causing air to flow preferentially through collateral pathways. In severe emphysema ventilation through openings directly through the chest wall into the parenchyma (spiracles) could bypass airway obstruction and increase alveolar ventilation via transpleural expiration. During lung transplant operations, spiracles occasionally can occur inadvertently. We observed transpleural expiration via spiracles in three subjects undergoing lung transplant for emphysema. During transpleural spiracle ventilation, inspiratory tidal volumes (TV) were unchanged; however, expiration was entirely transpleural in two patients whereas the expired TV to the ventilator circuit was reduced to 25% of the inspired TV in one. At baseline, mean PCO2 was 61 ± 5 mm Hg, which decreased to a mean PCO2 of 49 ± 5 mm Hg (P = .05) within minutes after transpleural spiracle ventilation and further decreased at 1 to 2 h (36 ± 4 mm Hg; P = .002 compared with baseline) on unchanged ventilator settings. This observation of increased alveolar ventilation supports further studies of spiracles as a possible therapy for advanced emphysema.

Massive pulmonary emboli can cause an abrupt onset of symptoms simultaneous with large pulmonary artery occlusions. In contrast, the temporal relationship between pulmonary vascular occlusion by smaller emboli and the development of symptoms of pulmonary infarction is unknown. We describe the time interval between embolization and the onset of clinical symptoms and signs compatible with pulmonary infarction.We examined the records of 56 patients with hereditary hemorrhagic telangiectasia (HHT) who underwent therapeutic balloon embolization of pulmonary arteriovenous malformation (PAVM) in a single center after noting that some of them experienced symptoms and signs compatible with pulmonary infarction. Because both the times of embolization and the onset of clinical symptoms were documented in medical records, we were able to calculate the time interval between embolic occlusion of vessels and the onset of symptoms.The records of 56 patients who underwent therapeutic embolization for HHT were examined. Five patients experienced a single episode of pleuritic pain postembolization, and one patient experienced episodes of pleuritic pain after each of two separate embolization procedures. Four of these pleuritic pain events evolved into a complex compatible with pulmonary infarction. The time intervals between embolization and the onset of pleuritic pain in those experiencing the infarction symptoms and signs were 24 hours, 48 ± 4 hours, 65 hours, and 67 hours, respectively.The clinically silent time interval between embolization of a pulmonary artery and the onset of symptoms and signs compatible with lung infarction is 24 hours or greater.

Patients with terminal illnesses often require surrogate decision makers. Prior research has demonstrated high surrogate stress, and that despite standards promoting substituted judgment, most patients do not want their surrogates to make pure substituted judgments for them. It is not known how best to help loved ones fulfill the surrogate role.To test the effectiveness of an intervention to help surrogate decision makers.One hundred sixty-six patients (41% with amyotrophic lateral sclerosis and 59% with gastrointestinal cancers) and their surrogates at two university medical centers were randomized to an intensive nurse-directed discussion of the end-of-life decision control preferences of the patient (TAILORED) or a discussion of nutrition (CONTROL); 163 completed baseline interviews and underwent the intervention.Twelve patients died during follow-up and 137 dyads completed the study. Post-intervention, using all available data, TAILORED patients and surrogates became more likely to endorse mutual surrogate decision making, that is, a balance of their own wishes and what the surrogate thinks best (adjusted odds compared with baseline for patients = 1.78, P = 0.04; adjusted odds for surrogates = 2.05, P = 0.03). CONTROL patients became 40% less likely to endorse mutual surrogate decision making (P = 0.08), and CONTROL surrogates did not change significantly from baseline (adjusted odds = 1.44, P = 0.28). Stress levels decreased for TAILORED surrogates (impact of events scale = 23.1 ± 14.6 baseline, 20.8 ± 15.3 f/u, P = 0.046), but not for CONTROL (P = 0.85), and post-intervention stress was lower for TAILORED than CONTROL (P = 0.04). Surrogates' confidence was uniformly high at baseline and did not change. Caregiver burden (Zarit) increased from 12.5 ± 6.5 to 14.7 ± 8.1 for TAILORED (P 0.01), while not changing for CONTROL, yet satisfaction with involvement in decision making was higher at follow-up for TAILORED than for CONTROL (71% vs. 52%, P = 0.03).TAILORED patients and surrogates who completed the study adopted a more mutual decision-making style, balancing their own wishes with what the surrogate thinks would be best for them. Surrogates reported less stress and more satisfaction. Confidence was high at baseline and did not change. There was a modest increase in caregiver burden. These findings suggest that interventions like TAILORED might positively impact surrogate decision making.

