An expert panel Delphi consensus statement on the use of palliative care in the management of patients with pulmonary arterial hypertension.

Mortality in pulmonary arterial hypertension (PAH) remains high and referral to palliative or supportive care (P/SC) specialist services is recommended when appropriate. However, access to P/SC is frequently a challenge for patients with a noncancer diagnosis and few patients living with PAH report P/SC involvement in their care. A modified Delphi process of three questionnaires completed by a multidisciplinary panel (N = 15) was used to develop expert consensus statements regarding the use of P/SC to support patients with PAH. Panelists rated their agreement with each statement on a Likert scale. There was a strong consensus that patients should be referred to P/SC when disease symptoms become unmanageable or for end-of-life care. Services that achieved consensus were pain management techniques, end-of-life care, and psychosocial recommendations. Palliative or supportive care should be discussed with patients, preferably in-person, when disease symptoms become unmanageable, when starting treatment, when treatment-related adverse events occur or become refractory to initial intervention. Care partners and patient support groups were considered important in improving a patient's overall health outcomes, treatment adherence, and perception of care. Most patients with PAH experience cognitive and/or psychosocial changes and those who receive psychosocial management have better persistence and/or compliance with their treatment. These consensus statements provide guidance to healthcare providers on the "who and when" of referral to palliative care services, as well as the importance of focusing on the psychosocial aspects of patient care and quality of life.

Serious illness conversations in pulmonary hypertension.

Pulmonary arterial hypertension has evolved from a fatal disease with few treatment options to a chronic condition with improved survival. This improvement is possible through development of effective therapies as well as the expansion of risk stratification scores to assist clinical decision making. Despite improved disease control, quality of life, and overall prognosis, many challenges remain. The treatment itself is burdensome, with significant impact on quality of life. Many patients with pulmonary arterial hypertension still present with advanced, often end-stage disease. Increased use of mechanical circulatory support and catheter-based interventions have expanded use of extracorporeal life support and right ventricle assist devices. For these reasons as well as the long-term relationships pulmonary hypertension physicians have with patients and their families, navigating the course of the illness in a considered, proactive way is essential. Understanding individual goals and revisiting them as they change over time requires comfort with the conversation itself. There are many barriers and challenges to having effective, compassionate conversations in the clinical setting with time constraints being the most often cited. Compressed visits are necessarily focused on the clinical aspects, therapy and medication adherence and tolerance. Clinicians are sometimes wary of diminishing hope in the face of ongoing treatment. Having sufficient experience and comfort with these discussions can be empowering. In this paper, we discuss the challenges involved and propose a framework to assist in incorporating these discussions into clinical care.

Trust-Based Partnerships Are Essential - and Achievable - in Health Care Service.

When people think about trust in the context of health care, they typically focus on whether patients trust the competence of doctors and other health professionals. But for health care to reach its full potential as a service, trust must also include the notion of partnership, whereby patients see their clinicians as reliable, caring, shared decision-makers who provide ongoing "healing" in its broadest sense. Four interrelated service-quality concepts are central to fostering trust-based partnerships in health care: empathetic creativity, discretionary effort, seamless service, and fear mitigation. Health systems and institutions that prioritize trust-based partnerships with patients have put these concepts into practice using several concrete approaches: investing in organizational culture; hiring health professionals for their values, not just their skills; promoting continuous learning; attending to the power of language in all care interactions; offering patients "go-to" sources for timely assistance; and creating systems and structures that have trust built into their very design. It is in the real-world implementation of trust-based partnership that health care can reclaim its core mission.

Transitioning Parenteral or Inhaled Treprostinil to Oral Treprostinil Diolamine: Case Series and Review of the Literature.

Treprostinil diolamine is the first oral dosage preparation of a prostacyclin analogue for use in treatment naive pulmonary arterial hypertension (PAH). This case series and review of the available literature describes the experience of patients with PAH receiving treprostinil by intravenous (IV), subcutaneous (SQ), or inhalation route who were transitioned to treprostinil diolamine. At our institution, 3 patients were transitioned to treprostinil diolamine who received treprostinil administered by each of the alternative routes: IV, SQ, and inhalation. All patients tolerated the transition without significant worsening of disease end points. In the literature, 5 additional reports representing 48 patients were transitioned to treprostinil diolamine from an alternate route of administration. A majority (92%) of patients were hospitalized during the cross-titration phase and tolerated the transition without changes in disease markers or significant adverse effect. Six (13%) patients required reinitiation of parenteral therapy due to clinical decline. The most common dosing frequency utilized for treprostinil diolamine was 3 times per day. In patients with stable PAH receiving parenteral or inhaled treprostinil, a transition to treprostinil diolamine was a safe approach in a highly select population meeting clinical end points. Additional studies are required to further describe this clinical strategy before accepted in clinical practice.

