Children With Medical Complexity: The 10-Year Experience of a Single Center.

Children with medical complexity (CMC) have chronic, multisystem health conditions, substantial health care needs, major functional limitations, and high resource use. They represent <1% of US children yet account for more than one-third of total pediatric health care costs. Health care systems designed for typical children do not meet the unique needs of CMC. In this special article, we describe the experience of our Comprehensive Care Program for CMC in a pediatric tertiary care center, from its launch in 2007 to its present model. We review the literature, describe our collective lessons learned, and offer suggestions for future directions.

Contextual Factors Influencing Implementation of Evidence-Based Care for Children Hospitalized With Asthma.

BACKGROUND AND OBJECTIVES: The translation of research findings into routine care remains slow and challenging. We previously reported successful implementation of an asthma evidence-based care process model (EB-CPM) at 8 (1 tertiary care and 7 community) hospitals, leading to a high health care provider (HCP) adherence with the EB-CPM and improved outcomes. In this study, we explore contextual factors perceived by HCPs to facilitate successful EB-CPM implementation. METHODS: Structured and open-ended questions were used to survey HCPs (n = 260) including physicians, nurses, and respiratory therapists, about contextual factors perceived to facilitate EB-CPM implementation. Quantitative analysis was used to identify significant factors (correlation coefficient ≥0.5; P ≤ .05) and qualitative analysis to assess additional facilitators. RESULTS: Factors perceived by HCPs to facilitate EB-CPM implementation were related to (1) inner setting (leadership support, adequate resources, communication and/or collaboration, culture, and previous experience with guideline implementation), (2) intervention characteristics (relevant and applicable to the HCP's practice), (3) individuals (HCPs) targeted (agreement with the EB-CPM and knowledge of supporting evidence), and (4) implementation process (participation of HCPs in implementation activities, teamwork, implementation team with a mix of expertise and professional's input, and data feedback). Additional facilitators included (1) having appropriate preparation and (2) providing education and training. CONCLUSIONS: Multiple factors were associated with successful EB-CPM implementation and may be used by others as a guide to facilitate implementation and dissemination of evidence-based interventions for pediatric asthma and other chronic diseases in the hospital setting.

Quality Improvement Initiative to Improve Abuse Screening Among Infants With Extremity Fractures.

OBJECTIVES: The aim of this study was to evaluate the effectiveness of clinical pathway implementation and quality improvement (QI) interventions to increase the percentage of infants with extremity fractures undergoing evaluation for suspected physical abuse, including skeletal survey (SS), and consultation with social work, and/or Child Protection Team. METHODS: Charts were retrospectively reviewed to establish percentage of infants less than 12 months old with extremity fractures undergoing an SS and consultation during the prepathway (January 1, 2012 to December 31, 2013) and postpathway (January 1, 2014 to June 30, 2015) periods. Using an Ishikawa framework, key process drivers were identified and additional QI interventions (clinical decision support and provider education) were developed and implemented. Impact of QI interventions on study metrics during active QI (July 1, 2015 to June 30, 2016) and post-QI periods (July 1, 2016 to December 31, 2016) was monitored using statistical process control charts. Logistic regression assessed predictors of obtaining an SS, consultation use, and occult fracture detection. RESULTS: Skeletal survey use pre- and postpathway averaged 40%, surpassing 60% on average during active QI and post-QI periods. Consultation performance averaged 46% pre- and postpathway, increasing to nearly 67% during active QI; consultation performance decreased during post-QI to 60%. A lack of trauma history and presence of femur or humerus fracture were associated with increased SS use and consultation (both P < 0.001). Overall 20% of SS revealed occult fractures.

Acquired von Willebrand Syndrome Associated to Secondary IgM MGUS Emerging after Autologous Stem Cell Transplantation for AL Amyloidosis.

Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic disorder that occurs in patients with no prior personal or family history of bleeding. Here, we describe a case of AVWS occurring after autologous stem cell transplantation (ASCT). Interestingly, AVWS developed after bortezomib-based induction and conditioning regimens. Recent evidence suggests that the proximity of the bortezomib therapy to the collection of stem cells with consequent depletion of regulatory T cells after the conditioning regimen could explain some of the unusual autoimmune complications reported in patients receiving bortezomib prior to ASCT. In addition, this patient developed a secondary MGUS post-ASCT, which may have also contributed to the AVWS. To the best of our knowledge, this is the first case of post-ASCT AVWS reported. Prospective data is needed to better elucidate the mechanisms by which these unusual complications occur in patients receiving bortezomib prior to ASCT.

Improving Pediatric Asthma Care and Outcomes Across Multiple Hospitals.

BACKGROUND AND OBJECTIVES: Gaps exist in inpatient asthma care. Our aims were to assess the impact of an evidence-based care process model (EB-CPM) 5 years after implementation at Primary Children's Hospital (PCH), a tertiary care facility, and after its dissemination to 7 community hospitals. METHODS: Participants included asthmatics 2 to 17 years admitted at 8 hospitals between 2003 and 2013. The EB-CPM was implemented at PCH between January 2008 and March 2009, then disseminated to 7 community hospitals between January and June 2011. We measured compliance using a composite score (CS) for 8 quality measures. Outcomes were compared between preimplementation and postimplementation periods. Confounding was addressed through multivariable regression analyses. RESULTS: At PCH, the CS increased and remained at >90% for 5 years after implementation. We observed sustained reductions in asthma readmissions (P = .026) and length of stay (P < .001), a trend toward reduced costs (P = .094), and no change in hospital resource use, ICU transfers, or deaths. The CS also increased at the 7 community hospitals, reaching 80% to 90% and persisting >2 years after dissemination, with a slight but not significant readmission reduction (P = .119), a significant reduction in length of stay (P < .001) and cost (P = .053), a slight increase in hospital resource use (P = .032), and no change in ICU transfers or deaths. CONCLUSIONS: Our intervention resulted in sustained, long-term improvement in asthma care and outcomes at the tertiary care hospital and successful dissemination to community hospitals.

Parent perceptions of quality of life and healthcare satisfaction for children with medical complexity.

PURPOSE: To describe parent perceptions of their child's quality of life (QOL) and their satisfaction with health care for a group of children with medical complexity (CMC), and to determine whether parent perceptions of child well-being are associated with QOL and health care satisfaction. METHODS: Participants were parents or legal guardians of children enrolled in a novel program of intensive outpatient care for CMC. Participants completed 7-item questionnaires to ascertain their perceptions of their child's well-being, QOL and health care satisfaction. RESULTS: One hundred and ninety-one participants completed questionnaires (response rate 100%). 85% rated their child's QOL as excellent, very good, or good. 87% reported satisfaction with their child's health care. Fair or poor mental health was associated with fair or poor QOL (OR 1.09, p= 0.0002). More pain was associated with lower QOL (OR 1.07, p= 0.0022). Fewer days of play or school attendance were associated with lower satisfaction with health care (OR 1.09, p= 0.0003). CONCLUSION: Our study suggests that more pain and worse mental health are associated with parental perceptions of the quality of life for their CMC. Clinicians who care for these children and their families should carefully and repeatedly assess for these important symptoms.

Emergency department use among children with tracheostomies: Avoidable visits.