When you have a serious illness, you may suffer from physical discomfort, such as pain, difficulty breathing, nausea (stomach upset) or fatigue. You may also have feelings of psychological distress, such as anxiety or depression, or feelings of spiritual distress. Your healthcare providers may provide medical therapies to treat or manage your illness, for example, antibiotics to treat pneumonia. In addition, you may receive care that helps you feel more comfortable, but does not treat or cure your illness. This type of care is called "comfort" or palliative (PAL-lee-uh-tiv) care.

Objective:There is a growing body of literature describing the characteristics of patients who plan for the end of life, but little research has examined how caregivers influence patients' advance care planning (ACP). The purpose of this study was to examine how patient and caregiver characteristics are associated with advance directive (AD) completion among patients diagnosed with a terminal illness. We defined AD completion as having completed a living will and/or identified a healthcare power of attorney.Method:A convenience sample of 206 caregiver–patient dyads was included in the study. All patients were diagnosed with an advanced life-limiting illness. Trained research nurses administered surveys to collect information on patient and caregiver demographics (i.e., age, sex, race, education, marital status, and individual annual income) and patients' diagnoses and completion of AD. Multivariate logistic regression was employed to model predictors for patients' AD completion.Results:Over half of our patient sample (59%) completed an AD. Patients who were older, diagnosed with amyotrophic lateral sclerosis, and with a caregiver who was Caucasian or declined to report an income level were more likely to have an AD in place.Significance of results:Our results suggest that both patient and caregiver characteristics may influence patients' decisions to complete an AD at the end of life. When possible, caregivers should be included in advance care planning for patients who are terminally ill.

Introduction:Many patients with advanced heart failure (HF) experience the life-extending benefits of implantable cardioverter-defibrillators (ICD), but at the end stage of HF, patients may experience shocks with increasing frequency and change the plan for end-of-life (EOL) care including the deactivation of the ICD. This report describes family members’ experiences of patients with ICD making decisions at EOL. Understanding the decision-making of patients with ICD at EOL can promote informed decision-making and improve the quality of EOL care.Methods:This pilot study used a mixed methods approach to test the effects of a nurse-guided discussion in decision-making about ICD deactivation (turning off the defibrillation function) at the EOL. Interviews were conducted, audiotaped, and transcribed in 2012 to 2013 with 6 family members of patients with advanced HF and ICDs. Three researchers coded the data and identified themes in 2014.Results:Three main themes described family members’ experiences related to patients having HF with ICDs making health-care decision at EOL: decision-making preferences, patients’ perception on ICD deactivation, and communication methods.Discussion:Health-care providers need to have knowledge of patients’ decision-making preferences. Preferences for decision-making include the allowing of appropriate people to involve and encourages direct conversation with family members even when advance directives is completed. Information of ICD function and the option of deactivation need to be clearly delivered to patients and family members. Education and guidelines will facilitate the communication of the preferences of EOL care.

Objective:Persons with ALS differ from those with other terminal illnesses in that they commonly retain capacity for decision making close to death. The role patients would opt to have their families play in decision making at the end of life may therefore be unique. This study compared the preferences of patients with ALS for involving family in health care decisions at the end of life with the actual involvement reported by the family after death.Methods:A descriptive correlational design with 16 patient–family member dyads was used. Quantitative findings were enriched with in-depth interviews of a subset of five family members following the patient's death.Results:Eighty-seven percent of patients had issued an advance directive. Patients who would opt to make health care decisions independently (i.e., according to the patient's preferences alone) were most likely to have their families report that decisions were made in the style that the patient preferred. Those who preferred shared decision making with family or decision making that relied upon the family were more likely to have their families report that decisions were made in a style that was more independent than preferred. When interviewed in depth, some family members described shared decision making although they had reported on the survey that the patient made independent decisions.Significance of results:The structure of advance directives may suggest to families that independent decision making is the ideal, causing them to avoid or underreport shared decision making. Fear of family recriminations may also cause family members to avoid or underreport shared decision making. Findings from this study might be used to guide clinicians in their discussions of treatments and health care decision making with persons with ALS and their families.