Role of Kindness in Cancer Care.

The wonders of high-tech cancer care are best complemented by the humanity of high-touch care. Simple kindnesses can help to diffuse negative emotions that are associated with cancer diagnosis and treatment-and may even help to improve patients' outcomes. On the basis of our experience in cancer care and research, we propose six types of kindness in cancer care: deep listening , whereby clinicians take the time to truly understand the needs and concerns of patients and their families; empathy for the patient with cancer, expressed by both individual clinicians and the care culture, that seeks to prevent avoidable suffering; generous acts of discretionary effort that go beyond what patients and families expect from a care team; timely care that is delivered by using a variety of tools and systems that reduce stress and anxiety; gentle honesty, whereby the truth is conveyed directly in well-chosen, guiding words; and support for family caregivers, whose physical and mental well-being are vital components of the care their loved ones receive. These mutually reinforcing manifestations of kindness-exhibited by self-aware clinicians who understand that how care is delivered matters-constitute a powerful and practical way to temper the emotional turmoil of cancer for patients, their families, and clinicians themselves.

When Patients and Their Families Feel Like Hostages to Health Care.

Patients are often reluctant to assert their interests in the presence of clinicians, whom they see as experts. The higher the stakes of a health decision, the more entrenched the socially sanctioned roles of patient and clinician can become. As a result, many patients are susceptible to "hostage bargaining syndrome" (HBS), whereby they behave as if negotiating for their health from a position of fear and confusion. It may manifest as understating a concern, asking for less than what is desired or needed, or even remaining silent against one's better judgment. When HBS persists and escalates, a patient may succumb to learned helplessness, making his or her authentic involvement in shared decision making almost impossible. To subvert HBS and prevent learned helplessness, clinicians must aim to be sensitive to the power imbalance inherent in the clinician-patient relationship. They should then actively and mindfully pursue shared decision making by helping patients trust that it is safe to communicate their concerns and priorities, ask questions about the available clinical options, and contribute knowledge of self to clinical decisions about their care. Hostage bargaining syndrome is an insidious psychosocial dynamic that can compromise quality of care, but clinicians often have the power to arrest it and reverse it by appreciating, paradoxically, how patients' perceptions of their power as experts play a central role in the care they provide.

Recommendations for the clinical management of patients receiving macitentan for pulmonary arterial hypertension (PAH): A Delphi consensus document.

In patients treated with macitentan (Opsumit®, Actelion Pharmaceuticals Ltd., Basel, Switzerland) for pulmonary arterial hypertension (PAH), prevention and/or effective management of treatment-related adverse events may improve adherence. However, management of these adverse events can be challenging and the base of evidence and clinical experience for macitentan is limited. In the absence of evidence, consensus recommendations from physicians experienced in using macitentan to treat PAH may benefit patients and physicians who are using macitentan. Consensus recommendations were developed by a panel of physicians experienced with macitentan and PAH using a modified Delphi process. Over three iterations, panelists developed and refined a series of statements on the use of macitentan in PAH and rated their agreement with each statement on a Likert scale. The panel of 18 physicians participated and developed a total of 118 statements on special populations, add-on therapy, drug-drug interactions, warnings and precautions, hospitalization and functional class, and adverse event management. The resulting consensus recommendations are intended to provide practical guidance on real-world issues in using macitentan to treat patients with PAH.

A Communications Bundle to Improve Satisfaction for Critically Ill Patients and Their Families: A Prospective, Cohort Pilot Study.

