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Rationale: Pulmonary lymphangioleiomyomatosis is a progressive cystic lung disease that is associated with infiltration of atypical smooth muscle-like cells. Previous descriptions of clinical characteristics of subjects with lymphangioleiomyomatosis have been based on a limited number of patients.Objectives: To describe the clinical characteristics of subjects with pulmonary lymphangioleiomyomatosis, both sporadic and tuberous sclerosis-related forms.Methods: Over a 3-yr period, from 1998 to 2001, 243 subjects with pulmonary lymphangioleiomyomatosis were enrolled into a national registry; 13 subjects who had already undergone lung transplantation were excluded for the purposes of this report.Measurements and Main Results: All 230 subjects were women, aged 18 to 76 yr (mean +/- SE, 44.5 +/- 0.65 yr). The average age at onset of symptoms was 38.9 +/- 0.73 yr and at diagnosis was 41.0 +/- 0.65 yr. Tuberous sclerosis complex was present in 14.8% of subjects. Pulmonary manifestations, most commonly spontaneous pneumothorax, were the primary events leading to the diagnosis in 86.5% of cases. Nearly 55% of the subjects were being treated with a progesterone derivative. An obstructive pattern on pulmonary function testing was observed in 57.3% of the subjects, whereas 33.9% had normal spirometric results. Women with tuberous sclerosis-related lymphangioleiomyomatosis were younger and had less impaired lung function compared with those with the sporadic form.Conclusions: The age range of women afflicted with pulmonary lymphangioleiomyomatosis is broader than previously appreciated and the degree of pulmonary function can be quite variable, with one-third of subjects having normal spirometry at enrollment into this registry. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To determine whether the dose of narcotics and benzodiazepines is associated with length of time from mechanical ventilation withdrawal to death in the setting of withdrawal of life-sustaining treatment in the ICU. DESIGN: Retrospective chart review. SETTING: University-affiliated, level I trauma center. PATIENTS: Consecutive critically ill patients who had mechanical ventilation withdrawn and subsequently died in the ICU during two study time periods. RESULTS: There were 75 eligible patients with a mean age of 59 years. The primary ICU admission diagnoses included intracranial hemorrhage (37%), trauma (27%), acute respiratory failure (27%), and acute renal failure (20%). Patients died during a median of 35 min (range, 1 to 890 min) after ventilator withdrawal. On average, 16.2 mg/h opiates in morphine equivalents and 7.5 mg/h benzodiazepine in lorazepam equivalents were administered during the time period starting 1 h before ventilator withdrawal and ending at death. There was no statistically significant relationship between the average hourly narcotic and benzodiazepine use during the 1-h period prior to ventilator withdrawal until death, and the time from ventilator withdrawal to death. The restriction of medication assessment in the last 2 h of life showed an inverse association between the use of benzodiazepines and time to death. For every 1 mg/h increase in benzodiazepine use, time to death was increased by 13 min (p = 0.015). There was no relationship between narcotic dose and time to death during the last 2 h of life (p = 0.11). CONCLUSIONS: We found no evidence that the use of narcotics or benzodiazepines to treat discomfort after the withdrawal of life support hastens death in critically ill patients at our center. Clinicians should strive to control patient symptoms in this setting and should document the rationale for escalating drug doses. [ABSTRACT FROM AUTHOR]