CONTEXT Medical education in the clinical clerkship years emphasises the systematic collection and organisation of patient information to be combined with domain-specific knowledge of disease processes. Eventually, novice clinical problem solvers will learn to recognise patterns within the patient data ('illness scripts') which suggest the main diagnostic possibilities. Before novice problem solvers develop these illness scripts, pattern recognition may not be effective for solving clinical problems. METHODS This discussion paper describes the application of a decision framework adapted from cognitive psychology (the search-inference framework) to basic medical problem solving. Emphasis is placed on problem solving by novices who have not yet developed a full compliment of illness scripts. CONCLUSIONS The search-inference framework is similar to the approach taken by laypersons to diagnose their own symptoms or solve other problems. It may be especially useful for students who have not yet developed a sizeable repertoire of illness scripts, and the principles described may also be invoked by experienced clinicians confronting difficult clinical problems.

Many patients, as they approach death, are unable to speak for themselves. Healthcare professionals usually turn to loved ones to speak on behalf of such patients, generally without any notion of the manner in which these patients would have healthcare professionals or loved ones approach these decisions. The two major standards suggested in the ethical and legal literatures are the best interests standard (deciding what they think would be best for the patient) and substituted judgment (attempting to discern what the patient would have wanted and deciding accordingly, whether through a living will or other knowledge of the patient’s values).1,2 The literature in medical ethics and many legislative and judicial opinions have privileged substituted judgment as the preferred manner of deciding for others, yet one study has suggested that patients receiving dialysis would give surrogates wide latitude in making such decisions.3 Another study has shown that a slight majority of terminally ill patients would prefer that the decision be made by a loved one even if it contradicted a hypothetically “perfect” living will.4 Moreover, multiple studies have demonstrated that substituted judgments are far from perfectly accurate.5,6 It is known that patients who have intact decisionmaking capacity vary considerably in their decision control preferences.7-10 Some make decisions independently, some defer decision-making authority to loved ones or physicians, and most would opt for shared decisionmaking. It has recently been shown, similarly, that patients with terminal diagnoses vary considerably in the role they would assign to their loved ones and physicians should they no longer be able to speak for themselves.11 Some would have substituted judgments, and others would have their loved ones or physicians decide what they believe is best, but most would have some combination of these two approaches. The stability of the patient’s choice of decision-making style has not been studied. Advance directives have, in the opinion of many, failed to deliver on their promise to improve healthcare decisionmaking at the end of life.12,13 The problems encountered in a simplistic reliance on advance directives have led to calls for a broader approach that has been dubbed “advance care planning.”14 One hypothesis to explain the failure of advance directives is that patients may change their views not just of what interventions they would want, but also of how decisions ought to be made as they become sicker and closer to death. This article reports on a long-term series of measurements of terminally ill patients’ decision control preferences regarding decisions made on their behalf. It was hypothesized that, as patients came closer to death, they would be more likely to defer decision-making authority to their loved ones and physicians. Factors associated with patients’ decision control preferences for decisions made on their behalf in the event of decisional incapacity were also examined.

Interstitial lung disease (ILD) is a final common pathway for a large number of lung insults. It is characterized by progressive scarring of the lung leading to restriction and diminished oxygen transfer. Clinically, the presenting symptoms of ILD are nonspecific (cough and progressive dyspnea on exertion) and are often attributed to other diseases, thus delaying diagnosis and timely therapy. ILD occurs most commonly in older individuals and is increasingly encountered by internists. Both the diagnosis and treatment can be daunting: patients frequently have irreversibly impaired lung function at diagnosis, and therapeutic modalities are limited and associated with significant adverse effects. This review will assist internists in the recognition and management of ILD, provide a benchmark for pulmonary referrals, and offer guidance in advising patients with this life-threatening disorder.

Clinicians frequently see the “right middle lobe” referred to in radiology reports, textbooks, and medical journals. Yet, there is no “left” middle lobe to distinguish it from, save a rare patient with Kartagener syndrome. We do not refer to the lingula as the “left” lingula. “Right” adds nothing to our understanding of the anatomic position of the lobe. So “right” is redundant when talking about the middle lobe. Yet, PubMed has 5,266 right middle lobe references, and a Google Scholars search found 22,900 publications used the phrase in the past 161 years. Th at is the equivalent of the length of two and one-half football fi elds worth of wasted words in just a Google search, nevermind those found in radiology reports, textbooks, or Index Medicus references (in Calibri font size 12, “right” 5 1 cm 3 22,900 search results 5 228.99 m).