CONTEXT: Communication skills training with simulated patients is used by many academic centers, but how to translate skills learned in simulated settings to improve communication in real encounters has not been described. OBJECTIVES: We developed a communications bundle to facilitate skill transfer from simulation to real encounters and improve patient and/or family satisfaction with physician communication. We tested the feasibility of its use in our hospital's medical intensive care unit (MICU). METHODS: This prospective cohort 2-week feasibility study included patients admitted to the MICU with APACHE IV predicted mortality >30% and/or single organ failure. The communications bundle included simulation communication training for MICU physicians, scheduling a family meeting within 72 hours of MICU admission, standardized pre- and post-meeting team huddles with the aid of a mobile app to set an agenda, choose a communication goal, and get feedback, and documentation of meeting in the electronic medical record. The intervention group receiving the communications bundle was located in a geographically separate unit than the control group receiving standard of care from MICU physicians who had not received training in the communications bundle. Patient satisfaction surveys were given within 48 hours of the family meeting and scores compared between the two groups. We also compared trainee self-perceived communication preparation. RESULTS: The intervention group (N = 15) scored significantly higher on satisfaction than the control group (N = 16) (P = 0.018). Intervention group trainees reported improvement in self-perceived communication preparation. CONCLUSION: Use of the communications bundle proved feasible in the MICU and suggests association with improved patient satisfaction and trainee self-perception of communication preparedness.

Low Rate of Adverse Events Associated With Inpatient Parenteral Prostacyclins.

Parenteral prostacyclin is the most-effective therapy for patients with pulmonary arterial hypertension. Administration is complex, and administration errors are potentially life threatening. Hospital policies to minimize the risk to patients are necessary, but their effectiveness has not been well studied. We quantified the adverse event incident rate per at-risk patient day in a tertiary care hospital with an established parenteral prostacyclin policy. Patients on parenteral prostacyclin including new initiations from January 2003 to January 2013 were identified, encompassing 386 discrete admissions. Reports of adverse events were obtained from the inpatient risk feedback-reporting process and detailed chart review. Policy-divergent events were analyzed both categorically and by the degree of severity. Overall, 153 total policy-divergent events were identified. Data analysis indicated an incident rate of 45.9 per 1,000 patient days. In total, 21 of 153 potential errors reached the patient, translating to an incident rate of 6.3 per 1,000 patient days. Incident rate for "serious symptomatic" or "catastrophic" policy-divergent events was 3.3 per 1,000 patient days. Even with specific prostacyclin training and administration policy, there remains a small risk of adverse events in hospitalized pulmonary hypertension patients receiving parenteral prostacyclin.

Inhaled Iloprost for Chronic Thromboembolic Pulmonary Hypertension (CTEPH) During Pregnancy: A Case Report.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a subset of pulmonary hypertension caused by acute and recurrent pulmonary emboli. Pulmonary thromboendarterectomy is the treatment of choice, but 10-50% of patients are ineligible for this procedure. We describe the case of a 25-year-old, morbidly obese (228-kg, body mass index 83.5 kg/m(2) ) pregnant woman (G3 P2 ) who presented at 24 weeks' gestation; bilateral pulmonary angiography revealed filling defects and confirmed the diagnosis of CTEPH. The patient was evaluated and deemed to present too high of a risk for pulmonary thromboendarterectomy, so a multidisciplinary team initiated medical therapy. Sildenafil 20 mg orally 3 times/day was started at week 24 of gestation, and inhaled iloprost was added at 26 weeks and titrated to 5 µg inhaled every 2 hrs in order to optimize hemodynamic status prior to a cesarean section delivery scheduled to be performed 6 weeks later. At 32 weeks of gestation, the patient's pulmonary arterial systolic pressure was 77 mm Hg, right atrial pressure was 15 mm Hg, and pulmonary capillary wedge pressure of 16 mm Hg, and a healthy 1741-g male infant was delivered by cesarean section. The patient was transferred back to the medical intensive care unit in stable condition and discharged home 9 days following the procedure. Pharmacotherapeutic strategies for patients with CTEPH who become pregnant are limited to phosphodiesterase type 5 inhibitors and prostacyclin analog therapies due to the teratogenicity of the other drug classes used to treat the disorder (endothelin receptor antagonists and soluble guanylate cyclase stimulators). To our knowledge, this is the first case report of inhaled iloprost use in addition to oral sildenafil to improve patient symptomatology and hemodynamics during the peripartum period of a young pregnant patient with inoperable CTEPH. This drug therapy was used safely, with no noted adverse effects to the newborn or to the patient.

