Hematology Service Research Articles

A Real-World Evidence Systematic Literature Review of Health-Related Quality of Life, Costs and Economic Evaluations in Newly-Diagnosed Multiple Myeloma Patients

IntroductionCurrent treatment for multiple myeloma (MM) is not curative, but aims to provide patients with improved disease control to extend survival. Newly diagnosed MM (NDMM) can have significant implications on the health-related quality of life (HRQoL) of a patient and increase the economic burden to both the patient and healthcare system. Strict protocols followed for clinical trials mean results seen in clinical trials may not reflect routine clinical practice. To assess the burden of illness of NDMM, systematic reviews of real-world humanistic and economic data was conducted.MethodsTwo systematic literature reviews (SLRs) of the humanistic and economic burden in NDMM were conducted, covering the published literature from Jan 2007 to June 2016. Data sources reviewed included Medline, Embase, PubMed (for e-publications ahead of print), and select hematology, oncology and health services research conferences. Eligibility criteria were limited to NDMM patients receiving first-line treatment (including induction, stem cell transplant [SCT], consolidation, and maintenance therapy) reported in observational studies or cost effectiveness analysis (CEA).ResultsEleven studies met the inclusion criteria for the HRQoL SLR and 29 studies for the economic SLR. The study populations were heterogeneous in terms of patient and clinical characteristics (Table 1).A summary of HRQoL measures and baseline values are shown in Table 2. Cancer or myeloma specific tools were used to assess HRQoL in 5 studies; 2 studies utilized a generic HRQoL tool (EQ-5D and SF-36). Symptoms of interest associated with MM and MM treatment - either pain, fatigue, or neuropathy - were assessed in 8 of the included studies.The humanistic burden was pronounced in patients with NDMM. Across studies, HRQoL was poor at diagnosis; the European Organization for Research and treatment of Cancer Quality of Life Questionnaire (EORTC-QLQ-C30) global scores ranged from 55 to 64 at diagnosis (scale from 0-100 with higher values indicating better HRQoL), and improved as patients received therapy. Domains related to physical attributes, including symptoms, were more adversely affected than those related to mental domains. Pain and fatigue were consistently highlighted as the most problematic symptoms throughout the treatment period; however, both symptoms improved over the course of therapy. Neuropathy was measured in 3 studies, each in selected populations; patients who were ineligible for SCT reported greater burden due to neuropathy, and neuropathy increased over the course of first-line treatment. Financial difficulties also contributed to impairment on HRQoL for patients throughout the measured period.Of the 29 studies identified as eligible for the economic review, 22 included either direct or indirect costs, and 7 studies were CEA; 11 studies included estimates of real-world resource use. Costs varied across studies, mainly due to different cost inputs considered and currencies reported. Overall, direct costs ranged from 7,534 USD 2014 per patient per month (unadjusted medical and pharmacy costs for 1st line lenalidomide treatment) to 213,166 USD 2014 per patient (direct costs associated with autologous SCT and one year follow-up). The primary cost drivers of direct medical costs were largely medical care, including hospitalizations and ambulatory care. Two studies reported out-of-pocket expenses, ranging from 3,478 USD 2013 (annual cost to patient in 1st year after diagnoses) to 4,666 USD (year not reported; per treatment episode for 1st year after treatment initiation). The results from the CEA studies differed significantly (Table 3).ConclusionsMM specific HRQoL measures indicate reduced HRQoL at diagnosis which gradually improves throughout the treatment period in newly diagnosed patients (before they experience relapse). Pain, fatigue and financial difficulties were domains reported as problematic to patients. Resource use burden studies primarily reported in- and out-patient visits. Indirect costs were reported to be as high as 4,666 USD, which further supports the financial difficulties reported in the HRQoL studies. The burden of NDMM for a patient is physical, emotional, and financial. Real-world evidence on HRQoL and economic outcomes reported from the patient perspective is complementary to evidence from clinical trials and provides an insight into the burden of NDMM. [Display omitted] [Display omitted] [Display omitted] DisclosuresYong:Takeda: Employment. Korol:ICON plc: Employment. Khan:ICON plc: Employment. Lin:Takeda: Employment. Thompson:Takeda: Consultancy. Valovicova:Takeda Pharmaceutical Company Ltd: Consultancy. Luptakova:Takeda Oncology: Employment. Seal:Millennium Pharmaceuticals, Inc., a wholly owned subsidiary of Takeda Pharmaceutical Company Limited: Employment, Equity Ownership.