Background. Although it is known that many evidencebased therapies are underutilized, the causes of the research-practice gap are not well understood. The authors sought to determine if there is a bias in the evaluation of new evidence that leads to low rates of adoption of beneficial therapies compared to abandonment of harmful ones. Methods. Two case vignettes describing hypothetical clinical trials were administered to 2 independent samples of pulmonary and critical care practitioners. Each vignette was presented in 2 different ways; in one version, the results of the hypothetical trial showed that a treatment was harmful, and in the other version, the same treatment was shown to be beneficial. Prospective respondents from each sample were randomized to receive 1 version of each vignette (intersubject design). The main outcome was respondent's willingness to apply the results of the hypothetical trial to patient care. Results. There were 174 participants for trial 1 and 138 participants for trial 2 (enrollment rates of 44.2% and 41.8%, respectively). For trial 1, respondents were 2.3 times less likely to change clinical practice based on results indicating benefit as opposed to harm (33.3% v. 76.5%; P < 0.0001). Similarly, for trial 2, respondents were 2.57 times less likely to change practice when trial results showed that early use was beneficial as opposed to showing that early use was harmful (37.1% v. 95.3%; P < 0.0001). Conclusions. When evaluating clinical trials, physicians demonstrate less willingness to adopt beneficial therapies than to abandon harmful ones. This difference may contribute to the research-practice gap.

Background Pulmonary and critical care physicians routinely make complex decisions, but little is known about cognitive aspects of this process. Omission bias and status quo bias are well-descibed cognitive biases that can cause lay decision makers to prefer inaction that preserves the status quo even when changing the status quo through action is more likely to lead to the best outcomes. It is unknown if these biases influence trained decision makers such as pulmonologists. Study objectives To determine whether omission bias and status quo bias influence the medical decisions of pulmonologists. Design and interventions The study was a randomized controlled trial conducted within a cross-sectional survey of pulmonologists’ opinions about the relevance of various factors in pulmonary and critical care decision making. We designed case vignettes that presented patient information with an associated patient management choice. The status quo state and the action/omission distinction were varied in two forms of otherwise identical vignettes. One form of each case vignette pair (A and B) was administered randomly to each prospective respondent during the first mailing of the opinion survey. Participants Five hundred pulmonologists selected randomly from the membership of the American College of Chest Physicians. Measurements and results There were 125 respondents, including 59 for form A and 66 form B (enrollment rate, 25%). In vignettes involving evaluation of pulmonary embolism and treatment of septic shock, respondents were more likely to choose a suboptimal management strategy when an omission option was present that allowed preservation of the status quo (71% vs 53%, p = 0.048; 50% vs 29%, p = 0.016, respectively). In a vignette involving a hypothetical clinical trial and the decision to prescribe tube feeding, the omission option was not significantly associated with the decision to prescribe tube feeding (54% vs 50%, p = 0.67). Conclusion Pulmonary and critical care decisions are susceptible to the influence of omission and status quo bias. Because of the great number of decisions that are made each day involving choices between maintaining or changing the status quo, this finding could have far-reaching implications for patient outcomes, cost-effectiveness, resource utilization, clinical practice variability, and medical errors.

Although providing spiritual support to patients has received growing attention in the nursing and medical literature, little has been written about how to screen new patients to determine whether a more in-depth spiritual assessment is in order. In many hospitals, newly admitted patients are simply asked whether they are affiliated with a specific religious denomination. This question alone provides little insight into potential spiritual needs that may require attention. Questions that inquire about patients' religious practices and the importance of religion in their lives may be more useful as screening questions to identify the need for a more detailed spiritual assessment. As a part of a longitudinal study on decision control preferences in terminal illness, data were collected on enrollment about religious practices and the importance of religion in a group of subjects recently diagnosed with a life-threatening illness. This study examines cross-sectionally the relationship between religious practices, importance of religion, and demographic variables. Recommendations are presented on how health professionals can use the responses to these questions to determine the need for further spiritual assessment and intervention.

