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Sulfasalazine-induced hypersensitivity syndrome (SIHS) is a serious systemic delayed adverse drug reaction that is associated with significant morbidity and mortality. Here, we report the first case, to our knowledge, of a patient with previously unidentified SIHS who developed a significantly more rapid and extreme recurrence on re-exposure to sulfasalazine. The patient is a 58-year-old woman with asymptomatic Crohn’s disease who, 10 days after initiating sulfasalazine, developed fevers, diffuse rash, pancytopenia, hypotension and hepatitis without a definitive source of infection. Sixteen days after her first hospitalisation, she was restarted on sulfasalazine and was readmitted within 10 hours with a similar but more serious presentation, requiring vasopressors. She did recover completely without any further recurrence to date, after definitively discontinuing sulfasalazine. This case demonstrates the importance of recognising SIHS early in patients to prevent re-exposure to sulfasalazine and to ensure timely initiation of appropriate treatment.

Therapeutic hypothermia (ARCTIC, or Advanced Resuscitation Cooling Therapeutics and Intensive Care protocol) is a widely recommended intervention to improve mortality and neurologic outcomes after cardiac arrest. However, neurologic outcomes are difficult to predict soon after cardiac arrest in the setting of hypothermia, as illustrated by this case report. A 60-year-old man had witnessed cardiac arrest at home. He was defibrillated twice, with return of spontaneous circulation, and cooled to 33°C for 24 hours. Neurologic exam on Day 6 revealed limited brainstem reflexes, and the intensive care unit team discussed with the patient's family that his prognosis for neurologic recovery was poor. Palliative care was consulted to participate in a goals-of-care meeting. Just prior to the meeting on Day 7, the patient awoke. He fully recovered and walked out of the hospital on Day 18. Prior to induced hypothermia, indicators of poor outcome included lack of one or more brainstem reflexes (pupillary or corneal reflex), absence of motor response at 72 hours, myoclonus, status epilepticus, electroencephalogram with generalized suppression, and absent bilateral cortical N20 response to somatosensory-evoked potentials. However, several studies have found these indicators to be unreliable after hypothermia. This may be the result of sedatives, which can affect physical examination and electroencephalogram results, and delayed clearance. Because of the unreliability of prognostication tests within the first 72 hours of hypothermic protocols in the setting of sedation, it appears prudent in some cases to delay final prognosis discussions until at least six days postcardiac arrest and after neurologic evaluation is done with patients sedative-free.

Purpose Many seriously ill patients with cancer do not discuss prognosis or advance directives (ADs), which may lead to inappropriate and/or unwanted aggressive care at the end of life. Ten years ago, patients with cancer said they would not like to discuss ADs with their oncologist but would be willing to discuss them with an admitting physician. We assessed whether this point of view still held. Patients and Methods Semi-structured interviews were conducted with 75 consecutively admitted patients with cancer in the cancer inpatient service. Results Of those enrolled, 41% (31 of 75) had an AD. Nearly all (87%, 65 of 75) thought it acceptable to discuss ADs with the admitting physician with whom they had no prior relationship, and 95% (62 of 65) thought that discussing AD issues was very or somewhat important. Only 7% (5 of 75) had discussed ADs with their oncologist, and only 23% (16 of 70) would like to discuss ADs with their oncologist. When specifically asked which physician they would choose, 48% (36 of 75) of patients would prefer their oncologist, and 35% (26 of 75) would prefer their primary care physician. Conclusion Fewer than half of seriously ill patients with cancer admitted to an oncology service have an AD. Only 23% (16 of 70) would like to discuss their ADs with their oncologist but nearly all supported a policy of discussing ADs with their admitting physician. However, fully 48% (36 of 75) actually preferred to discuss advance directives with their oncologist if AD discussion was necessary. We must educate patients on why communicating their ADs is beneficial and train primary care physicians, house staff, hospitalists, and oncologists to initiate these difficult discussions.

Existential suffering in patients with serious illness significantly impacts quality of life, yet it remains a challenge to define, assess, and manage adequately. Improving upon understanding and practice in the existential domain is a topic of interest for palliative care providers.As a quality improvement project, our palliative care team created an existential assessment tool utilizing a dialogue-oriented approach with four questions designed to identify sources of existential distress as well as strengths and challenges in coping with this distress. The tool utilized the mnemonic CASH, with each letter representing the core objective of the question. Providers who requested the palliative care consult were asked to evaluate the CASH assessment. On completion of the project, palliative care consultants evaluated the appropriateness of the CASH assessment tool.Patient responses to the CASH questions were insightful and reflected their beliefs, priorities, and concerns. Eight of nine providers found that the assessment enabled understanding of their patient. Seven noted a positive impact on their practice, and five reported an improvement in patient care after the assessment. The palliative care consultants who used the tool enjoyed using it, and half of them suggested changes to patient care based on their assessment. The most common reasons for not using the CASH assessment were inappropriateness to the consult, lack of perceived patient/caregiver receptiveness, or consultation service too busy.Our quality improvement project demonstrated that the CASH assessment tool is useful in ascertaining existential concerns of patients with serious illness. It enhances patient care by the primary team as well as the palliative care team. As a brief set of questions with an easy-to-remember mnemonic, the CASH assessment tool is feasible for a busy palliative consult service. Furthermore, the positive results of this project merit more rigorous evaluation of the CASH assessment tool in the future.

Octreotide, a synthetic analogue of the hormone somatostatin, is primarily used in palliative medicine because of its antisecretory effect and has been shown to be effective in the management of bowel obstruction, nausea, and diarrhea. Octreotide also has been successfully used for the management of bronchorrhea in both inpatient and outpatient settings. We report the case of a 47-year-old female with a history of bronchioloalveolar cell carcinoma whose copious bronchial secretions were controlled with octreotide. Octreotide should be further evaluated as a first-line treatment for bronchorrhea.