Treatment of patients with chronic thrombo embolic pulmonary hypertension: focus on riociguat.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease of the pulmonary vascular bed that is characterized by elevations in the mean pulmonary artery pressure in the setting of perfusion defects on ventilation-perfusion scan, and subsequently confirmed by pulmonary angiography. CTEPH, or World Health Organization (WHO) group 4 pulmonary hypertension, is a result of unresolved thromboembolic obstruction in the pulmonary arteries. Pulmonary endarterectomy (PEA) is the treatment of choice for CTEPH as it is a potentially curative therapy. However, up to one-third of patients are not candidates for the surgery, either due to distal and inaccessible nature of the lesions or comorbid conditions. Due to remodeling that occurs in nonobstructed pulmonary vessels, a portion of patients who have undergone PEA have residual CTEPH after the procedure, attributable to high shear stress prior to PEA. This phenomenon has led to the understanding of a so-called "two-compartment model" of CTEPH, opening the door to pharmacologic treatment strategies. In 2013, riociguat, a soluble guanylate cyclase stimulator, was approved in the US and Europe for the treatment of inoperable or persistent/recurrent CTEPH. This article reviews the current management of CTEPH with a focus on riociguat.

Impact of a Simulation-Based Communication Workshop on Resident Preparedness for End-of-Life Communication in the Intensive Care Unit.

Introduction. Although residents frequently lead end-of-life (EOL) discussions in the intensive care unit (ICU), training in EOL care during residency has been required only recently, and few educational interventions target EOL communication in the ICU. This study evaluated a simulation-based intervention designed to improve resident EOL communication skills with families in the ICU. Methods. Thirty-four second-year internal medicine residents at a large urban teaching hospital participated in small group sessions with faculty trained in the "VitalTalk" method. A Likert-type scale questionnaire measured self-assessed preparedness before, immediately following, and approximately 9 months after intervention. Data were analyzed using Wilcoxon rank-sum analysis. Results. Self-assessed preparedness significantly improved for all categories surveyed (preintervention mean; postintervention mean; p value), including discussing bad news (3.3; 4.2; p < 0.01), conducting a family conference (3.1; 4.1; p < 0.01), discussing treatment options (3.2; 3.9; p < 0.01), discussing discontinuing ICU treatments (2.9; 3.5; p < 0.01), and expressing empathy (3.9; 4.5; p < 0.01). Improvement persisted at follow-up for all items except "expressing empathy." Residents rated the educational quality highly. Conclusion. This study provides evidence that brief simulation-based interventions can produce lasting improvements in residents' confidence to discuss EOL care with family members of patients in the ICU.

Early initiation of prostacyclin in portopulmonary hypertension: 10 years of a transplant center's experience.

BACKGROUND: Portopulmonary hypertension (PoPH) is a subgroup of Group 1 pulmonary arterial hypertension (PAH) with particularly poor prognosis. Delay in initiation of parenteral therapy may be the reason for poor outcome. METHODS: We conducted a prospective observational study of all patients with Group 1 PoPH evaluated at Henry Ford Hospital between January 2002 and July 2012. The cohort of the REVEAL Registry patients with PoPH was used as the comparator group. The patient survival rates at 5 years after diagnosis and 2 years after enrollment, treatment trends, and the freedom from all-cause hospitalization rates at 2 years after enrollment were compared using χ (2) analysis. RESULTS: Twenty-one patients were enrolled in the PH Clinic with PoPH from January 2002 through July 2012. Our patients were significantly more likely to be on prostacyclin IV at 90 days as compared to REVEAL PoPH patients (67 vs. 31 %; p = 0.002). Despite this, early outcomes were not significantly different between the groups: 2-year survival from enrollment (70 and 67 %, respectively; p = 0.77) and 2-year freedom from hospitalization (35 vs. 49 %, respectively; p = 0.29). However, 5-year survival from time of diagnosis was significantly higher in our cohort of PoPH patients (71 and 40 %, respectively; p = 0.02). CONCLUSIONS: Early initiation of parenteral prostacyclin therapy in PoPH patients at a single institution was associated with improved 5-year patient survival from diagnosis as compared to the REVEAL Registry of PoPH patients and allowed for clearance for transplant in 52 % of patients within 1 year.