Pandemic influenza or other crises causing mass respiratory failure could easily overwhelm current North American critical care capacity. This threat has generated large-scale federal, state, and local efforts to prepare for a public health disaster. Few, however, have systematically engaged the public regarding which values are most important in guiding decisions about how to allocate scarce healthcare resources during such crises.The aims of this pilot study were (1) to test whether deliberative democratic methods could be used to promote engaged discussion about complex, ethically challenging healthcare-related policy issues and (2) to develop specific deliberative democratic procedures that could ultimately be used in a statewide process to inform a Maryland framework for allocating scarce healthcare resources during disasters. Using collaboratively developed focus group materials and multiple metrics for assessing outcomes, we held 5-hour pilot community meetings with a combined total of 68 community members in two locations in Maryland. The key outcomes used to assess the project were (1) the comprehensibility of the background materials and ethical principles, (2) the salience of the ethical principles, (3) the perceived usefulness of the discussions, (4) the degree to which participants' opinions evolved as a result of the discussions, and (5) the quality of participant engagement.Most participants were thoughtful, reflective, and invested in this pilot policy-informing process. Throughout the pilot process, changes were made to background materials, the verbal introduction, and pre- and post-surveys. Importantly, by holding pilot meetings in two distinct communities (an affluent suburb and inner city neighborhood), we discerned that participants' ethical reflections were framed in large part by their place-based life experiences.This pilot process, coupled with extensive feedback from participants, yielded a refined methodology suitable for wider-scale use and underscored the need for involvement of diverse communities in a statewide engagement process on this critical policy issue.

T HIS OFFICIAL STATEMENT OF THE A MERICAN T HORACIC S OCIETY WAS ADOPTED BY THE ATS B OARD OF D IRECTORS , M ARCH 1997. SUMMARY American Thoracic Society (ATS) members, as health care practitioners caring for critically ill patients in intensive care units (ICUs) or as ICU directors, commonly must make decisions that allocate ICU resources. Some decisions necessitate prioritization of ICU beds while others limit access to particular scarce resources. Although these decisions arise when providing medical care for ICU patients, they occur in a larger context that includes ethical, economic, social, and legal considerations. In this context health care systems in the United States and in other countries face the consequences of increasing demand for expensive health care services in times of limited financial resources and competing societal needs. If one accepts the premise that demand for health care resources will inevitably outstrip financial constraints, one then must face the basic question of how to allocate those resources fairly. How health care organizations will meet increasing demand for services within financial constraints raises specific questions relating to the fair allocation of ICU resources

The development of efficient DNA sequencing techniques as well as more recent advances in recombinant DNA technology have brought about a new phase in the diagnosis and treatment of pulmonary diseases. The Human Genome Project will likely identify abnormal genes that contribute to a significant number of pulmonary disorders (Table 1). This Project will provide the foundation for the development of numerous genetic tests, and it will eventually lead to the treatment of genetic diseases. As methods for genetic testing and treatment are developed and used, the complexities of decision-making by pulmonologists will increase, forcing them to face questions not previously encountered. Answering these questions will require a greater understanding of the medical and social implications of genetic disorders. In any discussion of medical genetics, it is important to realize that the scientific process generally evolves in three phases. The first is the descriptive phase, in which traits or clinical characteristics are recognized and familial associations are noted. The second phase is the information phase, in which the genes linked to particular traits or disorders are identified, enabling the development of diagnostic and prognostic genetic tests. The third phase is the development of treatment techniques based upon discoveries in the second phase. The chronologic gap between the development of diagnostic and therapeutic procedures for genetic disorders (Figure 1) creates ethical dilemmas that cannot be resolved by simply referring to the old ethics. Currently, because of the infancy of the field of gene identification, pulmonary physicians are more likely to encounter ethical questions pertaining to the information phase, including the issues of informed consent for testing, test result confidentiality, and selective abortion based on genetic information. The ethical implications of both somatic and germ-line gene therapy, though perhaps not of immediate concern to a practitioner, are likely to become increasingly important in the next several decades. Through the use of examples familiar to most pulmonologists, an effort is made to present guidelines that can be used in confronting ethical dilemmas pertaining to both genetic testing and gene therapy.