Background: No studies have assessed whether clinicians obtain informed consent for invasive medical procedures, and there are no explicit national standards to guide the process.Hypothesis: Informed consent practices are inconsistent for commonly performed invasive medical procedures.Methods: A simple questionnaire was electronically mailed and/or faxed to training program directors of critical care medicine and internal medicine departments, and to ICU directors in the state of Connecticut. The questionnaire listed common invasive medical procedures and asked the respondents to check those for which practitioners routinely obtain informed consent in their hospital.Results: The three samples, national intensivists (88 respondents), Connecticut intensivists (29 respondents), and national internists (56 respondents) demonstrated heterogeneity of consenting practices. The rate of obtaining consent for common vascular access procedures ranged from 20 to 90%. The rates of obtaining consent for Foley catheterization and nasogastric intubation were uniformly < 10%, and those for endoscopic procedures were > 90%. Separate consent (beyond the general consent to treat) was not uniformly obtained for the transfusion of blood products (range, 74 to 93%) and common diagnostic medical procedures (eg, thoracentesis, paracentesis, or lumbar puncture; range, 77 to 96%). Surgical intensivists reported that the obtaining of consent for invasive procedures was less routine compared to medical intensivists. Lower rates of consent were noted by those respondents who used a "blanket" consent form, which was signed at the time of hospital admission, to cover subsequent procedures.Conclusions: In this relatively small sample, there was no uniform practice of informed consent for commonly performed invasive medical procedures. Consent was routinely obtained for GI endoscopy, bronchoscopy, and medical research, and was not obtained for Foley catheterization and nasogastric intubation. The obtaining of consent for vascular cannulation and diagnostic procedures was not routine in the ICUs of a substantial number of respondents. Explicit standards that delineate specifically which procedures require consent may be required to assure more uniform practices. [ABSTRACT FROM AUTHOR]

Objective: To describe the perspectives of family members to the care provided to critically ill patients who died in the ICU.Design: Multicenter, prospective, observational study.Setting: Six university-affiliated ICUs across Canada.Methods: Patients who received mechanical ventilation for > 48 h and who died in the ICU were eligible for this study. Three to four weeks after the patient's death, we mailed a validated questionnaire to one selected family member who made at least one visit to the patient in the ICU. We obtained self-rated levels of satisfaction with key aspects of end-of-life care, communication, and decision making, and the overall ICU experience.Main Results: Questionnaires were mailed to 413 family members; 256 completed surveys were returned (response rate, 62.0%). In the final hours before the death of the patient, family members reported that patients were "totally comfortable" (34.8%), "very comfortable" (23.8%), or "mostly comfortable" (32.0%). Family members felt "very supported" (57.0%) and "supported" (30.7%) by the health-care team. Most (82.0%) believed that the patient's life was neither prolonged nor shortened unnecessarily. Most family members (90.4%) preferred some form of shared decision making. Overall, 52% of families rated their satisfaction with care as "excellent," 31% rated care as "very good," 10% as "good," 4% as "fair," and 2% as "poor." Overall satisfaction with end-of-life care was significantly associated with completeness of information received by the family member, respect and compassion shown to patient and family member, and satisfaction with amount or level of health care received.Conclusions: The majority of families of patients who died in participating ICUs were satisfied with the end-of-life care provided. Adequate communication, good decision making, and respect and compassion shown to both the dying patient and their family are key determinants to family satisfaction. [ABSTRACT FROM AUTHOR]

Lymphoid interstitial pneumonia (LIP) is regarded as both a disease and a nonneoplastic, inflammatory pulmonary reaction to various external stimuli or systemic diseases. It is an uncommon condition with incidence and prevalence rates that are largely unknown. Liebow and Carrington originally classified LIP as an idiopathic interstitial pneumonia in 1969. Although LIP had since been removed from that category, the most recent consensus classification sponsored by the American Thoracic Society and the European Respiratory Society recognizes that some cases remain idiopathic in origin, and its clinical, radiographic, and pathologic features warrant the return of LIP to its original classification among the idiopathic interstitial pneumonias. LIP also belongs within a spectrum of pulmonary lymphoproliferative disorders that range in severity from benign, small, airway-centered cellular aggregates to malignant lymphomas. It is characterized by diffuse hyperplasia of bronchus-associated lymphoid tissue. The dominant microscopic feature of LIP is a diffuse, polyclonal lymphoid cell infiltrate surrounding airways and expanding the lung interstitium. Classically, LIP occurs in association with autoimmune diseases, most often Sjögren syndrome. This has led to consideration of an autoimmune etiology for LIP, but its pathogenesis remains poorly understood. Persons who are seropositive for HIV, and children in particular, are at increased risk of acquiring LIP. Some studies suggest causal roles for both HIV and Epstein-Barr virus. The incidence of LIP is approximately twofold greater in women than men. The average age at diagnosis is between 52 years and 56 years. Symptoms of progressive cough and dyspnea predominate. There is great variability in the clinical course of LIP, from resolution without treatment to progressive respiratory failure and death. Although LIP is often regarded as a steroid-responsive condition, and oral corticosteroids continue to be the mainstay of therapy, response is unpredictable. Approximately 33 to 50% of patients die within 5 years of diagnosis, and approximately 5% of cases of LIP transform to lymphoma. [ABSTRACT FROM AUTHOR]

