Palliative Cytoreductive Surgery With or Without Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Carcinomatosis: Is It Safe and Effective?

INTRODUCTION: Palliation is a controversial indication for cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Patients with peritoneal carcinomatosis (PC) are living longer, and the roles of palliative CRS and HIPEC are increasingly challenged. The purpose of this study is to evaluate indications, morbidity, and symptom improvement from CRS/HIPEC in advanced PC. METHODS: A retrospective review of patients undergoing CRS and/or HIPEC with a palliative intent at a single institution from February 2008 to February 2018 was performed. Main end points included symptom improvement, symptom-free interval, and overall survival. RESULTS: Two hundred and seventy seven patients were referred for CRS/HIPEC during the study period and 17 underwent 20 palliative procedures. Appendiceal (n = 6) and colorectal cancers (n = 6) were the most common malignancies. Ascites (n = 8) and bowel obstruction (n = 8) were the most common indications for intervention. The postoperative complication rate was 50% and major complication rate was 20%. Partial symptom improvement or resolution of symptoms was achieved in 18 (90%) cases. A durable symptom control at 90 d was achieved in 13 (65%) cases. The median time to symptom recurrence was 5.1 mo (interquartile range: 2-11.4), and the median overall survival was 11.6 mo (interquartile range: 3.8-28.5). CONCLUSIONS: Palliative CRS and/or HIPEC achieve symptom improvement in patients with advanced PC. Risk assessment and expected time to recovery from surgery remain paramount for patient selection.

Evaluating the Outcomes of an Organizational Initiative to Expand End-of-Life Resources in Intensive Care Units With Palliative Support Tools and Floating Hospice.

BACKGROUND: There is evidence that palliative care and floating (inpatient) hospice can improve end-of-life experiences for patients and their families in the intensive care unit (ICU). However, both palliative care and hospice remain underutilized in the ICU setting. OBJECTIVES: This study examined palliative consultations and floating hospice referrals for ICU patients during a phased launch of floating hospice, 2 palliative order sets, and general education to support implementation of palliative care guidelines. METHODS: This descriptive, retrospective study was conducted at a level I trauma and academic medical center. Electronic medical records of 400 ICU patients who died in the hospital were randomly selected. These electronic medical records were reviewed to determine if patients received a palliative consult and/or a floating hospice referral, as well as whether the new palliative support tools were used during the course of care. The numbers of floating hospice referrals and palliative consults were measured over time. RESULTS: Although not significant, palliative consults increased over time (P = .055). After the initial introduction of floating hospice, 27% of the patients received referrals; however, referrals did not significantly increase over time (P = .807). Of the 68 patients who received a floating hospice referral (24%), only 38 were discharged to floating hospice. There was a trend toward earlier palliative care consults, although this was not statistically significant (P = .285). CONCLUSION: This study provided the organization with vital information about their initiative to expand end-of-life resources. Utilization and timing of palliative consults and floating hospice referrals were lower and later than expected, highlighting the importance of developing purposeful strategies beyond education to address ICU cultural and structural barriers.

Advance directives and intensity of care delivered to hospitalized older adults at the end-of-life.

BACKGROUND: Older adults prefer comfort over life-sustaining care. Decreased intensity of care is associated with improved quality of life at the end-of-life (EOL). OBJECTIVES: This study explored the association between advance directives (ADs) and intensity of care in the acute care setting at the EOL for older adults. METHODS: A retrospective, correlational study of older adult decedents (N = 496) was conducted at an academic medical center. Regression analyses explored the association between ADs and intensity of care. RESULTS: Advance directives were not independently predictive of aggressive care but were independently associated with referrals to palliative care and hospice; however, effect sizes were small, and the timing of referrals was late. CONCLUSION: The ineffectiveness of ADs to reduce aggressive care or promote timely referrals to palliative and hospice services, emphasizes persistent inadequacies related to EOL care. Research is needed to understand if this failure is provider-driven or a flaw in the documents themselves.

Clinical features related to statin-associated muscle symptoms.