Factors Influencing Hematology Career Choice in Hematology and Oncology Fellows at a Major Academic Institution

INTRODUCTION/BACKGROUNDThe majority of graduating fellows from hematology/oncology subspecialty training programs in the U.S. pursue careers in medical oncology or in non-academic fields. A workforce shortage of physicians specializing in hematology has been predicted for the next decade. Large-scale surveys have identified a number of factors responsible for this trend, including financial considerations, educational debt, work-life balance, and fellowship exposure. Qualitative studies in other subspecialty fields have yielded unique insights into specific factors motivating career decisions, but no such study has been conducted in hematology. We performed a qualitative study to identify factors influencing trainees' perceptions of careers in hematology.METHODSSenior or graduating fellows in the hematology/oncology training program at Yale School of Medicine were invited to participate in this study. Semi-structured interviews lasting between 30 and 90 minutes were conducted. Recordings were transcribed and analyzed using the framework method.RESULTSEight trainees participated in the study, five in their senior year of training, three shortly after graduation. Five were female. Three held dual M.D. and Ph.D. degrees. Most were planning on an academic career or a career with an academic affiliation; two were specifically pursuing careers in benign or malignant hematology. Trainees' career decisions and perceptions of hematology revolved around five major themes: serendipity, mentorship, clinical exposure and teaching, intellectual interest, and lifestyle factors. Most trainees identified seminal, career-defining experiences prior to medical school but were not influenced by medical school classroom experiences and were not differentiated at the start of fellowship with respect to a hematology- or oncology-oriented career. By the end of their first year of fellowship, almost all had decided to pursue careers in solid tumor oncology owing to clinical exposure and research opportunities. Mentorship was identified as the single most important determinant, with mentorship opportunities tending to occur "randomly" or by "happenstance," and with oncology having "more" "available mentors" who played a "huge role" in the career-decision process. Trainees characterized hematology as having a less desirable work/life balance than oncology, and trainees' experiences on the inpatient hematology services caused some of them to feel "burned out." Despite this, fellows commonly perceived hematology to be more "interesting" and "challenging" than oncology, with "much more complex" disease pathology. Financial considerations were of importance to only a minority of trainees.CONCLUSIONSMentorship and research opportunities are the major factors guiding career decisions among hematology/oncology fellows. An increase in structured mentorship opportunities in hematology may lead to greater trainee recruitment. Trainees view hematology as an intellectually engaging field although lifestyle factors render it less desirable than oncology. DisclosuresPodoltsev:Incyte: Consultancy, Honoraria; Ariad: Consultancy, Honoraria.

Evaluation of the Imatinib Treatment of Patients with Chronic Myeloid Leukemia (CML). a Retrospective Study from Algerian Working Group on CML (GAT-LMC)