Objectives: Patients' views of physician skill in providing end-of-life care may vary across different diseases, and understanding these differences will help physicians improve the quality of care they provide for patients at the end of life. The objective of this study was to examine the perspectives of patients with COPD, cancer, or AIDS regarding important aspects of physician skill in providing end-of-life care.Design: Qualitative study using focus groups and content analysis based on grounded theory.Setting: Outpatients from multiple medical settings in Seattle, WA.Patients: Eleven focus groups of 79 patients with three diseases: COPD (n = 24), AIDS (n = 36), or cancer (n = 19).Results: We identified, from the perspectives of patients, the important physician skills for high-quality end-of-life care. Remarkable similarities were found in the perspectives of patients with COPD, AIDS, and cancer, including the importance of emotional support, communication, and accessibility and continuity. However, each disease group identified a unique theme that was qualitatively more important to that group. For patients with COPD, the domain concerning physicians' ability to provide patient education stood out as qualitatively and quantitatively more important. Patients with COPD desired patient education in five content areas: diagnosis and disease process, treatment, prognosis, what dying might be like, and advance care planning. For patients with AIDS, the unique theme was pain control; for patients with cancer, the unique theme was maintaining hope despite a terminal diagnosis.Conclusions: Patients with COPD, AIDS, and cancer demonstrated many similarities in their perspectives on important areas of physician skill in providing end-of-life care, but patients with each disease identified a specific area of end-of-life care that was uniquely important to them. Physicians and educators should target patients with COPD for efforts to improve patient education about their disease and about end-of-life care, especially in the areas defined above. Physicians caring for patients with advanced AIDS should discuss pain control at the end of life, and physicians caring for patients with cancer should be aware of many patients' desires to maintain hope. Physician understanding of these differences will provide insights that allow improvement in the quality of care. [ABSTRACT FROM AUTHOR]

Study Objective: Opinions regarding do-not-resuscitate (DNR) decisions differ between individual physicians. We attempted to determine whether the strength of DNR recommendations varies with medical specialty and experience.Design: Written survey.Participants: Physicians from the pulmonary/critical-care medicine (PCCM), cardiology, internal medicine, gastroenterology, hematology/oncology, and infectious disease services as well as the Department of Medicine house staff at our tertiary-care referral center participated in the study.Interventions: Physicians were asked confidentially to quantify the strength of their opinions on discussing and recommending DNR orders for each of 20 vignettes made from the summaries of actual cases. Reasons for their opinions and demographic data also were recorded.Measurements and Results: One hundred fifteen of 155 physicians (74%) responded. PCCM physicians (mean [+/- SD] DNR score, 157 +/- 22) more strongly recommended DNR orders than cardiologists (mean DNR score, 122 +/- 32; p = 0.006), house staff (mean DNR score, 132 +/- 24; p = 0.014), and general internists (mean DNR score, 129 +/- 30; p = 0.043). PCCM physicians also trended toward recommending DNR orders for more of the 20 patients described in the vignettes compared to cardiologists (mean DNR number, 16.5 +/- 3.0 vs 11.9 +/- 5.8, respectively; p = 0.066). There were no differences between PCCM physicians and hematology/oncology, infectious disease, and gastroenterology specialists. Among the house staff, the likelihood of recommending a DNR order correlated significantly with increasing years of experience (r = 0.45; p = 0.002). The opposite trend was present in the specialty staff groups. No significant differences in opinion by gender, religion, or personal experiences were found.Conclusions: The strength of DNR order recommendations varies with medicine specialty and years of training and experience. An awareness of these differences and the determination of the reasons behind them may help to target educational interventions and to ensure effective collaboration with colleagues and communication with patients. [ABSTRACT FROM AUTHOR]

