Tuesday, March 3, 2015

Sickle cell in the Medical Literature | March - April 2015

1.
Thromb Res. 2015 Feb;135 Suppl 1:S46-8. doi: 10.1016/S0049-3848(15)50442-8. Epub 2015 Feb 9.

Abstract
Recent data strongly suggest an increased risk of venous thromboembolism in subjects with sickle cell disease and to a lesser extent, sickle cell trait. However, most studies have been retrospective, case-control or cross-sectional based on data obtained from administrative databases. More data from adequately powered prospective studies that include matched controls are needed to definitely establish the link between venous thromboembolism during pregnancy and sickle hemoglobin disorders. Similarly, there remains a need for properly designed randomized control trials to establish the safety of various hormonal contraceptive methods in women with sickle cell disorders. 
© 2015 Elsevier Ltd. All rights reserved.

PMID: 25903535 [PubMed - in process]



2.
Br J Haematol. 2015 Apr 19. doi: 10.1111/bjh.13424. [Epub ahead of print]
Abstract
Blood transfusions can induce alloantibodies to antigens on red blood cells (RBCs), white blood cells and platelets, with these alloantibodies affecting transfusion and transplantation. While transfusion-related alloimmunization against RBC antigens and human leucocyte antigens (HLA) have been studied, transfusion-related alloimmunization to minor histocompatibility antigens (mHA), such as H-Y antigens, has not been clinically characterized. We conducted a cross-sectional study of 114 children with sickle cell disease (SCD) and tested for antibodies to 5 H-Y antigens and to HLA class I and class II. Few patients had H-Y antibodies, with no significant differences in the prevalence of any H-Y antibody observed among transfused females (7%), transfused males (6%) and never transfused females (4%). In contrast, HLA class I, but not HLA class II, antibodies were more prevalent among transfused than never transfused patients (class I: 33% vs. 13%, P = 0·046; class II: 7% vs. 8%, P = 0·67). Among transfused patients, RBC alloantibody history but not amount of transfusion exposure was associated with a high (>25%) HLA class I panel reactive antibody (Odds ratio 6·8, 95% confidence interval 2·1-22·3). These results are consistent with immunological responder and non-responder phenotypes, wherein a subset of patients with SCD may be at higher risk for transfusion-related alloimmunization.
© 2015 John Wiley & Sons Ltd.

PMID: 25891976 [PubMed - as supplied by publisher]


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3.
Adv Skin Wound Care. 2015 May;28(5):206-10. doi: 10.1097/01.ASW.0000462327.30245.ae.
Abstract
OBJECTIVE:
Refractory leg ulcerations are common in homozygous sickle cell anemia. In this case series, patients were treated with transdermal continuous oxygen therapy (TCOT), based on the hypothesis that oxygen deprivation caused by arteriovenous shunting may be remedied by providing oxygen directly to the wound bed. The authors believe this is the first attempt to treat sickle cell ulcers with TCOT.
CASE PRESENTATION:
Five patients with long histories of recurring sickle cell disease ulcers that would not heal with various conventional and/or other adjunctive wound healing modalities were treated with TCOT. The patients had recurring nonhealing wounds for 30, 21, 20, 20, and 15 years, respectively. All 5 patients healed or showed substantial improvement in the treatment periods of 3 to 36 weeks.
CONCLUSION:
The authors conclude that TCOT may be a novel, effective, and inexpensive modality in treating patients with sickle cell disease ulcers. Improvement was typically noticeable within 2 weeks. Further clinical trials may be considered to evaluate the efficacy of TCOT in sickle cell ulcers.

PMID: 25882658 [PubMed - in process]