PURPOSE: To characterize high emergency department (ED) use by children with tracheostomies and complex chronic conditions, to distinguish avoidable from unavoidable ED visits, and to describe the financial impact of avoidable visits. METHODS: Children with tracheostomies in a pediatric tertiary care center with the highest ED utilization were identified via analysis of administrative data. Six experts in interdisciplinary dyads reviewed the records from all ED visits for these children, and distinguished avoidable from unavoidable visits. Hospital cost data for avoidable visits is described. RESULTS: Among 75 children with tracheostomies and complex chronic conditions, 23 (31%) were high ED utilizers. These 23 children accounted for 74% of all ED discharges the total group of 75 children from 2008 to 2011. Four of these 23 children with high utilization were excluded, leaving 19 subjects for review. These 19 children had 312 ED visits, of which 103 (33%) were deemed avoidable. Leading reasons for avoidable visits were uncomplicated upper respiratory infections, gastrointestinal infections, and enteral feeding system problems. Avoidable visits cost the hospital {$}67,940. CONCLUSIONS: One-third of ED visits by children with tracheostomies and complex chronic conditions may be avoidable. Increased ambulatory access to interdisciplinary teams of providers familiar with these children's unique needs might reduce avoidable ED visits and improve health outcomes. Further studies on how this model of ambulatory care might affect ED utilization and total healthcare costs are needed.

Adult sickle cell disease epidemiology and the potential role of a multidisciplinary comprehensive care center in a city with low prevalence.

The aim of this study was to determine the characteristics of the sickle cell disease population in a city of low prevalence and compare them to those reported in the literature. We performed a retrospective cross-sectional study of all sickle cell disease patients seen in the Calgary Health Region, Calgary, Alberta, Canada from 2006 to 2010. Data on clinical endpoints including emergency department (ED) visits, hospital admissions, transfusions, as well as laboratory parameters were collected. A total of 37 adult sickle cell disease patients were identified. Over 5 years, they were represented by a total of 49.2 ED presentations/year, 29.2 (59.0%) of these requiring admission. Eighty-three percent of these presentations were for acute pain episodes. We concluded that the number of ED visits, hospital admissions and several other parameters in our cohort were similar to those in other centers of higher prevalence. This suggests that guidelines representing regions of high prevalence may be applicable to smaller centers, where patients experience similar clinical outcomes.

Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status.

We investigated a case of acquired von Willebrand syndrome (AVWS) secondary to a nonneutralizing anti-von Willebrand factor (VWF) antibody associated with an autoimmune disorder. At diagnosis, VWF activity (VWF:Act), antigen (VWF:Ag), multimers, and factor VIII coagulant activity were virtually absent. VWF propeptide (VWFpp) was elevated with an infinitely high VWFpp to VWF:Ag ratio (VWFpp:Ag) consistent with rapid VWF clearance. Immunosuppressive treatment resulted in phenotypic remission 1 with normalization of VWF/factor VIII levels and multimer pattern. However, VWFpp:Ag remained elevated (∼2× normal), consistent with ongoing VWF clearance by the remaining anti-VWF antibody still present by enzyme-linked immunosorbent assay. This suggests that increased VWF secretion was compensating for the incomplete remission state. Relapse occurred when VWFpp:Ag was again infinitely high, with associated decreased VWFpp but unchanged anti-VWF titers; switching the balance to favor VWF clearance over secretion. Complete remission with undetectable anti-VWF occurred only when VWFpp:Ag was normal. This case of relapsing-remitting AVWS demonstrates the use of VWFpp:Ag for predicting remission status.

Costs and infant outcomes after implementation of a care process model for febrile infants.

OBJECTIVE: Febrile infants in the first 90 days may have life-threatening serious bacterial infection (SBI). Well-appearing febrile infants with SBI cannot be distinguished from those without by examination alone. Variation in care resulting in both undertreatment and overtreatment is common. METHODS: We developed and implemented an evidence-based care process model (EB-CPM) for the management of well-appearing febrile infants in the Intermountain Healthcare System. We report an observational study describing changes in (1) care delivery, (2) outcomes of febrile infants, and (3) costs before and after implementation of the EB-CPM in a children's hospital and in regional medical centers. RESULTS: From 2004 through 2009, 8044 infants had 8431 febrile episodes, resulting in medical evaluation. After implementation of the EB-CPM in 2008, infants in all facilities were more likely to receive evidence-based care including appropriate diagnostic testing, determination of risk for SBI, antibiotic selection, decreased antibiotic duration, and shorter hospital stays (P < .001 for all). In addition, more infants had a definitive diagnosis of urinary tract infection or viral illness (P < .001 for both). Infant outcomes improved with more admitted infants positive for SBI (P = .011), and infants at low risk for SBI were more often managed without antibiotics (P < .001). Although hospital admissions were shortened by 27%, there were no cases of missed SBI. Health Care costs were also reduced, with the mean cost per admitted infant decreasing from $7178 in 2007 to $5979 in 2009 (-17%, P < .001). CONCLUSIONS: The EB-CPM increased evidence-based care in all facilities. Infant outcomes improved and costs were reduced, substantially improving value.