Study Objectives: Although long-term ventilation (LTV) has been shown to extend the lives of individuals with Duchenne muscular dystrophy (DMD), initiating LTV is still considered controversial. The purpose of the study was to describe the LTV-related attitudes and practices of Canadian physicians who follow up patients with DMD.Design: The study consisted of a mail questionnaire supplemented by face-to-face interviews.Participants: Forty-five physicians who follow up patients with DMD through Canadian neuromuscular clinics.Measurements: A mail questionnaire of 66 closed-ended questions related to practice and attitudes was completed by all respondents. Qualitative semistructured interviews were conducted with six volunteer physicians, and were audiotaped and transcribed.Results: The results indicated that 25.0% of physicians do not discuss LTV with all of their DMD patients. The most frequently cited reason for advising against LTV was poor patient quality of life (52.6%). Three themes emerged from the qualitative data: mentioning and discussing LTV are discrete events with different purposes, nighttime and full-time LTV decisions are approached differently, and physicians modify their discussions to influence outcome.Conclusions: The study demonstrated considerable agreement among the physicians regarding disclosure practices. Concerns are raised by the number of physicians who do not disclose to all patients and families and the role of quality-of-life judgments in decision making. It is suggested that because of their subjective nature, quality-of-life judgments should not be made without the participation of the patient and family, and that an initial disclosure is the minimum requirement of informed consent/decision making. [ABSTRACT FROM AUTHOR]

Background: Patient-centered decision making, which in the United States is typically considered to be appropriate, may not be universally endorsed, thereby harboring the potential to complicate the care of patients from other cultural backgrounds in potentially unrecognized ways. This study compares the attitudes toward ethical decision making and autonomy issues among academic and community physicians and patients of medical center outpatient clinics in Japan and the United States.Methods: A questionnaire requesting judgments about seven clinical vignettes was distributed (in English or Japanese) to sample groups of Japanese physicians (n = 400) and patients (n = 65) as well as US physicians (n = 120) and patients (n = 60) that were selected randomly from academic institutions and community settings in Japan (Tokyo and the surrounding area) and the United States (the Stanford/Palo Alto, CA, area). Responses were obtained from 273 Japanese physicians (68%), 58 Japanese patients (89%), 98 US physicians (82%), and 55 US patients (92%). Physician and patient sample groups were compared on individual items, and composite scores were derived from subsets of items relevant to patient autonomy, family authority, and physician authority.Results: A majority of both US physicians and patients, but only a minority of Japanese physicians and patients, agreed that a patient should be informed of an incurable cancer diagnosis before their family is informed and that a terminally ill patient wishing to die immediately should not be ventilated, even if both the doctor and the patient's family want the patient ventilated (Japanese physicians and patients vs US physicians and patients, p < 0.001). A majority of respondents in both Japanese sample groups, but only a minority in both US sample groups, agreed that a patient's family should be informed of an incurable cancer diagnosis before the patient is informed and that the family of an HIV-positive patient should be informed of this disease status despite the patient's opposition to such disclosure (Japanese physicians and patients vs US physicians and patients, p < 0.001). Physicians in both Japan and the United States were less likely than patients in their respective countries to agree with physician assistance in the suicide of a terminally ill patient (Japanese physicians and patients vs US physicians and patients, p < 0.05). Across various clinical scenarios, all four respondent groups accorded greatest authority to the patient, less to the family, and still less to the physician when the views of these persons conflicted. Japanese physicians and patients, however, relied more on family and physician authority and placed less emphasis on patient autonomy than the US physicians and patients sampled. Younger respondents placed less emphasis on family and physician authority.Conclusions: Family and physician opinions are accorded a larger role in clinical decision making by the Japanese physicians and patients sampled than by those in the United States, although both cultures place a greater emphasis on patient preferences than on the preferences of the family or physician. Our results are consistent with the view that cultural context shapes the relationship of the patient, the physician, and the patient's family in medical decision making. The results emphasize the need for clinicians to be aware of these issues that may affect patient and family responses in different clinical situations, potentially affecting patient satisfaction and compliance with therapy. [ABSTRACT FROM AUTHOR]