Introduction: The advent of anti-tyrosine kinase has revolutionized the treatment of chronic myeloid leukemia. Indeed, from 2000, the IMATINIB has become internationally the gold standard of treatment for CML chronic phase, while the allogeneic bone marrow transplant was previously, the 1st intention choice, when an HLA-matched donor is available. The aim of this study is to evaluate the efficiency and the toxicity of a treatment with Imatinib(copy), drug used in Algeria to treat patients with a CML chronic phase. The main objective is to evaluate the overall survival and the progression-free survival to these patients.Materials and methods: This is a longitudinal study, National, multicenter, retrospective, which included Algerian patients with chronic phase CML and treated with Imatinib between January 2007 and December 2013. A technical form was established and distributed to different hematology services nationwide, to collect and analysis the following data: Patient’s general characteristics, disease circumstances of discovery, clinical and para-clinical examinations at diagnosis (blood count, blood smear, bone marrow aspiration, karyotype, molecular biology, Sokal prognostic classification score and Eutos score). The treatment: Imatinib 400 mg / d, a therapeutic assessment is made according to the ELN recommendations adapted to our conditions and capabilities in Algeria: The complete hematologic response (CHR) at 03 months and molecular response and / or cytogenetic and / or Fish at 03, 06.12, 18.24 months and more according to capabilities. At 03mois and / or 6 months we search a bcr / abl rate <10%. At 12 months we research a major molecular response (MMR), defined by a bcr / abl ratio lower than 0, 1% according to the ELN. A ratio between 0.1 to 1% is considered a good response according to GAT-LMC (the CML study Algerian group) so the Imatinib treatment is continued. The median follow-up of patients in December 2014 is 48 months (12-84 months). Overall survival and progression-free survival are determined by using the Kaplan-Meier method. The descriptive analysis of the quantitative variables by calculating averages, medians and the qualitative variables, by using percentages and 95% confidence interval. The Chi2 test is used to compare between two variables.Results: From 1024 collated sheets, 1007 are assessable; the median age of patients was 45.7 years (06-87 years), it’s about 516 men and 491 women with a sex ratio M / F 1.05. The Diagnosis of CML is done by cytogenetic examination in 337 patients (33%), by Fish 214 patients (21%) and by molecular biology in 401 patients (39%). The prognostic classification (PC), according to the Sokal score, found a low risk in 18.7%, 55.5% as intermediate and a high risk in 25.8%. The Eutos score is less than 87 in 97% and more than 87 in 03%. A CHR at 03mois was found in 907 patients (90.1%). There is no correlation between the RHC at 03 months and the SOKAL PC (p = 0.23), by cons we found a significant correlation with the Eutos score (p <10-3). Molecular assessment at 03 and 06 months is performed in 222 patients and a bcr / abl ratio <10% was found in 66.5%. A molecular evaluation at 12 months showed an MMR in 55.4%. Cytogenetic evaluation (FISH) has found a 28.6% CCyR at 3 months, 45% at 6 months, 64.2% at 12 months (IRIS = 68%), 75.7% at 18 months (IRIS = 76.2%) and 85% at 24 months. Overall survival was 84% at 08 years and it is significantly correlated to Sokal score (p <10-6). A failure to TRT was found in 11.5% of the cases and a 10, 1% relapse rate, related to non-adherence to TRT in 50% of the cases and a lack of monitoring by a regular molecular control in the other half of the cases. Event-free survival at 08 years was 76%. A good clinical and biological tolerance is noted in 90% of the cases. Only 8% of patients were switched to a 2nd generation TKIs because of intolerance. A non-adherence to TRT was found in 14.4%.Conclusion: Imatinib, used in Algeria, is a very interesting molecule both efficiency side and tolerance level. However, we must ensure a molecular monitoring for a patients optimal follow up, and an adequate patient education for a better adherence. DisclosuresNo relevant conflicts of interest to declare.

Prophylaxis in Patients with Hemophilia a and Its Impact on Quality of Life Using the Specific Index Haem-a-Qol at a Hospital in Paraguay

Prophylactic replacement therapy with FVIII is considered the standard of care for patients with hemophilia although a protocol for administering the prophylaxis has yet to be defined by the World Federation of Hemophilia. Gaona in 2012 demonstrated a significant reduction in the number of hospitalizations due to bleeding episodes in patients with Hemophilia A treated with secondary prophylaxis at the Hematology Service of the Hospital Central del Instituto de Prevision Social (HCIPS) with a regimen of 25 IU/Kg per week in two infusions by reporting results from such regimen that had started at the HCIPS in 2010. This is a lower dose to those most frequently used in high resource countries like the Malmo or Utrecht protocols.The goal of this study was to assess the impact of our lower dose prophylaxis regimen on bleeding events, hospitalizations and quality of life as an alternative for low resource countries that cannot afford to provide their patients with higher dose treatments.The Dutch group Haemo-QoL designed the specific questionnaire for adult patients with hemophilia, the Haem-A-QoL with a Spanish version available for patients aged 17 and up.Results: The HCIPS currently has 36 patients receiving prophylactic treatment with FVIII. From these 18 patients consented to filling out the questionnaire. The mean age of the patients was 28. 3 of the patients were married, 2 were in a stable relationship and 13 were single. 62% of patients lived within 20 km of the Hospital and 38% lived further away going up to 200 km. 17 of the 18 patients had the highest level of education possible for their age. Only one patient, the oldest one, had stopped his education at primary school. All of them had severe disease with one or more affected joints.The average FVIII use per year was 68,000 IU ranging from 8,000 IU to 96,000 IU or 917 IU/kg/year. The strongest determinant for receiving less FVIII was missing appointments. Distance to the Hospital was not of significance. Bleeding episodes prior to prophylactic treatment was 2-3 minor traumatic bleeding episodes per week (after brushing their teeth, easy bruising from minor trauma), 2-3 joint bleeds per month at which point they would seek medical assistance and 1-2 major bleeding episodes per year requiring hospitalization. In the last year 5 patients were hospitalized once. One for pneumonia, one for phlebitis, one for dengue fever and two for hemathrosis. They reported a decrease in bleeding episodes at home from 2-3 joint bleeds per month to 1 every other month. Patients reported after traumatic events their bleeding was "normal" (i.e. when brushing their teeth if they started bleeding they would continue bleeding for days at a time whereas when in prophylaxis they would bleed right after the brushing and then it stopped which they took to be normal).Converting the results from the Haem-A-QoL questionnaire to a scoring system of 1-100 being 1 the best QoL and 100 the worst, the average QoL was 52. With scores of 60 for physical health, 50 for feelings, 52 for view of themselves, 48 for work/school, 53 for treatment, 56 for future, 47 for family planning and 45 for dating. Scores for sports and leisure though only amounting to 64, 33% of patients said that category did not apply to them since they did not practice any sports and the dealing category with a score of 37 was the lowest of all. When compared to results published by a Blood Center in Brazil with an on demand regimen patients with severe hemophilia in that study showed a physical health score of 55, 40 for feelings, 35 for self-perception, 60 for sports and leisure, 35 for work and school, 20 for coping, 45 for treatment, 45 for future and 25 for dating with an average overall score of 40. With an annual average usage of 63,683 IU on the severely affected patients.In summary, the proposed dosing of 25IU/kgs/week seems at first glance to reduce bleeding episodes among patients with severe hemophilia as well as hospitalizations. In general patients treated at the HCIPS have a regular to poor quality of life as measured by the Haem-A-QoL questionnaire which contrasts with results from the Brazilian study on quality of life from patients with on demand treatment and roughly same FVIII usage per year showing their patients with severe hemophilia had an overall quality of life from regular to good. Perhaps the psychological factor comes in to play? A follow up study after patients receive proper psychological evaluations may help clarify results. DisclosuresNo relevant conflicts of interest to declare.