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4.
BMC Psychiatry. 2015 Apr 11;15:78. doi: 10.1186/s12888-015-0461-6.
bstract
BACKGROUND:
The majority of available studies have shown that children with sickle cell disease (SCD) have a higher risk of depressive symptoms than those without. The present study aimed to: assess the prevalence of depression in a sample of children with SCD; evaluate the association between disease severity, social support and depression, and the combined and/or singular effect on health-related quality of life (HRQL) in children with SCD; and show the predictive value of social support and disease severity on depression.
METHODS:
A total of 120 children were included in the study, 60 (group I) with SCD and 60 matched, healthy control children (group II). Depression was assessed in both groups using the Children's Depression Inventory (CDI) and the Children's Depression Inventory-Parent (CDI-P). Children with CDI and CDI-P scores of more than 12 were interviewed for further assessment of depression using the Diagnostic Interview Schedule for Children Version IV (DISC-IV). The Pediatric Quality of Life Inventory Version 4.0 Generic Core Scales (PedsQL 4.0) was used to assess HRQL in both groups, and social support was measured with the Child and Adolescent Social Support Scale (CASSS).
RESULTS:
Eight (13%) of the 60 children with SCD had CDI and CDI-P scores of more than 12 (CDI mean score 14.50 ± 1.19, CDI-P mean score 14.13 ± 1.12), and were diagnosed as having clinical depression using the diagnostic interview DISC-IV. For group I, HRQL was poor across all PedsQL 4.0 domains in both self- and parent-reports (P < 0.001) compared with group II. A higher level of parent support was a significantly associated with decreased depressive symptoms, demonstrated by lower CDI scores. Better quality of life was shown by the associated higher total PedsQL 4.0 self-scores of children with SCD (B = -1.79, P = 0.01 and B = 1.89, P = 0.02 respectively).
CONCLUSIONS:
The present study demonstrates that higher levels of parent support were significantly associated with decreased depressive symptoms and better quality of life in children with SCD. Interventions focused on increasing parent support may be an important part of treatment for depression in children with SCD.
PMCID: PMC4394397 Free PMC Article 

PMID: 25880537 [PubMed - in process]


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5.
Pak J Med Sci. 2015 Jan-Feb;31(1):203-8. doi: 10.12669/pjms.311.4104.
Abstract
OBJECTIVE:
The purpose of this study was to determine the impact of mean platelet volume (MPV) on the frequency and severity of vaso-occlusive and cerebrovascular events in patients with sickle cell anemia (SCA).
METHODS:
The 238 cases diagnosed with SCA were evaluated retrospectively with respect to the occurrence of painful crisis for the previous year. The incidence, severity and type of the vaso-occlusive crises of the patients with SCA between March 2010 and March 2011 were recorded. The last MPV values in patients who were free of erythrocyte transfusion for the last three months and who had no current vaso-occlusive crises were evaluated. All the patients were grouped according to the frequency of the crises for the previous year preceding the data collection. Group 1: 1 to 3 crises, Group 2: 4 to 5 and Group 3: 6 or more crises annually.
RESULTS:
In accordance with the results obtained during the evaluation of the cases diagnosed with sickle-cell anemia, MPV value was found to be significantly higher in patients with cerebrovascular events. Also MPV values increased with increasing incidence of the crises (r=0.297) (p=0.001).
CONCLUSION:
One of the contributing factors for this clinical heterogeneity may be related to the MPV values in patients with sickle cell anemia. The higher MPV values may be an early predictor of future cerebrovascular events in patients with sickle cell anemia and may require close follow-up and additional measures.
PMCID: PMC4386187 Free PMC Article 

PMID: 25878644 [PubMed]


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6.
West Indian Med J. 2015 Mar 5;63(7). doi: 10.7727/wimj.2014.012. [Epub ahead of print]
Author information:
Abstract
OBJECTIVE:
To determine the clinical factors associated with the length of hospitalization and mortality in patients with sickle cell disease (SCD).
METHODS:
All patients with SCD admitted to the medical wards of the University Hospital of the West Indies, Jamaica, over a five-year period, January 1 to December 31, 2010, were reviewed. Data were extracted from hospital charts and comprised demographic and clinical information, investigations, interventions, duration of stay, pathological data and outcomes.
RESULTS:
There were 105 patients reviewed; 84% were genotype Hb SS. Females accounted for 59% and males 41%. Overall mean age was 32.5 years (SD 13.7, range 12-66 years). The mean length of hospitalization was 10.2 days (SD 10.9, range 1-84 days). The main admission diagnoses were painful crisis, acute chest syndrome, severe anaemia, sepsis, hepatic sequestration, congestive cardiac failure and renal failure. The mean value for the following laboratory investigations were: haemoglobin 7.7 g/dL (SD 2.8), total white blood cell count 21.7 × 109/L (SD 14.2), platelet count 320 × 109/L (SD 191.9), blood urea 9.8 mmol/L (SD 11.9) and serum creatinine 198 umol/L (SD 267.9). Medical interventions included: blood transfusions in 20.9%, 55% received antibiotics and 74% received narcotic analgesia. There were 40 deaths with four autopsies done. The mortality rate for SCD was 38%. There were 189 repeat SCD admissions.
CONCLUSION:
Sickle cell disease still carries a high morbidity and mortality in patients admitted to hospital. Recurrent admissions are a concern, as they impact on patient's morbidity and quality of life.