To assess the long-term clinical impact of a broad-based ethics education program for medical houseofficers with specific emphasis on appropriate care for patients who have do-not-resuscitate (DNR) orders.Prospective, with an initial randomized phase.The medical service of a university teaching hospital.Medical houseofficers and their inpatients.A pilot program in 1988, and a full program with a two-year curricular cycle from 1989 to 1991.The authors measured compliance with specific standards of care by reviewing charts of patients who had DNR orders at baseline (n = 39, 1988), after the pilot phase (n = 57, 1989), and at the end of the first curricular cycle (n = 56, 1991), noticing who wrote the DNR order, whether the reasons for the order and appropriate consent were documented, and whether there was documented attention to any of 11 concurrent care concerns (CCCs), such as spiritual needs, the appropriateness of tube feedings or pressors, and adjustment of analgesic dose. The percentage of DNR orders written by houseofficers increased from 26% in 1988 to 67% in 1991 (p0.01). The percentage of charts documenting the rationale and consent for the DNR order was consistently high. The percentage of charts documenting attention to any CCC increased from 68% in 1988 to 86% in 1991 (p0.01). The mean number of CCCs addressed per DNA order increased from 1.34 in 1988 to 2.14 in 1991. The mean number of CCCs addressed per DNR order for patients who had AIDS increased from 0.89 in 1988 to 2.25 in 1991 (p = 0.03).The quality of care for patients who had DNR orders, both overall and for those who had AIDS, improved over long-term observation in the setting of an ethics education program for medical houseofficers. The results suggest that ethics education may alter physician practices and improve patient care.

Surrogate accuracy in predicting patient treatment preferences (i.e., what patients want) has been studied extensively, but it is not known whether surrogates can predict how patients want loved ones to make end-of-life decisions on their behalf.To evaluate the ability of family members to correctly identify the preferences of seriously-ill patients regarding family involvement in decision making.Cross-sectional survey.Twenty-five pancreatic cancer and 27 amyotrophic lateral sclerosis (ALS) patients and their family members (52 dyads total).Patients and family members completed the Decision Control Preferences (DCP) scale regarding patient preferences for family involvement in health care decisions using conscious and unconscious scenarios.Patient and family member agreement was 56% (29/52 dyads) for the conscious scenario (kappa 0.29) and 46% (24/52 dyads) for the unconscious scenario (kappa 0.15). Twenty-four family members identified the patient's preference as independent in the unconscious scenario, but six of these patients actually preferred shared decision making and six preferred reliant decision making. In the conscious scenario, preference for independent decision making was associated with higher odds of patient-family agreement (AOR 5.28, 1.07-26.06). In the unconscious scenario, cancer patients had a higher odds of agreement than ALS patients (AOR 3.86; 95% CI 1.02-14.54).Family members were often unable to correctly identify patient preferences for family involvement in end-of-life decision making, especially when patients desired that decisions be made using the best-interest standard. Clinicians and family members should consider explicitly eliciting patient preferences for family involvement in decision making. Additional research is still needed to identify interventions to improve family member understanding of patient preferences regarding the decision-making process itself.

Objective:Several studies have reported high levels of distress in family members who have made health care decisions for loved ones at the end of life. A method is needed to assess the readiness of family members to take on this important role. Therefore, the purpose of this study was to develop and validate a scale to measure family member confidence in making decisions with (conscious patient scenario) and for (unconscious patient scenario) a terminally ill loved one.Methods:On the basis of a survey of family members of patients with amyotrophic lateral sclerosis (ALS) enriched by in-depth interviews guided by Self-Efficacy Theory, we developed six themes within family decision making self-efficacy. We then created items reflecting these themes that were refined by a panel of end-of-life research experts. With 30 family members of patients in an outpatient ALS and a pancreatic cancer clinic, we tested the tool for internal consistency using Cronbach's alpha and for consistency from one administration to another using the test–retest reliability assessment in a subset of 10 family members. Items with item to total scale score correlations of less than .40 were eliminated.Results:A 26-item scale with two 13-item scenarios resulted, measuring family self-efficacy in decision making for a conscious or unconscious patient with a Cronbach's alphas of .91 and .95, respectively. Test–retest reliability was r = .96, p = .002 in the conscious senario and r = .92, p = .009 in the unconscious scenario.Significance of results:The Family Decision-Making Self-Efficacy Scale is valid, reliable, and easily completed in the clinic setting. It may be used in research and clinical care to assess the confidence of family members in their ability to make decisions with or for a terminally ill loved one.