Manifestaciones musculoesqueléticas en niños y niñas con leucemia linfoblástica aguda

IntroductionMusculoskeletal pain is prevalent in children. The causes are: trauma, growing pains, pressing as infections. However, it is necessary to expand the differential diagnosis to neoplastic diseases, with ALL, being the most common in the latter process. The objective is to evaluate the musculoskeletal manifestations of ALL in children to define an epidemiological profile of the manifestations. Materials and methodsA case series study was carried out between February 2010 and February 2011 including 44 patients admitted to the Hematology Service of the Pediatric Hospital Fundación La Misericordia, Bogotá. We evaluated the clinical manifestations in patients between 0 and 18 years of age with diagnosis of ALL. The following variables were recorded: age, gender, anatomic location of signs and symptoms, relation with radiographic findings, haemoglobin levels, C-reactive protein, leukocytes, lactate dehydrogenase (LDH). ResultsWe found graeter affection in male children, mean age 7.7 years (greater number 0 to 5 years) preponderant phenotype B and associated with greater musculoskeletal symptomatology. The majority presented normal leukocyte numbers, high LDH levels directly proportional to symptoms. Imaging findings in decreasing order were osteopenia, metaphyseal lines, column infiltrates, permeative patterns and periosteal reactions. ConclusionOur findings are similar to other studies. ALL is related to musculoskeletal manifestations (a common reason for orthopedic, pediatric and emergency consultations) being masked by other processes prevalent in pediatric patients. It is important to approach it as a differential diagnosis, since the prognosis of the disease depends on early diagnosis. Level of evidenceIV. Case study series

Preventing intraluminal occlusion in peripherally inserted central catheters.

Peripherally inserted central catheters (PICCs) are used in a variety of specialties. Infective and thrombotic complications are widely reported but little has been written about the complications of persistent withdrawal occlusion (PWO) or complete luminal occlusion in PICCs. An audit was conducted of all patients who had a PICC in situ for a 6-week period, using a combination of reviewing clinical documentation and local departmental databases, physical review of inpatients and discussion with the relevant clinical nurse specialists in haematology, oncology, outpatient antibiotic therapy and the vascular access team, to determine the degree of PWO or occlusion but also to review possible financial implications in the introduction of a more expensive product. It was difficult to determine a rate for either PWO or complete occlusion owing to the diversity of the patient cohort and the tertiary nature of the organisation; the occlusion rate was estimated at 5%, but there was general agreement this was underestimating the problem. A needle-free connector was subsequently introduced for all PICCs in haematology, oncology and hepatobiliary services. Following the change in needle-free connector, 180 PICCs were inserted by the vascular access team on one of the organisation's sites over a 12-month period, with a total dwell time of 9702 catheter days (mean: 53.9 days, median: 35 days), a PWO rate of 1/1000 catheter days and total occlusion rate of 0.4/1000 catheter days. The time for insertion to complication of PWO or occlusion ranged from 9 to 144 days. Despite poor baseline data the results suggested the introduction of the bi-directional needle-less connector had a positive impact on the PWO and occlusion rates in PICCs.

Pulmonary Embolism Response Teams.