INTRODUCTION: Statins reduce cardiovascular disease risk and are generally well tolerated, yet up to 0.5% of statin-treated patients develop incapacitating muscle symptoms including rhabdomyolysis. Our objective was to identify clinical factors related to statin-associated muscle symptoms (SAMS). METHODS: Clinical and laboratory characteristics were evaluated in 748 statin-treated Caucasians (634 with SAMS and 114 statin-tolerant controls). Information was collected on statin type, concomitant drug therapies, muscle symptom history, comorbidities, and family history. Logistic regression was used to identify associations. RESULTS: Individuals with SAMS were 3.6 times (odds ratio [OR] 3.60, 95% confidence interval [CI] 2.08-6.22) more likely than statin-tolerant controls to have a family history of heart disease. Additional associations included obesity (OR 3.08, 95% CI 1.18, 8.05), hypertension (OR 2.24, 95% CI 1.33, 3.77), smoking (OR 2.08, 95% CI 1.16, 3.74), and statin type. DISCUSSION: Careful medical monitoring of statin-treated patients with the associated coexisting conditions may ultimately reduce muscle symptoms and lead to improved compliance. Muscle Nerve 59:537-537, 2019.

Treat the Patient, Not the Test When a Hospitalized Patient in Status Epilepticus Transitions to Comfort-Focused Goals of Care.

Status epilepticus is a common and under-recognized cause of unconsciousness among hospitalized patients. It can clinically mimic delirium and other causes of acute mental status change, especially when clinically relevant seizure activity is not appreciated on physical examination. While the successful treatment of status epilepticus may require anesthetic dosing of antiepileptics such as barbiturates, these seemingly drastic therapeutic measures are justified when goals of care are life prolonging as they may allow a patient to regain consciousness and meaningfully interact with loved ones. However, medical burden from electroencephalogram (EEG) monitoring and other care associated with its diagnosis and treatment can contribute to distress for patients who may be dying from a comorbid illness. Furthermore, when goals of care transition to comfort, care challenges can result regarding the ongoing management of barbiturates or other sedatives, previously prescribed to treat the status epilepticus. In this case study, the lack of clinically significant seizure activity led us to conclude that the discontinuation of a barbiturate infusion and the EEG monitoring was the clinically appropriate approach for a dying patient with comfort goals of care and nonconvulsive status epilepticus.

Palliative Care Training in Cardiology Fellowship: A National Survey of the Fellows.

OBJECTIVES: To address perspectives of cardiology fellows on the current state of palliative education and palliative and hospice resource utilization within their fellowship experiences. METHODS: We conducted an online national survey of cardiology fellows during the 2015 to 2016 academic year. Survey questions aimed to assess perceived importance of palliative care education, level of palliative care education during fellowship, and the structure of palliative care support at respondent institutions. Responses were collected anonymously. A total of 519 programs, including subspecialty programs, were contacted. RESULTS: We received 365 responses, a number that represents roughly 14% of all cardiology fellows nationwide during the 2015 to 2016 academic year. Fellows reported discordance in the quality of education between general cardiology and palliative care principles as it relates to care of the patient approaching the end of life. Fellows infrequently received explicit training nor were observed or mentored in delivering end-of-life discussions. Respondents reported an underutilization of palliative care and hospice resources during fellowship training and also a perception that attending faculty were not routinely addressing goals of care. CONCLUSIONS: Our survey results highlight a need for enhanced palliative care and end-of-life training experiences for cardiology fellows and also suggest underutilization of hospice and palliative care resources for patients with advanced cardiac diseases. These findings create a platform for future work that might: (1) confirm this training deficit, (2) lead to exploration of educational models that could reconcile this deficit, and (3) potentially help improve palliative care support for patients and families facing advanced heart disease.

Factors associated with palliative care use in patients undergoing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

BACKGROUND: Peritoneal carcinomatosis represents widespread metastatic disease throughout the abdomen and/or pelvis. Cytoreductive surgery/hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) improves the overall survival compared to standard therapy alone. The role palliative care (PC) plays however, remains poorly studied among these patients. METHODS: Patients who had previously undergone HIPEC and who underwent an inpatient admission from 7/1/2013 to 6/30/2014 were identified to determine which patients were referred for inpatient or outpatient palliative consultation. Multivariable logistic regression analysis was performed to identify risk factors associated with the use of PC. RESULTS: Of the 60 patients analyzed, 23 (38.3%) had a PC consultation with a median time to PC referral of 310 (IQR: 151-484 days). Patients who were prescribed opioids (no PC referral versus PC referral: 46.0% versus 91.3%, P < 0.001), patients who reported the use of a cancer-related emetic (35.1% versus 87.0%, P < 0.001), patients reporting the use of total parenteral nutrition (16.2% versus 39.1%, P = 0.046), and patients dependent on a gastric tube for nutrition (5.4% versus 43.5%, P < 0.001) were more likely to be referred to a PC consultation. On multivariable analysis, use of opioids, use of a cancer-related antiemetic, and the use of a G-tube were independently associated with a greater odds for being referred to PC (all P < 0.05). CONCLUSIONS: Approximately one-third of patients were referred to PC following cytoreductive surgery/hyperthermic intraperitoneal chemotherapy. Palliative care referrals were most commonly used for patients with chronic symptoms, which are difficult to manage, especially toward the end of life.