PMID: 25867578 [PubMed - as supplied by publisher]




7.
Br J Haematol. 2015 Apr 9. doi: 10.1111/bjh.13447. [Epub ahead of print]
Author information:
  1Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Abstract
Many studies report estimated pulmonary artery systolic pressure (ePASP) in patients with sickle cell disease (SCD) screened by echocardiography. To better understand the prevalence and outcomes of elevated ePASP in clinically stable SCD patients, we conducted a random-effects meta-analysis. A total of 45 studies, representing 15 countries and including 6109 individuals, met our inclusion criteria. In most (70%) studies, elevated ePASP was defined by a tricuspid regurgitant velocity of 2·5 m/s. The prevalence of elevated ePASP was 21% (17-26%) in children and 30% (26-35%) in adults. After adjustment for sex, SCD genotype, haemoglobin, hydroxycarbamide (hydroxyurea) treatment, country and publication year, age remained associated with elevated ePASP, yielding a 12% (0·4-23%) higher adjusted prevalence in adults. Few studies reported 6-min walk tests or mortality outcomes, and estimates were highly heterogeneous. In random effects meta-analyses, patients with elevated ePASP walked an estimated 30·4 (6·9-53·9) metres less than those without elevated ePASP and had an associated mortality hazard ratio of 4·9 (2·4-9·7).
© 2015 John Wiley & Sons Ltd.

PMID: 25854714 [PubMed - as supplied by publisher]


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8.
Physiol Rep. 2015 Apr;3(4). pii: e12338. doi: 10.14814/phy2.12338.

Abstract
Physiologic contributors to reduced exercise capacity in individuals with sickle cell anemia (SCA) are not well understood. The objective of this study was to characterize the cardiopulmonary response to maximal cardiopulmonary exercise testing (CPET) and determine factors associated with reduced exercise capacity among children and young adults with SCA. A cross-sectional cohort of 60 children and young adults (mean 15.1 ± 3.4 years) with hemoglobin SS or S/β(0) thalassemia and 30 matched controls (mean 14.6 ± 3.5 years) without SCA or sickle cell trait underwent maximal CPET by a graded, symptom-limited cycle ergometry protocol with breath-by-breath, gas exchange analysis. Compared to controls without SCA, subjects with SCA demonstrated significantly lower peak VO2 (26.9 ± 6.9 vs. 37.0 ± 9.2 mL/kg/min, P < 0.001). Subjects demonstrated slower oxygen uptake (ΔVO2/ΔWR, 9 ± 2 vs. 12 ± 2 mL/min/watt, P < 0.001) and lower oxygen pulse (ΔVO2/ΔHR, 12 ± 4 vs. 20 ± 7 mL/beat, P < 0.001) as well as reduced oxygen uptake efficiency (ΔVE/ΔVO2, 42 ± 8 vs. 32 ± 5, P < 0.001) and ventilation efficiency (ΔVE/ΔVCO2, 30.3 ± 3.7 vs. 27.3 ± 2.5, P < 0.001) during CPET. Peak VO2 remained significantly lower in subjects with SCA after adjusting for age, sex, body mass index (BMI), and hemoglobin, which were independent predictors of peak VO2 for subjects with SCA. In the largest study to date using maximal CPET in SCA, we demonstrate that children and young adults with SCA have reduced exercise capacity attributable to factors independent of anemia. Complex derangements in gas exchange and oxygen uptake during maximal exercise are common in this population.
© 2015 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of the American Physiological Society and The Physiological Society.
Free Article

PMID: 25847915 [PubMed]