Iliac vein compression syndrome in an active and healthy young female.

Iliac vein compression syndrome is a condition involving external compression of the left common iliac vein by the right iliac artery, which was first described in the 1850s. It predominates in females typically between the third and fourth decade of life and has been associated with thrombophilias. Importantly, the syndrome is amenable to endovascular treatment. Here, we describe a case of a young athletic female with an incidental finding of a left iliac vein thrombosis while taking oral contraceptives, who was identified as having iliac vein compression syndrome on follow-up MR venography with positive testing for Factor V Leiden mutation.

Life-threatening hemolytic anemia due to an autoanti-Pr cold agglutinin: evidence that glycophorin A antibodies may induce lipid bilayer exposure and cation permeability independent of agglutination.

BACKGROUND: The hemoglobin of a 29-year-old man fell below 35 g/L over 5 days, despite 14 units of red blood cells (RBCs), due to an anti-Pr cold agglutinin (CA). His hemolytic anemia necessitated respiratory support in intensive care for 4 weeks. STUDY DESIGN AND METHODS: The hemolysis was investigated by the effects on blood group-compatible RBCs of this anti-Pr and an anti-I CA and of a rabbit anti-human glycophorin A (GPA) immunoglobulin G (IgG) antibody on Ca(2+) permeability and of phosphatidylethanolamine (PE) exposure. 1) The anti-Pr CA (in a plasmapheresis product from the patient) was absorbed and eluted from RBC ghosts and its immunophenotype was determined by agarose electrophoresis and immunofixation. 2) Ca(2+) permeability was measured by the response of Fluo-3-labeled RBCs to addition of external Ca(2+). 3) Exposed PE was measured with streptavidin-labeled biotinylated peptide Ro 09-0198 (cinnamycin). RESULTS: 1) The patient's anti-Pr CA was a polyclonal IgG. 2) The anti-Pr and the rabbit anti-human glycophorin IgG, but not an anti-I CA, rapidly increased Ca(2+)-dependent fluorescence upon addition of external Ca(2+) in a fraction (15%-25%) of RBCs that also became positive for cinnamycin. 3) Trypsin treatment of RBCs reduced the Ca(2+) influx due to the anti-Pr IgG, but neither trypsin nor neuraminidase changed the responses to the rabbit anti-human GPA IgG. CONCLUSIONS: The anti-Pr CA and rabbit anti-human GPA increased exposure of PE and increased membrane Ca(2+) permeability that may have caused hemolysis. The difference in the responses to these antibodies to enzyme treatment of RBCs suggests that they react with different epitopes on GPA.

Influenza virus infection in infants less than three months of age.