Pulmonary embolism is a common and often life-threatening event. Treatment options include anticoagulation alone, catheter-directed therapies, and surgical thromboembolectomy. While guidelines exist, there is often controversy over which treatment is most appropriate, particularly for intermediate-risk patients. The traditional care model, in which the primary team is responsible for consulting the appropriate specialists, may be inadequate and inefficient for emergent situations, as ensuring coordination and communication between various consulting services can be a time consuming and confusing process. The Pulmonary Embolism Response Team (PERT) model was developed to improve the quality and efficiency of care for patient with intermediate- and high-risk pulmonary embolism. Activation of the PERT allows for rapid, multidisciplinary discussion among dedicated specialists, which typically includes members of the cardiology, emergency medicine, hematology, pulmonary/critical care, and surgical services. While the majority of patients are still treated with anticoagulation alone, in the event that a more invasive approach is deemed necessary, the team expedites this process. Over the last several years, the PERT model has been adopted at more than 75 health care institutions and may represent a new standard of care.

Novel tool utilized as a trigger for advance care planning in hospitalized oncology patients.

22 Background: Study Purpose: reduce inpatient oncology readmissions; determine if there is a need to standardize trigger for Advance Care Planning in the inpatient oncology population. Methods: Investigational Quality Improvement Study January-June 2014. Retrospective chart review of oncology readmissions on the BMC Hematology and Oncology service during a 6 month period, January-June 2013 (N = 68). Data abstracted included: demographics (age, race/ethnicity, primary language, education, marital status, ECOG); type and stage of cancer; type of consults (palliative, spiritual, social services, integrative, PT/OT, hospice, visiting nurse). Developed a Severity of Illness Scale for use in the oncology inpatient patients at BMC. Results: Mortality rate within 1 year of discharge: 32.8% (non-elective readmissions) 19/58; mean days to death from readmission discharge: 65 days (range 0-252); 84.2% of those who died had metastatic cancer in index admission; only 37% ever had a palliative care consult placed (all on readmit); only 17% had a documented EOL discussion on index admission; 38.5% of patients had lung CA (22% of all non-elective readmissions). Conclusions: (1) There is a need to standardize triggers to goals of care discussions and access to Palliative Care in the Hematology/Oncology Inpatient Population at this Institution. (2) Readmissions may be reduced with better Advance Care Planning. (3) There is a communication gap amongst interdisciplinary teams in regards to Advance Care Planning at this institution. Currently IRB Approval for use of novel severity of illness scale on a maximum 450 patient sample (current N = 300) at daily rounds administered in all inpatients admitted to the Hematology/Oncology Service January 4, 2016-June 30, 2016. Preliminary data shows 25-30% of study sample scoring in for Advance Care Planning with a score of greater than or equal to 4 on novel tool (BMC Cancer Care Severity Of Illness Tool).

Effect of supportive care consultation on reducing 30-day readmissions to oncology inpatient services at an academic hospital.

181 Background: Decreasing 30-day hospital readmissions is an important quality care measure and signifies high-quality care. This study seeks to identify the benefits of Supportive Care consultations on 30-day readmissions to oncology inpatient services in an academic hospital. Methods: A retrospective review of all patients that had at least one 30-day readmission to the medical oncology or hematological malignancy service during a 2 year period (2011-2012). Data was collected on age, sex, cancer type, stage, number of readmissions, and presence of inpatient Supportive Care consultation. Results: During the study period 410 patients were identified with at least one 30-day readmission to an oncology service. Patients with a supportive care consult, 111 (27.7%), had an average of 2.5 readmissions and patients without a consultation, 299 (72.9%), had an average of 2.0 readmissions (p = 0.014). After Supportive Care consultation a reduction of 0.87 admissions (p &lt; 0.001) was observed. Stage IV disease was observed in 203 (49.5%) of patients studied. Conclusions: Supportive Care is often consulted for patients with significant symptoms and suffering during advanced illness. The higher observed average readmissions in patients seen by Supportive Care may reflect the complex assistance needed by this patient population. This study demonstrates a benefit of Supportive Care consultation in reducing 30-day readmissions after consultation.

Hodgkin's Lymphoma - evaluation of patients submitted to Autologous transplantation of hematopoietic cells in the Hematology Service of the Hospital Walter Cantídio - Fortaleza, Brazil.