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9.
Cochrane Database Syst Rev. 2015 Apr 6;4:CD004448. [Epub ahead of print]
Author information:
  1Paediatrics Department, National Hospital, Plot 132 Central District (Phase II), PMB 425 Garki, Abuja, Nigeria.
Abstract
BACKGROUND:
Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines (medicine derived from plants in their original state) encompass many of the plant remedies from traditional healers which the populations most affected would encounter. There has been little systematic appraisal of their benefits. This is an update of a Cochrane Review first published in 2010 and updated in 2013.
OBJECTIVES:
To assess the benefits and risks of phytomedicines in people with sickle cell disease of all types, of any age, in any setting.
SEARCH METHODS:
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, the International Standard Randomised Controlled Trial Number Register (ISRCTN) and the Allied and Complimentary Medicine Database (AMED).Dates of most recent searches: Haemoglobinopathies Trials Register: 13 October 2014; ISRCTN: 17 January 2015; AMED: 20 January 2015.
SELECTION CRITERIA:
Randomised or quasi-randomised trials with participants of all ages with sickle cell disease, in all settings, comparing the administration of phytomedicines, by any mode to placebo or conventional treatment, including blood transfusion and hydroxyurea.
DATA COLLECTION AND ANALYSIS:
Both authors independently assessed trial quality and extracted data.
MAIN RESULTS:
Two trials (182 participants) and two phytomedicines Niprisan® (also known as Nicosan®) and Ciklavit® were included. The Phase IIB (pivotal) trial suggests that Niprisan® was effective in reducing episodes of severe painful sickle cell disease crisis over a six-month period. It did not affect the risk of severe complications or the level of anaemia. No serious adverse effects were reported. The single trial of Cajanus cajan (Ciklavit®) reported a possible benefit to individuals with painful crises, and a possible adverse effect (non-significant) on the level of anaemia.
AUTHORS' CONCLUSIONS:
While Niprisan® appeared to be safe and effective in reducing severe painful crises over a six-month follow-up period, further trials are required to assess its role in the management of people with sickle cell disease and the results of its multicentre trials are awaited. Currently no conclusions can be made regarding the efficacy of Ciklavit®. Based on the published results for Niprisan® and in view of the limitations in data collection and analysis of both trials, phytomedicines may have a potential beneficial effect in reducing painful crises in sickle cell disease. This needs to be further validated in future trials. More trials are required on the safety and efficacy of phytomedicines used in managing sickle cell disease.

PMID: 25844571 [PubMed - as supplied by publisher]


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10.
Creat Nurs. 2015;21(1):38-46.
Abstract
INTRODUCTION:
Sickle cell disease (SCD) is an inherited autosomal recessive disorder. In the United States, most individuals with SCD are African Americans, withan incidence of 1 in 400 to 1 in 500 live births. SCD is a lifelong disorder with no known cure.
BACKGROUND:
SCD causes anemia, frequent painful episodes, and reduced life ex- pectancy. The most disturbing clinical problem associated with SCD is severe pain episodes, the most common reason for hospitalization. Pharmacological interventions have been the mainstream for treatment; however, psychological interventions such as cognitive behavioral therapy (CBT) may complement current medical treatment, lead- ing to better coping and overall improved quality of life.
METHODS:
In a quasi-experimental one-group pretest-posttest study, 9 African American individuals with SCD completed 3 weekly educational sessions learning CBT methods.
RESULTS:
Participants demonstrated increased frequency of use of CBT methods post-intervention, including diverting attention, coping self-statements, and behavioral activities, leading to better pain control. However, quality of life and role limitation did not show significant improvement.
DISCUSSION:
CBT may be beneficial to those suffering from SCD when combined with conventional treatment options; however, there are still barriers to incorporating psychological interventions into practice.
CONCLUSIONS:
CBT shows promise for individuals with chronic conditions such as SCD, but more investigation into its efficacy is needed with larger sample sizes over longer periods of time.

PMID: 25842524 [PubMed - indexed for MEDLINE]