International audience; Background HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults. Objective The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease. Methods The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches. Results The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.

Summary Routine testing and prophylaxis is considered in terms of haematological disorders, biochemical testing, hormonal testing, screening for gestational diabetes and nutritional deficiencies. Within these headings the place of routine supplementation of prognant women with iron, vitamins, trace elements and an increased protein/calorie intake is discussed. Screening for haemoglobinopathies, irregular blood group antibodies and gestational diabetes is dealt with in detail. The place for routine prophylaxis with anti-D is considered. Biochemical and hormonal testing is covered with particular reference to the use of biochemical and hormonal assays as placental function tests and their use in assessing fetal well-being. In this respect the use of biochemical and hormonal tests to screen a pregnant population for intrauterine growth retardation is also discussed.

Hypocapnic constriction has been proposed as a mechanism by which collateral pathways might rapidly alter ventilation to match perfusion. We studied the changes in response to hypocapnia with age in sheep, a species with collateral resistance (Rcoll) similar to those measured in humans. Measurements of Rcoll were made with either 5 or 10% CO2 and with air (hypocapnia) in 29 anesthetized sheep, ages 6 mo to 10 yr, with the wedged bronchoscope technique. Rcoll was 0.42 +/- 0.12, 0.58 +/- 0.18, 0.32 +/- 0.18, and 0.17 +/- 0.04 (SE) cmH2O.ml-1.min in 6-mo- and 1-, 2-, and 10-yr-old animals, respectively. These values were unchanged with hypocapnia. Despite the lack of a change in Rcoll with hypocapnia, administration of histamine aerosol (8 animals) through the bronchoscope increased Rcoll by 151 +/- 35% (P less than 0.05). These data suggest that although collateral pathways exist in sheep and are capable of constriction, they do not respond to hypocapnia. Furthermore, the response to hypocapnia is not influenced by age.

To determine the role terminally ill patients would opt to have their loved ones and physicians play in healthcare decisions should they lose decision-making capacity and how this changes over time.Serial interviews.The study institutions were The Johns Hopkins Medical Institutions in Baltimore, Maryland, and St. Vincent's Hospital, in New York.One hundred forty-seven patients with cancer, amyotrophic lateral sclerosis, or heart failure, at baseline and 3 and 6 months.Patients' baseline decision control preferences varied widely, but most opted for shared decision-making, leaning slightly toward independence from their loved ones. This did not change significantly at 3 or 6 months. Fifty-seven percent opted for the same degree of decision control at 3 months as at baseline. In a generalized estimating equation model adjusted for time, more-independent decision-making was associated with college education (P=.046) and being female (P=.01), whereas more-reliant decision-making was associated with age (P.001). Patients leaned toward more reliance upon physicians to make best-interest determinations at diagnosis but opted for physicians to decide based upon their own independent wishes (substituted judgment) over time, especially if college educated.Terminally ill patients vary in how much they wish their own preferences to control decisions made on their behalf, but most would opt for shared decision-making with loved ones and physicians. Control preferences are stable over time with respect to loved ones, but as they live longer with their illnesses, patients prefer somewhat less reliance upon physicians.

Over the past third of a century, there has been a revolution in the way that health-care providers and patients make most medical decisions. Paternalism has slowly gone the way of the long-play record, and in its place has emerged a consent process in which the patient is a more fully informed and active participant. This process takes time however, and for the busy health-care provider there is often the temptation to hand the patient a consent form to sign. It is important to realize that signing a consent form does not constitute informed consent. True informed consent is a process, and, as such, it requires that the health-care provider enter into a discussion that ultimately leads to the patient understanding of their options, and the risks and benefits of the alternative courses of action. The purpose of this article was to describe, in some detail, the consent process in practical clinical terms, and to note when and how it should be obtained.