The Autologous HSCT is an important alternative for refractory or recurrent HL patients in terms of survival and improved quality of life. This study analyzes the results of autologous BMT performed in HL patients in the Transplant Unit of the HUWC/ HEMOCE (Fortaleza - CE, Brazil). Fifty-two transplanted patients were studied from January 2009 to October 2015, among them, 30 men and 22 women, mean age of 28.2 years. All of them received GCS-F during the mobilization, in some cases associated with Vinorelbine or Plerixafor, with CD34 collection averaging 4.8 CD34/kg. The conditioning was performed with BEAC, NEAM or BEAM and the grafting with an average of 10 days. The evaluation on D + 100 showed: CR - 42 (82.7%), PR - 08 (13.5%) and 02 (3.8%) deaths, three and six days after cell infusion. After the D+100, 08 patients in CR showed HL recurrence from 06 to 36 months; 03 died and 05 are being treated with brentuximab; among the 08 patients in PR, 01 died due to HL activity, 04 months after BMT and 07 patients are undergoing treatment. The final evaluation of HL transplant patients showed an OS of 88.5% and a DFS of 61.5% in 6 years, with OS of the chemosensitive patients of 81% and of the chemoresistant ones, of 72.6%. It is possible to conclude that the Autologous HSCT has shown to be an excellent rescue therapy regarding tolerance, as well as the overall survival.

Expanding care through a layered learning practice model.

The outcomes of a patient-centered layered learning practice model (LLPM) in which the clinical specialist acted as the attending pharmacist and managed a pharmacy team to provide direct patient care were evaluated. Two 30-day evaluations were conducted on the acute care malignant hematology and medical oncology services of the University of North Carolina Medical Center in 2011. The primary objective of this study was to design an LLPM that used a team to expand the pharmacist care services offered. The primary outcome was the frequency of pharmacy team encounters at discharge (medication reconciliation and counseling), termed the discharge capture rate. During the study months, 42 and 78 malignant hematology and medical oncology patients were eligible for study inclusion, respectively. The overall discharge capture rate was 51%. Sixty-one patients received discharge medication reconciliation services during patient counseling. Patients included in the malignant hematology group received a mean of 11 prescriptions at discharge, compared with 9.83 in the medical oncology group. Means of 1.26 and 2.1 medication-related problems per patient were identified in the malignant hematology and medical oncology studies, respectively, during discharge medication reconciliation. The overall mean face time spent per patient was 21.3 minutes. Patients in malignant hematology and medical oncology services were counseled and provided discharge medication reconciliation by a pharmacy student or resident whose activities were managed and reviewed by an attending pharmacist using an LLPM, resulting in an improvement in all clinical outcomes and measures.

Advance directives: ethical issues of information to patients and caregivers training

BackgroundIf the law called “Leonetti” enacted in 2005 allows patients to prepare and submit their advance directives (AD), its ownership in hospital services is still very punctual, even in clinical hematology services where illnesses are severe and can be life-threatening. The objective of our study was to evaluate the impact of a dual approach: caregiver training and information to patients on AD. The secondary objectives are to analyze the obstacles to their appropriation by caregivers and better understanding of the meaning patients give to the AD.

EVALUATION OF AN ELECTRONIC PAEDIATRIC INTENSIVE CARE UNIT (PICU) MEDICATION RECONCILIATION (MR) FORM

AimTo gather opinions from doctors and pharmacists to improve the design of the PICU MR form generated by the electronic prescribing and clinical notes system to support transfer of care...

Intracranial Aneurysms in Sickle-Cell Disease Are Associated With the Hemoglobin SS Genotype But Not With Moyamoya Syndrome.

Intracranial aneurysms and aneurysmal subarachnoid hemorrhage may occur more frequently in sickle-cell disease (SCD), and this could be related to the sickle genotype and moyamoya syndrome seen in SCD. Records from a total of 1002 patients with SCD attending 2 specialized adult hematologic services were retrospectively reviewed. We analyzed data of a cohort of 767 patients attending 1 SCD clinic between 2002 and 2013 and of 235 patients from the other clinic who have had neurovascular imaging between 2007 and 2014. We identified 4 patients in the cohort who had an aneurysmal subarachnoid hemorrhage during 9063 patient-years. The highest incidence rate was seen among women in the age group 30 to 39 years with the hemoglobin SS (HbSS) genotype (440 per 100 000 patient-years). Unruptured intracranial aneurysms were found in 20 of the 324 patients, who had imaging data; the prevalence was significantly higher in patients with HbSS genotype compared with other sickle genotypes with the highest prevalence (15%) observed in women in the age group 30 to 39 years. Fifty-one HbSS patients had a moyamoya vasculopathy, but only 3 of these had concomitant intracranial aneurysms. Intracranial aneurysms are common in HbSS SCD. There was also a trend toward more common occurrence of aneurysmal subarachnoid hemorrhage in HbSS; women in the age group 30 to 39 years were most at risk. There was no correlation between the occurrence of intracranial aneurysms and moyamoya syndrome.