OBJECTIVE: We evaluated the presentation, outcomes, and the risk of serious bacterial infection (SBI) in infants <3 months old with influenza virus infection. PATIENTS AND METHODS: We identified demographic, hospitalization, and microbiologic data from computerized medical records for all infants and children <24 months of age, with laboratory confirmed influenza infection cared for at a tertiary care children's hospital during 4 winter seasons (2004-2008). We compared those <3 months of age with older groups. RESULTS: We identified 833 children <24 months of age with laboratory-confirmed influenza. Of those, 218 were <3 months old. Influenza accounted for 3.6% of all evaluations of febrile infants and 12% of febrile infant encounters during winter. Infants <3 months of age were less likely to have a high risk chronic medical condition, but were more likely to be hospitalized than children 3 to <24 months old (P < 0.005). Infants <3 months with influenza had fewer prolonged hospital stays than those 3 to <6 months old [P = 0.056; OR: 0.5 (0.24-1.0)] and 6 to <12 months old [P = 0.011; OR: 0.43 (0.24-0.83)]. Five (2.3%) infants <3 months old had SBI. CONCLUSIONS: Infants <3 months of age with influenza virus infection often present with fever alone. Although they are more likely to be hospitalized than those 3 to <24 months old, hospital stays are short and outcomes generally good. Infants with influenza virus infection have a low risk of concomitant SBI.

Nerve-sparing prostatectomy and urinary function: a prospective analysis using validated quality-of-life measures.

OBJECTIVES: To prospectively study whether urinary function and bother are directly related to neurovascular bundle preservation at radical prostatectomy using validated quality-of-life questionnaires. METHODS: A total of 91 consecutive patients undergoing radical prostatectomy were prospectively studied using the University of California, Los Angeles, Prostate Cancer Index and the International Prostate Symptom Score. The patients were divided into 2 groups (>50% nerve sparing vs 50% nerve-sparing during prostatectomy. Their mean age at surgery was 59 +/- 6 years, and the mean follow-up was 16 +/- 9 months. Of the 91 patients, 29 (32%) had

Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema.

BACKGROUND: We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) in 2004. OBJECTIVE: To ensure that this consensus remains current. METHODS: In collaboration with the Canadian Network of Rare Blood Disorder Organizations, we held the second Canadian Consensus discussion with our international colleagues in Toronto, Ontario, on February 3, 2006, and reviewed its content at the Fifth C1 Inhibitor Deficiency Workshop in Budapest on June 2, 2007. Papers were presented by international investigators, and this consensus algorithm approach resulted. RESULTS: This consensus algorithm outlines the approach recommended for the diagnosis, therapy, and management of HAE, which was agreed on by the authors of this report. This document is only a consensus algorithm approach and continues to require validation. As such, participants agreed to make this a living 2007 algorithm, a work in progress, and to review its content at future international HAE meetings. CONCLUSIONS: There is a paucity of double-blind, placebo-controlled trials on the treatment of HAE, making levels of evidence to support the algorithm less than optimal. Controlled trials currently under way will provide further insight into the management of HAE. As with our Canadian 2003 Consensus, this 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of HAE was formed through the meeting and agreement of patient care professionals along with patient group representatives and individual patients.

Chronic opioid use is a risk factor for the development of central sleep apnea and ataxic breathing.

BACKGROUND: Chronic opioid therapy for pain management has increased dramatically without adequate study of potential deleterious effects on breathing during sleep. METHODS: A retrospective cohort study comparing 60 patients taking chronic opioids matched for age, sex, and body mass index with 60 patients not taking opioids was conducted to determine the effect of morphine dose equivalent on breathing patterns during sleep. RESULTS: The apnea-hypopnea index was greater in the opioid group (43.5/h vs 30.2/h, p < .05) due to increased central apneas (12.8/h vs 2.1/h; p < .001). Arterial oxygen saturation (SpO2) in the opioid group was significantly lower during both wakefulness (difference 2.1%, p < .001) and non-rapid eye movement (NREM) sleep (difference 2.2%, p < .001) but not during rapid eye movement (REM) sleep (difference 1.2%) than in the nonopioid group. Within the opioid group, and after controlling for body mass index, age, and sex, there was a dose-response relationship between morphine dose equivalent and apnea-hypopnea (p < .001), obstructive apnea (p < .001), hypopnea (p < .001), and central apnea indexes (p < .001). Body mass index was inversely related to apnea-hypopnea index severity in the opioid group. Ataxic or irregular breathing during NREM sleep was also more prevalent in patients who chronically used opioids (70% vs 5.0%, p < .001) and more frequent (92%) at a morphine dose equivalent of 200 mg or higher (odds ratio = 15.4, p = .017). CONCLUSIONS: There is a dose-dependent relationship between chronic opioid use and the development of a peculiar pattern of respiration consisting of central sleep apneas and ataxic breathing. Although potentially significant, the clinical relevance of these observations remains to be established.