Pulmonary arterial hypertension (PAH) is a progressive and ultimately fatal disease of the pulmonary circulation. There has never been an investigation of the end-of-life symptoms in patients with PAH. In this investigation, we surveyed surrogates of recent decedents with PAH. We evaluate their responses to better understand the end-of-life experience of patients with PAH.The survey instrument includes demographic information and the Edmonton Symptoms Assessment Scale. Accredo Therapeutics mailed the survey to surrogates of recent decedents with PAH, and responses were anonymously returned to investigators at Virginia Commonwealth University and used in our descriptive analysis.Of 100 surveys distributed over 24 months (February 2009 to February 2011), we obtained 36 responses (response rate 36%). We found that most patient deaths (90%) were related to PAH, that the majority of patients died in the hospital (67%), with the majority of in-hospital deaths (83%) occurring in intensive care. Palliative care was infrequently involved in patients' care, and many surrogates were unaware of palliative care and hospice services available to the decedents. Patients died with a high symptom burden, especially dyspnea.In this cohort, patients with PAH usually died from their disease, often in the hospital setting with a high symptom burden. Further study will be needed to confirm the findings from this study and to better understand the forces leading to the trends uncovered in this investigation.

Abnormalities in taste and smell are commonly reported in patients receiving chemotherapy and may hinder appetite, dietary intake, nutritional well-being, and quality of life. Oral zinc has been used to treat taste and smell abnormalities in several altered physiologic states, including renal failure, liver disease, head trauma, and pregnancy, with varying results. The authors conducted a double-blinded, placebo-controlled randomized clinic trial over 3 months. Eligible patients were those taking chemotherapy that had alterations in taste and/or smell. The measurement of the primary end point, improvement in altered taste and smell, was made using a 0–100 scale (100 describing no loss or distortion in taste and smell, and 0 describing the worst distortion or loss of taste and smell). Twenty-nine subjects were enrolled in each treatment group, of whom 31 were white, 26 African American, and 1 Native American. Forty-one patients were female. A wide range of cancer types was represented, with breast the most common (21 patients). The zinc dose was 220 mg orally twice daily (equivalent of 50 mg elemental zinc twice daily). There was no statistically significant improvement in loss or distortion of taste or smell with the addition of zinc. There was a trend toward improvement over time in all groups, except in the zinc group where there was a nonsignificant worsening in loss of smell over time. Zinc at standard doses did not provide significant benefit to taste or smell in patients receiving chemotherapy.

Donation after cardiac death (DCD) or donation of organs after removal of life support is an accepted means of organ retrieval that usually occurs in the setting of sudden illness but has not been described in people with progressive neurologic illness. We report DCD in two people with progressive amyotrophic lateral sclerosis (ALS).Case series at an academic medical center of two men with progressive ALS who underwent withdrawal of artificial life support, rapid cardiac death, and subsequent organ donation. The primary outcome was donation of organs in concordance with patient and family wishes.Both patients underwent peaceful withdrawal of life support in the presence of family, and multiple organs were donated.Patients may legally and ethically refuse life-sustaining care. These patients considered their lives to be more burdensome than beneficial near the end of their lives, both carefully planned the time and circumstance of their deaths, and both fulfilled a long-standing desire to donate their organs. This study describes a potential opportunity for patients with progressive neurologic illness.

Most cancer patients do not have an explicit discussion about prognosis and treatment despite documented adverse outcomes. Few decision aids have been developed to assist the difficult discussions of palliative management. We developed decision aids for people with advanced incurable breast, colorectal, lung, and hormone-refractory prostate cancers facing first-, second-, third-, and fourth-line chemotherapy. We recruited patients from our urban oncology clinic after gaining the permission of their treating oncologist. We measured knowledge of curability and treatment benefit before and after the intervention. Twenty-six of 27 (96%) patients completed the aids, with a mean age of 63, 56% female, 56% married, 56% African American, and 67% with a high school education or more. Most patients (14/27, 52%) thought a person with their advanced cancer could be cured, which was reduced (to 8/26, 31%, P = 0.15) after the decision aid. Nearly all overestimated the effect of palliative chemotherapy. No distress was noted, and hope did not change. The majority (20/27, 74%) found the information helpful to them, and almost all (25/27, 93%) wanted to share the information with their family and physicians. It is possible to give incurable patients their prognosis, treatment options, and options for improving end-of-life care without causing distress or lack of hope. Almost all find the information helpful and want to share it with doctors and family. Research is needed to test the findings in a larger sample and measure the outcomes of truthful information on quality of life, quality of care, and costs.

Oncologists often do not give honest prognostic and treatment-effect information to patients with advanced disease. One of the primary reasons stated for witholding this information is to "not take away hope." We could find no study that tested if hope was influenced by honest clinical information.We tested decision-aids in 27 patients with advanced cancer who were facing first-, second-, third-, and fourth-line chemotherapy. These aids had printed estimates of treatment effect and the patient's chance of survival and being cured (always zero). We measured hope using the Herth Hope Index, which ranks patients' responses to 12 questions and yields a maximum score of 48.The scores on the Herth Hope Index did not change and the patients remained uniformly hopeful about their future. The pretest score was 44.2 (SD 3.9), and it increased to 44.8 (SD 3.86; P = .55 by paired Student's t-test).Hope is maintained when patients with advanced cancer are given truthful prognostic and treatment information, even when the news is bad.