Initial Experience with "Honoring Choices Wisconsin": Implementation of an Advance Care Planning Pilot in a Tertiary Care Setting.

BACKGROUND: Although previous research on advance care planning (ACP) has associated ACP with improved quality of care at the end of life, the appropriate use of ACP remains limited. OBJECTIVE: To evaluate the impact of a pilot program using the "Honoring Choices Wisconsin" (HCW) model for ACP in a tertiary care setting, and to understand barriers to system-wide implementation. DESIGN: Retrospective review of prospectively collected data. SETTING/SUBJECTS: Patients who received medical or surgical oncology care at Froedtert and the Medical College of Wisconsin. MEASUREMENTS: Patient demographics, disease characteristics, patient satisfaction, and clinical outcomes. RESULTS: Data from 69 patients who died following the implementation of the HCW program were reviewed; 24 patients were enrolled in the HCW program while 45 were not. Patients enrolled in HCW were proportionally less likely to be admitted to the ICU (12.5% vs. 17.8%) and were more likely to be "do not resuscitate" (87.5% vs. 80.0%), as well as have a completed ACP (83.3% vs. 79.1%). Furthermore, admission to a hospice was also higher among patients who were enrolled in the HCW program (79.2% vs. 25.6%), with patients enrolled in HCW more likely to die in hospice (70.8% vs. 53.3%). The HCW program was favorably viewed by patients, patient caregivers, and healthcare providers. CONCLUSIONS: Implementation of a facilitator-based ACP care model was associated with fewer ICU admissions, and a higher use of hospice care. System-level changes are required to overcome barriers to ACP that limit patients from receiving end-of-life care in accordance with their preferences.

Locked-In Syndrome: Case Report and Discussion of Decisional Capacity.

Locked-in syndrome (LIS) is a rare neurologic disorder rendering an individual quadriplegic and anarthric with preserved self-awareness and normal if not near-normal cognition. A lesion to the ventral pons causes the classic form of LIS, and patients can typically interact with their environment with eye/eyelid movements. LIS patients may live for years with preserved quality of life (QoL) and cognitive function, but with severe disability. However, medical providers and family often underestimate the patient's QoL, and choose less aggressive care. Prompt assessment of decisionality in LIS patients is challenging, but it must be done to allow these patients to participate in their care. We present the case of a 54-year-old man with LIS. The medical team recommended comfort measures, but the family advocated involving the patient in goals of care discussions. The patient was determined to be decisional during the acute hospitalization, and he elected for life-prolonging care. This case emphasizes the importance of unbiased shared decision making, but also the importance of utilizing a practical framework to assess the decision-making capacity in these patients. We provide a suggested approach to determining decision-making capacity in similar cases or conditions.

Palliative Care Training in Surgical Oncology and Hepatobiliary Fellowships: A National Survey of Program Directors.

BACKGROUND: Despite previous literature affirming the importance of palliative care training in surgery, there is scarce literature about the readiness of Surgical Oncology and hepatopancreaticobiliary (HPB) fellows to provide such care. We performed the first nationally representative study of surgical fellowship program directors' assessment of palliative care education. The aim was to capture attitudes about the perception of palliative care and disparity between technical/clinical education and palliative care training. METHODS: A survey originally used to assess surgical oncology and HPB surgery fellows' training in palliative care, was modified and sent to Program Directors of respective fellowships. The final survey consisted of 22 items and was completed online. RESULTS: Surveys were completed by 28 fellowship programs (70 % response rate). Only 60 % offered any formal teaching in pain management, delivering bad news or discussion about prognosis. Fifty-eight percent offered formal training in basic communication skills and 43 % training in conducting family conferences. Resources were available, with 100 % of the programs having a palliative care consultation service, 42 % having a faculty member with recognized clinical interest/expertise in palliative care, and 35 % having a faculty member board-certified in Hospice and Palliative Medicine. CONCLUSIONS: Our data shows HPB and surgical oncology fellowship programs are providing insufficient education and assessment in palliative care. This is not due to a shortage of faculty, palliative care resources, or teaching opportunities. Greater focus one valuation and development of strategies for teaching palliative care in surgical fellowships are needed.