Apolipoprotein E genotype and response of carbon monoxide poisoning to hyperbaric oxygen treatment.

RATIONALE: Hyperbaric oxygen (HBO2) reduced the incidence of cognitive sequelae 6 weeks after carbon monoxide (CO) poisoning compared with normobaric oxygen (NBO2). The apolipoprotein (APOE) epsilon4 allele predicts unfavorable neurologic outcome after brain injury and stroke. OBJECTIVES: To assess the effects of the epsilon4 allele on 6-week cognitive sequelae after CO poisoning. METHODS: We tested APOE genotypes in 86 of 152 CO-poisoned patients from our randomized trial. Logistic regression was used to control for risk factors while testing for effects with the epsilon4 allele or interactions with epsilon4 and treatment on 6-week and 6- and 12-month cognitive sequelae. MEASUREMENTS AND MAIN RESULTS: We enrolled 86 patients: 44 received HBO2 and 42 NBO2 therapy. A total of 31 (36%) patients had at least one epsilon4 allele. Six-week cognitive sequelae rates for patients treated with HBO2 and NBO2, respectively: epsilon4 allele absent, 11% (3/27) and 43% (12/28); epsilon4 allele present, 35% (6/17) and 29% (4/14). The epsilon4 allele was not associated with 6-week cognitive sequelae, 27% (15/55) without and 32% (10/31) with the epsilon4 allele (P = 0.323). The interaction between the epsilon4 allele and treatment was significantly associated with 6-week cognitive sequelae (P = 0.048). The interaction between the epsilon4 allele and treatment was not associated with 6- and 12-month cognitive sequelae. CONCLUSIONS: HBO2 therapy reduces cognitive sequelae after CO poisoning in the absence of the epsilon4 allele. Because apolipoprotein genotype is unknown at the time of poisoning, we recommend that patients with acute CO poisoning receive HBO2.

Carbon monoxide poisoning: risk factors for cognitive sequelae and the role of hyperbaric oxygen.

RATIONALE: Carbon monoxide poisoning is common and causes cognitive sequelae. Hyperbaric oxygen (HBO(2)) reduces cognitive sequelae incidence, but which patients may benefit from HBO(2) is unclear. OBJECTIVES: Risk factor determination for 6-wk cognitive sequelae from CO poisoning and risk modification with HBO(2). METHODS: Patients were from a randomized controlled trial, enrolling acutely CO-poisoned patients more than 15 years of age. Patients eligible but not enrolled in the randomized trial, and not receiving HBO(2), were followed during the study interval. In patients not receiving HBO(2), we performed univariate analyses including risk factors identified by randomized trial subgroup analyses. A multivariable analysis was performed using univariate results with and without HBO(2). MEASUREMENTS AND MAIN RESULTS: In 163 patients not receiving HBO(2), 68 (42%) manifested sequelae. Risk factors for sequelae from subgroup analyses were loss of consciousness, age of 36 years or more, and carboxyhemoglobin levels greater than or equal to 25%. By univariate analyses, risks for sequelae were age of 36 years or more (odds ratio [OR], 2.6; 95% confidence interval [CI], 1.3-4.9; P = 0.005), and exposure intervals greater than or equal to 24 hours (OR, 2.4; 95% CI, 1.2-4.8; P = 0.019). Including 75 patients receiving HBO(2), cognitive sequelae was reduced in patients age of 36 years or more (OR, 0.3; 95% CI, 0.2-0.6; P < 0.001). Exposure intervals greater than or equal to 24 hours are an independent risk factor for sequelae (OR, 2.0; 95% CI, 1.0-3.8; P = 0.046). CONCLUSIONS: HBO(2) oxygen is indicated for patients with acute CO poisoning who are 36 years or older or have exposure intervals greater than or equal to 24 hours. In addition, subgroup analyses support that patients with loss of consciousness or higher carboxyhemoglobin levels warrant HBO(2).