11.
J Pain. 2015 Apr 1. pii: S1526-5900(15)00603-3. doi: 10.1016/j.jpain.2015.03.010. [Epub ahead of print]

Abstract
This analysis examined the influence of quantifiable parameters of daily sleep continuity, primarily sleep duration and sleep fragmentation, on daily pain in adults with Sickle Cell Disease (SCD). Seventy-five adults with SCD completed baseline psychosocial measures and daily morning (sleep) and evening (pain) diaries over a three-month period. Mixed-effect modeling was used to examine daily between- and within-subjects effects of sleep continuity parameters on pain, as well as the synergistic effect of sleep fragmentation and sleep duration on pain. Results revealed nights of shorter sleep duration and time in bed, increased fragmentation, and less efficient sleep (relative to one's own mean) were followed by days of greater pain severity. Further, the analgesic benefit of longer sleep duration was attenuated when sleep fragmentation was elevated. These results suggest that both the separate and combined effects of sleep duration and fragmentation should be considered in evaluating pain in adults with SCD.
PERSPECTIVE:
Subjective parameters of sleep continuity (e.g. sleep duration, fragmentation, and efficiency) predict clinical pain in individuals with SCD. Additionally, sleep duration should not be considered in isolation and its association with pain may be qualified by sleep fragmentation. Research and practice should include assessments of both when addressing pain severity.
Copyright © 2015 American Pain Society. Published by Elsevier Inc. All rights reserved.

PMID: 25842346 [PubMed - as supplied by publisher]


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12.
Pediatr Emerg Care. 2015 Apr 1. [Epub ahead of print]
Author information:
  1From the *Department of Medicine, and †Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, MD.
Abstract
OBJECTIVE:
The aim of this study was to identify the factors associated with delays in treatment of sickle pain crisis in the pediatric emergency department with the goal of discerning whether earlier pain management is correlated with better clinical outcome.
METHODS:
This retrospective study examined data collected from clinical records of patients, aged 21 years or younger, who was treated for sickle cell pain crisis between January and June 2012. Demographic and clinical characteristics were extracted from electronic records, as well as time of registration, triage, initial pain assessment, analgesic administration, and pain reassessment.
RESULT:
A total of 160 sickle cell pain crises visits by 67 unique patients were identified. Opiates were the most common initial pain medication prescribed and administered. The mean time to initial analgesic administration and pain reassessment was 89 and 60 minutes, respectively. Patients with orders for imaging studies experienced significant delays in time to initial analgesic medication and pain reassessment. In addition, higher triage pain score correlated with shorter time to first dose of pain medication. However, age, sex, and final disposition did not affect time to administration of analgesic medications.Earlier pain management resulted in shorter ED length of stay for all patients regardless of disposition. However, earlier pain management did not affect the total length of hospitalization for patients admitted to the inpatient services.
CONCLUSIONS:
Pediatric patients with sickle cell pain crises experienced significant delays to initial analgesic medication. A standardized approach to pain management may improve ED management of SCD crises.

PMID: 25834959 [PubMed - as supplied by publisher]


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13.
Adv Ther. 2015 Apr 2. [Epub ahead of print]
Abstract
The lifespan of patients with sickle-cell disease (SCD) continues to increase, and most affected individuals in high-resource countries now live into adulthood. This necessitates a successful transition from pediatric to adult health care. Care for transitioning patients with SCD often falls to primary care providers who may not be fully aware of the many challenges and issues faced by patients and the current management strategies for SCD. In this review, we aim to close the knowledge gap between primary care providers and specialists who treat transitioning patients with SCD. We describe the challenges and issues encountered by these patients, and we propose a biopsychosocial multidisciplinary approach to the management of the identified issues. Examples of this approach, such as transition-focused integrated care models and quality improvement collaboratives, with the potential to improve health outcomes in adulthood are also described.

PMID: 25832469 [PubMed - as supplied by publisher]


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14.
Hemoglobin. 2015 Apr 1:1-7. [Epub ahead of print]
Author information:
  1School of Medicine, University of Pittsburgh , Pittsburgh, Pennsylvania , USA .
Abstract
Patients with sickle cell disease frequently experience severe pain events that lead to unplanned healthcare utilization. Mobile health tools (mHealth) may help prevent these events by providing remote monitoring and self-management support. This article describes the feasibility of the Sickle cell disease Mobile Application to Record symptoms via Technology (SMART), an mHealth app developed to help sickle cell disease patients monitor and manage their day-to-day symptoms. Fifteen patients recorded their pain intensity using a paper visual analog scale (VAS) and then repeated this measurement using an electronic VAS pain measure on SMART. Patients continued using SMART to record clinical symptoms, pain intensity, location and perceived severity, and treatment strategies for at least 28 days. Patient median age was 29 years (range 16-54); 60.0% were male. There was a high intraclass correlation between pain measurements entered on the paper VAS and SMART on the iPhone and the iPad We found a strong association between patient perceived pain severity and pain intensity entries using SMART (b = 1.71; p < 0.01). Daily compliance with SMART entries was a mean 75.0%, with a high of 85.7% in week 1 and low of 57.9% in week 4; however, one-third (n = 5) of the patients were 100.0% compliant even in week 4. Patients who were over age 35 or used an iPad for the study had the highest compliance rates. This study showed that SMART is a useable and feasible method for monitoring daily pain symptoms among adolescents and adults with sickle cell disease-related pain.