THE FACTOR VII DEFICIENCY IN MOROCCAN CHILDREN

The Factor VII deficiency is a rare causes of bleeding disorders in children. 12 cases were diagnosed and treated in hematology and oncology service between 2003 and 2016. The clinical manifestations are extremely variable. The severity of bleeding is variable and does not seem related to the importance of the deficit; a deep deficit may remain asymptomatic or result in more or less severe hemorrhagic syndrom. 33% of cases were asymptomatic, 66% of cases had a hemorrhagic syndrom: its Mucocutaneous bleeding (33%), gastrointestinal bleeding (17%), Urinary bleeding (hematuria) (8%), Articular bleeding (haemarthrosis ankle) (8%). Our patients have low PT ranging from 12% to 55%. Family investigation is required, including the dosage of Factor VII in parents and siblings. The Factor VII deficiencyis suspected ahead of the combination of an elongated prothrombin time and a normal PTT, it is confirmed by the dosage of factor VII activity, by chronometric method.Replacement therapyof this deficit is not systematic, it is indicated in the case of an acute hemorrhagic stroke, or prophylactically prior to surgery, it is based on the recombinant Factor.

Reducing patient wait time on an inpatient hematology and transplant unit.

87 Background: The University of Wisconsin Hematology and Bone Marrow Transplant services admit 250 patients per year for scheduled treatment. Time from patient arrival to chemotherapy initiation averages 7 hours. This long time leads to patient dissatisfaction and prolonged length of stay. Chemotherapy often begins in the evening when physicians and pharmacists are not on site to clarify treatment questions. Methods: A multidisciplinary team was formed in April 2015 as part of the ASCO Quality Training Program. The team developed the aim to reduce time from 7 hours to 5 hours to allow for more daytime nursing shift chemotherapy starts. The group created a process map and an Ishikawa diagram to identify causes of treatment delays. The team observed that treatment orders were often written and signed after hospital admission, though the plan of care was available prior to admission. Using a prioritized list, the initial process change was to have physicians write and sign chemotherapy treatment plans prior to admission. The process measure was the percentage of orders signed before admission. The outcome measure was time from arrival to chemotherapy start. Balance measures included narrative comments from physician and nursing staff about the process change. Data were obtained from EHR time stamps. Physicians were asked to begin making signed treatment plans available after August 1, 2015. Results: Data from 55 patients prior to the change (May 1-July 31) and 52 patients post-change (August 1-October 16) were analyzed. The number of treatment plans signed prior to admission increased from 27% to 58% (p = 0.0018). Mean time from admission to treatment pre-process change was 404 minutes and 383 minutes post-change. When plotted on a control chart, a sustained shift in the process was not apparent. Feedback from physician and nursing staff did not identify efficiency or safety concerns with the new process. Conclusions: Though the percentage of orders signed prior to admission increased, there was no improvement in the time from patient admission to treatment initiation with this change. Future PDSA cycles will include increasing the percentage of orders signed prior to admission as well as address other sources of delay identified in the process map.

Risk factors for visual impairment in patients with sickle cell disease in London.

Dramatically improved health care in recent years has increased the life expectancy of patients with sickle cell disease (SCD) as well as the prognosis for its ocular complications. We sought to identify risk factors for visual impairment in patients with SCD in London 4 decades after Goldberg's seminal studies. Patients 16 years and older with SCD (genotypes HbSS, HbSC, HbSβ-thalassemia) attending hematology and ophthalmology services were offered ocular examination. Retinopathy was graded according to the Goldberg classification. Visual impairment was defined as corrected distance visual acuity of 20/40 or poorer. In total, 182 eyes of 182 patients (mean ± SD age, 37.2 ± 12.8 years; female, 65.9%) were included. Women were significantly older than men (mean ± SD age, 38.8 ± 13.1 vs 34.2 ± 11.8 years; p = 0.0174). There was no difference in mean age of each genotype group (p&gt;0.15). Risk factors for sight-threatening proliferative sickle retinopathy (PSR) were age over 35 years (odds ratio [OR] 2.01; 95% confidence interval [CI] 1.05-3.89; p = 0.0359) and HbSC genotype (OR 4.06; 95% CI 2.07-7.98; p&lt;0.0001). Although visual impairment was related to the presence of sight-threatening PSR (OR 7.23; 95% CI 1.50-35.0; p = 0.0138), it was not related to hemoglobin genotype (p&gt;0.50). We present the largest study of ocular findings in SCD in the United Kingdom. Sight-threatening PSR is a risk factor for visual impairment, but hemoglobin genotype status is not.