Current approaches to end-of-life decision making are widely considered inadequate. We explored these complexities by examining how patients with terminal diagnoses would choose to involve their physicians and loved ones in making medical decisions, assuming they were able and unable to participate. Cross-sectional interviews of 130 patients recently diagnosed with fatal conditions were conducted. Patients were recruited from two academic medical centers using a modification of the Decision Control Preferences Scale, ranging from independent decision making to decision making that relies upon others. Patients were asked how they would balance their own wishes relative to the input of physician and loved ones in making medical decisions, and to weigh the input of loved ones relative to physician. Most patients (52%), assuming they had the capacity, would opt to share decision making with their physicians, but 15% would defer to their physicians and 34% would make decisions independently. Similarly, 44% would share decision making with their loved ones, but fewer (6%) would defer to their loved ones. Thirty-nine percent would rely upon their physicians' judgments about what would be best for them rather than their own wishes if they became unconscious, compared with 15% who would do so if they were conscious (P < 0.001). Nonetheless, patients were more likely to weigh their loved ones' input more heavily than their physicians' input if they were unconscious (33%) than if they were conscious (7%, P = 0.05). Race, religion, gender, diagnosis, and health status were largely unassociated with patients' decision control preferences. Patients with terminal diagnoses report a wide diversity of decision control preferences, but most would opt to share decision making with their physicians and loved ones. If unable to decide for themselves, they shift toward greater reliance on physician input relative to their own wishes but would weigh loved ones' input more heavily than physician input. Deciding for patients who cannot speak for themselves may be more complex than has previously been reflected in law, policy, or clinical ethics.

Widespread disparities in US healthcare have been documented with attendant speculation about their causes, including the potential role of the physician as a healthcare decision-maker. However, the current evidence on physician decision-making is inadequate to draw firm conclusions on how it relates to healthcare inequalities. In this article, we review the available evidence on physician decision-making as it relates to healthcare disparities, with an emphasis on its shortcomings, discuss potential sources of bias, including interpersonal factors and physician preferences, and make suggestions for further research in this area.

Pergolide is an ergot-derived dopamine agonist used in Parkinson's disease and, increasingly, in restless legs syndrome. We report a patient with a 2.5-year history of weight loss, pleuropulmonary fibrosis, and exudative pleural effusion that developed insidiously while taking this medication. The extensive and invasive workup that preceded the diagnosis highlights the difficulty in attributing such a process to a drug reaction. This is the second report of such a reaction to pergolide, which is one of the increasing number of ergot-derived compounds in common clinical use.

Patients' loved ones often make end-of-life treatment decisions, but the accuracy of their substituted judgments and the factors associated with accuracy are poorly understood.To assess the accuracy of judgments made by surrogate decision makers; ascertain the beliefs, practices, and clinical and sociodemographic factors associated with accuracy of surrogates' decisions; assess the preferences of patients for life-sustaining treatments; and compare differences in accuracy across diagnoses.Cross-sectional paired interviews.Outpatient practices of three university hospitals.250 patients with terminal diagnoses of congestive heart failure, AIDS, amyotrophic lateral sclerosis, lung cancer, and chronic obstructive pulmonary disease (50 patient-surrogate pairs in each group) and 50 general medical patients and their surrogates.The accuracy of surrogate predictions was measured by using scales based on 10 potential treatments in each of three hypothetical clinical scenarios.Preferences varied according to mode of treatment and scenario. On average, surrogates made correct predictions in 66% of instances. Accuracy was better for the permanent coma scenario than for the scenarios of severe dementia or coma with a small chance of recovery (P0.001). In a binary logit model, the accuracy of substituted judgments was positively associated with the patient having spoken with the surrogate about end-of-life issues (odds ratio [OR], 1.9 [95% CI, 1.6 to 2.3]), the patient having private insurance (OR, 1.4 [CI, 1.1 to 1.7]), the surrogate's level of education (OR, 1.5 [CI, 1.2 to 1.9]), and the patient's level of education (OR, 1.7 [CI, 1.4 to 2.2]). Accuracy was negatively associated with the patient's belief that he or she would live longer than 10 years (OR, 0.6 [CI, 0.5 to 0.7]), surrogate experience with life-sustaining treatment (OR, 0.4 [CI, 0.3 to 0.5]), surrogate participation in religious services (OR, 0.67 [CI, 0.50 to 0.91]), and a diagnosis of heart failure (OR, 0.6 [CI, 0.5 to 0.8]). Age, ethnicity, marital status, religion, and advance directives were not associated with accuracy.The accuracy of substituted judgments is associated with multiple clinically apparent patient and surrogate factors. This information can help clinicians identify conditions under which substituted judgments are likely to be accurate or inaccurate and can help target populations for education designed to improve the accuracy of surrogate decision making.