Association of common variants in the human eyes shut ortholog (EYS) with statin-induced myopathy: evidence for additional functions of EYS.

INTRODUCTION: Of the nearly 38 million people in the USA who receive statin therapy, 0.1-0.5% experience severe or life-threatening myopathic side effects. METHODS: We performed a genome-wide association study (GWAS) in a group of patients with severe statin myopathy versus a statin-tolerant group to identify genetic susceptibility loci. RESULTS: Replication studies in independent groups of severe statin myopathy (n = 190) and statin-tolerant controls (n = 130) resulted in the identification of three single-nucleotide polymorphisms (SNPs), rs9342288, rs1337512, and rs3857532, in the eyes shut homolog (EYS) on chromosome 6 suggestive of an association with risk for severe statin myopathy (P = 0.0003-0.0008). Analysis of EYS cDNA demonstrated that EYS gene products are complex and expressed with relative abundance in the spinal cord as well as in the retina. CONCLUSION: Structural similarities of these EYS gene products to members of the Notch signaling pathway and to agrin suggest a possible functional role in the maintenance and regeneration of the structural integrity of skeletal muscle.

Neuromuscular manifestations in a patient with ehlers-danlos syndrome type IV.

We present a case of Ehlers-Danlos Syndrome (EDS) type IV with associated neuromuscular manifestations. The patient presented with nonspecific myopathic-type symptoms and later developed a spontaneous median mononeuropathy in the forearm. We believe that the case presented here complements current published neuromuscular manifestations occurring in EDS IV patients and illustrates the urgency of making the diagnosis, given the increased mortality associated with EDS IV.

Genetic risk factors associated with lipid-lowering drug-induced myopathies.

Lipid-lowering drugs produce myopathic side effects in up to 7% of treated patients, with severe rhabdomyolysis occurring in as many as 0.5%. Underlying metabolic muscle diseases have not been evaluated extensively. In a cross-sectional study of 136 patients with drug-induced myopathies, we report a higher prevalence of underlying metabolic muscle diseases than expected in the general population. Control groups included 116 patients on therapy with no myopathic symptoms, 100 asymptomatic individuals from the general population never exposed to statins, and 106 patients with non-statin-induced myopathies. Of 110 patients who underwent mutation testing, 10% were heterozygous or homozygous for mutations causing three metabolic myopathies, compared to 3% testing positive among asymptomatic patients on therapy (P = 0.04). The actual number of mutant alleles found in the test group patients was increased fourfold over the control group (P < 0.0001) due to an increased presence of mutation homozygotes. The number of carriers for carnitine palmitoyltransferase II deficiency and for McArdle disease was increased 13- and 20-fold, respectively, over expected general population frequencies. Homozygotes for myoadenylate deaminase deficiency were increased 3.25-fold with no increase in carrier status. In 52% of muscle biopsies from patients, significant biochemical abnormalities were found in mitochondrial or fatty acid metabolism, with 31% having multiple defects. Variable persistent symptoms occurred in 68% of patients despite cessation of therapy. The effect of statins on energy metabolism combined with a genetic susceptibility to triggering of muscle symptoms may account for myopathic outcomes in certain high-risk groups.

Core competencies in neurology resident education: a review and tips for implementation.

BACKGROUND: The Accreditation Council for Graduate Medical Education (ACGME) Core Competency project for graduate medical education was developed in the late 1990s and is now being implemented in residency programs across all specialties. Program directors and residents in neurology are seeking national guidance in how to adjust educational curricula and establish new evaluation tools that meet ACGME standards. REVIEW SUMMARY: This review summarizes the competency movement and outlines specific guidelines and resources for neurology programs including national efforts underway through the American Academy of Neurology (AAN), Neurology Residency Review Committee, and other subspecialty organizations. Resident and program director concerns for implementation are highlighted, with a focus on use of national resources and established evaluation tools. CONCLUSIONS: All neurology programs must comply with new requirements generated by the competencies that will require significant time and energy for program directors. An effective approach to implementation involves careful review of existing evaluation tools established by other subspecialties and expansion of reliable tools used already in place in most programs such as the mock oral board and RITE In-Training Examination. The individual program director is encouraged to monitor resources nationally through the AAN Program Director's Consortium and locally through institutional projects which can incorporate neurology residents.