The impact of a community-based food skills intervention on cooking confidence, food preparation methods and dietary choices - an exploratory trial.

OBJECTIVE: To evaluate the feasibility of undertaking a food skills intervention study in areas of social deprivation aimed at altering cooking confidence, food preparation methods and dietary choices. DESIGN: A standardised skills programme was implemented in community-based settings. Pre- (T1) and post-intervention (T2) and 6-month follow-up (T3) measures (7-day diaries and self-administered questionnaires) were undertaken in intervention and comparison groups. SETTING: Eight urban communities in Scotland. SUBJECTS: One hundred and thirteen adults living in areas of social deprivation. RESULTS: It was clear that many subjects led fragmented lives and found commitment to intervention classes problematic. Sixty-three subjects completed the final (T3) assessments. The response to each component varied due to inability to attend sessions, illness, study requirements, employment, moving out of the area, change in circumstances, loss of interest and loss of postal questionnaires. At baseline, reported consumption of fruit and vegetables was low (mean frequency 8.1 +/- 4.78 times per week). Fruit intake increased significantly (P < 0.05) between T1 and T2 in the intervention group (1.7 +/- 2.36 to 2.7 +/- 3.28 times per week) only. Between T1 and T3, there was a significant increase (P < 0.05) in intervention subjects who reported confidence in following a recipe (67-90%,). CONCLUSIONS: This exploratory trial shows that a food skills intervention is likely to have a small but positive effect on food choice and confidence in food preparation. A full-scale randomised controlled trial in this hard-to-reach group would require a range of flexible approaches rather than a fully defined intervention, and presents challenges for trial design.

Upfront double high-dose chemotherapy with DICEP followed by BEAM and autologous stem cell transplantation for poor-prognosis aggressive non-Hodgkin lymphoma.

A single center, prospective clinical trial was conducted evaluating 2 cycles of induction high-dose chemotherapy for adults younger than 65 years of age with aggressive non-Hodgkin lymphoma (NHL) and 2 to 3 Age-Adjusted International Prognostic Index risk factors. Patients received one cycle of standard dose cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) followed by one cycle of dose-intensive cyclophosphamide 5.25 g/m(2), etoposide 1.05 g/m(2), cisplatin 105 mg/m(2) (DICEP), then underwent autologous blood stem cell collection, followed by one cycle of high-dose carmustine (BCNU) 300 mg/m(2), etoposide 800 mg/m(2), Ara-C 1600 mg/m(2), melphalan 140 mg/m(2) (BEAM), and autologous stem cell transplantation (ASCT) and radiotherapy to prior bulk. From June 1998 to August 2004, 55 patients aged 20 to 63 years (median 44 years) were accrued, 51 (92%) of whom had diffuse large B-cell NHL. Poor prognostic factors included stage 4 (n = 46), elevated lactate dehydrogenase (LDH; n = 47), Eastern Cooperative Oncology Group (ECOG) performance status 2 to 4 (n = 43), bulky mass more than 10 cm (n = 34), and marrow involvement (n = 16). Only one patient experienced nonrelapse mortality. With a median follow-up of 49 months, 4-year event-free survival (EFS) and overall survival (OS) rates for all 55 patients are 72% (95% confidence interval [CI] = 60%-84%) and 79% (95% CI = 69%-90%), respectively. In conclusion, CHOP-DICEP-BEAM is feasible and gave encouraging EFS and OS for patients with poor-prognosis aggressive NHL.