PMID: 25831427 [PubMed - as supplied by publisher]


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15.
Blood. 2015 Mar 30. pii: blood-2014-09-551564. [Epub ahead of print]
.
Abstract
Neurological complications are a major cause of morbidity and mortality in sickle cell disease. In children with sickle cell anemia, routine use of transcranial Doppler screening coupled with regular blood transfusion therapy has decreased the prevalence of overt stroke from approximately 11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with sickle cell disease and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurological deficit includes evaluation by a multi-disciplinary team (a hematologist, neurologist, neuroradiologist and transfusion medicine specialist); prompt neuro-imaging, and an initial blood transfusion (simple followed immediately by an exchange transfusion or only exchange transfusion), is recommended if the hemoglobin is > 4 gm/dL and < 10 gm/dL. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation. We will discuss our strategy of acute and long-term management of strokes in sickle cell disease.
Copyright © 2015 American Society of Hematology.

PMID: 25824688 [PubMed - as supplied by publisher]


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16.
Br J Haematol. 2015 Mar 30. doi: 10.1111/bjh.13348. [Epub ahead of print]
Author information:
  1Department of Haematology, Guy's and St Thomas' NHS Foundation Trust, London, UK.

PMID: 25824256 [PubMed - as supplied by publisher]




17.
Blood. 2015 Mar 25. pii: blood-2014-09-551887. [Epub ahead of print]
Author information:
  1The James Buchanan Brady Urological Institute and Department of Urology, The Johns Hopkins University School of Medicine, Baltimore, MD, United States;
  2Division of Hematology, Department of Medicine, Department of Oncology, and Institute for Cellular Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD, United States.
  3The James Buchanan Brady Urological Institute and Department of Urology, The Johns Hopkins University School of Medicine, Baltimore, MD, United States; aburnet1@jhmi.edu.
Abstract
Priapism is a disorder of persistent penile erection unrelated to sexual interest or desire. This pathologic condition, specifically the ischemic variant, is often associated with devastating complications, notably erectile dysfunction. Because priapism demonstrates high prevalence in patients with hematological disorders, most commonly sickle cell disease (SCD), there is significant concern for its sequelae in this affected population. Thus, timely diagnosis and management are critical for the prevention or at least reduction of cavernosal tissue ischemia and potential damage consequent to each episode. Current guidelines and management strategies focus primarily on reactive treatments. However, an increasing understanding of the molecular pathophysiology of SCD-associated priapism has led to the identification of new potential therapeutic targets. Future agents are being developed and explored for use in the prevention of priapism.
Copyright © 2015 American Society of Hematology.

PMID: 25810489 [PubMed - as supplied by publisher]


17.
Pediatr Blood Cancer. 2015 Feb 7. doi: 10.1002/pbc.25439. [Epub ahead of print]
Abstract
Hematopoietic stem cell transplant is curative of sickle cell disease (SCD) but limited by donor availability. Searches for 85 patients with SCD without matched sibling donors from 2009-2013 using modern techniques (allele-level HLA matching for unrelated donors and higher total nucleated cell doses for umbilical cord blood (UCB)) showed potential match probabilities of 20% for 8/8 HLA-matched unrelated donors, 84% for 7/8 donors, and 97% for two 4-6/6 UCBs suitable for ex-vivo expanded/double cord transplant. Searches performed by age 43 months would have a 90% chance of finding a suitable 5-6/6 UCB. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.