Oral anticoagulant therapy interruption in children: A single centre experience

BackgroundThe use of anticoagulant therapy in paediatrics is common, with vitamin K antagonists remaining the most commonly prescribed therapy. There is a weak evidence base behind many of the recommendations for anticoagulant therapy in paediatric patients. One of the areas requiring further research is the management of anticoagulant therapy interruption. Interruption to anticoagulation is the period surrounding a planned invasive procedure whereby long term anticoagulation is ceased, and recommenced post procedure. The word bridging refers to the use of low molecular weight heparin or unfractionated heparin to anticoagulate during the period of sub therapeutic INR. To date institutional protocols for bridging anticoagulation are based on adult guidelines. However, there are currently no studies validating the extrapolation of these guidelines to paediatrics. This study seeks to review the clinical outcomes associated with current bridging practices employed at a tertiary metropolitan children's hospital. MethodsThe patient population was selected from the warfarin management registry of a Clinical Haematology service of a major metropolitan children's hospital. The admission history of these patients was queried to identify admissions where anticoagulation interruption would typically be required. Namely, these were dental extraction, cerebral or cardiac angiography, or cardiac catheterization. Data relating to demographics, anticoagulant therapy interruption plan, and clinical outcomes were recorded. ResultsA total of 61 admissions for children aged between 1year and 17years and 11months were analysed for this study. Congenital heart disease (CHD) was the primary underlying disease for which long-term oral anticoagulation with warfarin was indicated. Children with Moyamoya in this cohort were treated more consistently compared to the other disease groups. There were no instances of major bleeding (n=0) or thrombotic events (n=0). ConclusionThis study describes the current practices and outcomes associated with anticoagulant therapy interruption at one institution thereby filling an evidence gap in the paediatric anticoagulant management. It achieved this by analysing the largest and most representative cohort to date. This project is a stepping stone from which future studies of safety and efficacy of paediatric anticoagulation interruption management can be developed.

Evaluating laboratory request forms submitted to haematology and blood transfusion departments at a hospital in Northwest Nigeria.

BackgroundThe laboratory request form (LRF) is a communication link between laboratories, requesting physicians and users of laboratory services. Inadequate information or errors arising from the process of filling out LRFs can significantly impact the quality of laboratory results and, ultimately, patient outcomes.ObjectiveWe assessed routinely-submitted LRFs to determine the degree of correctness, completeness and consistency.MethodsLRFs submitted to the Department of Haematology (DH) and Blood Transfusion Services (BTS) of Aminu Kano Teaching Hospital in Kano, Nigeria, between October 2014 and December 2014, were evaluated for completion of all items on the forms. Performance in four quality indicator domains, including patient identifiers, test request details, laboratory details and physician details, was derived as a composite percentage.ResultsOf the 2084 LRFs evaluated, 999 were from DH and 1085 from BTS. Overall, LRF completeness was 89.5% for DH and 81.2% for BTS. Information on patient name, patient location and laboratory number were 100% complete for DH, whereas only patient name was 100% complete for BTS. Incomplete information was mostly encountered on BTS forms for physician’s signature (60.8%) and signature of laboratory receiver (63.5%). None of the DH and only 9.4% of BTS LRFs met all quality indicator indices.ConclusionThe level of completion of LRFs from these two departments was suboptimal. This underscores the need to review and redesign the LRF, improve on training and communication between laboratory and clinical staff and review specimen rejection practices.

Update on inherited disorders of haemostasis and pregnancy.

Inherited bleeding disorders have the potential to cause bleeding complications during pregnancy, childbirth and the postpartum period as well as effect fetal outcomes. There is an evolving understanding of the need for specialised and individualised care for affected women during these times. The aim for each patient is to estimate the risk to mother, fetus and neonate; to implement measures to minimise these risks; and to anticipate complications and develop contingencies for these scenarios. This includes accurate diagnosis, preconceptual care, prenatal diagnostic options, antenatal care, delivery and postpartum care as well as care of an affected neonate. An understanding of the physiologic haemostatic changes associated with pregnancy as well as the scope of defects, inheritance and management of inherited bleeding disorders is paramount when caring for these women. Collaborative and prospective management in conjunction with haematology services underpins the approach advocated. This review draws on the available literature, and outlines the principles of care for women with inherited bleeding disorders before, during and after pregnancy, as well as their babies, based on both available data and collective clinical experience.