Monday, March 2, 2015

Sickle Cell News for March –April 2015

Raising Public Awareness of Sickle Cell and Thalassaemia - New Outreach resources on the United Kingdom Government website and available for public use
The United Kingdom (UK) National Health Service Sickle Cell and Thalassaemia Screening Programme (NHSSCTSP) have published new resources as part of their strategy to improve public understanding of sickle cell disease, thalassaemia and genetic screening.
Education and outreach have been important for the NHSSCTSP because it was the first national genetic screening programme in the UK National Health Service. In 2009, the UK Sickle Cell Society was commissioned to deliver the NHSSCTSP public outreach on sickle cell.  There was a lack of awareness of sickle cell and lots of stigma around the condition particularly in the high risk African and Caribbean communities most at risk of inheriting sickle cell so it was important to educate about sickle cell, testing and associated myths. 
These resources now published include a good practice guide for people who commission, fund, deliver and evaluate outreach programmes, anoverview of the work delivered and the research underpinning it. The resources capture the learning from years of outreach work. They include a detailed guide that explains the learning, video clips from public events and interviews with service users and people who delivered the outreach. Resources can be accessed from: https://www.gov.uk/search?q=sickle+cell+outreach (For further information contact: iyamide.thomas@sicklecellsociety.org)

ODEP – Free resource – Work Accommodation Ideas for Sickle Cell Anemia athttps://askjan.org/media/sickle.htm  A great resouce

Track  the new bill in Congress - H.R. 1807 Sickle Cell Disease Research, Surveillance, Prevention, and Treatment Act of 2015 at https://popvox.com/bills/us/114/hr1807 orhttps://www.govtrack.us/congress/bills/114/hr1807

Stem cell transplant resource pagehttp://www.dana-farber.org/health-library/what-happens-during-the-stem-cell-transplant-process-.aspx

Local mom starts new career to help sons fight sickle cell disease http://wric.com/2015/04/16/local-mom-starts-new-career-to-help-sons-fight-sickle-cell-disease/

Years ago, Tiffany was a school teacher. But after she became a mom, her 9 to 5 changed. “I was a stay at home mom,” Tiffany told 8News Anchor Ava-joye Burnett.

Both of Tiffany’s sons were born with sickle cell disease; the disorder can lead to serious health challenges and excruciating pain.

“You get a child who is crying, ‘Mom, Mom help me, I’m in pain,’ explains Tiffany, “and there is nothing that you can do because they are on morphine and the morphine pump. The doctor or the nurse can possible say, ‘I can’t give your child enough morphine because the pain is immeasurable.’”

The boys are now 12 and 8, trying to live normal lives with a tough disease. Tiffany also has a new career: working at Virginia Blood Services. There’s now a big push here in central Virginia to get more blood donations from African Americans.

“I think a lot of times, people, they don’t see where [their] blood goes, so it’s hard for them to really think and put their heads around that,” says Michelle Westbay, Marketing Director for Virginia Blood Services. “But in the end, you really are supporting lives.” Donations help with blood transfusions, and transfusions help to restore a patient’s body when the disease attacks. There are so many side effects—one of them being stroke—even for kids.

But another major part of the job at Virginia Blood Services is done in labs. Researchers are always looking for rare blood that will go only to patients who desperately need it. In some cases, it’s a patient’s only hope of survival.

Beth Johnson, of Virginia Blood Services, says they handle rare blood in a special way. “Once we find the rare blood, we stash it away so no body else can have it, except for us and we send it out to re patients who absolutely need it. Aside from working at Virginia Blood Services, Tiffany Dews is also continuing her work outside of the labs, within local communities. Her mission is to get more African Americans to donate blood that could help sickle cell patients—like her two sons.

“No one can see the damage it’s doing to their body as they get older,” Tiffany says. “A lot of times people give up, because no one understands the pain that they go though.”

Santonio Holmes Raises Awareness of Sickle Cell Diseasehttp://www.theroot.com/photos/2015/04/santonio_holmes_raises_awareness_of_sickle_cell_disease.html

NFL player Santonio Holmes knows the pain of sickle cell disease. He sees it in his 11-year-old son, T.J., as the boy struggles against the blood disorder that primarily affects people of African descent. A disease that causes intense pain and even life-threatening situations, it’s a tax on the body and the wallet as families often scramble to pay for expensive treatments. Holmes wants to change that. President of the III & Long Foundation, Holmes was in Washington, D.C., recently to meet with members of Congress and health care influencers, advocating for sickle cell research, awareness and treatment.

“As a parent of someone who has been diagnosed with sickle cell, I know how financially expensive and emotionally taxing the hospital visits, bills, treatment and medication can be,” said Holmes. “My goal is to raise awareness and help families that cannot afford the proper treatments by providing grants to organizations that assist these deserving families.”